Small cell neuroendocrine carcinoma (SNEC) is a rare form of malignancy. It mainly presents as bronchogenic neoplasm, and the extrapulmonary form accounts for only 0.1% to 0.4% of all cancers. These extrapulmonary tumors have been described most frequently in the urinary bladder, prostate, esophagus, stomach, colon and rectum, gall bladder, head and neck, cervix, and skin. Primary SNEC of the sinonasal tract is extremely rare with only less than 100 cases reported in the literature. Because of extreme rarity and aggressiveness of the tumor, the management for this entity varies considerably mandating multimodality approach. In this paper, we report a patient presented with left-sided facial swelling, and the histopathologic examination confirmed primary SNEC of left sinonasal tract. The tumor involved multiple paranasal sinuses with invasion into the left orbit and left infratemporal fossa and metastasized to cervical lymph nodes and bone. The patient encountered devastating outcome in spite of optimal medical management and treatment with palliative chemotherapy highlighting the necessity for further research of primary SNEC of head and neck. Neuroendocrine tumors constitute broad spectrum of malignant epithelial neuroendocrine neoplasms. They are further subdivided into typical carcinoid (well-differentiated), atypical carcinoid (moderately differentiated), and small cell carcinoma (poorly differentiated neuroendocrine carcinoma) . Small cA 54-year-old African-American chronic smoker woman presented to Emergency Department with worsening left-sided facial swelling and blurry vision for one week. Five months ago, the patient was evaluated for left neck mass with aspiration at the other facility. The pathology was benign and she was treated for infection with antibiotics. Then, the patient failed to follow up. Since then, the patient noted intermittent swelling of left neck mass for several months which for past one week has progressively gotten worse, associated with periorbital swelling, left-sided blurry vision, and left nasal congestion and epistaxis. She had 10–15 pounds of unintentional weight loss over last couple of months with increasing fatigue. She has a known history of HIV , asymptomatic hepatitis B virus carrier, and chronic kidney disease.Comprehensive physical examination revealed a nontendered, nonmobile, hard, large left-sided neck mass with left-sided facial and orbital swelling. She was also noted to have dry mouth, bilateral pale conjunctiva, mass in left nasal chamber, excessive lacrimation, intermittent alternating exotropia, and restricted left eye extraocular movement. Systemic review demonstrated no organomegaly and no other lymphadenopathy.Laboratory results were significant for anemia, thrombocytopenia, elevated lactate dehydrogenase, and uric acid levels. Computerized tomography (CT) scan of neck showed extensive lymphadenopathies, left greater than right, and a soft tissue mass involving left nasal cavity and multiple paranasal sinuses with invasion into left orbit and left infratemporal fossa Figures . The patDuring the course, the patient had acute chronic renal failure and worsening of hematological parameters requiring hemodialysis and blood products, respectively. Given the extent of her disease, the patient was started on palliative chemotherapy with carboplatin (etoposide was omitted due to thrombocytopenia) urgently as inpatient. The patient was not felt to be a candidate for cisplatin based chemotherapy due to her renal dysfunction. Treatment with combined carboplatin and etoposide was planned initially. But etoposide was omitted due to severe thrombocytopenia. There was a good clinical response after receiving the first cycle of chemotherapy; however, due to worsening anemia and thrombocytopenia, further chemotherapy was delayed. Unfortunately, the patient succumbed to the disease. The autopsy was performed and there was no definite mass found below the neck. Extensive sampling of lung, gastrointestinal tract including liver and spleen, and genitourinary tract were unremarkable. Only the vertebra showed small blue cells which was further confirmed by CD56 stain.Small cell undifferentiated neuroendocrine carcinomas of sinonasal tract, also known as poorly differentiated neuroendocrine carcinomas, are extremely rare and represent a histological spectrum of differentiation. It has been reported to be highly aggressive with very poor prognosis. Most prevailing malignancy of head and neck is squamous cell carcinoma, followed by adenocarcinoma . Larynx Most frequent clinical features of patients with neuroendocrine carcinoma of the head and neck, which were also seen in our case, are recurrent epistaxis, nasal obstruction, nasal discharge, proptosis, paresthesia, and anosmia. Occasionally, the presenting symptoms may be exophthalmos, facial pain, and swelling . These tMicroscopically, it is indistinguishable between SNEC of head and neck from those of bronchogenic origin. These cells are typically densely cellular, oval to spindle-shaped, and pleomorphic nuclei, frequent mitoses, and necrosis . CytokerAs witnessed in our case, tumors of this type are often disseminated at diagnosis; thus, it is essential to perform thorough metastatic workup before initiating the treatment. Multimodality therapy is increasingly used in small cell neuroendocrine carcinoma as it is an aggressive malignancy with high rates of local recurrence and metastases. Treatment modalities include chemotherapy, radiotherapy, and possibly surgery, depending on the extent of disease or the primary site. Surgical option is reserved for local relapse with no evidence of metastases because surgical results for this tumor have been disappointing. The combination of chemotherapy and radiation therapy, with or without surgery, has been recommended since late 1990s . PlatinuSNEC of sinonasal tract is an uncommon neoplasm with tendency toward recurrence and distant metastasis. Because of its rarity and aggressive nature, diagnosis and management of SNEC remain a challenge. Further research is needed to develop more specific, targeted approach of SNEC of head and neck in order to improve patient's survival and quality of life.


Ovarian teratomas rarely undergo new neoplastic transformation and account for a small percentage of malignant ovarian germ cell neoplasms. Here we report a case of a 51-year-old woman with multiple endocrine neoplasia type I (MEN I) who was found to have an ependymoma and neuroendocrine tumor (trabecular carcinoid) associated with mature cystic teratoma of her left ovary. The ependymoma component displayed cells with round nuclei and occasional small nucleoli which were focally arranged in perivascular pseudorosettes and true rosettes. Rare mitoses were identified. No necrosis was present. Immunohistochemical staining was positive for S-100 and GFAP. The Ki67 proliferation index was very low (2-3%). In contrast, the endocrine tumor component was composed of small uniform cells with eosinophilic cytoplasm, round nuclei, and speckled chromatin. Immunohistochemical staining was positive for synaptophysin and focally positive for chromogranin. This rare case illustrates that MEN I may have an influence on the pathogenesis of ovarian teratomas as they undergo malignant transformation. Mature cystic teratomas (MCTs) are the most common germ cell tumors of the ovary . TeratomCarcinoid tumors arising from the ovary are exceedingly rare and they are usually associated with MCT . PrimaryMultiple endocrine neoplasia (MEN I) is a disease entity that is classically understood to cause parathyroid adenomas, enteropancreatic endocrine tumors, and pituitary tumors; however, it actually causes combinations of over 20 different endocrine and nonendocrine tumors . Among sA 51-year-old woman with a history of MEN I was found to have a left ovarian mass on an outpatient computed tomography (CT) scan during a work-up for dysfunctional uterine bleeding. The patient had a long-standing history of recurrent primary hyperparathyroidism associated with her endocrine disease. She previously required multiple surgical resections of parathyroid adenomas, the first being in 1988, with additional resections in 2008 and 2012.Following a diagnostic laparoscopy and hysteroscopy with dilation and curettage to address dysfunctional uterine bleeding, the patient was referred to the University of Louisville Department of Gynecologic Oncology by her primary care OBGYN. She presented with no clinical symptoms related to her incidental ovarian mass. She underwent a total robotic hysterectomy with bilateral salpingooophorectomy and cystoscopy. An intraoperative frozen section was sent for pathologic evaluation, which revealed a cystic teratoma. An immature component could not be completely ruled out, thus prompting the patient to be surgically staged.Intraoperatively, sectioning through the left ovary revealed fatty to tan-pink and focally calcified cut surfaces, which were surrounded by a 5.5 × 3.0 × 1.0 cm uniloculated cyst containing coarse black hair. There were two discrete solid nodules arising from the cyst wall . One nodMicroscopic examination of the larger solid nodule revealed a heterogeneously cellular tumor with fibrillary background. The cells were uniform and round with occasional nucleoli. Frequent perivascular pseudorosettes as well as true rosettes with small to large tubules were noted . No necrMicroscopic examination of the second nodule showed a trabecular and cord-like arrangement of the tumor cells surrounded by dense fibromatous stroma. The tumor cells were uniform oval with finely dispersed chromatin . A diagnApart from these two components, the remaining teratoma was comprised of skin and mature glial tissue. The tumor was completely confined to the left ovary. Nine lymph nodes as well as all biopsies from the surgical staging were negative for metastatic neoplasm.Immunohistochemical staining was performed on the tissue samples for pathologic confirmation of the diagnosis. The ependymoma component stained positive for S-100 and GFAP . EpendymFinal pathologic staging was International Federation of Gynecology and Obstetrics (FIGO) stage IA. The patient was scheduled for a 6-month follow-up appointment. It was made clear to her that while her prognosis was good based on staging, close clinical follow-up would be needed. She has remained symptom-free since her surgery 12 months ago.Both MEN I and new neoplastic transformation of MCT are rare clinical entities. Our patient is exceptionally rare in that she was affected by both diseases. Based on the type of tumors that developed from the MCT seen in our patient, it raises the possibility that MEN I may alter the pathogenesis of MCTs as they undergo new neoplastic transformation. To our knowledge, this is the first reported case in the literature that would show synchronous association of ependymoma and neuroendocrine tumor with a MCT in a patient with MEN I.In addition to the classic presentation of parathyroid adenomas, enteropancreatic endocrine tumors, and pituitary tumors that are most often associated with MEN I, carcinoid tumors are rarely encountered. They can present in the bronchi, gastrointestinal tract, pancreas, and thymus . The proThe development of ependymomas in patients with MEN I is an extremely rare entity. As of 2010, there were only four reported cases in the literature . Three oThe most recent case of spinal ependymoma was recently reported in a 53-year-old man with MEN I .The MEN I gene is a putative tumor suppressor gene located on chromosome 11q13 . Tumor dMCTs, by definition, can develop into tissues derived from any of the three embryonic germ cell lines. The secondary malignancy that develops in these fairly common neoplasms will be influenced both by the patient's underlying genetic mutations and environmental factors. Our patient developed two separate tumors in her MCT that have both been documented to occur in association with MEN I. To our knowledge, this is the first case that would illustrate a potential relationship between MEN I and the subsequent components of a MCT following malignant transformation. While the case is exceedingly rare, it raises the following question: “do the genetic alterations associated with MEN I predispose women with MCTs to undergo malignant transformation at higher rates?” Such knowledge could have a significant impact on the screening algorithms of MEN I patients for MCTs. The data is currently limited and further study is required.


