We report a case of a 33 year-old pregnant woman who was diagnosed at the time of the first trimester ultrasound with a multilocular solid arising form the right ovary. An abdominal MRI was performed afterwards and it revealed a pelvic mass, developing from the right ovary, with a liquid component but with a major solid area. CA 125 was within the normal range values. A laparotomy followed by right salpingo-oophorectomy was performed at 14 weeks of gestation and both the frozen section and the definitive histology revealed a borderline mucinous ovarian tumor. Ovarian tumors of low malignant potential comprise 10%–20% of all ovarian malignancies. They carry an excellent prognosis with 95%–99% long-term survival. Whereas in the past, radical surgery  was standard regardless of the age of the patient, unilateral salpingo-oophorectomy with or without staging has become the recommended management for women who desire childbearing. In the absence of large prospective randomized trials it is difficult to know which are the best management practices and especially to determine the right moment during pregnancy to perform surgery in these patients. The incidental finding of an adnexal mass in pregnancy is becoming more common with the increased use of ultrasound. Data from the literature supports a high rate of spontaneous resolution and an extremely low rate of clinically relevant pathology. It is also important to note that no prospective, randomized trials are available to evaluate the various diagnostic and therapeutic options. The overall incidence of adnexal masses in pregnancy can vary greatly based on study population, use of sonography, and gestational age at presentation , 2. It iWe report a case of a 33-year-old gravida 2 para 1 that was referred to our department after the first trimester ultrasound scan (performed in another institution) had revealed a huge ovarian mass. The patient was asymptomatic, denying weight loss and abdominal pain or distention. A more detailed medical record was obtained and there was a positive family history of borderline ovarian tumors (BOTs). The physical examination showed a palpable abdominal mass that reached the umbilicus. The ultrasound performed in our institution showed a 12-week singleton pregnancy and an adnexal mass, measuring approximately 17 × 11.5 cm, with a heterogeneous and highly vascular solid component, arising from the right ovary . An abdoAfter discussion with the patient secondary staging surgery  was performed and no evidence of disease was found. Currently the patient is being followed (36 months after the first surgery) and all imaging data show no signs of disease relapse.BOTs are a distinct histological entity and they account for nearly 10%–20% of all ovarian epithelial tumors , 4. ApprAccording to the literature, BOTs are frequently diagnosed during the first trimester and usually they are detected in routine ultrasound exams. When symptomatic, patients may refer to unspecific abdominal pain . Pelvic ultrasound remains the mainstay for evaluating the adnexa. The primary purpose is to characterize the mass and identify features that have been associated with an increased risk of malignancy, such as size >7 cm, solid components, “complex” appearance, papillary structures, internal septations, bilaterality, irregular borders, ascites, increased vascularity, and low-resistance blood flow. Bearing these features in mind, in our patient, all sonographic features were highly suspicious of malignancy. Accordingly, when there is a strong suspicion for malignancy, surgery is often indicated –6. AlthoAnother point of discussion is the surgical route. Laparotomy is usually the most definitive and traditional approach to remove adnexal masses during pregnancy. A midline vertical incision is often indicated to maximize visualization and to allow an optimal exploration of the abdomen. Bearing in mind once again the imaging features of the adnexal mass of our patient, a midline vertical laparotomy was performed not only due to the size of the tumor but also due to the high risk of malignancy. Laparoscopy may be an option because it has been shown as an effective and safe method for removing adnexal masses during pregnancy –3, 5, 6.Confirmed low malignant potential tumors (in the frozen section examination) during pregnancy can be treated conservatively by salpingo-oophorectomy, peritoneal washing, and abdominal exploration . Due to In conclusion, data from the literature is not consensual on the management of BOTs during pregnancy.


Coincidental occurrence of hepatocellular carcinoma (HCC) and cholangiocarcinoma, known as “collision tumors”, within a cirrhotic liver is rare. Herein, we report a case of liver transplantation (LT) in a patient with such collision tumors. Our patient was a 56-year-old woman with hepatitis C virus-related cirrhosis and 2 focal hepatic lesions, measuring 1.5 and 3 cm, in the liver segments 8 and 5, respectively. The lesion on segment 8 showed the typical radiological characteristics of HCC; however, the lesion in segment 5 showed an atypical vascular pattern and was closely associated with the inferior vena cava. Serum alpha-fetoprotein level was normal and serum carbohydrate antigen 19-9 (CA19-9) level was slightly elevated (63 U/mL); the extrahepatic spread of HCC was ruled out. The patient underwent an uneventful deceased-donor LT. Histopathological examination of the explant confirmed that the lesion on segment 8 was an HCC, but surprisingly, the lesion on segment 5 was found to be a cholangiocarcinoma. Six months after LT, the serum CA19-9 level was markedly elevated (255 U/mL), and the patient began experiencing abdominal pain. Magnetic resonance imaging showed enlarged hilar and paraaortic lymph nodes that were suggestive of metastases; histopathological analysis using ultrasound (US)-guided biopsy confirmed recurrent cholangiocarcinoma. Unfortunately, the patient died because of tumor recurrence 9 months after LT.Collision tumor resulting from the co-existence HCC and cholangiocarcinoma in a cirrhotic liver is rare and has a negative impact on the outcome of LT. Atypical vascular pattern and elevated serum CA19-9 levels are suggestive of such tumors; patients with these findings should undergo a targeted biopsy to rule out the coincidental occurrence of HCC and cholangiocarcinoma. Focal hepatic lesions in cirrhotic livers are common and suggest the presence hepatocellular carcinoma (HCC). Appropriate diagnosis of these lesions is crucial in choosing appropriate treatment strategies, ranging from liver transplantation (LT) to regular follow-ups . Recent 6 IU/mL. The findings of a liver biopsy revealed the recurrence of hepatitis C virus (HCV) infection. The patient was administered 180 µg of peginterferon alpha-2a (weekly) and 800 mg of ribavirin (daily). Three months later, the level of liver enzymes increased rapidly and the measured viral load was undetectable. A repeat liver biopsy suggested moderately acute cellular rejection. The patient was treated with a 3-day course of pulse steroids, which resulted in a significant decline in the level of liver enzymes. Ultrasonography revealed a mass in the porta hepatis. Serum CA19-9 level was markedly elevated (255 U/mL), and the patient began experiencing vague abdominal pain. MRI confirmed the findings of enlarged hilar and paraaortic lymph nodes that were suggestive of metastases (We report the case of a 56-year-old woman presenting with decompensated cirrhosis caused by hepatitis C virus (Genotype 4). As a part of our routine pre-transplant workup, we performed dynamic abdominal computed tomography (CT), which showed 2 focal hepatic lesions, measuring 1.5 and 3 cm, in the liver segments 8 and 5, respectively. On dynamic imaging, the lesion in segment 8 showed the typical vascular pattern of an HCC , but thatastases ; ultrasotastases .Combined HCC and cholangiocarcinoma is a rare form of primary liver cancer that usually presents as "mixed tumors", wherein regions of hepatocellular and biliary epithelial differentiation are intimately mixed within the same tumor -10. HoweCollision tumor resulting from combined HCC and cholangiocarcinoma in a cirrhotic liver is rare and has a negative impact on the outcome of LT. Atypical vascular pattern and elevated serum CA19-9 levels are suggestive of such tumors; patients with these findings should undergo a targeted biopsy to rule out the coincidental occurrence of cholangiocarcinoma LT is considered.


