Dear Sir,Carcinosarcoma is a rare type of malignant neoplasm, which was first described by Virchow in 1864. It is composed of two types of neoplastic cells, epithelial, and mesenchymal ones. This neoplasm has a poor prognosis and may develop in many organs, including the uterus and ovaries of older women, the head and neck region, and the lungs and kidneys. Within the gastrointestinal tract, carcinosarcoma is most commonly found in the esophagus [An 83-year-old male was admitted to the surgical ward with signs of massive lower gastrointestinal tract bleeding. The patient had congestive heart failure, diabetes, and chronic respiratory failure secondary to venous thromboembolism. On digital rectal examination, a stenotic, bleeding tumor of the rectum, infiltrating the perianal tissues on the right side could be felt. The distal margin of the tumor was palpable at the level of anorectum, while its superior margin was unreachable. Anal endosonography revealed a tumor (30 mm × 53 mm × 40 mm in size) occupying the right half of the anal canal, infiltrating the sphincter muscles , with areas of necrosis Fig. .Fig. 1aDue to the deterioration of the patient’s overall condition and continued signs of active bleeding from the tumor, the patient qualified for emergency surgery (ASA IV).Intraoperatively, on rectal examination, a stenotic tumor with an ulceration at the right side of the rectal wall was confirmed. The necrotic tissue was partially removed, the rectum was rinsed with normal saline, then, the site of bleeding was coagulated, and internal packing of the rectum was carried out. An exploratory laparotomy was performed through an infraumbilical midline incision. A loop sigmoidostomy was created due to necrosis of the rectal wall and perirectal tissue. The remaining abdominal organs did not reveal any abnormalities on palpation . There were no complications in the early postoperative period. However, due to the patient’s poor overall condition, he was not a candidate for oncological treatment. The patient was discharged home on postoperative day 14 and advised to return for the histopathology results. He died 5 weeks after the surgery, due to cardiorespiratory failure.The histopathology examination of the specimen revealed carcinosarcoma of the rectum which was confirmed by immunohistochemical staining which was positive for cytokeratin, vimentin, and smooth muscle antigen Fig. . This miCarcinosarcoma of the colon is a rare neoplasm composed of both carcinomatous and sarcomatous elements. It has also been called as sarcomatoid carcinoma, carcinoma with mesenchymal stroma, carcinoma with sarcomatous change, spindle cell carcinoma, and pleomorphic anaplastic carcinoma . The proSurvival in the reported cases of rectal carcinosarcoma was never more than 6 months. Our patient died within a mere 5 weeks, which should be attributed to the advanced local state of the cancer at the time of diagnosis and the patient’s poor general condition which prevented further treatment. The case presented in this article is, to the best of our knowledge, the first involving bleeding into the gastrointestinal tract secondary to perforation of the rectal wall as a complication associated with this neoplasm. Therefore, the rare diagnosis of carcinosarcoma should be considered in patients with gastrointestinal bleeding.


Secondary bladder neoplasms are uncommon and they represent only 2% of all malignant bladder tumors.The authors present a case of a 59-year-old Caucasian man with a primary gastric adenocarcinoma that had been surgically removed 10 years before he developed bladder metastasis. He presented with low abdominal pain after 10 years without any symptoms. Cystoscopy and an abdominal computed tomography scan showed a bladder tumor. A transurethral resection of the bladder tumor was performed. A histological examination revealed an adenocarcinoma, which turned out to be a metastasis of the primary gastric tumor. One year later, abdominal surgery revealed peritoneal metastases.This is the first known case in Europe where bladder metastasis occurred 10 years after surgical removal of a primary gastric neoplasm. There are only four cases in the literature where metastases of the peritoneum developed 11 years after surgical removal of a primary gastric tumor. Bladder tumor is the eighth most common neoplasm in Hungary (the fifteenth most common in women and the sixth most common in men) . More thWe report a single case in a man with late urinary bladder metastasis 10 years after the surgical removal of the primary gastric adenocarcinoma.A 59-year-old Caucasian man presented to his doctor with gastric complaints. Gastroscopy showed an ulcer with a diameter of 5cm along the lesser curvature in the prepyloric antrum. A biopsy revealed adenocarcinoma of the stomach. The patient underwent a Billroth II resection of the stomach, omentectomy and D2 lymph node dissection. An anatomical preparation showed an exulcerated and infiltrative tumor invading all layers of the stomach Figure . HistopaImmunohistochemical staining of tumor cells detected epithelial membrane antigen (EMA)+, cytokeratin (CK)7+, CA19.9 +, CK20-, carcinoembryonic antigen (CEA)+, while prostate-specific antigen (PSA) was irrelevant. Because of its EMA positivity and CK20 negativity this urinary bladder tumor turned out to be secondary to a gastric neoplasm. Endoscopic control of the primary gastric tumor was performed after a pathologic examination, and a biopsy from the scar showed no sign of local recurrence. Positron emission tomography - computed tomography (PET-CT) presented residual malignancy in the bladder. Cystoscopy showed mucosal hyperemia and edema on the left lower part of the posterior wall and necrosis on its surface. The patient received five cycles of epirubicin, cisplatin and capecitabine (ECX) chemotherapy. He responded well to treatment and control PET and CT scans were negative. He became asymptomatic. One year later, a colonoscopy detected a 4cm sessile polyp in the ascending colon. A histopathologic evaluation revealed adenoma with high-grade dysplasia. An exploratory laparotomy was performed and, surprisingly, peritoneal dissemination was found. A histopathological examination proved metastases of the primary gastric neoplasm for a second time Figure . The tumAntibodies: CK7: , Ki67: , EMA: , CEA: , CK20: , CDX-2: , CA19.9: , TTF-1: , PSA: .Bladder metastases are rare and represent only 2% of all bladder tumors. Metastases to urinary bladder could not only originate from the direct spread of a primary tumor, but also from implantation of detached pelvic/ureteric neoplastic cells, lymphogenic/hematogenous or even peritoneal dissemination from a distant primary neoplasm. Gastrointestinal origin is suspected if a biopsy reveals signet-ring cell adenocarcinoma ,9. A priIn our case, the metastatic tumor in the bladder occurred 10 years after the primary neoplasm. Surgical intervention was decided, as we could not detect any metastasis elsewhere and could not prove local recurrence in the stomach. The literature suggests chemotherapy in case of multiple metastases. We first diagnosed bladder metastasis and one year later peritoneal metastases. During the redactation of present article the patient was still alive. Peritoneal metastases can be detected in only 17% of gastric carcinomas , which cThere are only four cases in the literature where peritoneal metastases occurred 10 years after resection of a primary neoplasm. Two cases were reported from Japan, two reports from Korea, but none from Europe. In all four cases, serosal surfaces and lymph nodes were also histologically affected. In these cases, peritoneal metastases occurred within five years after surgery but a 10-year interval for metastasis manifestation is very rare -15.The authors presented a rare case where, 10 years after the surgical removal of a primary gastric neoplasm, first a bladder and a year later peritoneal metastases occurred. This is the first known case in Europe where, more than 10 years after surgical removal of the primary gastric neoplasm, vesical and peritoneal metastases occurred. In a case of bladder adenocarcinoma, one should always think of the possibility of metastasis, even years after surgical removal of a primary neoplasm. In a case of an unknown primary tumor, further investigations are advised in order to find a possible source of the metastasis. As a solitary bladder metastasis of gastric cancer is rare, one should always search for peritoneal involvement as well. In our case, one year after the diagnosis of a bladder metastasis peritoneal metastases were found during abdominal surgery. Imaging techniques showed no sign of peritoneal involvement. All of the three surgical specimens  contained the same histopathologic type of tumor.With our case presentation, we would like to stress the necessity at routine disease control to search for distant metastases even 10 years after the primary intervention.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.All authors declare that they have no competing interests.CA was a major contributor in writing the manuscript. LT performed and interpreted the histological examinations. JP participated in and interpreted the gastric tumor operation. EC was a contributor in writing the manuscript and did the literature search. MT interpreted the surgical anamnesis and performed the imaging examinations. ZC performed the endoscopy, and gastroenterological controls, and interpreted these data. ZG analyzed the data of the surgical controls. AE participated in and interpreted the second operation. TF performed and interpreted the urological controls and examinations. ZH did the final draft proofreading and approval. All authors read and approved the final manuscript.