Pancreatic hamartoma is an extremely rare benign disease of the pancreas. Only 30 cases have been reported to date.A 68-year-old man presented with an asymptomatic solid and multi-cystic lesion in the uncus of the pancreas, incidentally detected on abdominal enhanced computed tomography. The tumor was found to be a well-demarcated solid and multi-cystic lesion without any enhancement, measuring 4 cm in diameter. After 28 months of follow-up, the tumor enlarged. At 31 months after initial diagnosis, the patient underwent surgical resection because it was difficult to clinically determine whether the tumor was malignant or not. Macroscopically, the solid tumor consisted of yellow adipose tissue with a smooth thin capsule confined to the pancreatic uncus. The inner structure of the tumor consisted of multiple cysts with a white nodule between the cysts. Histologically, the solid part and the multi-cystic portion consisted of mature adipose tissue and colonization of dilated pancreatic ducts with mild fibrosis, respectively. Immunohistochemical findings revealed cytokeratin 7 and 19 positive staining in the epithelial cells of the ducts. Adipose tissue showed positive staining for S-100 protein and there were only a few MIB-1 positive cells. The tumor was then diagnosed as a pancreatic hamartoma.Beside on the above findings, we suggest that the term “well-demarcated solid and cystic lesion with chronological morphological changes” could be a clinical keyword to describe pancreatic hamartomas.The online version of this article (doi:10.1186/s12876-016-0419-2) contains supplementary material, which is available to authorized users. Pancreatic tumor-like cystic lesions are significantly less common than solid lesions, and they account for less than 1 % of all pancreatic tumors . TherefoA 68-year-old man presented with an asymptomatic solid and multi-cystic mass in the uncus of the pancreas, incidentally detected on abdominal enhanced CT during a health examination. The patient had no relevant medical history, including chronic pancreatitis. The tumor was demonstrated to be a well demarcated solid and cystic lesion, measuring 4.2 × 3.9 cm in diameter by enhanced CT Fig. . The tumThe patient visited for re-examination 21 months after the initial visit because he had no symptoms. The size of the pancreatic tumor had become smaller, measuring 3.9 cm in maximum diameter, and the internal structure showed morphological changes with smaller cysts and a larger solid lesion by plain CT Fig.  and MRI/On coronal T2WI imaging, ringed-multilocular cysts with high intensity were surrounded by a smooth superficial mass, and a small nodule was observed inside of the cyst ring Fig. . These iA soft and elastic tumor was confirmed in the uncus of the pancreas. The tumor was not exposed and there were no inflammatory changes around the pancreas. Intraoperative ultrasound showed a well demarcated honeycomb-like cystic lesion in the pancreatic uncus, and there was no evidence of venous or ductal invasion Fig. . PylorusA solid tumor consisting of yellow adipose tissue with a smooth thin capsule measuring 40 × 40 × 28 mm in size was confirmed in the pancreatic uncus. The inner structure of the tumor consisted of multiple cysts, and a white nodule was found between the cysts Fig. .Fig. 4MaThe solid part of the mass consisted of mature adipose tissue, and the cystic lesion consisted of colonization of the dilated pancreatic ducts with mild fibrosis and infiltration of monocytes in the circumference. There was no evidence of mucinous products in the cysts, and no evidence of malignancy in the epithelial cells of the cysts. A few normal scattered acini were observed in the solid portion of the lesion, and the nodule located between the cysts consisted of stromal fibrosis without atypical cells Fig. .The epithelial cells of the ducts expressed cytokeratin 7 and cytokeratin 19, but were negative for cytokeratin 20. The adipose tissue was positive for S-100 protein, and mostly negative for MIB-1 (Ki-67). These results suggest that the tumor was non-proliferative Fig. . Based oAfter surgical resection, the patient received follow-up examination with plain CT every three to six months and there was no recurrence for 50 months.Pancreatic hamartoma is an extremely rare benign disease of the pancreas. With the improvements in diagnostic imaging equipment over the last decade, it has become increasingly common to discover rare pancreatic tumors such as those listed above. Although these tumors are usually not aggressive, they typically require surgical resection because of the difficulty in prospective clinical diagnosis . AccuratAlbrecht  first inTable Regarding the morphological features of these cases, 15 cases showed a solid pattern, and 14 cases showed both a solid and cystic pattern (this was unknown in two cases). A solitary tumor was observed in 22 patients, and multiple tumors were found in 6 cases (this was unknown in three cases). All cases showed a well-demarcated line and non-invasive growth. The median size of the main tumor was 4.4 cm . Twenty-one of the reported hamartomas were located in the head, four were located in the body, and 4 in the tail of the pancreas. Two cases were identified as diffuse tumors.There were only a few patients who could be followed for a long period of time. In the present case, we were able to follow the patient over 2 years, during which we observed chronological morphological changes in the tumor. Sueyoshi et al.  reportedRegarding the pathological findings, almost all cases demonstrated disarranged acinar and ductal cells embedded in the fibrous stroma. The solid lesion consisted of fibrous and adipose tissue, and the cystic lesion consisted of dilated ducts. The ducts varied in size and were lined by columnar epithelium without atypical cells. The acinar cells were well differentiated without normal lobular structures. In contrast, normal islets of Langerhans were confirmed in only 9 cases. Pauser et al.  and YamaIn immunohistochemical studies, several authors reported that the acinar cells of pancreatic hamartomas were positive for exocrine markers (amylase and trypsin), and that the ductal cells were positive for epithelial markers  , 11, 17,Nagata et al.  reportedPancreatic hamartoma is an extremely rare tumor of the pancreas. Recently, this disease has become clearly recognized, the clinical imaging diagnosis remains some of difficult. We suggest that the term “well-demarcated solid and cystic lesion with chronological morphological changes” could be a clinical keyword to describe pancreatic hamartomas.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.