Cutaneous neuroendocrine carcinoma (cNEC) is rarely seen in the external ear. In this paper, we newly describe a patient with cNEC in his right external auditory canal, followed by a further discussion on the clinical features, diagnosis, and treatments of cNEC of the external ear. A review of the literature showed that cNEC of the external auditory canal generally presents as asymptomatic and that pathology yields the most confirmative diagnosis. A wide resection with adjuvant radiotherapy and chemotherapy is recommended. The overall prognosis of this condition is poor. Neuroendocrine carcinoma (NEC) constitutes a heterogeneous group of neoplasms that have been postulated to originate from a common precursor cell population. Though commonly associated with the gastrointestinal tract and bronchopulmonary system, a substantial number of these tumors originate in less common anatomical sites and can range from indolent, unrecognized entities to highly active, metastatic secretory tumors. In 1972, Toker , and onlIn this paper, we newly describe a patient with cNEC in his right external auditory canal, followed by a further discussion on the clinical features, diagnosis, and treatment of cNEC of the external ear.A 62-year-old, otherwise healthy male was referred to our hospital in November 2007 with a two-year history of a painless mass in his right external auditory canal. The patient had surgical history positive for removal of a small keratoacanthoma in his right external auditory canal in 2001. Since then he had not experienced any discomfort or symptoms until a mass was found in his right external auditory canal during a routine physical exam in 2005. As the lesion was asymptomatic at that time, the patient refused immediate resection proposed by his doctor and decided to postpone treatment until after he retired. Myringoscope revealed a mass in the deep right auditory canal near the tympanic membrane . The masThe mass was excised through a microscope-guided surgery, during which we removed the flesh-colored mass that was adherent to the surface of tympanic membrane and preserved the canal wall and the tympanic membrane. The excised mass was then sent to pathology.Tissues were fixed in 10% formalin, imbedded by paraffin, routinely sliced, and HE stained. To our surprise, through light microscopy, we found that the tissues demonstrated cord, trabeculae, or cluster proliferations of moderately differentiated cells in dermal and proper layers, with pleomorphic nuclei and inconspicuous nucleoli, in addition to frequent mitoses. There were also scattered cell fragments with pyknotic nuclei, indicating apoptosis. The cuticular layer was not involved Figures .In order to confirm the diagnosis, immunohistochemistry was carried out on paraffin sections using standard techniques with antibodies against cytokeratin (CK), epithelial membrane antigen (EMA), vimentin, neurone specific enolase (NSE), S-100 protein, smooth muscle actin (SMA), synaptophysin, glial fibrillary acidic protein (GFAP), and chromogranin (ChG). The result revealed strongly positive for CK and NSE Figures  and 4 anAs we did not see normal tissues on certain regions of the tumor margin, a secondary surgery was proposed for complete clearance of the tumor. The patient underwent a PET scan two days after initial surgery, which did not show evidence of local lymph node involvement or metastasis. Nine days after the first surgery, the patient underwent a sleeve mastoidectomy and tympanotomy, during which we removed the tympanic membrane and performed a sleeve excision of skin and bone in the right external auditory canal. The tympanic cavity was clean. A temporoparietal fascial flap was used to reconstruct tympanic membrane and a postauricular free flap was created to cover the external auditory canal. One week after the second surgery, the patient received a chemotherapy combined of cisplatin, calcium folinate, and tegafur for a three-day course. In addition, a radiotherapy protocol was added, that a level of 200 cGy radiation was delivered each time for a total of 30 times. The patient did not experience any signs of adverse reaction throughout the entire postoperative treatment. He completed the treatment three months after his admission, and was asked for routine followup every two months in the first six months and every half a year thereafter. The total follow-up period was 2 years, no evidence of tumor recurrence was found in the patient.cNEC that occurs in the head and neck is much more commonly seen in cheeks, nose, and larynx than in the external ear . Up to nIt is generally believed that the risk factors for cNEC include exposure to radiation, immunosuppression, and old age. Yet only the age factor was presented in our patient. Consistent with most case reports that we identified, our patient presented no obvious symptoms or signs. Unlike NEC occurred in the lung or pancreas, where secretion of tumor cells might lead to noticeable symptoms, cNEC rarely causes carcinoid syndrome. This is perhaps due to the fact that the tumor cells of cNEC are usually functionally silent.In this case, evidences including patient's medical history and preoperative tests indicated that the mass was most likely benign, but the more conclusive postoperative pathology confirmed it as malignant. Clinical signs and symptoms were negligible so that this tumor may lack features distinct from other tumors that commonly occur in the external ear, such as basal cell carcinoma, melanoma, lymphoma, and ceruminous gland carcinoma. In fact, early diagnosis of cNEC of the external ear should be a challenge for both patients and clinicians because it often appears as a tiny, indolent, and unspecific entity. However, once there is local lymph node involvement or metastasis, which are not rarely seen until the later stage of this tumor, the overall prognosis is far worse due to the advanced presentation of the disease . TherefoPathologic examination and immunohistochemistry are essential to confirm the diagnosis of cNEC and the latter one may provide useful information for the differential diagnosis of other tumors that appear similar under microscope. Our case refers to a moderately differentiated histotype, as characterized by tumor cells with a relatively small to medium-sized uniform appearance, in addition to pleomorphic nuclei and inconspicuous nucleoli. There were mitotic figures and scattered cell fragments with pyknotic nuclei, indicating apoptosis. It would also be helpful to confirm the diagnosis of cNEC if neurosecretory granules present within the cytoplasm of tumor cells.131I-MIBG, 111In-octreotide, or 90Y-lanreotide, was observed in NECs of digestive system [Surgical intervention is generally accepted as the standard treatment of care and the only approach that can achieve a cure in patients with NEC at any site. The most common method is wide resection of the primary tumor. The role of prophylactic neck dissection in the standard treatment of cNEC of the external ear remains unclear, but may reduce the recurrence rate. In addition, adjuvant treatments of radiotherapy to the primary site and primary nodal chains, as well as combination chemotherapy, are also recommended because they have shown considerable short-term and long-term control rates in clinical trials , 10. OurPatient's age and physical condition, tumor size, tumor staging, histological differentiation, and treatment are all prognosis factors for cNEC. Particularly, TNM staging is considered to be the most consistent and independent predictor for survival rate in MCC cases . AccordicNEC rarely occurs in the external ear and usually presents as MCC. Clinical features alone do not lead to an easy diagnosis. Therefore, biopsy or excision is essential for early detection of cNEC if an indolent mass is found in the external ear. Only histological diagnosis is conclusive and the contributions of pathologic examination and immunohistochemistry are crucial. A wide resection with adjuvant radiotherapy and chemotherapy may minimize the risk of tumor recurrence. The overall prognosis of cNEC of the external ear is poor, but several prognosis factors can influence its final outcome.