Paravertebral malignant tumors constitute 4.8% of cancer cases in pediatric oncology and are mostly composed of neuroblastoma (46.4%) and soft tissue sarcomas (35.7%). We describe the case of a Caucasian 6-year-old boy who was admitted for middle back pain radiated to limbs and progressively increasing weakness of the legs, suggesting a spinal cord disease. The exploration revealed two paravertebral masses extending through the neural foraminae into the epidural space. The association with elevated serum neuron specific enolase suggested at first the diagnosis of neuroblastoma, but the pathological examination revealed a Burkitt's lymphoma. This is a rare location of sporadic Burkitt's lymphoma with neurologic syndrome as first symptoms. Paravertebral malignant tumors constitute 4.8% of cancer cases in pediatric oncology and are mostly composed of neuroblastoma (46.4%) and soft tissue sarcomas (35.7%) .  Neuroblastoma (NBL), ganglioneuroblastoma, and ganglioneuroma are embryonal tumors of the sympathetic nervous system which derive from the primitive neural crest. Those primary tumors can arise at any localization coinciding with normal sympathetic nervous system structure, for example, the adrenals, the sympathetic chain, or abdominal paraganglia. Thoracic, abdominal, and pelvic paravertebral tumors can extend through the neural foraminae and compress nerve roots and the spinal cord. Catecholamines are usually elevated in the urine and serum neuron-specific enolase is a useful but not specific marker. Soft tissue sarcomas (STSs) of the paravertebral region represent about 3.3% of the pediatric STS. These lesions tend to invade the spinal extradural space and are composed of extraosseous Ewing's sarcomas or undifferentiated sarcomas in 55% of the cases .Childhood non-Hodgkin lymphomas (NHLs) differ from the common types of adult lymphoma by their pathological, immunological, and clinical characteristics. While adult lymphomas are more commonly of low or intermediate grade, more than 90% of childhood NHL are of high grade and belong to four major histological subtypes: Burkitt's lymphoma (BL), diffuse large B-cell lymphoma, lymphoblastic lymphoma, and anaplastic large cell lymphoma. Two types of BL can be defined: the sporadic (Europe and the Americas) and the endemic  varieties. The sporadic form typically occurs in the abdomen—most often the ileo-caecal area—that is, affected in more than 90% of the children with sporadic BL. Other sites include cervical lymphadenopathies, testis, bone, skin, bone marrow and central nervous system. Paravertebral involvement is a very rare presentation (<2%) of BL . A European 6-year-old male was admitted in our hospital for nonspecific constant middle back pain radiated to the limbs. Complaints include discomfort, progressive weakness of legs with abnormal sensations leading to unusual walk and limping. Symptoms increase with bending back and forward. He also suffered from chills, fever, anorecia and weight loss for 6 weeks without any infectious or traumatic context anorexia. On examination he was pale, irritable, in poor condition and painful. No tingling was described; no bladder or bowel loss of control was present. Cutaneous sensitivity and motor reflexes seemed normal. Abdominal palpation was normal. Blood investigations showed isolated anemia, major systemic inflammatory response (ESR = 109), and elevation of LDH 2328 U/L) and neuron-specific enolase (NSE = 69.5 ng/mL). Alkaline phosphatase and BUN were normal. Total spine X-ray and CT scan were normal and excluded any osseous vertebral abnormality; bone 99 Tc-scintigraphy was also normal. Sagittal spine MRI showed heterogeneity of gadolinium captation in some vertebrae, suggesting bone marrow infiltration . Ultraso328 U/L aThe association of paravertebral masses, serum NSE elevation and probable bone marrow infiltration suggested at first a neuroblastoma diagnosis.A transparietal tumoral biopsy was performed and pathological examinations revealed homogenous basophilic cells of intermediate size with hyperchromatic nuclei containing nucleoli . The tumThe patient was immediately treated by chemotherapy alone. Neurological symptoms rapidly resolved and the patient is now well and in first complete remission at 5 years.In our patient, neurological symptoms were the first manifestations of a primary paravertebral tumor. The initial presentation with back pain, progressive weakness of legs, and difficulties to walk suggested a spinal cord disease (SCD). Plain X-ray, spine CT scan, and bone scintigraphy were normal, excluding a lesion originating from the vertebra. CSF was also normal, without pleocytosis or hyperproteinorachy. MRI demonstrated two paravertebral masses extending through the neural foraminae and compressing the spinal nerve roots. The differential diagnosis of paravertebral masses includes mainly infectious and tumoral processes. Paravertebral abscesses usually develop after untreated spine infections involving vertebral body and adjacent disk interspace; this diagnosis was excluded in our patient. A variety of tumors should be differentiated when paraspinal mass is discovered, including neurogenic, neuroendocrine tumors, menigiomas, ependymomas, sarcomas, processes arising from lymphoid, connective, and osseous tissues. In children, the most frequent are sympathetic nervous system tumors (neuroblastomas), extraskeletal form of Ewing sarcomas which show a predilection for the paravertebral and costal niches and peripheral nerve sheath tumors, occurring usually in older patients. The association of paravertebral masses and serum NSE elevation in our child suggested at first the diagnosis of neuroblastoma.  Neuron specific enolase (NSE) is an isozyme of the glycolytic pathway specifically found in brain and neuroendocrine tissue. It is a tumoral marker for neuroendocrine neoplasms, namely, neuroblastoma in pediatric oncology. Nevertheless, the presence of NSE has also been demonstrated in miscellaneous tumors, including malignant lymphomas , 7 and s CNS extension occurs in 8.8% of childhood BL and most often consists of meningeal disease . Spinal Spinal cord disease is usually diagnosed at a late stage. Early symptoms may fluctuate in intensity and the findings on neurological examination may even be normal in the presence of a spinal tumoral mass. Clinical features of SCD include back pain, motor deficits, sensitivity abnormalities, sphincter disturbances, and later on paraplegia –14. As B Bilateral nodular nephromegaly—as seen in our patient—has been described in Burkitt's lymphoma . Paravertebral malignant tumors constitute 4.8% of cancer cases in pediatric oncology and are mostly composed of neuroblastoma (46.4%) and soft tissue sarcomas (35.7%) . Paraspi Even though malignancies involving the spine are not common, they account for a disproportionate degree of morbidity. Remission may be achieved, but neglected neurological deficits almost always result in permanent disability. Since the outcome of SCD is primarily determined by the patient's neurologic status at treatment initiation, the goal must be to establish the underlying diagnosis before irreversible spinal cord damage. Owing to the effectiveness of chemotherapy in BL, surgery should be rarely considered and should be reserved to rapid neurological deterioration or to diagnostic purposes.  Despite this unusual location, BL should be included in the differential diagnosis in patients with nerve root symptoms and in paravertebral processes.


The present report refers to a case of a 7-year-old male child presenting an extensive skin lesion that began when he was 5 years of age. Two biopsies were evaluated using the CD3, CD4, CD8, CD56, βF1, and TIA markers. A dense infiltrate of CD3+, CD4−, CD8+, CD56−, βF1+, and TIA+ pleomorphic lymphocytes was found in the subcutis. The previous biopsy showed cytophagic histiocytic panniculitis with a small focus on CD8+ and βF1+ malignant cells. The lesion regressed spontaneously. This case shows that prognosis may be excellent in SPTL (α/β). On the other hand, it also serves as an alert that a biopsy performed in an area of cytophagic panniculitis may lead to misdiagnosis.Subcutaneous panniculitis-like T-cell lymphomas (SPTLs)  While the 5-year overall survival rate in cases of SPTL (α/β) is generally around 82%, with Cγ/δ-TCL, the 5-year overall survival rate is 11% [Recently, the EORTC Cutaneous Lymphoma Study Group has evaluated 63 cases of SPTL and 20 cases of Ce is 11% .α/β) occurring in a child in whom involution was spontaneous.The objective of this paper was to describe an unusual case of SPTL (α/β) was made. The previous biopsy was reviewed. Anatomopathological and immunohistochemical studies. A dense and diffuse infiltrate of small and medium-sized lymphocytes with nuclear contour irregularity was found confined to the subcutaneous tissue. Rimming of the neoplastic cells surrounding individual fat cells was frequently found (βF1+ (α/β). Review of the previous biopsy then revealed vacuolated histiocytes and epithelioid cells, with few lymphocytes in the fat lobules. Only a small, peripheral focus of interstitial infiltration of malignant lymphocytes CD3+, CD8+, and βF1+ was found. Taking the diagnosis of the latest biopsy into account, this was then considered to constitute a case of SPTL (α/β) admixed with a cytophagic histiocytic panniculitis. Evolution. In the seven months that it took to reach a conclusive diagnosis, during which no treatment was given, the patient underwent a progressive and accentuated reduction in infiltration. After the definitive diagnosis, he was submitted to the following staging investigations, all of which were normal, complete blood cell count, blood chemistry panel, chest radiography, computed tomography scan of the chest and upper and lower abdomen, and bone scintigraphy. There was no evidence of T-cell infiltrate or hemophagocytosis in the bone marrow smear or biopsy specimen. It was decided not to treat the patient. Examination of the patient four months later, showed only pigmentation of the skin at the site of the lesion. At the latest follow-up visit 20 months later, the patient continued in full remission.A 7-year-old male Brazilian child presented an extensive skin lesion that began when he was 5 years of age associated with constant fever varying from 38 to 39°C. The patient was HTLV-1 negative and HIV negative. Dermatological examination revealed diffuse erythema and infiltration of the skin involving the infraumbilical area and right inguinal region extending to the anterior and internal surfaces of the upper two-thirds of his right thigh . Routinely found . Many adly found , CD20−, nd  affects a younger age group than Cγ/δ-TCL, the median age of patients being 36 years of age. This is a rare type of lymphoma; however, many cases of SPTL (α/β) may be overlooked and may have been misdiagnosed as panniculitis [βF1+ lymphocytes, confirming that this biopsy indeed corresponded to a case of SPTL (α/β).SPTL (iculitis . Weenig iculitis  describeIt is impossible to be sure whether the observed panniculitis consists in fact of areas of involution of the tumor or whether it is rather a case of SPTL that has developed from cytophagic histiocytic panniculitis (CHP). According to Marzano et al. , CHP mayα/β and γ/δ phenotypes [α/β) in children and adolescents of 18 years of age or less in which a differential diagnosis was performed with cutaneous γ/δ T-cell lymphomas by immunohistochemistry or by molecular biology [In many studies reported in the literature that include children and adolescents, no differentiation was made between the enotypes –12. On tenotypes , 14. The biology –20. Thes biology , 17. In  biology , 16 and  biology , 17. In  biology . Furtherα/β) rather than Cγ/δ-TCL because of the clinical, anatomopathological, and immunohistochemical features present. The confinement of the infiltrate to the subcutaneous tissue, as observed in the present case, constitutes a feature that is helpful in the diagnosis of SPTL (α/β). Furthermore, unlike cases of Cγ/δ-TCL, the malignant cells were CD4−, CD8+, CD56−, and βF1+ [The present case was diagnosed as SPTL (α/β) [α/β) from LED include epidermal involvement, interphase dermatitis, mucin deposition, and the presence of clusters of B cells or admixed plasma cells and reactive germinal centers [It is believed that some cases, originally diagnosed as lupus erythematosus profundus (LEP), may indeed have consisted of cases of SPTL (α/β) , 5. Impo centers , 21.α/β), characterized by a subcutaneous lesion with a waxing and waning course. Some distinguishing morphological features are present in ALLP including the absence of a greater density of infiltration, a much lesser degree of cytologic lymphoid atypia, a low proliferative index, the presence of a significant number of CD4+ lymphocytes, and lack of βF1 expression [α/β) is absent [α/β) cases in the literature, complete spontaneous resolution of the lesion occurred in the present case with no further treatment. Spontaneous involution of this disease has been previously observed in only two of the 63 cases recently reviewed in the literature [Considering that in the present case there was spontaneous involution of the disease, it is important to make a differential diagnosis with atypical lymphocytic lobular panniculitis (ALLP), a condition considered to represent a preneoplastic phase of SPTL  is suspected. A differential diagnosis between SPTL (α/β) and Cγ/δ-TCL must be performed in all pediatric cases, and all cases of SPTL (α/β) should be published in order to provide pediatric oncologists with more complete and exact data on the morbidity and therapeutic management of this disease.The current case shows that prognosis may be excellent with this form of lymphoma. On the other hand, it also serves as an alert that a biopsy performed in an area of cytophagic panniculitis may lead to misdiagnosis, hence, the importance of continued followup with repeated biopsies when SPTL (