Increased incidence world-wide of cancer and increased survival has also resulted in physicians seeing more complications in patients with cancer. In many cases, complications are the first manifestations of the disease. They may be insidious and develop over a period of months, or acute and manifest within minutes to days. Imaging examinations play an essential role in evaluating cancer and its complications. Plain radiography and ultrasonography (US) are generally performed initially in an urgent situation due to their wide availability, low cost, and minimal or no radiation exposure. However, depending on a patient’s symptoms, evaluation with cross-sectional imaging methods such as computed tomography (CT) and magnetic resonance imaging (MRI) is often necessary. In this review article, we discuss some of the most important acute noninfectious oncological complications for which imaging methods play an essential role in diagnosis. Cancer has become one of the leading causes of natural deaths worldwide. The high incidence of neoplasms increased the medical care related to complications of this disease in recent years. These complications may present as an acute life-threatening or insidiously, taking weeks or months to be recognized and treated. Moreover, it is not uncommon such complications being the first manifestation of of the disease .Cancer complications can be classified as the direct or indirect effects of a tumor. Direct or structural effects include invasion or mechanical compression of structures adjacent to the tumor. Indirect complications include systemic manifestations of the disease, such as hypercoagulability, immune suppression, and paraneoplastic syndrome. Surgical complications and those associated with the side effects of chemotherapy and/or radiation therapy are also significant ,2. TableImaging examinations play an essential role in evaluating cancer and its complications. Plain radiography and ultrasonography (US) are generally performed initially in an urgent situation due to their wide availability, low cost, and minimal or no radiation exposure. However, depending on a patient’s symptoms, evaluation with cross-sectional imaging methods such as computed tomography (CT) and magnetic resonance imaging (MRI) is often necessary .In this review article, we discuss some of the most important acute noninfectious oncological complications for which imaging methods play an essential role in diagnosis.Spinal cord compression occurs in 2.5–6% of patients with cancer . Early dMost (80%) cases of spinal cord compression syndrome occur in patients with previous cancer diagnoses . The mosThe thoracic vertebral column is most commonly affected (70% of cases) . The majMRI is the gold standard for the diagnosis of cord compression ,6. This On MRI, malignant tumors in the spinal column generally present with low signal intensity on T1-weighted images, high signal intensity on T2-weighted images, and contrast enhancement. Such tumors impress upon the spinal canal, dislocating and compressing the spinal cord. This can result in high signal intensity of the cord on T2-weighted images, which suggests edema  are common complications in patients with cancer because of their hypercoagulable state, local tumor effects, or treatment side effects . MalignaIt is found incidentally on imaging examinations performed for other reasons in about 4% of patients with cancer  and mainPTE is commonly asymptomatic or associated with non-specific symptoms. Acute onset dyspnea is the most suggestive symptom of PTE, followed by pleuritic pain. Massive PTE can cause pulmonary hypertension and signs of direct cardiac insufficiency. Chemotherapy, history of recent surgery, prolonged immobilization, or signs of deep vein thrombosis are associated with developing PTE -29.The differential diagnosis of PTE includes pulmonary tumor thrombotic microangiopathy (PTTM), an extremely rare and serious complication in patients with cancer caused by the presence of microemboli; it is associated with adenocarcinomas, mainly of gastric origin . A patieCT angiography is the method of choice to diagnose and evaluate the extent of PTE. CT should be performed using a specific angiographic protocol to achieve adequate contrast of the pulmonary arteries, with suitable venous access. Multiplanar reconstructions can be useful in identifying the thrombus -35.Acute PTEs are identified as one or more filling defects in a pulmonary arterial branch Figure . Some caIn patients who cannot undergo CT angiography, the method of choice is ventilation/perfusion pulmonary scintigraphy. However, this modality may be unavailable in urgent situations in many institutions. Another option is MRI of the thorax, which enables the identification of large thrombi with a balanced steady state free precession sequence, without the use of paramagnetic contrast Figure  36]. Th. Th36]. PTTM shows nonspecific signs of pulmonary hypertension on thoracic CT images. Because of changes to the small vessels, typical findings of infectious bronchiolitis, such as diffuse centrilobular opacities and a tree-in-bud pattern, have also been described -35.Intestinal obstructions are relatively common in patients with cancer and can be caused by benign etiologies or directly associated with the tumor ,38. BeniMalignant causes of small intestinal obstruction are less frequent than adhesions and inflammatory changes. Malignant obstruction of the small intestine is caused more frequently by metastatic tumors than by primary tumors of the small intestine, which are responsible for <2% of all gastrointestinal neoplasias . MalignaFocal intestinal lesions can also cause intussusception ,44. ThisRadiography can show signs of intestinal obstruction, with gaseous distension of the small bowel or bowel loops forming air-fluid levels on upright images . Small bIn intussusception, CT may shows pathognomonic changes, such as the target sign or a “loop within loop” pattern, with or without invaginated fat and mesenteric vessels Figure .Acute intestinal inflammatory changes are common in patients with cancer, and various etiologies may be involved in these processes -48. NeutClostridium difficile infection, commonly after antibiotic treatment, it should be considered especially in immunocompromised cancer patients with abdominal complaints [Pseudomembranous colitis is caused by mplaints ,52. It implaints .Malignant tumors can also be associated with mesenteric ischemia and ischemic colitis . IschemiThe small intestine and colon are sensitive to radiation therapy. Thus, actinic enteritis and colitis should be considered in the differential diagnosis when signs of an inflammatory process are present in previously irradiated areas .Ultrasound is useful in some cases, but CT is generally the best method for the evaluation of intestinal complaints ,40,46. CCT may show nonspecific inflammatory findings, including thickening and of the small intestine or colon, with or without effacement and striations of pericolic adipose tissue. The identification of pneumatosis, pneumoperitoneum, and pericolic collections often suggest developing necrosis or perforation requiring urgent surgical evaluation ,50. The Biliary obstruction can be secondary to biliary stasis in patients with diffuse metastatic infiltration of the liver, causing obstruction of the small intrahepatic bile ducts, or it can occur due to compression of the main bile ducts in patients due to more commonly metastatic disease or lymphoma or disease within the ducts, for example, from cholangiocarcinoma .Malignant tumors of the head of the pancreas and the ampulla of Vater are common causes of obstruction of the main bile ducts. Benign differential considerations include pseudocyst from pancreatitits .Bile duct obstruction manifests mainly as jaundice, dark urine and pale-colored stools. In a patient with abdominal pain and/or fever, the possibility of cholangitis associated with biliary obstruction should also be considered ,56.Imaging methods should be able to define the presence or absence, level, and cause of bile duct obstruction. Ultrasound is generally the first method used and has good diagnostic accuracy in detecting dilation of the intra- and extrahepatic bile ducts . A literOther methods for evaluating the bile ducts include endoscopic retrograde cholangiopancreatography (ERCP), which also allows biopsy. However, this is invasive, more complex and commonly less readily available than CT or MRI. Commonly, the second line test due to ease of availability is CT. MR also visualizes visceral structures, and one can employ heavily T2-weighted MR sequences for visualizing the ducts (MRCP) .In cases of diffuse metastatic infiltration of the liver, CT or MRI shows multiple hepatic lesions that are more commonly infiltrative or confluent depending on the primary tumor, together with focal dilatation of the small intrahepatic bile ducts adjacent to vessels of the hepatic triad .In cases of main bile duct compression, diffuse dilation of the intra- and extrahepatic bile ducts is present upstream of the obstruction site Figure . Such tuUrinary tract obstruction can occur in patients with retroperitoneal or pelvic tumors; these are more commonly gynecological or urological cancers of the cervix, ovaries, bladder, and prostate ,57. MetaUltrasound is the easiest way to detect the presence of hydronephrosis ,59. HoweCancer patients can develop bleeding complications secondary to thrombocytopenia, tumor rupture, or hemorrhage from a vascular neoplasm.Massive hemoptysis is generally used to describe the expectoration of a large amount of blood and/or a rapid rate of bleeding, and when it occurs secondary to malignant disease, the mortality rate may be as high as 60%. Bronchogenic carcinoma is the most common cause of massive hemoptysis in patients older than 40 years. Endobronchial metastases from carcinoid tumors, breast, colon or kidney cancer, melanoma and sarcomas may also cause hempoptysis. Hemoptysis in cancer patients may also be caused by nonmalignant conditions, such as fungal infections, or may be related to thrombocytopenia or other coagulation disorders ,61.In patients with cancer-related massive hemoptysis, chest radiography may demonstrate abnormalities such as tumors, cavitary lesions, pulmonary infiltrates Figure , and medSevere abdominal bleeding in cancer patients is a rare, but potentially fatal complication that requires prompt diagnosis and treatment. Malignant hemorrhage can occur in an organ parenchyma or subcapsular space due to direct tumor rupture or in the peritoneal cavity from carcinomatosis or extension of visceral tumor rupture. Hypervascular tumors, such as hepatocellular carcinoma, renal cell carcinoma, and melanoma, are the most commonly associated to spontaneous hemoperitoneum. Large size of the mass, a peripheral or subcapsular location and increased vascularity are the most important risk factors for intratumoral hemorrhage and subsequent spontaneous rupture. In addition, patients with hematologic malignancies can develop spontaneous hepatic or splenic rupture ,61.Intratumoral hemorraghe is suggested by hyperechogenicity at US or hyperattenuating components at unenhanced CT Figure . HematomAt unenhanced CT, hemoperitoneum manifests as high attenuation ascites . Subcapsular hematomas are elliptical high-attenuation collections bound by the organ capsule in which they originate. Thus, on CT images, the highest-attenuation hematoma, or sentinel clot, is that closest to the site of bleeding. Foci of active extravasation of intravenous contrast material extending into or around the hematoma indicate ongoing bleeding. Angiography of the visceral arteries not only helps identify the source of bleeding but also assists in treatment ,61,63.Nowadays, the number of oncological visits is increasing in clinical practice, especially those related to acute and insidious noninfectious oncologic complications. Imaging methods play an essential role in diagnosis and as soon as the support team recognizes these conditions, the most appropriate therapeutic approach may be provided improving the quality of life and survival of these patients.The authors or author’s institutions have no competing interest.MDG and AGVB drafted the manuscript and provided the presented cases. EM, RC, JLG and VK reviewed the manuscript and the presented cases. All authors read and approved the final manuscript.


Low-grade indolent lymphomas can be transformed into high-grade aggressive lymphomas ,2,3,4. V A 57-year-old male diagnosed with diffuse large B-cell lymphoma (DLBCL)  in 2002 completed 6 cycles of CHOP followed by radiotherapy. In 2006, 18F- fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) showed no active disease. In 2007 there was recurrence in the left obturator and external iliac nodes. Lymph node biopsy done outside our facility showed CD20+ B-cell lymphoma. The patient was advised to undergo intensive chemotherapy, but was lost to follow-up. In 2010, the patient came to our hospital with bilateral firm non-tender inguinal and right axillary lymphadenopathy without any organomegaly. 18F PET/CT revealed heterogeneous uptake in the left paraaortic, retrocaval, precaval, and bilateral internal iliac nodes. A previous diagnostic lymph node biopsy was reviewed, showing diffuse infiltration of large atypical cells, positive for CD20, CD30, MUM1, and Bcl2 with a Ki67 index of 80% and negative for CD3, CD5, and CD10, which was consistent with DLBCL . Biopsy The phenomenon of high- or intermediate-grade non-Hodgkin lymphoma recurring as a low-grade lymphoma is an uncommon form of transformation known as “downgraded” lymphoma. This downgrading may be due to: 1) recurrence of a low-grade lymphoma that was present as a minor component of the initial lymphoma or in a site not biopsied, or 2) development of a second lymphoma resulting from chemotherapy and/or an intrinsic propensity for lymphoma development in the patient . Relapse in DLBCL mainly occurs in the first 2 to 3 years, while late relapses after 5 years are rare, occurring in 3.6% of cases. Patients with DLBCL relapse usually have the same histology. However, relapse as indolent lymphoma following initial DLBCL may occur in about 17% of cases, predominantly as follicular lymphoma or rarely as nodal marginal zone lymphoma or as extranodal mucosa-associated lymphoid tissue lymphoma [