Objective. Primary intramedullary spinal germ cell tumors are exceedingly rare. As such, there are no established treatment paradigms. We describe our management for spinal germ cell tumors and a review of the literature. Clinical Presentation. We describe the case of a 45-year-old man with progressive lower extremity weakness and sensory deficits. He was found to have enhancing intramedullary mass lesions in the thoracic spinal cord, and pathology was consistent with an intramedullary germ cell tumor. A video presentation of the case and surgical approach is provided. Conclusion. As spinal cord germinomas are highly sensitive to radiation and chemotherapy, a patient can be spared radical surgery. Diverse treatment approaches exist across institutions. We advocate biopsy followed by local radiation, with or without adjuvant chemotherapy, as the optimal treatment for these tumors. Histological findings have prognostic value if syncytiotrophoblastic giant cells (STGCs) are found, which are associated with a higher rate of recurrence. The recurrence rate in STGC-positive spinal germinomas is 33% (2/6), whereas it is only 8% in STGC-negative tumors (2/24). We advocate limited volume radiotherapy combined with systemic chemotherapy in patients with high risk of recurrence. To reduce endocrine and neurocognitive side effects, cranio-spinal radiation should be used as a last resort in patients with recurrence. Germ cell tumors are similar in histology to germinal cells of the genital organs, and they may aberrantly arise in the central nervous system (CNS). Germ cell tumors account for 1% of all CNS tumors, and they are seen more frequently in Japan (3%) and East Asia 12.5%) [.5% 2–4]4]; howevWe present a patient with a primary intramedullary thoracic germ cell tumor. This patient is one of only a handful of reports of a primary spinal germ cell tumor from the western hemisphere .A 45-year-old man presented with numbness and weakness in his lower extremities, worsening over the course of a year. His weakness substantially worsened over the 4 months prior to presentation, during which he became fully dependent on a walker. He was also intermittently incontinent of urine and chronically constipated.On physical examination, from the T2 level and below, he had right loss greater than left loss of light touch, pinprick, and proprioception sensation. On motor examination, his right leg strength was 0/5 and his left leg strength was 4/5. He had bilateral clonus and muscle spasms in his legs. β-HCG were within normal limits. Magnetic resonance imaging (MRI) demonstrated intramedullary signal within the upper thoracic spinal cord with diffuse enhancement and cord expansion . ImagingDifferential diagnosis of this lesion included primary spinal cord neoplasm, such as astrocytoma and ependymoma, hemangioblastoma, intradural metastasis, and lymphoma. Ischemia and lupus myelitis were also included in the differential diagnosis. The patient was taken for open biopsy/resection of this lesion. http://dx.doi.org/10.1155/2013/798358, which demonstrates surgical approach and technique). The patient was placed prone on the operating room table. Motor and sensory neuromonitoring was established prior to incision. Motor evoked potentials (MEPs) and somatosensory evoked potentials (SSEPs) were absent below the level of the lesion. The correct levels were ascertained using fluoroscopy. Bilateral laminectomies were then performed at T3, T4, and T5. After the thecal sac was decompressed, attention was turned towards opening the dura and identifying the tumor. At this time, the microscope was brought into the operation. Under the microscope, the dura was opened. The cord was noted to have yellow discoloration, but otherwise no gross abnormality was observed  . Spinal Spinal germinomas occurred most frequently within the thoracic spinal cord (47%), followed by the thoracolumbar area (27%), the lumbar area (20%), and least frequently the cervical spinal cord (7%). Spinal germinomas are also primarily seen intramedullary in 70% of cases. They had both intramedullary and extramedullary components in 17% of cases and were purely extramedullary in 13% of cases. β-HCG, which may lead to CSF or serum elevation of this hormone, elevated β-HCG levels do not definitively indicate the presence of STGCs. Histological findings of CNS germinomas may have prognostic value if STGCs are found, as these are associated with poorer prognosis and a higher rate of recurrence . While Sβ-HCG, only 55% (6/11) of these 11 were STGC positive. However, 100% of cases that were STGC positive were also β-HCG positive (6/6) (We found that while 37% (11/30) of cases had elevated levels of ve (6/6) .The overall recurrence rate of spinal cord germinomas after treatment was 13% (4/30), bolded in Clinical symptoms and imaging features of primary spinal germ cell tumors are often indistinguishable from other spinal cord neoplasms. Only histological examination can establish a definitive diagnosis. If frozen-section biopsy during surgery suggests the possible diagnosis of germinoma, radical resection of the tumor should not be undertaken. However, if frozen section favors a high-grade tumor (as in our case), the extent of resection must be guided by preoperative neurological function, the reliability of neuromonitoring signals, the extent of cord compression, and the amount of medullary invasion.Like intracranial germinomas, spinal cord germinomas are also highly sensitive to radiation. Most cases can be successfully treated with external beam radiotherapy despite the lack of standard guidelines for fractionation and dose. In our particular case, 3D conformal external beam radiation therapy (EBRT) was utilized to treat the spinal cord from C7 to T7. This field was treated to a dose of 45 Gy in 25 fractions using 15 MV photons and AP/PA portals. Daily kV imaging was performed for image-guided radiotherapy (IGRT) to ensure precise patient positioning. In our review, we found the average amount of local radiation to be 35.7 ± 13.4 Gy and the average amount of craniospinal radiation to be 30.4 ± 12.1 Gy. In order to reduce the endocrine and neurocognitive side effects of craniospinal radiation, it should be reserved for patients with STGC-positive tumors or patients with recurrence. Intracranial germ cell tumors are also highly sensitive to chemotherapy, especially to platinum and alkylating agents. However, there are no standard guidelines for chemotherapy for spinal germinomas. In our case review, 50% of the patients received systemic chemotherapy. The common chemotherapeutic agents found to be effective for CNS germinomas are cisplatin, bleomycin, vinblastine, and etoposide. The treatment with chemotherapy alone in CNS germinomas has been investigated . In one One report describes the complete remission of a pineal gland germinoma after 2 cycles of BEP therapy  with no further need for radiotherapy . HoweverGenerally, some form of radiation is recommended to maintain long-term control. Although chemotherapy has the advantage of decreasing the total dose of radiation, chemotherapy can have significant side effects. A treatment regimen consisting of PVB therapy  or BEP therapy avoids the use of alkylating agents and can spare sterility; yet it can have significant renal and auditory toxicity . Other lAlthough primary spinal cord germinomas are extremely rare, there are reports of these tumors primarily in the Japanese literature. Definitive diagnosis is imperative. As spinal cord germinomas are highly sensitive to radiation and chemotherapy, a patient can be spared radical surgery. Recurrence of spinal germinomas is uncommon and associated with STGC positivity. We advocate surgical biopsy followed by local radiation, with or without adjuvant chemotherapy, as the optimal means of treatment of spinal germ cell tumors with low risk of recurrence. Taking cue from the management of intracranial germ cell tumors, limited volume radiotherapy combined with systemic chemotherapy should be offered in patients with high risk of recurrence, for example, those with high serum/CSF B-HCG levels and intratumoral presence of STGCs.Craniospinal irradiation should be used as a last resort (due to its many neuroendocrine and neurocognitive side effects) in patients with recurrence.Supplementary video consists of a complete narrated presentation of the clinical features, diagnostic considerations, imaging, surgical approach, pathology, and follow-up evaluations of the case presented. It includes intraoperative footage of surgical technique from positioning to tumor resection and closure. It also includes detailed pathological images with appropriate histological stains used in tissue evaluation and diagnosis. Finally it concludes with postoperative imaging and clinical status at long term follow-up.Click here for additional data file.