Myxomas are the most common benign tumors of the heart. They vary widely in size, and little is known about their growth rate. The present case report is, we believe the first in bibliography that provides images of an apical left ventricular myxoma from transthoracic echocardiography and computed tomography scans taken a year apart.We present the rare case of a 65-year-old asymptomatic Caucasian man with an apical left ventricular myxoma that grew over a 12-month period. Our patient underwent successful surgical excision of the tumor and had an uneventful postsurgical recovery period.Left ventricular myxomas are benign and curable tumors. They do not usually present with systemic symptomatology and most of them are diagnosed as sequelae of syncope caused by left ventricular outflow tract obstruction or systemic embolization. Because they are usually removed after diagnosis, the growth rate of myxomas is generally unknown. We present a rare case of the asymptomatic presentation of a left ventricular myxoma and its documented growth during a 12-month period. Whereas atrial myxomas are usually associated with symptoms and signs of systemic illness  , these set al. demonstrThe exact etiology of myxomas is unknown and most cases are sporadic. Familial atrial myxomas have an autosomal-dominant transmission, but these account for <10% of the total. Although familial myxomas may be transmitted without any associated disorders, they may present as a component of a Carney complex, an autosomal-dominant condition comprising myxomas at various sites, endocrine tumors, and spotty pigmentation of the skin. It is well accepted that myxomas can develop after cardiac trauma and that radiofrequency ablation for paroxysmal atrial fibrillation (PAF) increases the risk of thrombus or endocarditis in the atrium ,4.Because myxomas are usually removed once the diagnosis is made, their growth rate is generally unknown. The reported growth rates of left atrial myxomas from previous reports vary from no growth to between 1.3 to 6.9mm/month in diameter ,6. Theseen masse.Although recurrence has been reported, LV myxomas are usually benign and curable -9. The oThe surgical approach to ventricular myxomas tends to be site-specific and efforts have been made to improve procedural outcomes and minimize the potential postoperative complications . MinimalA 65-year-old Caucasian man with a history of PAF was referred to our hospital for further evaluation of a mass located in the apex of his left ventricle. The mass was discovered incidentally after a transthoracic echocardiographic (TTE) examination during hospitalization for paroxysmal atrial fibrillation.Our patient’s medical history was unremarkable apart from an unsuccessful pulmonary vein ablation for PAF, which took place 12 months previously. On admission, our patient had a heart rate of 80 beats/minute in sinus rhythm, blood pressure of 130/68mmHg, normal carotid pulse, and absence of murmurs or extra-cardiac sounds. A physical examination was normal and our patient was asymptomatic.A two-dimensional TTE was performed, which showed a large, pedunculated, mobile mass sized 15 × 18mm located at the apical interventricular septum of his left ventricle, with a normally contracting adjacent myocardium Figure a,b. FurtOur patient underwent urgent cardiac surgery. Under cardiopulmonary bypass and cardioplegic protection, a left apical 2.5cm longitudinal ventriculotomy was performed, which exposed the mass in full view Figure .Resection of the tumor was performed through the ventriculotomy with macroscopically uninvolved margins. A pathology examination demonstrated the typical features of a myxoma Figure . Apart fEchocardiographic follow-up has not shown evidence of recurrence eight months after the resection of the myxoma.Our case represents an unusual location of a cardiac myxoma. Only three cases of apical left ventricular myxoma have been published ,7,8 and,Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.CT: Computed tomography; LV: Left ventricular; PAF: Paroxysmal atrial fibrillation; TTE: Transthoracic echocardiography.The authors declared that they have no competing interests.PK was responsible for the design and writing of the case report. EG contributed to article drafting and editing. DT provided the images of the echocardiography studies of the patient and revised the article. OK provided the images of the computed tomography studies of the patient. SKa was responsible for editing the images of the computed tomography studies. SKy was responsible for editing the images of the echocardiography studies. LK was the pathologist and provided the microscopic image of the tumor. MK was the patient’s surgeon, and provided the intraoperative images. VV was responsible for approving the article. All authors read and approved the final manuscript.


In this study we report a case of cavernous hemangioma of the testis, which mimicked a testicular teratoma. A 42-year-old male presented with a left testicular swelling that had arisen suddenly and been present for three months. Scrotal ultrasound revealed a hypoechoic mass with several calcifications in the left testicle. The mass demonstrated blood flow in the color Doppler mode. A radical orchiectomy was performed. Pathological evaluation revealed a testicular cavernous hemangioma with thrombus organization and calcification. Cavernous hemangiomas are benign vascular tumors that may develop in any part of the body. They are composed of large vessels with dilated lumina and thin walls. The vessels may have abnormal walls and cannot be identified as arterial or venous. Thrombosis and calcification are commonly observed in cavernous hemangiomas.Testicular cavernous hemangiomas are a rare type of benign testicular tumor and distinguishing them from other common testicular tumors prior to surgery is challenging. The chief presenting symptom of testicular cavernous hemangioma is testicular enlargement. In the current study, we report a case of cavernous hemangioma of the left testis, which mimicked a testicular teratoma and was treated with radical orchiectomy, and provide a review of the literature.A 42-year-old male presented with a sudden episode of left testicular fullness for three months. The patient denied any history of hematuria, fever, scrotal trauma or urinary tract infection. The patient’s past medical history and family history were non-contributory.Physical examination revealed a palpable, non-tender, left testicular mass ∼3×2.5 cm in size. The left testis was swollen and stiff. The epididymis and spermatic cord were normal. The patient had a normal blood cell count and urinalysis. Laboratory examinations, including relevant tumor markers, particularly α-fetoprotein and β-human chorionic gonadotrophin, were normal. Scrotal ultrasound revealed a roundish, well-demarcated, hypoechoic mass in the left testicle  and seveCavernous hemangiomas are benign vascular tumors, which may develop in any part of the body. The occurrence of cavernous hemangioma in the testis is rare. The first case of testicular cavernous hemangioma was reported in 1944 and, to date, 23 cases have been reported . The majThe age of onset for testicular cavernous hemangiomas reported in the literature varies from 17 weeks to 77 years . TesticuIn conclusion, testicular cavernous hemangioma is rare. When a patient presents with a testicular mass where the ultrasound reveals a mass with calcifications of various sizes and negative tumor marker findings, a diagnosis of testicular cavernous hemangioma should be considered.


Struma ovarii is a rare form of ovarian neoplasm in a form of mature teratoma and is composed predominantly of thyroid tissue. In the literature review, there has only been 10 cases of this tumor, associated with ascites and pleural effusion (Meig's Syndrome) and increased CA125 so far. In such cases, the tumor mimics malignant ovarian tumor. In this article, the case of a 72-year-old symptomatic woman with a pelvic mass, pleural and peritoneal effusion and high level of serum CA125 is presented. Cytological evaluation for the pleural fluid was performed. She underwent hysterectomy and bilateral salpingo-oophorectomy. The result of pathologic diagnosis is presented in this paper. The patient was well in postoperative period and paraclinical tests including CA 125 were normal as well. Struma ovarii is a rare form of ovarian neoplasm derived from germ cells layers in a form of mature teratoma and is composed predominantly of thyroid tissue. The preoperative diagnosis is generally difficult . AlthougA72-year-old woman with a history of gravida 10, para 10 was referred to department of Obstetrics & Gynecology of Taleghani Hospital. She had a pelvic mass that was from 2 months before, and was admitted to a local hospital complaining of dyspnea. Her medical history had included hypertension since 4 years ago and her other medical history had been normal. In physical examination, she had a top normal size uterine and palpable mass in lower side of abdomen. No lymphadenopathy was detected in auxiliary, supraclavicular or inguinal regions. In paraclinical examination, chest imagings showed massive pleural effusion on right side . There was also free intraperitoneal fluid in pelvic cavity. The sonographic figure of her mass declared a case of serous cystadenocarcinoma.Then an abdominopelvic CT scan with IV and Oral contrast was performed Figure . The resCA19-9 = 62.72CA125 = 607.4CEA = 1.92Inhibin A < 0.1The patient went through operation. Her abdominal cavity had about 700 cc of free fluid sent for cytological examination. The uterine and left ovary was normal. There was a 9 cm-diameter mass on her right ovary with vegetative lesions on its surface that was resected and sent for the frozen section.The result of frozen study was granulosa cell tumor. Therefore, the abdomen was explored and no other involvement was detected. Omentectomy was performed which was intact. The hysterectomy and bilateral salpingo-oophorectomy was performed for the patient. The permanent pathological report was struma ovarii Figure . The uteThe patient recovered normally and went to home on the fifth postoperative day. After three months of surgery, no evidence of ascites and pleural effusion was detected and the serum level of CA 125 was in the normal range (9.3 Units/ml); 12 months following the surgery the CA125 level is 12.5 Units/ml, and there is neither pleural effusion in chest X-ray  and non-mesothelial cells  [We describe an additional case with struma ovarii associated with pseudo-Meigs syndrome and high level of CA 125. Serum levels of CA-125 can be elevated in Meig's syndrome, but the degree of elevation does not correlate with malignancy . The eleAssociation of an ovarian mass with ascites and high CA 125 level in women generally suggests a malignant process. Therefore, the present case with the clinical findings of ascites, hydrothorax, high level of CA 125 and a large pelvic mass in an elderly woman suggested pelvic malignancy before operation. After surgical resection of the tumor and pathologic investigation, the benign nature of the mass became evident and complete remission of the ascites, hydrothorax, and CA125 was obtained after surgery without any further treatment. The patient is on follow-up and now after about 12 months she is symptom free.Struma ovarii is usually non functional and only 8% of patients present with symptoms and signs of hyperthyroidism, as a result of autonomous activation of the thyroid tissue . At presAccording to this report, one should know that there are some benign ovarian masses like Struma Ovarii that resemble malignant conditions initially based on their clinical or laboratory aspects and morphological appearances.In the differential diagnosis of an ovarian mass in a patient presented with ascites, high CA 125 serum and pleural effusions, but with negative cytologic examination we should consider these benign gynecological conditions too.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.CT: Computed tomography; CEA: Carcinoembryonic antigen; CA 125: Carbohydrate antigen.The authors declare that they have no competing interests.AE drafted the manuscript. NM supervised the study. MF and NM are involved in design, interpretation and data preparation. KZ analyzed the patient's tissue samples. All authors had read and approved the final manuscript.NM, AE, MF: Department of Obstetrics and Gynecology, Taleghani Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.KZ: Department of pathology, Taleghani Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.