Supplemental digital content is available in the text Primitive neuroectodermal tumors (PNETs) arising directly from the lung are extremely rare but particularly aggressive neoplasms. Although thoracic PNET usually develops on the chest wall, there have been reports of primary Ewing sarcoma/PNET of the lung.We present the case of a 16-year-old male with PNET diagnosed following histologic and immunohistochemical examination of a video-assisted thoracic surgical biopsy. As typically occurring for these tumors, the diagnosis was initially delayed in our patient and prognosis was poor despite aggressive surgical resection and postoperative chemotherapy. We attempted to explore the use of targeted pharmacotherapy through high-throughput sequencing in this case. We found that this treatment using Avastin and Cetuximab could provide a new therapeutic direction for PNET.PNENTs arising from the lung parenchyma without pleural or chest wall involvement are extremely rare. Although uncommon, if the pathological features are similar to Ewing sarcoma, PNENTs should be kept in mind, and the target therapy may be a potent treatment for this disease. Reports have also noted the presence of PNET in the liver, kidneys, and adrenal glands.4 In the chest region, PNET commonly arises from the chest wall; this is usually referred to as an Askin tumor.6 Cases of PNETs arising from the lung parenchyma without pleural or chest wall involvement are extremely rare. To the best of our knowledge, multiple pulmonary PNTEs without chest wall involvement in 16-year-old adolescent have not been reported to date.Primitive neuroectodermal tumors (PNETs) belong to the Ewing sarcoma family, and most commonly arise in adolescents or young adults  with a slight male preponderance.A 16-year-old boy was admitted with the chief complaints of shortness of breath and occasional chest pain of about 3 days duration. He had no other complaints and his past history was unremarkable. He denied smoking and had no family history of malignancy. A review of systems was noncontributory. Peripheral blood count, baseline serum chemistry screening, and urinalysis were normal on admission, as were tumor biomarker tests , and squamous cell carcinoma antigen) and a purified protein derivative (PPD) for tuberculosis.max  of 1.0, and without correlative abnormality  was performed and 6 nodules in the lingual segment were removed. During the operation, a number of nodules were present on the surface of both left lung lobes with protrusion, but under the visceral pleura. As shown in Figure 2 day 1, 8), Cisplatin , and Endostatin , every 3 weeks at another facility. Since progressive disease was apparent after 2 cycles of this chemotherapy, the treatment regimen was changed to Ifosfamide , Etoposide , and Endostatin , every 3 weeks. After 1 cycle of this regimen, the disease continued to progress. Thus, the chemotherapy was changed to Paclitaxel  and Gemcitabine , every 3 weeks for 2 cycles. Although the chest CT scan did not image any obvious changes in the lung nodules, the patient complained of back pain, and multiple spinal metastases were imaged by magnetic resonance imaging.The patient had an uneventful postoperative recovery and was initially treated with the combined chemotherapy of Navelbine →D[Asp]), the remaining 7 genes were all synonymous mutations, as shown in Table MLH1 gene with the PNET in this patient, Avastin or Cetuximab could be used for the target therapy. However, this patient did not have the opportunity to receive this target therapy.Recently, in order to explore the molecular structure of the tumor, the mutations of 45 tumor-related driver genes were detected by a second-generation sequencing test . As shown in Complementary Table 1, Furthermore, we summarized the reported literatures for primary pulmonary primitive neuroectodermal tumor patients in Table 17 But new research suggest that while the degree of neuronal differentiation used to be applied to distinguish between classical ES and PNET, molecular biology studies have now shown that all these tumors share a common gene rearrangement involving the EWS gene on chromosome 22, so that this distinction is now obsolete. In addition, PNETs are further divided into central and peripheral tumors. Peripheral primitive neuroectodermal tumors (pPNETs) occur outside the central nervous system and originates from neuroectodermal differentiation disorders.18 PNETs may affect people of all ages and occur most often in adolescents or young adults, usually before the age of 35 years with slight male preponderance.1 PNETs can occur in numerous solid organs including the kidneys, breasts, gastrointestinal tract, prostate, endometrium, jaw, adrenal glands, and meninges; however, they rarely arise in the lungs. In 1979, Askin et al5 described a similar rare malignant small-cell tumor of the thoracopulmonary region in 20 children and adolescents with a mean age of 14 years; they were referred to as Askin tumors. To the best of our knowledge, less than 20 cases of primary lung PNETs have been described in the literature. As shown in Table 16 Small cell carcinomas exhibit consistent positive immunoreactivity to cytokeratins, and reactivity for chromogranin and thyroid transcription factor 1. Malignant lymphomas do not absorb stain for leukocyte common antigen. Although there are no specific antibodies or markers for PNETs, CD99 is generally positive in these tumors. Our case also showed positive expression of CD99 by IHC staining, which was consistent with this point. In some cases, fluorescence in situ hybridisation or reverse transcription and polymerase chain reaction can also be used to confirm the diagnosis; However, IHC results are equivocal. A study reported that 85% of patients with these aggressive malignant tumors had the identification t chromosome rearrangement and the detection of p30/32 cell surface antigen , and the remaining 15% of the patients had variants of this translocation, including 22q12, 21q12 (10% of cases) and 7p22, 17q12, 2q36 (<1% of cases).19 Another study also noted that the structural changes might occur more often than translocations, including trisomy 8 or 12, deletions of 9p21.20 In our case, because the staining of CD99 was positive and the diagnosis of primary lung PNETs was confirmed, detection of the translocation was not conducted.PNETs and Ewing sarcoma are widely regarded as clinically and histologically identical tumors; they consist of small round cells and belong to the Ewing/PNET tumor family. They are uncommon entities and comprise 5% of all cases of small round cell tumors.21 As shown in a literature review,16 8 of 20 (40%) patients underwent resection and adjuvant chemotherapy with or without radiation, while 36/20 (30%) patients underwent neoadjuvant chemotherapy followed by resection with or without adjuvant chemotherapy, 5/20 (25%) patients underwent resection, and 1/20 (5%) patient only received chemotherapy. The 2-year survival rates for the first three groups were 37%, 33%, and 60%, respectively, and the patient who only underwent chemotherapy lived for 17 months following the initial diagnosis. To the best of our knowledge, our case is the first to report a patient with inoperable thoracic PNET who only underwent the chemotherapy, and lived only 5 months after the initial diagnosis was made. Ewing tumor family is generally comprised of PNETS, Askin tumors , and extraosseous Ewing sarcoma. Due to the significant progression in understanding disease pathogenesis and multimodality treatments, 1-year survival rate of patients with localized Ewing sarcoma has increased up to nearly 70%. However, the 5-year survival rate is still very poor, less than 25% approximately.22 PNET, like other Ewing sarcomas, is a highly malignant tumor with poor prognosis. Once the diagnosis is made, multimodality treatments, including systemic chemotherapy (neoadjuvant and adjuvant), surgery, and radiation, are all considered as treatment alternatives depending on the disease status and patients condition.The treatment of PNET should be various combinations of early surgical resection as well as adjuvant chemotherapy and radiation therapy.23 For the patients with metastatic disease, the second-line regimens, including cyclophosphamide/topotecan, irinotecan/temozolomide, and high-dose ifosfamide, are often exploited.25 So far, there was no evidence comparing the therapeutic outcomes of surgery and radiotherapy for the localized Ewing sarcoma. Therefore, based on the clinical experience, surgical resection is the preferential alternative, followed with radiotherapy with a dosage <40 Gy for the positive margin.26The first-line drugs of proven efficacy for Ewing sarcoma include vincristine, doxorubicin, cyclophosphamide, etoposide, and ifosfamide.28 Previous studies have demonstrated that angiogenesis played an important role in the growth of Ewing sarcoma. In the COG-AEWS-0521 trial, it was found that the patients with recurrent Ewing sarcoma exhibited a high level of VEGF protein. And animal studies also proved a role for angiogenesis in Ewing sarcoma.30 Therefore, bevacizumab (Avastin) and sunitinib might be the good candidates of targeted therapy for Ewing sarcoma. In our case, the missense mutation of the MLH1 gene indicated that Avastin or Cetuximab was a possible targeted therapy alternative. However, it was unfortunate that this patient was not received these medications. Moreover, Ecteinascidin 743 (Trabectedin) has been used for the treatment of soft tissue sarcoma, but the effect still needs to be determined for Ewing sarcoma.31Targeted therapy is also an effective therapeutic alternative in Ewing sarcoma, and some potential biomarkers have been addressed.In view of the foregoing, the outcome of PNETs is very poor due to the limitations of traditional chemotherapy and radiotherapy. Furthermore, to date, the target therapy for this disease remains unproven. To the best of our knowledge, the literature contains no other similar cases with the formation of multiple nodules in both lungs. Our case highlights the outcome of detecting the mutations of oncogenes. Our results suggest that we could attempt a target medicine cure using Avastin and Cetuximab. Moreover, it might provide a new therapeutic direction.The patient signed the permission for the publication when he was admitted in our hospital at the first time.


Vascular complications from osteochondroma are rare and include essentially stenosis, occlusion, and pseudoaneurysms. The authors report an original case of intraoperative rupture of undiagnosed popliteal artery pseudoaneurysm during resection surgery for a distal femur osteochondroma. Exostoses are the most frequent benign bone tumors. They account from 10 to 15% of both benign and malignant bone tumors . OsteochA 22-year old man with hereditary multiple exostoses was scheduled for a surgical excision of distal right femur osteochondroma. The preoperative evaluation was unremarkable, distal pulses were presents and the leg was well perfused. Knee X-rays showed bilateral distal femoral exostosis During intervention a massive bleeding occurred, and for hemostasis, the surgeon was forced to lie the popliteal artery. Three hours later, the patient was admitted to our emergency department with a relative ischemia of the right lower limb, with normal vital signs. On physical examination, sensation was present but reduced to the foot, the popliteal and distal pulses were absents. Laboratory tests were normal except for haemoglobin; it was at 9 g/dl. An arteriography was performed and clearly showed a popliteal artery occlusion opposite the incision . The patOsteochondroma is one of the most common bone tumors. It is exceptionnaly responsible for vascular complications from which pseudo-anevrysm constitutes the most frequent entity. Hereditary multiple exostoses is a multi-phenotypic autosomal dominant disorder that affects 1 per 50000 in the general population for Caucasians . It is aOsteochondroma may cause various complications such as arterial pseudo-anevrysms. The vascular disease genesis is due to repeated exercices, such a s knee flexion which could chronically abrad the popliteal artery and produce an adventicial defect followed by a pseudo-anevrysm. Exostose is often asymptomatic. A chronic edema may reveal popliteal artery disease. It may also lead to many other problems: peripheral nerves compression, blood vessels, distal ischemia, phlebitis and pulsative mass . In our In addition to this patient, 2 other cases of rupture of popliteal artery have been reported in the literature. The pathologic diagnosis of osteochondroma is made by radiographic examination which demonstrated an osteocartilaginous exostosis . The cliThe first case is a 33 -year -old patient. Osteochondroma was located on the fibula. The rupture occurred after traumatisme.the popliteal artery was repaired by vein patch angioplasty . The secIn our patient, the exostosis was removed by an orthopaedic surgeon. No vascular ultrasonography or arteriography have been realized before surgery. After removing the exostosis, bleeding occurred and a ligation of the popliteal artery was reported by the orthopeadic surgeon. A part from the surgery, there was no history of trauma that might have caused a fracture of the exostosis. Pain, edema, pulsative mass are the most frequent pseudo-anevrysm clinical signs. However, the rupture may reveal a pseudo-anevrysm not yet known. In this case, surgery is required.Surgical repair and excision of the adjacent osteochondroma is considered as the treatment of choice. One case of successful transarterial embolization using helical microcoils in the treatment of pseudoaneurysm on osteochondroma that was located on the superficial femoral artery. The popliteal artery aren&39;t suitable to this kind of treatment. Our patient is young, the pseudoanevrysm was broken when he was admitted to our hospital and the popliteal artery was ligated by the orthopaedic surgeon. So, surgery was required to restitute a normal vascular status.Osteochondroma may be present as a solitary lesion or in the form of hereditary multiple exostoses. Vascular complications of exososes are infrequent and the association with false anevrysms is exceptional. Acute rupture is rare, In a recent review of the literature, 38 cases of popliteal pseudoaneurysm secondary to exostosis have been reported, and only two of which were ruptured. To our knowledge this is the first case of intraoperative rupture occurring during resection surgery for distal femur osteochondroma. A combined orthopaedic and vascular surgery must always be undertaken when there is exostose associated to a false anevrysme.