Introduction. Hydatid disease (HD) is an infestation that is caused by the larval stage of Echinococcus granulosus. The liver is affected in approximately two-thirds of patients, the lungs in 25%, and other organs in a small proportion. Primary retroperitoneal hydatid cyst is extremely rare. The most common complaint is abdominal pain; however, the clinical features of HD may be generally dependent on the location of the cyst. Case Presentation. A 43-year-old female was admitted with the complaint of abdominal pain. Her physical examination was normal. Computed tomography (CT) revealed a 17 × 11 cm cystic lesion, with a thick and smooth wall that is located among the left liver lobe, diaphragm, spleen, tail of the pancreas, and transverse colon and invading the splenic hilum. Total cystectomy and splenectomy were performed. Pathological examination was reported as cyst hydatid. Discussion. Cysts in the peritoneal cavity are mainly the result of the spontaneous or traumatic rupture of concomitant hepatic cysts or surgical inoculation of a hepatic cyst. Serological tests contribute to diagnosis. In symptomatic and large hydatid peritoneal cysts, surgical resection is the only curative treatment. Total cystectomy is the gold standard. Albendazole or praziquantel is indicated for inoperable and disseminated cases. Percutaneous aspiration, injection, and reaspiration (PAIR) technique is another nonsurgical option. Echinococcus granulosus [ Echinococcus granulosus [ Echinococcus granulosus enters to the lymphatic circulation via penetrating the intestinal mucosa and it is transported to the liver, lungs, and other organs [ Echinococcus granulosus infection have single-organ involvement and more than 70% of patients have only one cyst [Hydatid disease (HD) is an infestation that caused by the larval stage ofanulosus . It is panulosus . Human ianulosus . The annanulosus . The larr organs . The livr organs . Primaryr organs . 85% to one cyst , 5. HD ione cyst , 6.3 and eosinophil count of 30 (35%). Other biochemical investigations were normal. Indirect hemagglutination test (IHAT) for HD was 1/1280 (+). Chest X-ray revealed no pathological signs. An abdominal ultrasonography (USG) showed a 15 × 11 cm cystic lesion that extended from the left liver lobe to the transverse colon. Computed tomography (CT) revealed a 17 × 11 cm cystic lesion, with a thick and smooth wall that is located among the left liver lobe, diaphragm, spleen, tail of the pancreas, and transverse colon and invading the splenic hilum without any pathology of the intraabdominal organs (A 43-year-old female was admitted to our clinic with the complaint of abdominal pain localized in the left upper quadrant for the last 6 months. Her physical examination was normal and we could not find any palpable masses on abdominal exam. Routine blood analyses revealed a white blood cell count of 7900/mml organs . The patl organs . In ordel organs . Mean opThe retroperitoneal hydatid cyst is rare even in endemic areas . The oveIn conclusion, the possibility of HD in a patient presenting with a retroperitoneal cystic mass should be suspected especially in endemic areas such as sheep-raising Mediterranean Countries and the definitive diagnosis may require surgical removal of the cyst and histopathological examination of the resected specimen. Total cystectomy is the gold standard. When the complete resection is not feasible, unroofing and drainage followed by adjuvant antihelminthic therapy must be performed to prevent secondary recurrence of the cyst.


The authors describe a case of a retroperitoneal bronchogenic cyst in a 36-year-old female. She presented with abdominal pain, nausea, and vomiting. An MRI scan revealed an 8 cm cystic lesion in the left upper retroperitoneum, with intermediate signal on T2-weighted images, high signal on T1 weighted images, and lack of internal enhancement after gadolinium. After laparoscopic excision, the histology findings were compatible with a bronchogenic cyst, which is extremely uncommon in the retroperitoneum. Bronchogenic cysts are congenital lesions arising from abnormal budding of the embryonic foregut, during early embryogenesis. They develop most commonly in the mediastinum, posterior to the carina . RetropeA previously healthy 36-year-old female patient had episodes of abdominal pain in the last three months, associated with nausea and vomiting. Physical examination was unremarkable. Laboratory data showed no abnormalities: transaminases, serum amylase, and lipase were within normal values. An initial ultrasound revealed a large cystic lesion in the left upper quadrant. A contrast-enhanced abdominal MRI was performed, revealing an 8 cm retroperitoneal thin-walled cystic mass, with regular margins, located between the pancreatic tail, the upper pole of the left kidney, and the left adrenal gland. On GRE T1-weighted images see  it had hThe lesion was surgically removed via laparoscopy, and an 8 cm cystic lesion with a smooth outer surface was found in the retroperitoneum, next to the pancreatic tail.Histologically, the cyst wall was covered with ciliated pseudostratified columnar epithelium see . In the The postoperative course of the patient was uneventful and she was discharged from our institution in the seventh postoperative day, free of symptoms.Bronchogenic cysts are usually benign congenital lesions thought to arise from abnormal budding of the tracheobronchial analog of the primary foregut, which occurs between the 3rd and 6th weeks of gestation. If the abnormal bud retains some attachment to the primitive foregut, they are generally found near the tracheobronchial tree or oesophagus , 5. If tOn pathology they are mainly cystic lesions, lined by pseudostratified columnar epithelium that rests on a connective tissue wall, containing at least one of the following: seromucous glands, smooth muscle, or hyaline cartilage .Clinically, retroperitoneal bronchogenic cysts are usually asymptomatic, unless they are infected or large enough to cause compression of adjacent organs. The most common symptoms are vague abdominal discomfort and early satiety. There are described cases of pheochromocytoma-like symptoms, due to compression of the adrenal gland , 10, leaIn the case presented the symptoms were believed to be caused by the cyst, because they subsided after the surgery.In half of the cases these lesions are found incidentally . On ultrOn MRI, variable signal intensity has been described, probably due to a mixture of water and proteinaceous fluid. Most of the cysts are isointense or hyperintense to skeletal muscle on T1-weighted images. Generally they demonstrate high signal on T2 weighted images , 12. TheOn gadolinium enhanced T1-weighted images, there is enhancement of the cyst wall, aiding the delineation of the thin wall .The differential diagnosis of a retroperitoneal cystic lesion is large, including, among others, pancreatic pseudocyst, adrenal cyst, cystic lymphangioma, and teratoma.The treatment of bronchogenic cyst is surgical and the prognosis is excellent , 14.Retroperitoneal bronchogenic cyst is a very rare entity. However, it should be included in the differential diagnosis of a retroperitoneal cystic lesion, especially if it is found in the left upper quadrant.