This is a report on a 72-year-old postmenopausal woman who presented with passage of fetal bones through an umbilical fistula. She was diagnosed as a case of demised abdominal pregnancy, which had been retained for 26 years. She subsequently had exploratory laparotomy, evacuation of the abdominal pregnancy, hysterectomy, and bowel resection. The patient's condition remained unstable throughout the postoperative period and she died from septicemia on the eleventh day. Abdominal pregnancy is a rare form of ectopic pregnancy where the conceptus implants in the abdominal cavity . This isThe patient was a 72-year-old Para 4A2 widow from Sokoto, Nigeria, who was 26 years postmenopausal. She was referred from the surgical outpatient clinic (SOPD), with a 2-week history of passage of bony substances suspected to be fetal bones through the umbilicus Figures . There wThe patient was a Para 4A2, all females. Her 1st two children died at 3 and 9 years of age, respectively. Previous pregnancies were not booked for antenatal care and all her deliveries were unsupervised. She had no knowledge of contraceptives and had never used any form. She had not been sexually active since the demise of her spouse. Her past medical history was not significant. She was a pauper and was not formally educated.9/L, and the platelet count was 226 × 109/L. The liver and renal function tests all showed normal values. A plain abdominal X-ray revealed abdominal distension with multiple air fluid levels. The bowel loops, caliber, and distribution were normal. There was an irregularly outlined mass of calcific density projected over the pelvis, measuring about 9 cm × 9 cm. A sharply defined continuous structure was seen holding the mass. The underlying bone showed moderate osteoporosis and spondylosis. The impression was a huge bony calcific mass in the pelvis. A transabdominal ultrasound showed multiple calcifications in the pelvis, the size of which could not be measured. A CT-scan revealed a grossly calcified amorphous intraperitoneal tumor with adherence to the anterior abdominal wall and umbilicus. The mass measured 93 mm × 8.7 mm. There were matted omental and mesenteric tissues. The uterus was atrophic and it was difficult to delineate the adnexae.Physical examination revealed an elderly woman who was moderately pale and had pedal and periorbital edema. She was anicteric and afebrile to touch. The pulse rate was 108 beats per minute and the blood pressure was 110/70 mmHg. She weighed 47 kg. The chest was clinically clear and heart sounds were normal. The abdomen was distended with indurations in the periumbilical region but moved with respiration. The umbilical orifice was discharging a foul smelling purulent material. There were 3 human bones determined to be the fetal tibia and humerus presented by the patient. There was a hard, fixed pelvic mass of about 24 weeks size with irregular contours. The mass had both cystic and solid components. There was tenderness around both iliac fossae. The liver, spleen, and kidneys were not palpably enlarged. Pelvic examination revealed an atrophic vulva and vagina. The cervix was flushed to the upper part of the vagina and the cervical Os was closed. There was difficulty in moving the cervix and the patient declined further examination. A diagnosis of umbilical bony fistula following a neglected demised abdominal pregnancy to rule out ovarian germ cell tumour was made. General and specific investigations were requested. The PCV was 21%, WBC 6.8 × 10A wound swab was taken for microscopy and culture. She received intravenous antibiotics and oral anti-inflammatory drugs. Daily dressing of the umbilical fistula with EUSOL and hydrogen peroxide was instituted. She was transfused with 3 units of blood and had a review from the general surgeons. The patient was counseled and the social welfare department of the hospital accepted to foot her hospital bills. She was prepared for exploratory laparotomy. On 25/7/2013, the patient was taken to the theatre and had exploratory laparotomy, delivery of abdominal pregnancy, subtotal hysterectomy, adhesiolysis, bowel resection, and end-to-end anastomosis of the resected bowel segment, under general anesthesia.The intraoperative findings were a longitudinal uterine rent in the posterior uterine wall, extending to the fundus uterine. There was a well-formed fetal skeleton. There were protrusions of bony tissues—mainly small bones of the upper limbs, skull, the ribs, and spinal cord. It was difficult to visualize the uterine tubes, ovaries, and bladder due to dense adhesions. There were massive bowel adhesions and a damaged segment of the ileum, about 20 cm from the ileo-cecal junction. Adhesiolysis was done with difficulty and the damaged segment was resected and end-to-end anastomosis was performed. Subtotal hysterectomy was performed following the standard procedure.The estimated blood loss was 1,200 mL. The procedure was well tolerated by the patient, she had 2 units of blood intraoperatively, and 3 units were transfused postoperatively. Parenteral antibiotics were also given but the vital signs remained unstable. The patient died on the 11th postoperative day. The cause of death was septicemia.Abdominal pregnancy, though a rare condition, continues to generate interest because it is associated with high maternal and perinatal morbidity and mortality . The incThe clinical presentation of abdominal pregnancy depends on gestational age. In the first trimester, the symptoms are similar to those of tubal pregnancy. Thus, recent advances in imaging techniques such as ultrasound, magnetic resonant imaging (MRI) CT-scan, and immunochemistry have aided in some measures in the early recognition of abdominal pregnancy in the first trimester, but advanced abdominal pregnancy is a rare event and presents a diagnostic challenge. The diagnosis was made possible in the index case because of the clinical symptoms of persistent lower abdominal pain, suprapubic mass, and passage of fetal bones through a discharging umbilical sinus and also with the aid of imaging technology. In a 10-year review of abdominal pregnancy in Sokoto, Nigeria, Nnadi et al. observed that in most cases, persistent lower abdominal pain was likely to be present .If an abdominal pregnancy is neither diagnosed nor treated and if the fetus dies intraabdominally, the end result may be a calcified product of conception known as lithopedion. Intraabdominal calcification instead of spontaneous reabsorption of a dead mummified fetus is an unusual complication of abdominal pregnancy. Fistula formation is common in situations where the pregnancy is advanced, demised, undiagnosed, and retained for a very long time. This was the situation in this case where the pregnancy has been retained for 26 years. There was a reported case of lithopedion that had been retained for 29 years , 9. FistPitfalls in establishing diagnosis by ultrasound have been described . The treAdvanced abdominal pregnancy retained for 26 years in a 72-year-old postmenopausal woman is a very rare event. The patient's ignorance and poor utilization of medical services were contributing factors. Late presentation, prolonged retention of dead mummified fetus, extensive bowel adhesions, and advanced maternal age lead to unfavorable outcome.


Adenomyosis, also known as endometriosis interna, is the presence of endometrial glands and stroma within the myometrium. Its localised form is called adenomyoma and mimics a leiomyoma. Rarely, adenomyomas are located outside the uterus and some of them form uterus-like masses with a thick muscle wall and an endometrium-lined central cavity. They are generally located in the ovary or broad ligament, and, although they are closely related to endometriosis, their pathogenetic mechanisms are different from each other. Müllerian duct fusion defect and subcoelomic mesenchyme transformation theory are two possible pathogenetic mechanisms for this rare entity. Here we report abscessed uterine and extrauterine adenomyomas forming uterus-like masses in the left and right broad ligament and an ectopic adrenal tissue in the left paraovarian region in a 56-year-old woman. Although there is a reported abscessed adenomyosis in the literature, this is the first abscessed extrauterine uterus-like masses with synchronous pelvic pathologies like endometriosis, leiomyomas, adenomyosis, an endometrial polyp, an endocervical polyp, and an ectopic adrenal tissue. This benign lesion gives the impression of leiomyoma, a uterine malformation, or even malignancy preoperatively. Frozen section helps in differential diagnosis. An adenomyoma is a circumscribed, nodular aggregate of smooth muscle, endometrial glands, and, usually, endometrial stroma within the myometrium . The dow6/UL, 16.64 × 106/UL, and 165 × 106/UL, respectively. Hemoglobin was 9.8 g/dL; haematocrit was 28.5%. CRP was 249.1 mg/L. Urine culture was negative. Magnetic resonance imaging revealed a giant hypervascular lobulated mass measuring 8 × 12 × 13 cm, with central necrosis, filling the pelvis, indenting the uterus, rectosigmoid, and bladder. It was closely related to uterine corpus so it was thought to be originated from it  woman presented with menorrhagia after three months' amenorrhea and pelvic and lumbar pain. There was no history of a chronic disease, pelvic inflammatory disease, intrauterine device, or tubal pathology. On physical examination, she was found to have lobulated hard masses filling the pelvis up to the umbilicus. She had no fever. Transvaginal ultrasonography showed well-defined nodular masses around the uterus obscuring the discrimination between uterus and ovaries. Serum CA 19-9 and CA-125 levels were 32.94 U/mL and and 46.60 U/mL, respectively. In the whole blood, erythrocyte, leukocytes, and thrombocyte counts were 3.11 × 10 from it . It was Intraoperative evaluation revealed four leiomyoma-like masses in the right broad ligament measuring 5 to 10 cm in diameters. There was a 6-cm-diametered one in the left broad ligament and two other ones measuring 7 cm and 5 cm in the posterior and right walls of the uterus. There were also intraperitoneal adhesions and 20 cc fluid in the Douglas fossa. One of the masses located in the right broad ligament was sent for frozen evaluation. It was a peritonealised surfaced nodular mass with 8 × 7 × 5 cm dimensions and had a 4 × 1 cm central cavity containing pus. The cut surface of the mass was somewhat like a uterus with a thick muscle wall and an endometrial cavity-like central space with some nodularity in the wall. In microscopic examination, the cavity was lined by endometrial type glands and stroma and infiltrated by leukocytes, lymphocytes, plasmocytes, hemosiderin-laden macrophages, and hystiocytes . The infIn the hysterectomy specimen, there was a similar mass with 9.5 cm diameter in the right wall of the uterus. There were Nabothian cysts and a 15 × 10 × 4 mm polypoid lesion in the cervix and a 4 × 3 × 2 mm polyp in the endometrial cavity. Five leiomyomas up to 25 mm diameter and a few small bloody cysts were observed within the myometrium. The left wall of the uterus was yellowish in color. Ovaries contained small cysts, and there was a 4 mm orange colored nodule between the left ovary and left tuba. The right-sided uterine mass and intraligamentary masses had a similar microscopic feature with the one evaluated by frozen section. There were endometriotic foci and leiomyomas, one of which is palisaded type, in the myometrium. Ovaries contained follicular cysts. The orange colored nodule was consistent with an ectopic adrenal tissue mostly formed of adrenal cortical cells . In the microbiological examination, no microorganisms were isolated from the foci of abscess in both aerobic and anaerobic cultures, and there was not another primary source of infection. So, the cause of inflammation remained unknown. The patient's postoperative period was uneventful. The presence of tumor-like masses formed of endometrial glands, specialized endometrial stroma, and smooth muscle in extrauterine localisations is rare, and the terminology is somewhat arbitrary for these lesions. The term “extrauterine adenomyoma” is preferred by most authors as they are similar in most respects to their more common uterine counterparts , 8, 12. Although the first uterus-like mass is said to be described by Cozzutto in 1981, Cranstoun reported a cystic adenomyoma of uterus in 1922 , 19. TheCarinelli et al. stated that there are only 19 extrauterine adenomyomas in the English-language scientific literature . These uA Müllerian duct fusion defect is the possible mechanism for the pathogenesis of uterus-like masses. This theory is based on a developmental abnormality occurring during the formation of the female genital tract. Lack of fusion of the Müllerian ducts in a localized area, or throughout the length of the ducts, may explain various duplications or atresias of the uterus . In our Another theory is the subcoelomic mesenchyme transformation theory , 18. TheBatt et al. suggested the theory of müllerianosis—developmentally misplaced Müllerian tissue—for their case . In 2007The differential diagnosis includes endometrioma with a smooth muscle component, leiomyomatosis peritonealis disseminata, extrauterine leiomyoma with entrapped endometrioid glands and stroma (adenomyotic leiomyoma), –24, uterSurgical treatment followed by long-term GnRH agonist therapy is said to be effective in keeping the disease stable . As therThe presence of ectopic adrenal tissue in the adnexal region is an interesting finding in our case. Although it was incidentally found, the development of this ectopic gland may be related to the development of uterus-like masses embryologically because the adrenal cortex develops from the coelomic mesodermal epithelium, that is closely related to Müllerian system, in the fourth to sixth week of gestation as a cluster of cells between the root of mesentery and the genital ridge . EctopicIn Schechter's report, Morgagni was said to be first described yellowish nodules resembling adrenal tissue adjacent to the main glands in 1740 . AccessoThe clinical implication, of the adrenal rests are essential in the surgical approach of the patients. In patients who have undergone bilateral adrenalectomy due to pathologic ACTH production, compensatory hyperplasia of the ectopic adrenal tissue may be responsible for the recurrence of the disease. Another clinical aspect is the possibility of formation and development of malignant disease in the ectopic gland. Here we reported an interesting case with uterine and extrauterine abscessed adenomyomas with features of uterus-like masses. There was an ectopic adrenal tissue in the adnexal region. Although the ectopic tissue seems to be incidentally found, the development of these two separate entities may be related to each other embryologically.