It has been reported that cardiac malignant fibrous histiocytomas occur more frequently in the left side of the heart, especially in the left atrium, but rarely invade the mitral valve. We present a case with a giant malignant fibrous histiocytoma with an unusual localization involving almost the entire left atrium, mitral valve, and left superior pulmonary vein.We describe the case of a 54-year-old woman from Kosovo admitted to our emergency department with dyspnea. A transthoracic echocardiography demonstrated a giant mass localized on her left atrium. Our patient underwent emergent total surgical removal of the mass. The mass extended between her left superior pulmonary vein, and extended to her left atrium and the posterior mitral valve leaflet. We formulated a surgical plan for total separation of the mass from the endocardium. Total removal was performed and her left side pulmonary veins were entirely freed from the mass. We then performed a mitral valve replacement. The differential diagnosis included other masses of the left atrium, including thrombi, vegetations, and cardiac tumors. Postsurgical histopathologic results showed a pleomorphic malignant fibrous histiocytoma. Six monthly follow-up cardiac and abdominal sonographic examinations revealed no tumor recidivism.We reviewed 90 cases with malignant fibrous histiocytoma reported in the literature. Our case was especially unusual because of the primary location in the mitral valve, the pleomorphic variant, and the dimensions and extension. Complete surgical resection is mandatory to ameliorate symptoms and to obtain histologic information. In 2001 [et al.  analyzedA 54-year-old woman from Kosovo was admitted to our hospital with dyspnea. On clinical examination, our patient’s blood pressure was 100/60 mmHg, and her pulse rate was 130 beats per minute with a regular rate and rhythm. Cardiac auscultation revealed a diastolic murmur. End-inspiratory crackles suggested pulmonary edema. Two-dimensional transthoracic echocardiography revealed a giant mass originating from her posterior mitral valve leaflet, occupying almost her entire left atrial cavity. Cerebral, thoracic, and abdominal computed tomography was also performed, showing no evidence of additional tumors.Our patient underwent emergency surgical removal of the cardiac tumor. Intraoperative transesophageal echocardiography was performed, which confirmed the presence of the tumor Fig. . Our patMacroscopically, the excised lesion was composed of multiple irregular soft tissue fragments Fig. . After t2 of ifosfamide on days 1 to 3 and 80 mg/m2 of epidoxorubicin on day 1. The treatment was well tolerated with no unacceptable toxicities. Our patient was still alive with no signs of metastasis six months later.Our patient’s postoperative course was uneventful. Six weeks after surgery, she started a six-course chemotherapy regimen of 1.5 mg/mPrimary MFH is the second most common primary cardiac sarcoma, with an estimated incidence of ∼ 1.7 % of cases. It usually affects people of ages 14 to 77 years . After aClinical manifestations of primary MFH are dependent on their size and location. With small tumors, no clinical manifestations are evident. As the tumor grows, the most frequent symptoms are shortness of breath, palpitation, or chest discomfort. The MFH in our case is, to the best of our knowledge, the largest reported.Histologically, MFH is a variously shaped and multilobulated mass, sessile or pedunculated. It is a tumor of the fibroblasts, with giant cells and atypical nuclear and cytoplasmic findings. The term MFH has now become synonymous of undifferentiated high-grade pleomorphic sarcoma, because the fibrohistiocytic differentiation is not characteristic of a specific tumor type. Sarcomas of myoblastic or fibroblastic origin, including leiomyosarcoma, fibrosarcoma, myxoid fibrosarcoma, and pleomorphic sarcoma (MFH), occur most often as endocardium-based pathologies. The differential diagnosis of cardiac sarcoma in these circumstances is cardiac myxoma, especially in the case of a left atrial intimal-type sarcoma. MFH is a pleomorphic sarcoma composed of fibroblasts, myofibroblasts, and histiocyte-like cells. The pleomorphic variant of the MFH is rarely reported and only had mitral valve involvement in two of the 90 cases we found in the literature , 9.The treatment plan for MFH depends on the tumor status, size, histology, location, and metastatic spread. Complete surgical resection is mandatory to ameliorate symptoms and to obtain histologic information. In our case, once we had started surgery, we suspected that we had a malignant tumor. However, owing to the fact that the tumor had invaded her posterior mitral valve leaflet, causing severe mitral valve obstruction, we had to remove her mitral valve as this was the only measure to complete total tumoral mass removal and relieve the mitral valve stenosis.Although the prognosis of MFH is poor and the possibility of local recurrence and metastasis is high, patients benefit from surgery. Multiple studies have reported a median survival of six months for right-sided tumors, whereas left heart tumors seem to have a better prognosis . The rolOur case was especially unusual because of the primary location of an MFH in the mitral valve, the pleomorphic variant, and the dimensions and extension. Complete surgical resection, including mitral valve replacement, is mandatory to improve symptoms and to obtain histologic information.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Liposarcoma is a malignant tumor of soft tissue. Myxoid/round cell liposarcoma has a tendency to spread to extrapulmonary sites but the spine is an unusual location even for metastasis. Metastatic bone tumors in the spine are painful. The vertebral body augmentation procedures for treating painful metastatic spinal lesions are minimally invasive and are good alternatives to open surgery.A 41-year-old Turkish man was treated with radiofrequency tumor ablation and percutaneous vertebral augmentation for spinal metastasis. Asymptomatic perivertebral and segmental veins' cement leakage was detected on perioperative X-ray radiograms; at the follow-up computed tomography scan, no further migration of any cement material was seen, and his postoperative course was uneventful.The risk of cement leakage and embolism is increased with the treatment of some malignant lesions. The frequency of local leakage of bone cement is relatively high. Patients undergoing percutaneous vertebral augmentation of malignant spinal metastases need close monitoring. There is no agreement on the treatment strategy. Liposarcoma is one of the most common histologic types of soft tissue sarcoma . The freSpine metastasis can be debilitating, and have a significant impact on patients' quality of life. The treatment regimen for spinal metastasis is generally palliative and consists of a combination of medical therapies . Radiofrequency thermal ablation (Rf-TA) and vertebral augmentation procedures (VAPs) have been shown to be effective in metastatic bone lesions , 4. Our We describe the case of a 41-year-old Turkish man. Eight months before he was admitted to our clinic, a subcutaneous tissue mass was excised with a marginal excision from his right thigh; it was liposarcoma. He then received chemotherapy and radiotherapy with a good response but the liposarcoma had since progressed. He was admitted to our hospital, presenting with back pain with radiation and with no neurological deficits. He had a 3-month history of increased back pain, and he did not recall any trauma. A skeletal survey showed no lytic lesions, and a CT scan also showed no evidence of tumors in his spine. A spinal MRI revealed multilevel vertebral body edema  than those with osteoporosis (1 to 2 %) or spinal angiomas 2 to 3 %). The higher risk of extravasation in patients with spinal malignancy is because of the cortical destruction of vertebral body and higher vascularization. Some authors reported the use of a preinjection venogram to decrease the incidence of pulmonary embolism  %. The h.Conservative management with anticoagulants, antibiotics, and corticosteroids is reserved for smaller or peripherally located cement emboli. Paravertebral venous leakage and pulmonary embolization of cement occurs frequently but is clinically silent in most cases.In our case, his visual analog scale (VAS) before the procedure was 7 and after the procedure it was 2. In order to prevent or reduce cement leakage, we used high viscosity cement (4 ml volume) and a low-pressure injection. Before injection, we created a cavity with a drill and curette but cement leakages still occurred. It appears that the cement passed from the vertebral venous plexuses via the paravertebral veins.The vertebral augmentation with radiofrequency procedure significantly reduced associated spinal malignancy pain, improving functionality and quality of life. However, many patients' cement leakage remains asymptomatic, but late cement migration during follow-up did not occur in our case. Standard post-procedural CT scan of the treated vertebral body in vertebral augmentation is useful. Even though we used high viscosity cement and created a cavity, cement leakage did occur. Close monitoring of patients undergoing VAP and radiological follow-up are important.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Abdominal leiomyosarcoma arising from the mesentery is a rare malignancy. It is an aggressive entity with an overall 5 year survival rate between 20 and 30 %. Surgical resection is the cornerstone of primary treatment and may be curative for localized disease. However, patients often develop intra-abdominal relapse and/or metastatic disease. If surgical resection is not feasible, palliative chemotherapy is the treatment of choice. However, there are no clear guidelines regarding chemotherapy; neither in the adjuvant nor advanced setting.We present a 40 year-old woman, with a mesenteric leiomyosarcoma, who underwent radical tumor resection and did not receive adjuvant oncological therapy. Three months postoperatively, she developed metastatic disease to the lungs and liver. After multidisciplinary assessment she received an unconventional histological-subtype-tailored chemotherapy comprising 3–4 regimens. Initially, there was a decrease both in number and size of metastases. Ultimately, an almost complete radiological response was seen. Subsequent surgical resection and radiofrequency ablation of residual metastatic foci in the liver and lung brought her into complete clinical remission. She is presently tumor free, 36 months following diagnosis of metastatic disease.To our knowledge, this is the first report of a patient with metastatic mesenteric leiomyosarcoma who is in complete clinical and radiological long-term remission following very aggressive multimodal treatment; including intense poly-drug chemotherapy and without any demonstrable long-term side effects. Given the rarity of mesenteric leiomyosarcoma and lack of guidelines regarding oncological therapy, we suggest that multimodal therapy including aggressive chemotherapy, guided by a multidisciplinary team, is essential to achieve an optimal outcome. Leiomyosarcoma (LMS) represents between 10 and 20 % of all newly diagnosed soft tissue sarcomas . AlthougHere, we present a patient with an abdominal LMS, specifically a mesenteric LMS. This is a rare malignancy, with only a few cases reported in the literature –9. The LOur patient is a 40 year old woman who had no significant medical history except for an appendectomy when she was 20 years-old. She presented with a two-month history of left sided abdominal pain accompanied by abdominal discomfort and distention. Additionally, she suffered from nausea and vomiting without any changes in stool pattern.On physical examination, a large palpable mass was detected in the left quadrant. This mass was very tender upon palpation, but there were no signs of peritonitis. Blood tests revealed increased acute phase proteins and reduced haemoglobin but were otherwise unremarkable. Computerized tomography (CT) of the abdomen revealed an intraperitoneal tumor in the left quadrant measuring 8 × 7 cm axially and 7 cm craniocaudally Fig. a. The tuAbout two weeks later, the patient was operated via a midline laparotomy. The tumor, which was partly adherent to the sigmoid mesocolon, was removed en bloc together with 15 cm of small intestine and colon. An antiperistaltic side-to-side-anastomosis was established at the splenic flexure as well as a small intestinal anastomosis about 120 cm proximal to the ileocaecal valve. No intraperitoneal metastases were seen, but tumor perforation to the peritoneal cavity and resulting bloody ascites was documented perioperatively. Despite this high risk for tumor relapse, no adjuvant therapy was given to the patient in line with guidelines regarding adjuvant therapy for abdominal LMS [2) were found   and ifosfamide (5 g/m2) with granulocyte colony-stimulating factor (G-CSF) given at 3 weeks interval. The patient showed a radiological response, exhibiting partial remission with a reduction in size and number of liver and lung metastases . This induced a further reduction in the number and size of metastases in the liver and lungs. Due to side effects with water retention and muscle pain we switched to 3 cycles of high dose ifosfamide 12 g/m2. Subsequent radiological assessment confirmed an almost complete radiological response to this intense schedule of chemotherapies  [Despite the paucity of randomized trials, anthracyclines, either as a single-or-multi-agent chemotherapy, are the first-line of treatment for abdominal LMS , accordi2) , (2) a c2)  (which s2) , (3) a c2)  and (4) 2) . The rat2)  in achie2) . We chosFor advanced metastatic disease, surgery or radiofrequency ablation is usually not the treatment of choice, but may be considered in selected cases where the number of metastatic foci are few and amenable to intervention. Given the remarkable response of our patient to chemotherapy, we decided to remove remaining lesions in the lung and liver using surgical resection and radiofrequency ablation with the hope of achieving long-term, complete clinical remission . The ratTo our knowledge, this is the first report of a patient with metastatic mesenteric LMS who developed complete clinical and radiological remission without any demonstrable long-term side effects from chemotherapy, after receiving multimodal therapy including an unconventional histological-subtype-tailored chemotherapy comprising 3–4 regimens, surgical resection and radiofrequency ablation of metastatic foci. Given the rarity of mesenteric leiomyosarcoma and lack of guidelines regarding oncological therapy, we suggest that multimodal therapy including aggressive histological-subtype tailored chemotherapy can result in complete remission under the guidance of a multidisciplinary team.