Atypical Teratoid Rhabdoid Tumor (ATRT) is a rare malignant intracranial neoplasm more commonly diagnosed in young children. The authors report the case of an 11-year-old boy with a long standing history of slowly progressive weight loss, fatigue, and weakness over 1.5 years whose magnetic resonance imaging revealed a large heterogeneous enhancing dorsally exophytic lower brainstem mass. Examination revealed extreme cachexia, gaze-evoked nystagmus, dysphagia, dysarthria, bilateral dysmetria, and global weakness without ambulation. The protracted history and neuroimaging features were most suggestive of a low grade glioma. However, pathology revealed a hypercellular tumor with large hyperchromatic nucleoli and loss of INI-1 staining on immunohistochemistry consistent with a diagnosis of an ATRT. The child died shortly after surgery due to complications from his brainstem infiltrative disease. This case illustrates the diverse presentation of ATRT in childhood that can clinically and radiographically mimic that of low grade glioma. Primary childhood brain and central nervous system (CNS) brain tumors occur at an incidence of 5.1 per 100,000 in the United States with a slightly higher rate in males , 3. PrimAn 11-year-old previously healthy male presented to our hospital following a one-and-a-half year history of worsening vomiting, extreme cachexia, dysphagia, drooling, and a rapid decline with progressive weakness to the point of inability to ambulate over a course of 2-3 months. His neurological examination at presentation revealed bilateral lateral and down gaze nystagmus, diminished strength most noticeable in finger and wrist extensors of the right upper extremity, and mildly decreased rapid alternating movements of right upper and lower extremities. Magnetic resonance imaging (MRI) examination of the brain revealed a large heterogeneous enhancing mass on T2-weighted imaging centered in the medulla with dorsal exophytic extension into the fourth ventricle with internal areas of restricted diffusion but relative lack of surrounding edema . InternaATRT remains a significant challenge in pediatric neurooncology. Due to its aggressive nature and high rate of leptomeningeal dissemination, children often progress within months to a year of diagnosis , 8. DataATRTs can mimic supratentorial PNET and medulloblastoma both histologically and radiographically. One of the earliest studies looking at a series of infants with CNS ATRTs showed an association with an abnormality on chromosome 22 , 13. ATR


It is generally believed that malignant gliomas never metastasize outside the central nervous system (CNS). However, the notion that oligodendrogliomas (OGDs) cells cannot spread outside CNS is being challenged.MGMT promoter, and PTEN exon mutations. A search of worldwide literature was conducted for reports of metastatic OGDs using NCBI-PubMed, with the keywords “extracranial”, “extraneural”, “oligodendroglioma”, “oligodendrogliomas”, “metastatic”, “metastasis”, and “metastases”, in different combinations.We described in detail the clinical story of one patient with anaplastic OGD, which metastasized to lymph nodes, bone marrowand bones Genetic analyses included detection of 1p and 19q chromosomal arms, methylation status of MGMT promoter, and the G → A transversion at codon 234 of PTEN exon 2 were detected. Literatures review yielded 60 reports of metastatic OGDs from 1951 to the present, which with our patient makes 61 cases. Concerning these 61 patients, there were 110 infiltrated sites correlated closely with primary OGDs. The most frequent metastatic sites were bone and bone marrow , lymph nodes , liver , scalp , lung , pleura , chest wall , iliopsoas muscle , soft tissue , and parotid gland .An open biopsy of the infiltrated bones in our patient revealed that malignant cells had replaced the patient’s marrow. Moreover, the diagnosis of multiple-organ metastases of anaplastic OGD was confirmed based on immunohistochemical staining. Genetic analyses showed that the tumors originated from previously resected brain lesions. None of the lesions had 1p and 19q deletions, but hypermethylation of MGMT promoter, and characteristic PTEN exon mutations may help differentiate subtypes which are more prone to extracranial metastases.Extracranial metastases in anaplastic OGD are very rare but they do occur; bone and bone marrow may be the most common sites. Detection of certain molecular markers such as deletion of 1p and 19q chromosomal arms, hypermethylation of http://www.diagnosticpathology.diagnomx.eu/vs/8749838611478560.The virtual slide(s) for this article can be found here:  It has been widely believed by neurosurgeons and neurooncologists that malignant gliomas never metastasize outside the central nervous system (CNS) . HoweverOGD is an uncommon diffuse glial tumor of central neuroepithelial origin, accounting for ~4.2% of all primary brain tumors. It has been mostly identified in adults, with the highest incidence occurring in the fifth and sixth decades of life, although it has also been reported in children and adolescents . VariousHerein, we report a case of anaplastic oligodendroglioma (AO) that metastasized to multiple lymph nodes, bone marrow, and bones, including the bilateral iliac bones, the right acetabulum, and multi-vertebral bodies.-2 · d-1 for 5 days, repeated every 28 days.A 45-year-old male who initially presented with a short history of headache and vomiting was admitted to our hospital in September 2011. No focal neurological deficit was found on admission. Magnetic resonance imaging (MRI) showed a left solid occipital tumor with mild contrast enhancing -computed tomography (CT) scans revealed more multifocal invasion. A whole body s Figure . PET-CT s Figure A,B, the s Figure C and thes Figure D.A bone marrow smear of the right iliac bone showed no plasmacytoma cells Figure . An openWe collected and analyzed both the bone marrow from the above mentioned open biopsy and the brain tumor tissues resected in the first and second craniotomy. The Human Research Committee of the Fourth Military Medical University approved the use of human tissues, in accordance with the Approval of Research Involving Human Subjects. The patient’s custodians provided informed written consent. All specimens were handled and made anonymous in accordance with ethical and legal standards.260), and purity was verified by OD260/OD280 ≈ 1.8.The bone marrow from the biopsy was decalcified using ultrasonic decalcification with ethylenediaminetetraacetic acid (EDTA) as described previously  and paraSections from the brain tumors and metastatic lesions were evaluated with routine hematoxylin-eosin (H & E) staining. FISH assays were also performed for molecular characterization. Both the brain tumors and the metastatic lesions were analyzed to perform dual-color FISH assay as previously published . BrieflyMGMT promoter.DNA from the tumor tissues was analyzed by performing a methylation-specific polymerase chain reaction (MS-PCR) assay, as previously published , to detePTEN gene  of the PCR products and loading buffer  were mixed and centrifuged for 15 s, heat-denatured at 95°C for 7 min, snap-chilled on ice for 10 min, and resolved through an 8% non-denaturing polyacrylamide gel  containing 50 mM Tris-borate (pH 7.5) and 2.5 mM EDTA, and electrophoresed with 1× tris-borate-EDTA buffer for 16 h at 14°C at a voltage of 100 V. Silver staining was performed as previously described [SSCP analyses of all escribed .According to the PCR-SSCP results of genomic DNA, the present sample was considered PCR-SSCP positive based on the evident difference in the single strand strip number and electrophoresis transference location . GenomicNone of the tumors from the resected brain lesions or the metastatic lesions had the 1p Figure A or 19q MGMT promoter  have occurred after surgical excision of the primary tumor . The mosOGD is such a diffuse glial tumor. Extracranial metastasis of the primary intracranial neoplasm is infrequent generally, and it seemed to usually happen only in the setting of prior neurosurgical resection . OGDs arn = 47; 42.7%) followed by lymph nodes , liver , scalp , lung , pleura , chest wall , iliopsoas muscle , soft tissue , parotid gland , and adrenal gland, spleen, thoracic wall, pancreas, dorsal root ganglia, abdomen, spinal dura mater, breast, and thymus gland with one lesion each .Of the 61 reported metastatic OGDs, 33 (54.1%) were male, 17 (27.9%) were female, and in the remaining cases 18.0% gender was not reported , with 60-70% of patients responding . StudiesMGMT promoter, and PTEN exon mutations may help elucidate which subtypes of AO are more prone to extracranial metastases, which would benefit these patients.In summary, extracranial metastases in AO do occur, although they are very rare. Detection of molecular markers such as combined deletion of the 1p and 19q chromosomal arms, hypermethylation of the Written informed consent was obtained from the patient's family 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.OGD: Oligodendrogliomas; MRI: Magnetic resonance imaging; MGMT: O-6-methylguanine-DNA methyltransferase; PTEN: Phosphatase and tensin homolog; CNS: Central nervous system; AO: Anaplastic oligodendroglioma; WHO: World Health Organization; PET-CT: Positron emission tomography-computed tomography; EDTA: Ethylenediaminetetraacetic acid; IDH1: Isocitrate dehydrogenase-1; GFAP: Glial fibrillary acidic protein; EMA: Epithelial membrane antigen; MS-PCR: Methylation-specific polymerase chain reaction; PCR-SSCP: PCR single-strand conformation polymorphism; FISH: Fluorescent in situ hybridization; NCCN: National comprehensive cancer net.The authors declare that they have no competing interest.YZ and GG designed the study. GL, ZZ, SH and GC participated in designing and coordinating the study, and GL drafted the manuscript. ZZ and TJ worked together to draft the manuscript. JZ, HL, LG and HY participated in following up the patient, reviewing the relevant articles, and helped to improve the manuscript. All authors have read and approved the final manuscript.