A 22-year-old pregnant woman presented at the twenty-seventh week of gestation in the Emergency Department with acute abdominal pain and right iliac fossa tenderness. Urgent MRI was done and was suggestive of acute appendicitis. A laparoscopy was performed that confirmed an inflamed and purulent appendix that was removed. The technique used is described in detail. The histopathologic findings were those of acute appendicitis, carcinoid, and endometriosis of the appendix. We report the first case of this extremely rare triad presented in pregnancy. Acute appendicitis is the most common surgical emergency encountered by the general surgeon. Its incidence in pregnancy has been found to be less than 1 in 1500 . 2 saturations of 97% on air. Her chest was clear, and her abdomen was tender at the right iliac fossa with rebound tenderness but no guarding. Rovsing's sign was positive, and the bowel sounds were present and normal in nature. Vaginal examination was negative, and there was no uterine tenderness. Laboratory studies showed a white blood cell count of 13.14 × 109 g/L , neutrophils 9.20 × 109 g/L  and C-reactive protein level of 15.1 mg/L . A 22-year-old pregnant woman, gravida 2, para 1, presented at the twenty-seventh week of gestation in the Emergency Department with a 6-hour history of right-side abdominal pain. The pain started at rest and was stabbing in nature. It was worse with movements and better with simple analgesia. She was anorexic but had no nausea or vomiting. There was no history of vaginal bleeding or dysuria. Past medical and surgical histories were unremarkable. On examination, the patient was lying still and pale. She was apyrexial with a heart rate of 80 beats per minute, blood pressure of 110/60, and OThe patient's clinical presentation was highly suspicious of an acute appendicitis. An urgent (Magnetic Resonance Imaging) MRI scan was done to eliminate other differential diagnosis. This scan showed mild periappendiceal fat stranding, suggestive of early acute appendicitis . The patient underwent laparoscopy under general anesthesia. The fetus was monitored perioperatively. The first port was introduced via the left upper quadrant using Visiport 12 mm, which was followed by the 5 mm post at the right upper quadrant and then 12 mm post at the suprapubic midline under vision. We used pneumoperitoneum pressure of less than 14 mmHg. A small amount of free fluid was encountered in the right lower abdomen. After identification of the ileocecal region, an inflamed and nonperforated purulent appendix was found. The uterus was gravid, and the fallopian tubes and ovaries were normal. The mesoappendix was divided with diathermy to a healthy base. The latter was secured with PDS (polydioxanone) endoloop, and the appendix was removed in a retrieval bag via the suprapubic port. Thorough irrigation of the lower abdomen with normal saline was followed. The 12 mm port sites were closed with PDS 0 sutures. The patient had uneventful postoperative recovery. She was discharged home at the third postoperative day. Three months later, she had normal vaginal delivery of healthy full-term child.The entire specimen has been examined histologically. The appendix showed luminal pus, patchy mucosal ulceration, transmural acute inflammation, and overlying serositis, in keeping with an acute appendicitis.There was an abundant decidualised endometriosis  within tIn addition, at the tip of the appendix, there was a well-differentiated endocrine tumour  (G1, claThe simultaneous presentation of acute appendicitis, endometriosis, and carcinoid tumour of the appendix during pregnancy is extremely rare; to our knowledge, this is the first case described in the literature. Although acute appendicitis is the most common surgical emergency encountered by the general surgeon, its incidence in pregnancy has been found to be less than 1 in 1500 [Laparoscopy in pregnancy has become a more common and accepted surgical approach; however some clinicians still have concerns about the safety of pneumoperitoneum for the fetus and the mother. Large studies that compared laparoscopy and laparotomy for nonobstetric procedures found similar outcomes. Moreover, the length of stay, postoperative pain, and infection rates are generally lower in laparoscopy . We usedEndometriosis is defined as the presence of endometrial tissue outside the uterine lining and is a fairly common disease. The appendix is rarely implicated: 2.8–4.1% of all cases of endometriosis and 4/1,000 of the general population , 4. MostCarcinoid tumour is the commonest malignant neoplasm of the appendix. Others include: papillary mucinous cystadenocarcinoma, and nonpapillary adenocarcinoma . It was To our knowledge, this is the first report of the simultaneous occurrence of appendiceal carcinoid tumour and endometriosis presenting as acute appendicitis during pregnancy. Early surgical intervention of these patients will prevent complications such as perforation and peritonitis, which are potentially fatal for both the mother and the fetus. Laparoscopy enables the examination of the peritoneal cavity providing an excellent diagnostic and therapeutic procedure with minimal postoperative complications compared to the open surgery. Moreover, it can be safely performed during pregnancy. Finally, the definitive diagnosis of appendiceal endometriosis/carcinoid will be made under the microscope. We need to consider such conditions that may mimic or present with acute appendicitis and follow the patients up postoperatively as required.


Solitary fibrous tumour of the uterine cervix is an extremely rare phenomenon. We present a case of the largest cervical tumour of this type in this anatomical location reported so far.A 45-year old white female presented with abdominal pain, abnormal uterine bleedings and a 15 cm mass of the uterine cervix/left parametrium. Histological examination with immunohistochemistry of the tumour biopsy revealed diagnosis of solitary fibrous tumour. The patient underwent radical abdominal hysterectomy with bilateral salpingo-oophorectomy and pelvic lymphadenectomy. No recurrence has been observed for 8 months of follow-up.Solitary fibrous tumour can be occasionally found in patients with large cervical/parametrial masses. Immunohistochemistry was helpful in diagnosis and surgery was feasible and effective in treatment of our case of a large solitary fibrous tumour of the cervix. Solitary fibrous tumour (SFT) is a rare mesenchymal neoplasm accounting for less than 2% of all soft-tissue tumours . It may A 45-year-old white women, para 5 aborta 1 presented with lower abdomen pain. She had been treated for misdiagnosed chronic adnexitis for several months at a local clinic. Her obstetric and gynaecological history was significant for one miscarriage, two natural births, one premature breech delivery and two cesarean sections. A year before she had undergone a pelvic examination which did not reveal abnormalities. On speculum and bimanual examination there was a movable tumour of the uterine cervix and left parametrium of approximately 15 cm in size relocating normal size body of the uterus to the right side of the pelvis. Pap test was “negative for intraepithelial lesion or malignancy” and colposcopy revealed the external cervical os relocated to the right vaginal fornix, type 3 transformation zone and normal squamous epithelium covering the vaginal portio and the vaginal fornices. Transvaginal ultrasound showed a 15 cm polycyclic tumour of mixed echogenicity encompassing uterine cervix and isthmus. The uterine body of normal size and shape was relocated to the right side of the pelvis and normal adnexa were visualized. No free fluid was present in the pouch of Douglas. Abdominal and pelvic computed tomography (CT) scan revealed a 160 mm × 109 mm × 98 mm mass in the pelvis Figures  and 3. TRare histotypes of tumours such as solitary fibrous tumour can be occasionally found in patients with large cervical/parametrial masses and may cause abnormal uterine bleedings Table .Our case and all the other three described SFTs of the cervix had benign histology Table . In one Although there is no clearly defined morphologic and immunoreactivity criteria of SFT and the four described SFTs of the cervix differ somewhat in the immunoprofile Table , immunohSurgery was feasible and has been effective so far in the very large cervical SFT we treated, similarly as in the other two women with cervical SFTs for which follow-up data are available Table . The expWritten 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.SFT: Solitary fibrous tumour; CT: Computed tomography.The authors declare that they have no competing interests.AN took a major part in the diagnosis, treatment and follow-up of the patient and planned, wrote and reviewed the manuscript. WK provided histopathological work-up and description of the histological diagnosis. DW was involved in surgical treatment of the patient, provided selection of the CT scans. MSS took part in conception of the manuscript, literature search and review. WB supervised the treatment of the patient, took part in the conception of the manuscript. All authors critically reviewed and approved the manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1472-6874/14/3/prepub


ACC is a rare malignant tumor that affects most commonly the major and minor salivary glands and rarely the paranasal sinuses, lacrimal gland, larynx, ear, vulva, and so forth. The maxillary sinus when affected is considered having a poor prognosis due to delayed diagnosis and delayed treatment credited to its slow spread, late symptoms, and complex anatomy which hampers surgical resection. The expressions of tumor markers too have a significant role in determining the prognosis. The treatment of choice consists of wide radical resection of the tumor followed by radiotherapy. Rehabilitation options in cases with huge maxillary defects still need further exploration. Adenoid cystic carcinoma (ACC) is a rare malignancy, accounting for less than 5% of all head and neck cancers . ACC ariA 40-year-old male patient reported to the Department of Oral and Maxillofacial Surgery with a chief complaint of painless swelling on the left side of the face and obstruction in nasal breathing since 3 years. The swelling was slow growing, painless, and persistent in growth. There was no reduction in size of the swelling since the patient had noticed it. Personal history was negative for any detrimental habits. On extra oral examination, a large swelling was seen on left side of face, extending superoinferiorly from medial canthus of left eye till left commissure of lips and anteroposteriorly from the left lateral surface of nasal septal cartilage till 4 cm short of tragus of left ear. On nasal examination, there was severe deviation of nasal septum seen towards the right side, along with thick polyp-like mucosal obstruction in the left nostril. On eye examination, the left eye was virtually closed and raised due to the pressure from the swelling over orbit inferiorly and medially . EyeballMalignancies of the nasal cavity and paranasal sinuses represent only 3% to 5% of all head and neck carcinomas . After b