Solid pseudopapillary tumor is a rare primary neoplasm of the pancreas that typically affects young women. It is a relatively a benign tumor, with a favorable prognosis. We here report a 27-year-old woman with solid pseudopapillary neoplasm, who presented with mild jaundice, mildly elevated liver function tests and right upper quadrant pain. Ultrasound was suggestive of hemorrhagic hepatic adenoma; however, on magnetic resonance imaging, a heterogenous mass was found in the head of pancreas. Endoscopic ultrasound-guided fine needle aspiration (FNA) revealed tumor cells with papillary architecture and immunohistochemical analysis showing cells positive for markers including beta-catenin, vimentin, alpha 1 antitrypsin etc. These findings were consistent with solid pseudopapillary neoplasm. The patient underwent pancreaticoduodenectomy. Post-surgical biopsy confirmed the FNA findings with tumor localized to the pancreas. The patient was not given any adjuvant therapy. She remained asymptomatic and showed no signs of disease after four months follow-up. It is important to differentiate this tumor from other pancreatic neoplasms, because this type is amenable to cure after complete surgical resection, even in cases with capsular invasion, unlike malignant tumors of the pancreas. Solid pseudopapillary tumor (SPT) of the pancreas is a rare neoplasm, usually characterized by a well encapsulated mass, with low malignant potential. It occurs predominantly in young females, in their third decade of life. Histogenesis remains a debatable question: acinar, endocrine, ductal and progenitor cells have been postulated as possible beginnings of this tumor. It is frequently asymptomatic or minimally symptomatic . These tn = 15–41), alanine transaminase (ALT)-486 IU/L (n=14–54), alkaline phosphatase (ALP)-94 (n=33–116), total bilirubin-2.8 (n=0.2–1.5) with direct bilirubin-1.7 (n=0.1–0.5). Amylase, lipase and white blood cells (WBCs) were within normal limits.A 27-year-old African American woman with no prior medical history presented with complaints of epigastric and right upper quadrant (RUQ) pain for four days. The pain had a sudden onset, with intermittent attacks; it was stabbing in nature and radiating to her back. It was associated with nausea and vomiting. There was no precipitating, relieving or aggravating factor. Review of systems was otherwise unremarkable. Her vital signs were stable. On physical examination, she was mildly icteric, her abdomen was soft, non-tender and non-distended. There were no palpable masses, and bowel sounds were audible. Laboratory data revealed aspartate transaminase (AST)-286 IU/L  of the pancreas is a rare exocrine pancreatic tumor, which represents only about 1% of all tumors of the pancreas. It was first described by Frantz in 1959. Different names for this tumor were reported until it was defined by the World Health Organization (WHO) in 1996 as a “solid pseudopapillary tumor” of the pancreas . The orivimentin . The majvimentin . Despitevimentin . Neithervimentin . SurgeryIn conclusion, pancreatic pseudopapillary tumors are rare neoplasms with malignant potential. They may present as an abdominal mass, abdominal pain or, rarely, with jaundice, as in this case. Timely resection on diagnosis provides long-term survival.Conflict of interest: none declared.


Diaphragmatic herniation is an uncommon complication in the postquirurgic follow of the liver transplant. The associated symptoms are unspecific and may not suggest the correct diagnosis. It may explain why in many patients the diagnosis remains unmade or it is made only after a long interval of time. We present the case of a fifty-seven-year-old male who required an orthotopic liver transplant in 2010 due to a trifocal hepatocarcinoma. In postoperatory follow-up the patient showed alimentary regurgitation, vomiting, and dyspepsia. The diagnosis was made by an oesophagogastroduodenal transit with barium and an abdominal CT scan that showed a left diaphragmatic herniation with the gastric fundus into the thorax. With these findings we decided to perform a programmed surgery. After takedown of adhesions and replacement of the stomach into the upper abdomen, the palm-sized diaphragmatic opening was closed with a synthetic material. The patient's condition remained stable throughout the entire operation. The postoperative course was uneventful and he was discharged at the fifth day after surgery with a normal digestive intake. In a 12-month follow-up the patient shows no symptoms. Diaphragmatic herniation is an uncommon complication in the postquirurgic follow of the liver transplant. Few cases had been described in orthotopic liver transplants in adults , being mWe present the case of a fifty-seven-year-old male who required an orthotopic liver transplant in 2010 due to a trifocal hepatocarcinoma  with an alcoholic cirrhosis and hepatitis B (Child's class B (9 points) and a Model for End-Stage Liver Disease score of 18).Posteriorly he developed a stenosis of the biliary anastomosis that required hepaticojejunostomy in 2011, leading to a small leak which did not require reintervention. Two years later in 2013 he required quirurgic drainage of an abdominal collection. In postoperatory follow-up the patient showed alimentary regurgitation, vomiting, and dyspepsia. The diagnosis was made by an oesophagogastroduodenal transit with barium  and an aWith these findings we decided to perform a programmed surgery. After takedown of adhesions and replacement of the stomach into the upper abdomen, the palm-sized diaphragmatic opening  was closThe responsible mechanism of the muscle rupture after liver transplant may be a result of many factors, such a devitalized diaphragmatic muscle , a traumIschemia caused by diathermy to the diaphragm during hepatectomy with or without a raised abdominal pressure resulting from an oversized liver graft may also lead to diaphragmatic injury and necrosis, which can progress to herniation. In some cases, a degree of diaphragmatic injury is inevitable with the use of diathermy and suturing when hemostasis is achieved in the presence of diaphragmatic collaterals and portal hypertension . There iOne case of spontaneous diaphragmatic rupture has been reported 16 months after liver transplant in a fifty-eight-year-old patient who started to be treated with sirolimus two months after liver transplant for alcohol-related chronic liver disease and hepatocellular carcinoma . DecreasThe symptoms may be minimal and nonspecific, such as colic abdominal pain or digestion problems. In other cases the acute thoracic occupation may cause lung compression and mediastinum displacement leading to respiratory insufficiency and shock. The image study should include chest radiography, abdominal echography, an abdominal CT scan, and an oesophagogastroduodenal transit with barium in order to make the cause of the nonspecific symptoms clear and exclude other complications.The decision to perform a surgery on a patient with unspecific symptoms is difficult because the origin can be caused by another condition, such as subocclusive adhesions or an altered intestinal motility that may persist after the liver transplant. We suspected an iatrogenic diaphragmatic herniation because in the transplantation surgery in 2010 the patient had hepatomegaly of the left hepatic lobe and a difficult separation from the diaphragm may have caused an unseen injury.The approach to repair the diaphragm is arguable. The abdominal approach means the mobilization of the graft and bowels, which might be very laborious and harmful. In other cases the thoracic approach, which offers a good and extense exposure of the diaphragm but less control over the abdominal elements, can be preferred. We think that every case has to be taken under consideration separately, evaluating the characteristics of each patient. We decided on an open abdominal approach because of the previous interventions and the possible adherence that may make the hernia reduction through a thoracic approach difficult.Diaphragmatic hernias present with subtle symptoms but may be a cause of major bowel loss. Surgical repair should be undertaken immediately, and recurrence after the primary repair appears to be uncommon . In our


Leiomyoma originating from the scrotum is a scarce entity. We report a case of scrotal leiomyoma which was unique to us. A 35 year-old man sought medical care for a painless scrotal lesion that had enlarged over a 3-year period and was causing him discomfort. His medical history was otherwise unremarkable: there was no history of trauma, inflammation, or infection, and no significant urologic antecedent. Physical examination revealed a 2x2 cm painless skin-colored nodule of rubbery consistency on the left side of the scrotum, which was well circumscribed, pedunculate and clearly separate from the testicles and epididymis. There was no inguinal lymphadenopathy. Scrotal ultrasonography demonstrated that the scrotal contents were normal. Under local anesthesia, the scrotal mass and surrounding skin were surgically resected. Histologic examination was consistent with leiomyoma. There was no evidence of cyto-nuclear atypia. The patient's postoperative recovery was uneventful. On follow-up visit 10 days later, he did not have any complications, and the sutures were removed. At his last visit 12 months after the procedure, he was still asymptomatic and did not have any recurrence. Leiomyoma is a benign tumor of smooth muscles that may arise anywhere in the body. Smooth muscle tumors of scrotum were first described by Forsters in 1858. They arise from the dartos muscle and are extremely rare: less than 50 cases were reported in the literatures. Patients usually present with painless solitary small cutaneous lesion, and that's histology which confirms the diagnosis. Treatment is based on a simple surgical excision. Exceedingly rare, scrotal leiomyoma are usually misdiagnosed. Thus, this entity should be suspected in cases of scrotal masses.