Schwannomas are generally slow growing asymptomatic neoplasms that rarely occur in the GI tract. However, if found, the most common site is the stomach. Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract, and 60–70% of them occur in the stomach. Owing to their typical presentation as submucosal neoplasms, gastric schwannomas and GISTs appear grossly similar. Accordingly, the differential diagnosis for a gastric submucosal mass should include gastric schwannomas. Furthermore, GI schwannomas are benign neoplasms with excellent prognosis after surgical resection, whereas 10–30% of GISTs have malignant behavior. Hence, it is important to distinguish gastric schwannomas from GISTs to make an accurate diagnosis to optimally guide treatment options. Nevertheless, owing to the paucity of gastric schwannomas, the index of suspicion for this diagnosis is low. We report a rare case of gastric schwannoma in 53-year-old woman who underwent laparoscopic partial gastrectomy under the suspicion of a GIST preoperatively but confirmed to have a gastric schwannoma postoperatively. This case underscores the importance of including gastric schwannomas in the differential diagnosis when preoperative imaging studies reveal a submucosal, exophytic gastric mass. For a gastric schwannoma, complete margin negative surgical resection is the curative treatment of choice. Mesenchymal tumors of the gastrointestinal (GI) tract are mainly comprised of a spectrum of spindle cell tumors which include gastrointestinal stromal tumors (GISTs), leiomyomas or leiomyosarcomas, and schwannomas . Among tOwing to their typical presentation as submucosal neoplasms with spindle cell histology, gastric schwannomas and GISTs appear grossly similar , 6. BothNonetheless, a diagnostic difficulty exists in that preoperative conventional imaging techniques , although helpful, cannot always provide enough information to differentiate between these two tumors. Indeed, radiologic and endoscopic findings are nonspecific , 10. FurIn this paper, we present a 53-year-old woman with a gastric mass who underwent laparoscopic partial gastrectomy under the suspicion of a GIST preoperatively but confirmed to have a gastric schwannoma postoperatively.A 53-year-old woman was referred to surgical oncology for a gastric mass. The reason for her initial visit was chest pain. She only complained of intermittent gastric discomfort and dysphagia in response to solids. Her medical history was significant for hypertension, hyperlipidemia, diabetes mellitus, hypothyroidism, and uterine cancer, which was successfully treated with robotic hysterectomy 5 years prior.During the chest pain assessment, a gastric mass was incidentally detected on abdominal ultrasound . A subseThe patient then underwent an esophagogastroduodenoscopy (EGD), and a submucosal mass lesion was confirmed in the gastric antrum with normal overlying gastric mucosa. Biopsy specimens obtained at the endoscopy yielded only unspecific signs of mild inactive chronic inflammation without evidence of a malignancy. To facilitate the evaluation, we performed an endoscopic ultrasound (EUS) examination, which depicted a hypoechoic heterogeneous mass lesion with calcifications located in the gastric antrum . The masAfter presenting the case at our Gastrointestinal Multidisciplinary Tumor Board Conference, a consensus was reached to proceed with resection. The patient was counseled about the surgical options and offered an elective laparoscopic partial gastrectomy. After informed consent was obtained, the patient was taken to the operating room where she was placed in the supine position under general endotracheal anesthesia. The abdomen was prepped and draped in sterile fashion. Pneumoperitoneum was achieved at 15 millimeters of mercury, and four additional trocars were placed under direct vision as shown in The final pathologic study revealed that the resected neoplastic mass was comprised of spindle cells of varying cellularity with relatively bland cytology and focal nuclear palisading . There wSchwannomas, also known as neurilemmomas or neurinomas, are benign neurogenic tumors, originating from Schwann cells, which normally wrap around the axons of the peripheral nerves. Theoretically, schwannomas can develop anywhere along the peripheral course of nerve. However, they most commonly occur in the head and neck but rarely in the GI tract .Gastric schwannomas, the most common GI schwannoma, account only for 0.2% of all gastric tumors, and principally involve the submucosa and muscularis propria –6. They For a gastric submucosal mass, the main differential diagnosis is a GIST. Although rare, gastric schwannomas are also a primary GI mesenchymal tumor . Most imThe differentiation between gastric schwannomas and GISTs can be difficult preoperatively. While preoperative imaging studies, such as sonography, endoscopy, and CT, can demonstrate the presence or extent of invasion, none of these modalities have shown any distinct features unique to these neoplasms –13. FurtIn 2005, Levy et al. reported that gastric schwannomas are uniquely different from other schwannomas in that they show homogeneous attenuation on CT and that degenerative changes such as cystic changes are uncommon . In addiConsistent with the CT findings, endoscopic ultrasonogram of our case depicted a heterogeneously hypoechoic mass. It seemed that the cystic changes caused inhomogeneous echoes, and the dense composition of spindle cells caused the lower echogenicity . EndoscoDespite strong morphological similarities, GI mesenchymal tumors are heterogeneous in their immunophenotypes. In the past, gastric schwannomas were included in the GIST category . In 1988In summary, this case underscores the importance of including gastric schwannomas in the differential diagnosis when preoperative imaging studies reveal a submucosal, exophytic gastric mass. Thus far, the majority of previous series in the literature addressing gastric schwannomas regard these neoplasms as uniformly slow growing benign tumors. Owing to subclinical tumor growth, the diagnosis is usually delayed. However, to date, there is no clear evidence in the literature to suggest that gastric schwannomas have malignant potential. Additionally, recurrent disease has been only observed after incomplete resection –6. There


Auricular tophi are firm deposits of monosodium urate in crystal form, which may slowly develop in subcutaneous tissue of the ear. Ear is not usual locations for gout tophi, but when this growth does occur, helix and the antihelix are common sites.We present a 64-year-old man who had multiple painless nodules over bilateral helix. An excisional biopsy was performed. Hematoxylin-eosin staining of biopsy specimens revealed a proteinaceous matrix that surrounded dissolved crystals, consistent with gout tophi. Bilateral auricular tophi are not common and may resemble a number of other diseases including squamous cell carcinomas, Kaposi’s sarcoma, epidermal and dermoid cysts, rheumatoid nodules. Biopsy should be performed to rule out malignancy.Tophi of the auricle are usually asymptomatic but can become inflamed and occasionally ulcerate through the overlying skin. Chronic tophaceous gout is treated with dietary control and medication. Surgical excision is performed under local anesthetic if symptoms progression or cosmetically deformity is concerned. Auricular tophi are firm deposits of monosodium urate in crystal form, which may slowly develop in subcutaneous tissue. Gout tophi occurs around auricular area is not common. Common locations for this growth on the ear are the helix and the antihelix. We present a 64-year-old man who had multiple painless nodules over bilateral helix after he received anti-tuberculosis therapy. Auricular tophi are not