Type 1 neurofibromatosis (NF1) is a dominantly inherited neurologic disorder that affects primarily the skin, bones, and peripheral nervous system. It may be associated with a variety of clinical manifestations including cafe-au-lait spots, skinfold freckling, Lisch nodules, and visceral neurofibromas. Individuals affected by NF1 harbor an increased risk for both benign and malignant tumors. Malignant transformation is usually observed in the form of neurosarcoma. Rarely, NF1 affects the genital tract, and isolated vulvar localization is extremely rare. Here is reported a rare case of a solitary neurosarcoma of the vulva in a 43-year-old woman affected by NF1 syndrome treated with surgical excision. The purpose of this case is to underline the possibility of association between NF1 and genital tract sarcoma and to suggest an accurate evaluation of rapid growth vulvar mass in this setting. Type 1 neurofibromatosis is the most common autosomal dominant phakomatoses, with an incidence of 1 in 2500–3000 births. Most of cases are hereditary but about 30% arise de novo from spontaneous genetic mutations [Neurofibromatosis consists of at least two distinct dominantly inherited disorders: type 1 (NF1) that is responsible for about 85–90% of cases and type 2 (NF2) that was recently recognized as separate entities with responsible genes localized in chromosomes 17 and 22, respectively . Classic features of the clinical phenotype include the presence of café-au-lait spots, neurofibromas, axillary and inguinal freckling, Lisch nodules (hamartomas), and deformities of the skeletal system, as well as the risk of developing multiple tumors, especially in the central nervous system . NeurofiIndividuals affected by NF1 harbor an increased risk for both benign and malignant tumors. Malignant transformation is usually observed in the form of neurosarcoma , 7. TumoWe report the case of a solitary vulvar sarcoma in a woman with NF1.A 43-year-old Caucasian woman referred to our department because of an enlarging, bleeding, and painful vulvar mass. She has a diagnosis of NF1 since the age of 22 based on findings of multiple café-au-lait spots and subcutaneous nodules. Patient reported a mild asymmetry of the vulva since two years but she has noted a rapid enlargement of the lesion during the last month.On gynecological examination, the mass was localized on the right major labium of the vulva, measuring 15 centimeters in diameter, presenting a smooth surface and hard consistency, and was painful and easily bleeding. Urethral meatus, clitoris, vagina, cervix, and uterus appeared not to be involved. No enlarged inguinal lymph nodes were detected at palpation.The patient underwent preoperative evaluation including chest X-ray, ECG, routinary blood exams, anesthesiologic evaluation, and a magnetic resonance (MR) that confirmed the characteristics of the vulvar lesion. MR imaging revealed a 12 × 10 × 10 centimeter in size, parenchymatous mass without evidence of stromal invasion. Hepatic lesions highly suspected for metastasis were detected. No other pathological cerebral lesions were found at cerebral MR. However, a surgical resection of the lesion to reduce patient discomfort was needed. After informed consent, she underwent right hemivulvectomy with total resection of the mass . No intraoperative consultation or frozen section was performed. Histological evaluation revealed a vulvar carcinosarcoma with positivity for vimentin and focally positive for cytokeratin at immunohistochemistry analysis. Surgical margins were clear and distant about 30 millimeters from the neoplasia. No perioperative complications were reported. Adjuvant chemotherapy including 6 cycles of doxorubicin 60 mg/mq every 21 days and ifosfamide 10 g/mq for 4 days associated with mesna (AIM) every 21 days started 2 months after surgery. There is no evidence of recurrent disease on MRI of the chest, abdomen, and pelvis or on physical examination 3 months after surgery. Cosmetic and functional outcome at 4 months followup was good.Neurofibromatosis is a hereditary neurologic disorder firstly described by the German pathologist Frederich von Recklinghausen in 1882 . NF1 is caused by neurofibromin-1 gene mutations. Neurofibromin-1 is involved in the control of cellular proliferation by stimulating the intrinsic GTP-ase of p21-ras. Loss of function in NF1 results in reduced control of cell proliferation . For thiCase reports and case series have documented female genital neurofibromas, most commonly affecting clitoris with other areas of the genitourinary system or rarely the labium majors alone. Clinical presentation of genital neurofibromas is generally associated with pain, bleeding, and functional impairment, especially for advanced and infiltrating lesions .Diagnostic imaging is very important to evaluate the distinction between superficial and invasive tumors, and MR is the technique of choice to planning surgical resection of the lesion , 17.Management of this kind of lesions includes a multidisciplinary approach involving the gynecologist, plastic surgeon, dermatologist, and histopathologist. If the tumor is suitable of surgery, a surgical excision with wide margins of 3 to 5 cm of normal skin should be performed .Our patient presented a fast-growing cutaneous lesion, so the diagnosis was relatively early. Clinical management in this kind of patients was influenced by multiple factors such as age of patient, comorbidity, histopathological findings, size, location, and margins of the lesion. The comparison with other similar cases reported in the literature is difficult due to the paucity of series and the wide heterogeneity of cases relative to the original definition of histology and stage at presentation. Except for some cases diagnosed at early stage disease , 20, theFemale genital tract sarcomas are very rare; moreover, no cases of vulvar sarcoma in patients with NF1 have been ever described. For this reason, the purpose of our case is to underline the possibility of this association and to suggest an accurate evaluation of rapid growth vulvar mass in this setting.


No standard therapy for pulmonary epithelioid hemangioendothelioma (PEH) has yet been established due to the rarity of the disease, the lack of clear standards for treatment and the partial-to-complete spontaneous regression. This report describes three cases of PHE manifested as bilateral intrapulmonary masses with an initial diagnosis conducted by thoracoscopic lung biopsy. These patients demonstrated a partial response to combination chemotherapy with carboplatin, paclitaxel, bevacizumab or endostar, and an improvement in clinical status. Furthermore, we reviewed the literature regarding such patients who received chemotherapy and immunotherapy; this indicated that patients with PEH demonstrated a good partial response to chemotherapy with carboplatin, paclitaxel, bevacizumab, thalidomide and α-interferon. Overall, combination chemotherapy regimens may hold therapeutic potential for the treatment of this rare disease. Pulmonary epithelioid hemangioendothelioma (PEH) was originally named intravascular bronchiolo-alveolar tumor (IVBAT) by Dail and Liebow in 1975  scan revealed the presence of multiple nodules scattered in both lungs without hilar and mediastinal lymphadenopathy or pleural effusion . InitialThe histological diagnosis of PEH was based on the pathological examination. The pathological examination of the biopsied specimen revealed that the center of the pulmonary nodule was sclerotic and hypocellular, with hyalinization and calcification. The tumor cells were round with abundant eosinophilic cytoplasm, intracytoplasmic vacuolization and a signet ring-like appearance . ImmunohPEH disease progressed rapidly in this patient one month after pulmonary surgery. The T1-weighted magnetic resonance imaging (MRI) section examination revealed a nodular lesion in the brain, which was strongly suggestive of brain metastasis . The CT The patient was a 54-year-old female, non-smoker, who complained of chest pain, dyspnea and a dry cough for 11 months. A chest CT scan revealed intrapulmonary masses in the bilateral superior lobes, and a small right pleural effusion. Abdominal and pelvic CT scans did not reveal any lesions. A thoracoscopic lung biopsy from the right superior lobe was performed in order to examine the nodules. The postoperative course of the patient during follow-up was uneventful. Examination of the nodular sections revealed clusters of neoplastic cells as well as individual tumor cells. The normal pulmonary architecture was replaced by alveoli containing nodules of neoplastic cells and matrix. The histological features of pulmonary epithelioid hemangioendothelioma (EHE) were evident with confirmatory CD31 and CD34 immunohistochemical stains. No markers of mesothelial and muscular differentiation were observed. As a result, the patient was diagnosed with PEH. Immediately following confirmation of the diagnosis, combination chemotherapy with carboplatin, paclitaxel and bevacizumab (15 mg/kg) was initiated for six cycles, without distinct toxicities. The stabilization of the disease was evident, as the chest pain gradually subsided. Following eight months of stabilization, progression of the disease was evident. The patient survived for 15 months following the initial diagnosis.A 44-year-old female was admitted to our hospital for pleuritic pain and an irritable cough for two months. The patient did not have a history of smoking. A chest CT scan revealed innumerable nodules of various sizes (≤1.5 cm in diameter) in both lungs. Brushing and transbronchial biopsies demonstrated an inflammatory response with numerous eosinophils and no evidence of malignancy. A thoracoscopic lung biopsy from the superior middle lobe was performed. Microscopically, proliferation of histiocytic-like tumor cells with eosinophilic cytoplasm and round nuclei, and without apparent atypical morphology, were observed. Some of the cells contained intracytoplasmic vacuoles. Immunohistologically, the tumor cells were immunoreactive for CD31 and CD34, but negative for cytokeratin, S-100, CD68 and factor VIII. As a result of the examination and the laboratory findings, the patient was diagnosed with PEH. Six cycles of combination chemotherapy of carboplatin and paclitaxel were initiated. The chest CT revealed that there was no significant change in the size of the lung nodules over the period of chemotherapy, and no new metastatic lesions were detected. The patient’s symptoms of pleuritic pain and an irritable cough were improved, and a marked improvement in clinical status was observed. Following 10 months of stabilization, progression of the disease was evident. The patient survived for 25 months following the initial diagnosis.EHE (formerly known as IVBAT) is an uncommon tumor of vascular endothelial origin, with an intermediate course between hemangioma and conventional angiosarcoma –5. The dImmunohistochemical and electron microscopy studies revealed that IVBAT is of endothelial origin, and the tumor was renamed PEH –8. PEH iA previous study reviewed 15 cases of this rare tumor that involved the lungs , while aThe hallmark of PEH that is evident on chest radiographs or CT scans is the presence of multiple perivascular nodules with well- or ill-defined margins in both lungs; however, a single cavitary nodule is also observed in PEH patients . RadiogrPEH is difficult to diagnose and thus the diagnosis may be delayed. There are only two cases of PEH diagnosed by transbronchial biopsy that have been reported in the literature ,15. The The diagnosis of PEH is conducted on the basis of histological features and is confirmed immunohistochemically ,16. ImmuThe poor prognostic factors of PEH include the presence of respiratory symptoms or pleural effusion revealed by chest radiography on presentation; extensive intravascular, endobronchial or interstitial tumor spreading, hepatic metastases; peripheral lymphadenopathy; the presence of spindle cells in the tumor ; and, paet al. All patients demonstrated stabilization of the disease and a dramatic improvement in clinical status. These cases potentially aid efforts to find an effective treatment method for this rare disease. Further research and case reports are required to contribute to the data regarding the clinical findings and the natural history of this rare tumor.