Encapsulating peritoneal sclerosis (EPS) is classically described as progressive sclerosis and cocooning of the entire peritoneum; however, there has been limited number of reported cases of localized fibrosis as a variant form.We describe two cases of acute bowel obstruction with isolated transition points in the setting of long-term peritoneal dialysis.We postulate that some of the cases of small bowel obstruction with an obvious transition point in long-term peritoneal dialysis patients may represent a unique and localized form of EPS. We aim to emphasize the presence of macroscopic variations in presentation of EPS. Encapsulating peritoneal sclerosis (EPS) is a rare but devastating complication of long-term peritoneal dialysis (PD). It is characterized by progressive thickening and fibrosis of the peritoneal membrane, which eventually leads to encapsulation of the abdominal content by the abnormal fibrous tissue .The incidence of EPS increases with time on PD, and presents most frequently after cessation of the therapy. Kawanishi et al. reported an incidence of 17.2 % after 15 years of PD therapy . DuratioThere is an early inflammatory prodrome in many cases that can be missed. Manifestations include vague abdominal discomfort or anorexia, new-onset infusion pain, hemoperitoneum, and development of rapid transport status. Other clinical symptoms of EPS are directly related to disturbances in gastrointestinal transit, due to abnormal fibrous tissue formation around the bowel loops. The most common findings are abdominal pain, nausea, vomiting, anorexia, abdominal fullness, and eventually partial or complete small-bowel obstruction. Since the initial signs and symptoms of EPS are non-specific and subtle, a high index of suspicion is needed to establish an early diagnosis –9.The classical macroscopic appearance of advanced EPS is total encapsulation of the bowel by a fibrotic membrane . LaparotLocalized involvement of parts of the bowel with fibrosis has been reported in the past, and it has been suggested that it could be more common than previously recognized , 14.We report two cases of bowel obstruction with obvious transition point, appearing to be localized, which happened in patients after being on PD for many years. We postulate that localized fibrosis represents an atypical form of EPS.A 31-year-old woman was admitted with symptoms and signs of bowel obstruction. She had been diagnosed with systemic lupus erythematosus (SLE) about 14 years previously, and experienced multiple complications of SLE, including lupus nephritis, cerebritis and pleuritis. She had been on PD for 8 years. Other comorbidities included hypertension, hyperlipidemia and chronic anemia. She was on the waiting list for renal transplant at the time of presentation to the hospital. She had no history of peritonitis, and her PD treatment was uneventful. On initial 4-h peritoneal equilibrium test (PET), Dialysate/Plasma Creatinine (D/P Cr) was 0.71 (high-average transporter). Her lupus had otherwise been under control with no manifestations of active disease. There was no history of previous bowel surgeries or bowel obstruction.She presented with a history of nausea, vomiting and abdominal pain, with report of not having had flatus or bowel movements for a few days.Plain radiography showed dilated small bowel loops, with several air-fluid levels on the erect view Fig. .Fig. 1PlTo further investigate, computed tomography (CT) of the abdomen was obtained which showed dilated small bowel loops measuring up to 3.6 cm, with a transition point in the pelvis, where distal to this point the ileal loops were collapsed; the colon was relatively collapsed as well Fig. .Fig. 2CTInflammatory markers included declining serum albumin level for the past few months with a nadir of 23 g/L, and erythrocyte sedimentation rate (ESR) of 38 mm/first hour.The surgery service recommended non-operative management, with resting the bowels, and insertion of a nasogastric tube. The PD therapy was held in the setting of bowel obstruction; a hemodialysis line was inserted to initiate hemodialysis. The patient eventually recovered with this approach, and was tolerating oral intake upon discharge from the hospital. She elected to stay on hemodialysis after this presentation.A 57-year-old female was admitted to the hospital with signs and symptoms of bowel obstruction.Her past medical history included a diagnosis of anti-phospholipid antibody syndrome (APLAS) on anticoagulation, end-stage kidney disease, multiple previous arterial and venous thrombotic events, congestive heart failure, previous mitral valve replacement, atrial fibrillation, hepatitis C, and hypothyroidism. She had been started on PD in the form of continuous ambulatory PD (CAPD) 5 years prior to the current presentation. As with the first patient, there had been no peritonitis during her 5-year course of PD. There was no history of bowel surgery or bowel obstruction. On initial 4-h PET, D/P Cr was 0.78 (high-average transporter).Upon presentation to the emergency department, she reported a 10-day history of nausea, vomiting, abdominal distension and pain, and absence of bowel movement.To further investigate, a CT abdomen was performed which revealed fluid and gas distended loops of proximal small bowel, measuring up to 4.3 cm in diameter. This extended to the pelvis, where there was a focal cluster of distal loops displaced abnormally into the left lower pelvis with multiple transition points. The appearance was in keeping with a closed-loop distal small bowel obstruction (Fig. An exploratory laparotomy was performed during which a highly abnormal mass of small bowel loops was noted in the left lower quadrant, where the bowel loops could not be separated. Pathology report of the mass indicated a high degree of localized fibrous serosal adhesion. The abnormal mass of bowel loops was resected, with residual small bowel length of 170 cm. Similar to the previous case, serum albumin declined rapidly from a normal baseline over the preceding few months, with a nadir of 20 g/L. Other inflammatory markers were not obtained at this time. The PD catheter was removed during this operation, and the patient was permanently transitioned to hemodialysis.We present two cases of bowel obstruction with transition point in the context of long term peritoneal dialysis. Patients with longstanding PD and concurrent autoimmune disease have previously identified as being particularly at risk for EPS . EPS hasSimilarly Habib et al. reported 3 cases of “localized” EPS, where intra-operatively, an obvious sclerotic and thickened peritoneal membrane was observed, predominantly covering and constricting the terminal ileum .By reporting these cases, although surgical exploration was not performed in our first case, we aim to emphasize the presence of macroscopic variations in presentation of EPS. We speculate that some of the cases of localized small bowel obstruction, with obvious transition points, in the context of prolonged PD therapy, may in fact represent non-classic forms of EPS. The approach to management of these patients should therefore be similar to those proposed for the more generalized form of encapsulation. A conservative approach with resting the bowels should be tried, and in cases where the index of suspicion for EPS is significant, we suggest termination of PD and switching to a different dialysis modality. In case of persistent bowel obstruction despite conservative management, a surgical approach is suitable, as described in our second case, in addition to termination of PD. Further case studies are needed to investigate the use of pharmacologic agents such as tamoxifen or corticosteroids.It is suggested that clinicians have a high index of suspicion for EPS in patients on long-term PD therapy who present with acute bowel obstruction with an isolated transition point.The patients provided full informed consent for gathering the data and publishing the case.


Based on these findings, we diagnosed the patient with DIC. The patient was treated with nafamostat mesilate and fresh frozen plasma, but the DIC did not show any improvement. Subsequently, a hysterectomy was performed, after which the DIC improved. Clinicopathological findings indicated the presence of a leiomyoma with multiple vessels containing thromboemboli, and suggested that the DIC was caused by the leiomyoma. Therefore, it is essential to consider that that a benign leiomyoma may be a cause of DIC.A leiomyoma rarely causes disseminated intravascular coagulopathy (DIC). In the present report, we describe a case of DIC caused by leiomyoma. A 36-year-old nulliparous woman presented with hypermenorrhea and a lower abdominal mass. On magnetic resonance imaging, we detected a 14 cm uterine tumor, which was suspected to be a sarcoma. Blood tests at the preoperative examination indicated platelet count of 9.6 × 10 Leiomyoma occurs in 30–40% of women and is one of the most common benign gynecological diseases. Leiomyoma may present with a variety of symptoms, such as hypermenorrhea and dysmenorrhea. Furthermore, an advanced malignant disease may reportedly coexist with disseminated intravascular coagulopathy (DIC). However, cases wherein a leiomyoma has caused DIC are very rare. Magnetic resonance imaging (MRI) is useful for distinguishing between leiomyoma and sarcoma. In the present report, we describe a case wherein the tumor was suspected as sarcoma on MRI but was subsequently identified as a leiomyoma that also caused DIC.μL, Hb level was 11.7 g/dL, and platelet count was 9.6 × 104/μL, whereas the prothrombin time and activated partial thromboplastin time were normal. However, the thrombin/antithrombin III complex (TAT) level was elevated to 46.3 ng/mL , indicating that the blood coagulation system was activated. Moreover, the fibrin degradation product (FDP) level was elevated to 107.1 μg/mL , D-dimer level was elevated to 37.4 μg/mL , plasmin α2-plasmin inhibitor complex (PIC) level was increased to 6.4 μg/mL , and fibrinogen level was decreased to 54 mg/dL , indicating that the fibrinolysis system was also activated. These data suggest that DIC was present. Moreover, we performed thoracic and abdominal computerized tomography (CT) and blood examination for further diagnostic analysis; however, we did not identify any other disease causing the DIC, such as sepsis or malignant tumors other than uterine tumors. Thus, we suspected that the cause of the DIC was the uterine tumor.A 36-year-old nulliparous woman presented with a 4-5-year history of hypermenorrhea. The woman visited a clinic because of the presence of a palpable growing mass in her lower abdomen over several months. She presented with a uterine tumor of approximately 10 cm in diameter and anemia (hemoglobin (Hb) level: 4.7 g/dL) and was treated for the anemia with iron supplements. The woman was referred to our hospital for investigating the growing tumor. She had no atypical genital bleeding, except for hypermenorrhea. Physical examination indicated a mobile, firm, nontender solid mass arising in the pelvis and extending to below the umbilicus, by 1 fingerbreadth. Furthermore, vaginal sonography revealed a uterine tumor, 10 cm in diameter, which appeared as a uterine leiomyoma and had anonymous features. She had no remarkable medical history, had never used oral contraceptives, and had never received a blood transfusion. Moreover, she did not have any family history of thromboembolisms. We performed pelvic MRI with and without gadolinium contrast enhancement. On MRI, we identified a 14 cm uterine tumor in the pelvis. The uterine tumor grew from 10 cm to 14 cm within 1.5 months from the first visit. Furthermore, MRI indicated the presence of the uterine tumor with a small section of high-signal intensity on T1-weighted images (T1WI) , a heterThe woman was hospitalized and treated with 200 mg/day nafamostat mesilate  and tranIn the present report, we describe the case of a patient with DIC caused by leiomyoma. MRI suggested that the uterine mass was a sarcoma. The high blood flow, degeneration, bleeding, and necrosis caused by the multiple thrombi within the leiomyoma resulted in a sarcoma-like appearance on MRI. To our knowledge, this is the first report of a uterine leiomyoma causing DIC with specific MRI findings.In the present case, it is clear that the leiomyoma caused the DIC, as the DIC improved following the surgical removal of the uterus containing the tumor. The many causes of DIC include sepsis, severe burns, and placental abruption. Certain reports have indicated that malignant diseases may cause DIC; however, very few studies have investigated the link between benign disease, especially leiomyoma, and DIC. Four cases of leiomyoma accompanied by DIC have been reported in the English literature. Two cases were identified with degeneration of the leiomyoma during pregnancy , 3, and Leiomyoma is a benign disease, and it is important to distinguish this disease from malignant diseases such as sarcoma. The clinical symptoms of rapid growth and MRI findings are useful for the differential diagnosis between leiomyoma and sarcoma. In the present case, the uterine tumor demonstrated growth and appeared to be a sarcoma on an MRI scan. A typical leiomyoma shows an iso/low-intensity on T1WI and a low-intensity on T2WI on MRI. In contrast, sarcomas show high-intensity on T1WI, high-intensity on T2WI, and heterogeneous contrast enhancement. One study compared the MRI findings of 24 patients with either smooth muscle tumors of uncertain malignancy (SMTUMP), leiomyosarcoma, or leiomyoma clinically suspected as sarcoma . They reWe used nafamostat mesilate and FFP to treat DIC, but it did not show any improvement. Nafamostat mesilate is a synthetic serine protease inhibitor and is commonly used in Japan for the treatment of DIC . NafamosWe discussed an extremely rare case of DIC caused by leiomyoma, a common benign gynecological disease. In the present case, DIC developed because of the presence of multiple thromboemboli within the leiomyoma. The presence of necrosis, degeneration and hemorrhage due to intratumoral infarction, and multiple vessels containing thromboemboli of the leiomyoma resulted in a sarcoma-like appearance on MRI. In cases where sarcoma is suspected, based on the clinical symptoms and MRI findings, it is necessary to perform surgery and histopathological analysis for accurate diagnosis and treatment. Therefore, it is important to consider that benign leiomyoma can potentially cause DIC.