common and may resemble a number of other diseases including squamous cell carcinomas, Kaposi’s sarcoma, epidermal and dermoid cysts, rheumatoid nodules. Biopsy should be performed to rule out malignancy.A 64-year-old man was seen with a 6-month history of multiple bilateral auricular nodules. He also had a history of pulmonary tuberculosis and had been receiving antituberculosis therapy since December 2009. The protocol consisted of 4 drugs  administered for 2 months, followed by 4 months of treatment with isoniazid and rifampin. He denied any history of trauma or sun exposure.Physical examination revealed multiple 2.5-mm painless nodules over the helix Figure . These wThe differential diagnosis of auricular nodules is broad and includes sebaceous cysts, chondrodermatitis nodularis helicis, sarcoid nodules, leprosy, hemangioma, keratoacanthoma, chondroma, lipoma, squamous cell carcinomas, Kaposi’s sarcoma, melanoma, epidermal and dermoid cysts, elastotic nodules, tophaceous gout, rheumatoid nodules, and schwannoma . Biopsy Gout is a common systemic metabolic disease, but manifestations of it are infrequently encountered in the field of otolaryngology. It is caused by disordered purine metabolism, resulting in hyperuricemia. When the uric acid level in serum is above 7.0 mg/dL , urate crystals form a deposition in subcutaneous tissue, called tophi. Symptoms such as pain, erythema, and swelling are related to the precipitation of uric acid crystals in joint spaces and soft tissues . PrimaryThe external ear, especially the helix, is one of the most common sites of tophus formation in the head and neck region. Documented sites of involvement in the head and neck include the arytenoid, true vocal cord, hyoid bone, thyroid cartilage, nasal septum, temporomandibular joint, soft palate, cervical spine, and glossoepiglottic ligament  Tophi ofTophi of the auricle are usually asymptomatic but can become inflamed and occasionally ulcerate through the overlying skin. Chronic tophaceous gout is treated with dietary control and medication. Surgical excision is performed under local anesthetic if symptoms progression or cosmetically deformity is concerned. These lesions clinically resemble carcinoma and hence even asymptomatic auricular nodules should be biopsied for tissue diagnosis.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.The authors declare that they have no competing interests.PCW diagnosed, investigated, followed-up and managed the patient, and determined the medical significance. YCH collected the clinical pictures of the patient. HJC collected the data and wrote the manuscript. PCW and SHH revised the manuscript and provided important suggestions regarding medical content. All authors read and approved the final manuscript.


Pneumoperitoneum in neonates is not an uncommon condition. Free air in peritoneum may be secondary to host of pathological lesions. Usually the patient presents with signs of intraperitoneal sepsis, however presence of air in the scrotum as a concomitant sign is a rare event. Herein we report a 4-day-old neonate who presented with 2 days history of fever and scrotal swelling. Abdominal signs were subtle. Scrotum was hugely distended and tense. Workup of the patient revealed free intraperitoneal gas with air in the scrotum. At exploration, two perforations were found near the greater curvature of stomach and repaired. Post-operative course was uneventful. Free intraperitoneal gas on abdominal x-rays, the pneumoperitoneum, is not an uncommon presentation in neonates. Mostly it is taken as a sign of gut perforation and laparotomy is generally required [1]. It may also be found in neonates with severe respiratory distress, after aggressive resuscitation or with mechanical ventilation [1-4].Pneumoperitoneum presenting as pneumoscrotum secondary to gastric perforation without abdominal findings is rare. We report a case of neonate who presented with scrotal swelling and was found to have gastric perforations at laparotomy. Four days old (1.8 kg) full term male neonate presented with fever and gross scrotal swelling for two days. The baby was delivered at home and nothing significant found in perinatal history. He passed meconium within few hours of birth and had no vomiting.On examination, the baby was febrile (100 ºF), irritable, reluctant to feed, with heart rate of 130/min, and respiratory rate of 42/min. Chest was clear on auscultation and abdomen was non distended and soft. The umbilical stump was infected. There was a huge, irreducible, soft swelling at the right hemi-scrotum, extending proximally to the inguinal region. The scrotal skin was tense and shiny . Right testis could not be palpated. Initially a diagnosis of irreducible inguinal hernia was made.Laboratory parameters were within normal limits. X-ray abdomen and pelvis in erect posture revealed free gas under the right dome of diaphragm and in the scrotum . With the suspicion of gut perforation, laparotomy was performed.Pneumoperitoneum in neonates has multiple causes and varied presentations ranging from a vitally stable newborn with minimal or no abdominal signs to a sick baby with gross abdominal distension. Mostly it is taken as a sign of gut perforation. Rarely, extra abdominal cause may be the source of free intraperitoneal air [2-4]. Very rarely it may be without any surgical or medical cause, termed as benign pneumoperitoneum [1].Gastric perforation in neonates is a rare event and represents immediate surgical emergency. It usually occurs in premature neonates in intensive care setting. Proposed mechanisms of this perforation are trauma, ischemia, and one occurring spontaneously [5]. Traumatic perforations are usually iatrogenic, occurring due to vigorous nasogastric tube placement or massive gastric distension associated with aggressive resuscitation with bag mask ventilation or in newborns with respiratory distress and on mechanical ventilation [5]. Severe birth asphyxia, prematurity and sepsis are the common risk factors associated with ischemic perforations of stomach in newborns [6]. Spontaneous perforations occur usually in healthy newborns without gastrointestinal conditions, presenting within 2 - 7 days of life. No exact predisposing or risk factor can be identified in such newborns. Rarely congenital muscular deficiency of stomach may be the cause of gastric perforation. It has also been reported secondary to distal obstruction and atresias [6]. No such etiological factors were found in our patient and it may be suggested that the gastric perforations were spontaneous in nature.Clinically, a newborn with gastric perforation presents with gross abdominal distension compromising ventilation along with signs of hypovolemic shock and sepsis and radiologically, massive pneumoperitoneum is noticed . In our case, the baby had clinically normal abdomen and very small amount of free intraperitoneal air on x-ray.Although pneumoscrotum has been reported secondary to perforation of Meckel’s diverticulum, perforation of ileum secondary to atresia, after aggressive resuscitation, and with mechanical ventilation; scrotal pneumatocele secondary to gastric perforations and without signs of intraperitoneal sepsis is rarely reported. As processus vaginalis is patent in 80 - 95 % newborn males, free air in the peritoneum passing through the patent process vaginalis, causing gaseous distension of the scrotum is the possible explanation of pneumoscrotum in this case. Other mechanisms postulated in the development of pneumoscrotum are subcutaneous or retroperitoneal air dissecting down the dartos lining of the scrotal cord into the scrotal wall or may result from local production of gas secondary to infections .In conclusion, pneumoscrotum along with pneumoperitoneum without significant abdominal distension may be a presentation of neonatal gastric perforation and demands high index of suspicion to manage the condition effectively. Source of Support: NilConflict of Interest: None declared