Gastro-intestinal stromal tumors (GISTs) of the appendix are a rare entity. To date, only eight cases has been described in the literature, most of which have been of the benign type. We report a new case of an appendiceal GIST in a 75-year-old man. The tumor was discovered when the patient presented with acute appendiceacal peritonitis. Preoperative diagnosis of appendiceal GIST was rarely done as tumors were usually associated with appendicitis-like symptoms. Gastrointestinal stromal tumors (GIST) are the most common primary mesenchymal neoplasms of the gastrointestinal (GI) tract. GISTs occur most commonly in the stomach (60%) and the small bowel (30%). Appendiceal GISTs are extremely rare making up approximately 0.1% of all cases, with eight cases reported in the literature thus far, seven of these cases were benign. Only in one reported case, the malignant nature of the lesion was confirmed.3) and elevation of the C-reactive protein (CRP: 22 mg/dl). There were no other abnormal values on peripheral blood analysis or serum biochemical analysis. Sonography found a noncompressible distended appendix in the right lower quadrant and free fluid in the hole abdominal cavity.A 75-year-old man was brought to the emergency department with a 48-hour history of abdominal pain, emesis, and low fever. He reported that the pain was progressive in nature. The patient′s medical history was significant for hypertension. On physical exam, he was febrile (38.5°C), with normal vital signs. Physical examination demonstrated diffuse lower abdominal tenderness with peritoneal signs. Laboratory tests showed leukocytosis  are the most common mesenchymal tumours originating in the digestive tract. They have a characteristic morphology, are generally positive for CD117 (c-kit) and are primarily caused by activating mutations in the KIT or PDGFRA  They areAppendiceal GISTs are extremely rare making up approximately 0.1% of all cases , with eiBased on the previously reported 8 cases and our case, patients with appendiceal GISTs have a mean age of 67 years  with a remarkable predilection for men (3.5:1).Three tumors were associated with appendicitis-like symptoms in the absence of histologic evidence of acute appendicitis, suggesting that the symptoms were caused by the tumor , 7, 8. TConsistent with the high incidence of associated other malignancies in GIST patients in general , 4 of 9 In our case, six previous cases examined by immunohistochemistry showed a uniform coexpression of CD117 and CD34. None was positive for desmin, smooth muscle actin, or S-100. None of 3 patients with follow-up had evidence of progressive disease.The main problem with our patient was whether to consider the tumor as ruptured, as the patient was in general peritonitis, or not. In patients with tumoral rupture, it would be acceptable to administer imatinib for more than 3 years. On the other hand, our patient had general peritonitis due to a perforation of the apex of the appendix and the tumor was located in the mid portion, so that, adjuvant therapy was not administrated.Gastro-intestinal stromal tumors (GISTs) of the appendix are a rare entity. To date, only eight previous cases have been described in the literature, seven of which have been of the benign type. Preoperative diagnosis was rarely done as tumors were usually associated with appendicitis-like symptoms.


Hemangiomas are the most common benign tumors found in the liver, typically asymptomatic, solitary, and incidentally discovered. Although vascular in nature, they rarely bleed. We report a case of a 52-year-old woman with a previously stable hemangioma who presented to our hospital with signs and symptoms indicative of spontaneous rupture. We review the literature, focusing on diagnosis and management of liver hemangiomas. A 52-year-old Hispanic woman with history of a stable liver mass of undetermined etiology diagnosed over ten years ago presented to our institution with increasing right upper quadrant abdominal pain for one month that had intensified within the last two weeks. Pain intensity was rated 10 out of 10 and increased with deep inspiration. She denied any other associated symptoms, relieving or exacerbating factors. She denied any history of icteric illnesses in the past, any excessive alcohol use, or any parenteral risk factors for viral hepatitis. She had been on an oral contraceptive briefly in her mid-thirties. Her past medical history was negative for parenchymal liver disease but noted for hypertension, type 2 diabetes mellitus, dyslipidemia, obesity, and two previous uncomplicated pregnancies carried to term. Her surgical history was noted for a hysterectomy for dysfunctional uterine bleeding. Her social and family history was unremarkable. On exam, her vital signs were stable and her body mass index was 31. She was anicteric with a regular heart rate. Her lungs were clear, and abdominal exam was noted for right upper quadrant tenderness, nondistended abdomen without a palpable spleen. She had good peripheral pulses and capillary refill, without evidence of edema. Her laboratory investigation revealed an unremarkable complete blood cell count, complete metabolic panel, coagulation studies, and viral hepatitis serologies. A computed tomography of her abdomen revealed a 6.2 × 5.9 × 4.1-centimeter exophytic lesion arising from the posteroinferior aspect of the right liver lobe with a small amount of fluid tracking inferior to the lesion into the right parapelvic gutter suggestive of hemorrhage Figures . Hemangiomas are the most common benign tumors of the liver, found in one series up to 7% of autopsies . Most heThe imaging modality that is primarily used for the diagnosis of liver hemangiomas is the ultrasound. Characteristically, on ultrasound, the hemangioma is a hyperechoic lesion, predominantly found in the posterior segment of the right liver lobe located inferior to Glisson's capsule, without a peripheral hypoechoic halo and/or hypoechoic center , 13. It Grossly hemangiomas are described as “spongy” with vascular compartments of various sizes separated by fibrous tissue. Thrombi may be present and are well separated from the normal liver parenchyma despite the absence of a fibrous capsule. They express CD31, CD3 markers, and factor VIII-related antigen among other markers . HemangiLiver hemangiomas usually do not cause symptoms unless their diameter exceeds 4 cm. If smaller, they very rarely cause symptoms and almost universally the liver enzymes are within normal limits  . The majThere are a number of interventions used to treat symptomatic hemangiomas. The first report of surgery for a hemangioma was by Karp in 1931. In a study from Mayo clinic including 49 cases of hemangiomas exceeding 4 cm in diameter, 13 patients underwent surgery, from simple excision to hepatic lobectomy and 36 other patients were observed for 15 years . RuptureWe reported a case of a ruptured giant hepatic hemangioma in a nonpregnant woman that was managed with hepatic resection. In the literature, there are few reported cases of spontaneously ruptured liver hemangiomas, mostly cavernous type, most of which were treated urgently with resection or transarterial embolization due to extensive hemoperitoneum and hemodynamic instability. In our case, the patient presented and remained in a stable condition throughout the hospitalization until her surgery. Despite having imaging findings of giant hemangioma rupture, she never became hemodynamically unstable. We could not identify cases related to spontaneous rupture of a sclerosing, (ancient) liver hemangioma. A PubMed search of the English literature with the keywords “spontaneous rupture, hepatic-liver and sclerosed or sclerosing hemangioma,” yielded no results. Of course, the lack of references on spontaneous sclerosing liver hemangioma rupture may be partially due to insufficient or no reporting of pathology findings in some of the published cases. Our case suggests that spontaneous rupture may occur in a cavernous liver hemangioma with abundant sclerosing features; however, this may be responsible for a subacute presentation, not significant hemoperitoneum and no hemodynamic instability, as happened with our patient. In conclusion, a hemangioma is the most common benign lesion found in the liver. Although typically an incidental finding, discussed potentially severe complications can occur requiring prompt intervention.


We report on the evaluation and management of a 47-year-old white male found to have primary carcinoid tumor of the ileal segment of his diverting ileovesicostomy thirty-five months after initial creation. Subsequent to presentation with intermittent gross hematuria, CT urogram highlights an 8 mm enhancing lesion near the enterovesical junction of urinary diversion. Office cystoscopy confirms presence of a lesion that was later endoscopically resected and found to be a well-differentiated carcinoid tumor. Evaluation with serum markers, direct visualization utilizing endoscopy, and imaging was without finding of alternate primary or metastatic lesions. The patient ultimately had the proximal ileal portion of his ileovesicostomy excised and the distal portion converted into an ileal conduit. After briefly discussing the carcinoid tumor and the carcinoid syndrome it may cause, we review the literature on the incidence of carcinoid tumors in a population requiring the use of intestine in the urinary tract. Carcinorcinomas .A 47-year-old man, with paraplegia related to a T7 spinal cord injury suffered at the time of a motor vehicle collision nearly 30 years prior, originally presented carrying the diagnosis of neurogenic bladder that had been managed by indwelling foley catheter. At that time, physical exam demonstrated the manifestations of urethral erosion which would later be complicated by development of a necrotic phallus and eventual loss of this appendage by means of partial penectomy. As a means of urinary diversion, he underwent creation of an ileovesicostomy that was later revised due to redundancy of the efferent limb. At the time of revision, a concurrent bladder neck ligation was performed due to persistent urinary incontinence per urethra. Thirty-five-month status after original diverting procedure, a CT urogram was obtained to evaluate complaint of intermittent gross hematuria. This study demonstrated an enhancing 8 mm mass near the enterovesical anastamosis of the ileovesicostomy . Office According to the 2010 National Comprehensive Cancer Network (NCCN) guidelines for neuroendocrine tumors, triple phase CAT scan or MRI of the abdomen or pelvis is the first-line imaging modality for evaluation of a carcinoid tumor . OctreotTreatment of a localized carcinoid in the ileum should involve resection of the affected segment of ileum with associated mesentery. For patients with the carcinoid syndrome, octreotide is considered first line therapy. Depending on the organ system, therapy for metastatic carcinoid may differ. Treatment may include chemotherapy and, if feasible, resection of metastases. Precise treatment for each organ system affected by carcinoid syndrome is beyond the scope of this review. To our knowledge, this is the first case of a carcinoid tumor within an ileovesicostomy described in the urologic literature. A MEDLINE review of “carcinoid tumors” and “urinary diversion” reveals five results, a testament to the rarity of these tumors and among the reports of carcinoid in intestine used in other forms of the urinary tract; there are three cases seen in an ileal conduit –7 and onAmong the cases involving intestine used in urinary diversion, Frese et al. reported on the case of carcinoid in an ileal neobladder . Their pWe report a case of a unique occurrence, carcinoid in intestine used in the urinary tract. More specifically, we show the first case reported in the urologic literature of a carcinoid tumor in a segment of ileum used in a bladder augment. Due to the low incidence of this unique type of tumor in an urologic patient, definitive treatment algorithms are absent. Interestingly, this patient had a CT scan prior to his initial surgery of bladder augment with ileovesicostomy that was free of tumor burden. The question of whether the use of this segment of ileum in bladder augmentation contributed to the patient's carcinoid tumor is an interesting one. However, due to the low incidence of this tumor type, it is not possible to draw conclusions about the cause of the carcinoid tumor at this time.