We report an unusual case of gastric carcinosarcoma with rhabdomyosarcomatous and neudoendocrinal differentiation in a 71-year-old Japanese female. Gastric carcinosarcoma with rhabdomyosarcomatous and neuroendocrinal differentiation is a rare tumor. The tumor developed in the body of the stomach and was exophytic in appearance. By histochemical analysis, the tumor was shown a part of positive for desmin and myoglobin and a part of positive for synaphtophysin and vimentin.We conclude that, though rare, gastric carcinosarcoma with rhabdomyosarcomatous and neuroendocrinal differentiation thus is reviewed in the English literatures. Carcinosarcomas are rare, malignant, biphasic tumors. In the upper gastrointestinal tract, they are most frequently observed in the esophagus, while localization in the stomach has been less frequently reported –3. We prWe reviewed the scientific literature pertaining to gastric rhabdomyosarcoma and identified several distinctive clinical features of this type of tumor.A 71-year-old Japanese female was admitted to the National Kyushu Cancer Center in April, 2012. Anorexia and vomiting were not observed; the CEA and CA19-9 levels were below the cutoff levels. Endoscopic studies revealed a Bormann II type lesion in the middle stomach (Fig. Pathologically, the tumor was identified as carcinosarcoma with skeletal muscle and neuroendocrinal differentiation. In the submucosa, there was a proliferation of oval to polygonal cells with hyperchromatic nuclei, prominent nucleoli, and a small amount of eosinophilic cytoplasm, arranged in sheets and accompanied by thin fibro-vascular septa and prominent necrosis. Mitotic figures were frequently seen. Aggregates of histiocytes and granulation tissue were recognized in the surrounding gastric wall Fig. a.Fig. 2Immunohistochemical staining was performed using the ordinary avidine-biotin-peroxidase complex, and reaction products were visualized by benzidine reaction. The sections were slightly counterstained by hematoxylin. Immunohistochemically, the tumor cells were positive for AE1/AE3, synaptophysin, desmin, and myogenin Fig. b but negIn the Japanese and English scientific literature, about 60 cases of gastric carcinosarcoma have been reported. The most commonly reported carcinomatous component of this tumor is tubular or papillary carcinoma. The sarcomatous components have still not been adequately characterized in most of the reported cases. Distinct sarcomatous components showing heterologous differentiation such as leiomyosarcoma , 16–19, There is no indication that the gastric rhabdomyomatous component of gastric carcinosarcoma in our case or in other case reports represented a from some other site; indeed, metastases to the stomach by rhabdomyosarcomas are uncommon. De la Monte et al. reported that no instances of metastasis to the stomach in a review of 17 autopsies of 22 patients who died of embryonal and alveolar rhabdomyosarcoma at Johns Hopkins Hospital between 1929 and 1983 [Gastric carcinosarcoma with rhabdomyosarcomatous differentiation has been reported in twelve cases Table  4–13]. –13. 4–13The histogenesis of gastric carcinosarcoma remains controversial. Some authors have reported a bioclonal origin, which supports the collision tumor theory , 31. OthOur experience with the present case emphasizes that gastric carcinosarcoma with rhabdomyosarcomatous differentiation exhibits aggressive behavior, the tumor, however, is extremely rare.This report described a very rare case of gastric carcinosarcoma with rhabdomyosarcomatous lesions. This case has survived without tumor recurrence, though most cases of gastric carcinosarcoma with rhabdomyosarcomatous were poor prognosis.Patient consent for publication of images has been given in writing.


Spontaneous gastric perforation is a rare entity in neonates. We report a case of spontaneous gastric perforation in a neonate operated for in-utero rupture of omphalocele.  At 35th weeks of gestational age , a baby girl was delivered via emergency C/S for intrauterine growth retardation and suspicion of strangulation of eviscerated bowel through anterior abdominal wall defect. Her birth weight was 1900 gms and Apgar score was 9 at 1st minute. On examination she had a ruptured exomphalos sac with parts of stomach, small and large intestines lying outside the abdomen . Protruded bowel was dusky but improved after resuscitation. There was no thickening or matting of bowel. At operation, there was rupture of exomphalos sac measuring 3x3 cm in diameter. Bowel was reduced easily after milking out meconium through anus and primary repair done with umbilicoplasty. She was kept electively ventilated. On postoperative D-2 baby developed abdominal distension and deterioration of her general condition. An emergent abdominal x-ray revealed pneumoperitoneum. Emergency laparotomy done through supra-umbilical transverse incision showed a stomach perforation of 1x1 cm size at the fundus . Primary repair of perforation was done after ruling out distal obstruction. Baby was extubated from ventilatory support after 2 days and had a speedy recovery. She was discharged in good clinical condition. Three mechanisms have been proposed for gastric perforation (GP) in neonates: traumatic, ischemic, and spontaneous.[1-4] Spontaneous GP is considered secondary to congenital weakness of gastric wall smooth muscles and have been reported in otherwise healthy neonate in between 2nd and 7th day of life [1]. Perforation generally occurs on the fundus and greater curvature of the stomach but may involve the posterior wall as well . Our case had intrauterine rupture of exomphalos sac diagnosed at 35th week of gestation with growth retardation. The fetus was delivered via emergency C-section. The baby had a repair of exomphalos without much delay. She had perforation of stomach on postoperative D-2 when she was on mechanical ventilation. There was no evidence of traumatic injury by orogastric tube or inadvertent injury from vigorous resuscitation. We believe the perforation in the index case is of spontaneous nature as there is no evidence of ischemia or trauma by NG tube or vigorous resuscitation. Few cases of GP due to distal obstruction are reported [5] but not found in the index case. Source of Support: NilConflict of Interest: Nil


Intraosseous hemangiomas are benign vascular malformations which are extremely rare in the maxilla, but have been reported in the mandible, zygoma and orbital region. A 12 years old female presented to the oral and maxillofacial department with an ectopically positioned upper left canine in her zygomatic bone and buccal alveolar expansion between the upper left lateral incisor and upper left first premolar.This case shows the unusual presentation of an intraosseous hemangioma associated with an ectopically migrated upper left canine tooth. It is possible that this lesion caused migration of the tooth. The clinician should be aware of the possibility of this lesion for bony expansile lesions and the importance of radiographic examination in patients who present with delayed eruption of canine teeth.How to cite this article: Kalsi H, Scannell J. Unusual Presentation of an Intraosseous Hemangioma of the Maxilla and Displaced Canine. Int J Clin Pediatr Dent 2013;6(2):124-126. On examination there was firm, bony expansion between these teeth with associated root displacement. The 22 and 24 were not mobile. The overlying mucosa was normal and the patient was asymptomatic.Radiographic examination revealed an ectopic 23 which appeared to be within the maxillary sinus or a possible large cystic radiolucency surrounding the canine as well as an indistinct area of bony expansion between the 22 and 24 . There wThe patient had the 23 region explored under a general anesthetic, as well as a bone biopsy of the lesion and a left middle meatal antroscopy. The 23 was not visible during examination of the left maxillary sinus. The expanded buccal bone appeared firm but abnormal looking with slight bleeding . A diffeHistopathology results showed vital lamellar bone with uniform osteoblast layer with minimal evidence of resorption. The soft tissue component was a mixture of adipose and highly vascular fibrous tissue. Numerous blood vessels ranged widely in size, some having muscle wall and appeared to be displacing adipose tissue. These finding were compatible with a diagnosis of an IH.On review, there was no further evidence of bony expansion or active hemorrhage from the lesion. Although the 23 was positioned in the zygomatic bone, there was no cystic evidence and therefore it was not surgically removed. No further investigations were warranted as the patient was asymptomatic. The patient is currently reviewed on a 6-monthly basis with no clinical changes. No further intervention was required for this patient as she was asymptomatic.7 and maxillary IH have been reported in the literature.6 It is possible that this lesion caused migration of the canine tooth.IH of the maxilla are extremely rare and to the authors knowledge there has been no published cases of a maxillary IH associated with an ectopically placed tooth, although cases of mandibular IH8 The maxillary canines are usually palpable in the buccal sulcus between the ages of 10 to 11, with them erupting usually between the ages of 12 to 13. In this case, the true position of the canine and the presence of an unusual lesion could have been missed without the necessary radiographs.The prevalence of ectopic canines is reported to be 1.5% and is most commonly positioned in the palate.9 Clinical features are a firm, painless swelling of the bone, occasional pressure or discomfort, bleeding from the gingival around the lesion and mobile teeth.3IH are classified as true benign vasoformative neoplasms or developmental condition of endothelial origin by the World Health Organization.3Diagnosis can be difficult due to absence of symptoms and unspecific radiological changes, but is usually made by a combination of clinical features, radiographic appearance and histopathology. CT and magnetic resonance imaging (MRI) can aid diagnosis, as well as angiography which can confirm the vascular nature of the lesion by showing its extent and highlighting feeder vessels.1 Treatment requires interdisciplinary management.5 The type of treatment largely depends on the size and location of the lesion and age of the patient and can include surgery, radiotherapy, curettage and embolization.1 Embolization of large feeder vessels is usually carried out prior to surgical treatment to reduce intraoperative bleeding. En bloc resection is usually performed with ligation of feeder vessels. Reconstruction may involve bone grafts and placement of implants.Treatment of IH is not always required and depends upon degree of disfigurement or repetitive bleeding.Clinicians should be aware of the possibility of an IH in patients presenting with a bony expansile lesion of the maxilla or mandible. Failure to consider this lesion as a differential diagnosis could lead to significant hemorrhage loss during surgical intervention. This case also highlights the importance of radiographic examination in patients who present with delayed eruption of canine teeth.