Desmoplastic small round cell tumor is a rare malignant tumor that has a poor prognosis. It affects predominantly young males. In the current report, a 14-year-old male patient was admitted to the hospital for evaluation of abdominal distension, and abdominal pain. Imaging examination revealed a high prevalence of multiple intraperitoneal and liver parenchymal cystic and solid tumors. After an explorative surgery, the pathological findings confirmed the presentation of desmoplastic small round cell tumor. Diagnosis of desmoplastic small round cell tumor could easily have been overlooked since there was no specific evidence for this condition available in the clinical and imaging examinations. In the present study, ultrasound examination detected solid cystic masses, which suggested the presence of necrosis and hemorrhage. Immunohistochemistry and cytogenetic studies confirmed the diagnosis of desmoplastic small round cell tumor in this patient. Desmoplastic small round cell tumor (DSRCT) is a highly malignant small round cell tumor. It was first described by Gerald and Rosai in 1989 and formally named in 1991 . It is fA 14-year-old male patient was admitted to our hospital for evaluation of abdominal distension, and abdominal pain. A physical examination revealed an apparent abdominal mass and tenderness. The mass exhibited an unclear boundary and hard texture. A low degree of mobility was also observed. Ultrasonography (US) of the abdomen revealed multiple cystic-solid masses with regular shape and clear boundaries in the right liver and abdominal cavity with diameters of 12.1 cm to 4.9 cm. In addition, there was a mass in the hilum of the spleen , CK (+), Vim (+/-), epithelial membrane antigen.(EMA) (+/-), CD34 (-), CD117 (-), neuron-specific enolase (NSE) (-), WT-1 (-), and S-100 (-) Figures After the operation, it was planned he should receive seven courses of chemotherapy. Up to date, he has already undergone all cycles of chemotherapy and he is in good general condition.DSRCT has the same characteristics as those of other small round cell tumors, such as Ewing's sarcoma, small cell mesothelioma, neuroblastoma, and lymphoma . Thus, iIn most cases of DSRCT desmin, CK EMA,and vimentin are positive, of which desmin and CK being positive at the same time is considered DSRCT’s most specific immunological indexes. Stromal elements are vimentin positive, prompt from the muscle fiber mother cell.The direction of DSRCT cellular differentiation is largely uncertain. However, this tumor can express epithelial, mesenchymal, neuroendocrine, and other immunophenotypes and is cytogenetically specific. In addition, cells can generate the EWS-WT1 fusion gene .Since it may metastasize to the liver, most commonly, and lungs, and or lymph nodes of the groin,neck, or mediastinum, once abdominal imaging reveals multiple tumors, imaging of the chest with a CT scan and distant metastasis using a total body positron emission tomography (PET) scan, is recommended as the preferred. Percutaneous or open biopsy of the lesion should be evaluated by immunohistochemistry and cytogenetics to confirm the characteristics of DSRCT.DSRCT mainly occurs in children and adolescents. Primary peritoneal cases account for the vast majority of appearances of this disease, and a few cases are reported in single organs, such as the ovary, lung, kidney, pancreas, bone, and posterior cranial fossa -11. ClinSome patients may suffer from acute abdominal discomfort or cannot walk upright because of abdominal pain. The majority of patients develop a reduced body weight compared with that before the onset. Physical examination reveals an apparent mass that is palpable in the abdominal cavity that is accompanied by tenderness, an unclear mass boundary, hard texture, and a low degree of motility. Ultrasound, CT scanning, and magnetic resonance imaging (MRI) often display a large nodular mass within the pelvic or abdominal cavity, mostly in the greater omentum or mesentery, while no apparent primary lesion is detected in the parenchymal organs. In most cases, the lesions are multifocal, and some patients might present with single nodular lesions. In some cases, paravertebral involvement is also visible. Even the peritoneum may be thickened in a diffuse and nodular manner, similar to that of mesothelioma. In other cases, liver metastases occur, accompanied by ascites. Diagnosis only through noninvasive imaging examinations is difficult.In the present study, ultrasound examination detected cystic-solid masses, suggesting the occurrence of necrosis and hemorrhage. Abdominal ultrasonography findings were also highly suspicious and suggested a multifocal cystic-solid mass in the abdominal or pelvic cavity and other large organs consistent for the presentation of DSRCT. A definitive diagnosis is only obtained based on pathological findings.Current therapies are mainly dependent on surgical resection, chemotherapy, and radiotherapy. According to the patient’s condition, interventional therapy can be given, and preoperatively induced chemotherapy, surgery, and radiotherapy. Recent studies show that complete surgical excision, including a 1 to 2 mm- long implant, is necessary to achieve long-term disease control. Intraperitoneal hyperthermic chemotherapy using cisplatin is thought to be effective at reducing the recurrence rate of disease . NeverthDSRCT is a rare malignant tumor with a poor prognosis, and it mainly affects young males. Imaging examination alone is not sufficient for a definitive diagnosis, and immunohistochemistry and cytogenetic studies are suggested.The authors declare that we have no competing interests.JZ, and XX, carried out the pathology, JY, follow up cases, XL, and SF, participated in drafted the manuscript. All authors read and approved the final manuscript.


Meningitis is an uncommon complication of an untreated pituitary macroadenoma. Meningitis may occur in patients with macroadenomas who have undergone transsphenoidal surgery and radiotherapy and is usually associated with rhinorrhea. Less commonly, cerebrospinal fluid rhinorrhea has been reported as a complication of treatment of prolactinomas by dopamine agonists. Cerebrospinal fluid rhinorrhea in cases of untreated pituitary macroadenoma is reported only in isolated cases. Acute bacterial meningitis without rhinorrhea in patients with an untreated pituitary macroadenoma is an exceptional finding with only three previously reported cases.A 31-year-old male was urgently admitted for headache, fever and visual loss. Neuroimaging disclosed an invasive pituitary lesion. Cerebrospinal fluid leakage was not clinically detected. Lumbar puncture showed acute meningitis. Blood tests revealed increased inflammatory markers, a serum prolactin of 9000 ng/ml (2.5-11 ng/ml) and panhypopituitarism. Intravenous antibiotics and hydrocortisone replacement therapy were initiated, leading to a favorable clinical outcome. An endoscopic transsphenoidal debulking procedure was performed, it showed that the sphenoid floor was destroyed and the sinus occluded by a massive tumor.Meningitis should be ruled out in patients with a pituitary mass who present with headache and increased inflammatory tests, even in the absence of rhinorrhea. Typical signs leading to the diagnosis of invasive macroadenoma are visual impairment, hypogonadism and rarely signs of intracranial hypertension. Meningitis is an unusual first clinical manifestation of an invasive pituitary adenoma. A missed or delayed diagnosis could have a major impact on morbidity and mortality. Meningitis in a patient with an invasive pituitary macroadenoma is generally due to an infection of cerebrospinal fluid (CSF) leaking through the disrupted bony skull into the sphenoid sinus, allowing the entry of nasopharyngeal organisms ,2. CSF lCSF rhinorrhea in an untreated pituitary macroadenoma has been reported only in isolated cases. We report a case of an untreated invasive macroprolactinoma revealed by acute meningitis. The diagnostic and therapeutic challenges of bacterial meningitis in case of an invasive pituitary adenoma are discussed.A 31-year-old man presented at the Emergency Department with a six day history of severe headache and vomiting. No history of trauma or neurosurgery was reported. The patient mentioned an important visual loss in the left eye and a decreased libido for at least two years. No history of rhinorrhea was reported.On admission, clinical examination revealed a normal level of consciousness. Complete left eye blindness with no deficit of other cranial nerves was observed. No rhinorrhea was detected. Body temperature was of 38°C. Computerized tomography (CT) and magnetic resonance imaging (MRI) disclosed a large, invasive pituitary lesion, which extended to the cavernous sinuses, to the sphenoid sinus and to the third ventricle, compressing the optic chiasm Figure . No intrAt endoscopic transsphenoidal approach a large bony defect in the inferior part of the sphenoid sinus was detected, obstructed by a massive tumor protrusion. Intraoperatively, a dural defect was not clearly identified. A significant reduction in tumor size was achieved through surgery, confirmed by MRI imaging. Histopathology confirmed the diagnosis of pituitary adenoma. No evidence of hemorrhage or necrosis was detected. No recovery from visual loss and panhypopituitarism was observed. Treatment with cabergoline 0.5 mg twice a week and levothyroxine 75 μg per day was continued, with normalization of serum prolactin and free T4 Table . HydrocoThe incidence of meningitis in patients with untreated pituitary macroadenomas is low and difficult to be estimated, as most information comes from case reports or small series ,7. MeninStreptococcus pneumoniae[The first study assessing the incidence of nonsurgical CSF rhinorrhea among patients with pituitary adenomas was published in 2007 by Suliman et al. [neumoniae.Our patient presented with acute bacterial meningitis in an untreated pituitary macroadenoma not preceded by CSF leakage. Only three cases have been reported in the literature. Their major clinical characteristics are summarized in Table Meningitis is an unusual complication of untreated invasive pituitary adenomas and only represents the initial symptom leading to diagnosis of a macroadenoma in exceptional cases. Differential diagnosis between meningitis and pituitary apoplexy in a patient with an invasive macroadenoma presenting with headache and fever may be challenging. In the presence of inflammatory biology, a lumbar puncture must be performed without delay to treat rapidly meningeal infection. A missed or delayed diagnosis could have a major impact on morbidity and mortality. The absence of CSF rhinorrhea does not rule out the possibility of meningitis.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.CSF: Cerebrospinal fluid; CT: Computerized tomography; MRI: Magnetic resonance imaging.The authors declare that they have no competing interests.MB led the conception of this case report, performed the review of literature and drafted the manuscript. NB provided clinical care for the patient. DB performed radiologic interpretation. BC and FD critically reviewed the manuscript. All authors read and approved the manuscript.