Carcinosarcoma is a rare biphasic malignancy consisting of intermixed epithelial and mesenchymal elements. Carcinosarcoma is particularly rare among children. We accepted a 7 year old patient with retroperitoneal carcinosarcoma. The tumor was totally resected and no recurrence is found 11 months after operation. Literatures has been reviewed and there are few reports of primary retroperitoneal carcinosarcoma in children up to date. So we report the patient's clinical character, surgical resection, pathological and immunohistochemical analysis. Carcinosarcoma is a rare malignancy consisting of intermixed epithelial (carcinoma) and mesenchymal (sarcoma) elements, mostly arising from glands and female reproductive system ,2. CarciA 7 year old female was admitted due to two month history of epigastric pain with anorexia, who was found to have an epigastric mass. Past medical history was unremarkable. Abdominal exam revealed a distended abdomen with a palpable mass at the right upper quadrant. The mass was estimated to be around 15 cm, moderate hardness with clear edge, but not movable and no tenderness. No abdominal wall venous varicose was noticed. Bowel sounds were present and normal. Lower extremity edema was absent, bilaterally. Laboratory test showed normal hepatic and renal function. AFP 4.9 μg/L, CEA 0.6 μg/L, CA 19-9 11.7 U/ml. Ultrasound exam showed a large well-circumscribed solid mass with irregular internal echo signals located in the right upper quadrant extending. On CT, the right upper quadrant solid mass was measured at 20 cm longitudinally. Tumor image was mildly enhanced at artery phase, which rapidly phased out at venous phase and delayed phase. The adjacent kidney, liver, stomach, duodenum, transverse colon, abdominal aorta and vena cava were all compressed or distorted  was pushed leftward and anteriorly, right hepatic lobe appeared thinner. However, the liver was soft, no mass, cirrhosis change or other abnormalities were appreciated. The adrenal gland, duodenum, transverse colon, abdominal aorta and vena cava were all compressed by the tumor, but can be dissected from the tumor easily and no tumor invasion was detected among these adjacent organs. The reproductive system organs were free from the tumor compression. There was a tight adhesion between the tumor and the right kidney, making it hard to judge whether the tumor originated from the kidney or the tumor infiltrated the kidney. Frozen section of the kidney sample reported "malignant tumor without identifiable kidney glomerulus structure, possible tumor infiltration". Complete retroperitoneal tumor resection plus right nephrectomy was thus performed. Macroscopically, the tumor weighed 3.5 Kg, measured at 21 × 14.6 × 12.1 cm, encapsulated with fibrous tissue. Section of the tumor revealed "fish-meat" like greyish-white color inside with spotted bleeding and cystic necrosis cavities ranging 1.2 - 3.0 cm in diameter filled with brown color fluid  staining showed that the tumor tissue consisted of two well distinguished types of cells, large spindle cells lining in net with identifiable nuclear mitosis and epithelial nest or adenocarcinoma cells lining around microvascular structure Figure . Large aThe patient had an uneventful recovery following the surgery and was discharged home on postoperative day 7. On 11 months follow up, the patient did well and gained weight. She was symptom free with normal renal and hepatic function. There was no tumor recurrence was found on CT scanPrimary tumors arising from retroperitoneal space are rare, most are soft tissue tumors. The incidence of retroperitoneal malignancies  is reported as 2.7/10^6 and did not change significantly over the years ,4. CarciCarcinosarcoma consists of mixed epithelial carcinoma and mesenchymal sarcoma components. The ratio of these two components may vary, however, they are related and share many same biological characters . There hCarcinosarcoma has no specific clinical symptoms or signs. There is also no specific laboratory and imaging study reported. It is difficult to differ it from other retroperitoneal malignancies before surgical resection. The diagnosis of carcinosarcoma is thus mainly by pathological and immunohistochemical analysis. Most primary retroperitoneal tumors are found due to abdominal pain or abdominal mass. Ultrasound and CT imaging studies can help delineate tumor location and anatomy in relation to adjacent organs in order to make a treatment plan. Final diagnosis is usually immunohistological. H & E staining can clearly show characteristic epithelial and mesenchymal cell components and their differentiation degree. Immunohistological study can determine the tumor components by specific labeling techniques. Most used labeling techniques include vimentin, VI, CD99 for mesenchymal tissue and epithelial membrane antigen (EMA) and cytokeratins for epithelial tissue ,9,10. OuCarcinosarcoma is a highly malignant and invasive tumor. Retroperitonal carcinosarcoma can often occur insidiously without obvious signs or symptoms until the tumor grow large enough to compress adjacent organs. Currently, surgical resection is still the main and most effective treatment method ,5. SurgeCurrently, there has been no prognosis study on primary retroperitoneal carcinosarcoma. However, inferring from prognosis study of carcinosarcoma in other locations, prognosis of retroperitoneal carcinosarcoma can be very poor, given its highly malignant, rapid aggressive infiltration and easy postoperative metastatic nature ,17.Carcinosarcoma is a rare biphasic tumor with high malignancy, aggressive invasiveness and poor prognosis. Preoperative diagnosis is often difficult, especially for retroperitoneal carcinosarcoma, due to its insidious onset and lack of specific laboratory and imaging study available. Confirming diagnosis requires immunohistological studies. Surgical resection is still the most important treatment, plus adjuvant chemotherapy and possible targeted therapy could improve survival rate. However, satisfactory result is still hard to achieve. This is especially the case among children due to the severe complication from chemotherapy limiting it application in these patients. The prognosis of our patient could be get depending on further follow ups.Written informed consent was obtained from the patient's father 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.The authors declare that they have no competing interests.JMY proposed the study. FX and YQH wrote the first draft. XF and YQH analyzed the data. JMY is the guarantor. All authors read and approved the final manuscript.


Adrenocortical carcinoma is a rare malignancy and rare cause of Cushing’s syndrome.th day after diagnosis.A 65-year-old seemingly well male patient was referred to our clinic under the suspicion of hyperaldosteronism due to hypertension combined with hypokalemia. However, his serum aldosterone and plasma renin activity were within normal limits. Instead, Cushing’s syndrome was diagnosed by elevated urine free cortisol and a non-suppressible dexamethasone test. Abdominal computed tomography showed a 7.8 × 4.8 cm mass lesion at the right adrenal gland with liver invasion. Etomidate infusion was performed to reduce his cortisol level before the patient received a right adrenalectomy and liver wedge resection. The pathology report showed adrenocortical carcinoma with liver and lymph node metastasis. According to the European Network for the Study of Adrenal Tumors (ENSAT) staging system, the tumor was classified as T4N1M1, stage IV. Recurrent hypercortisolism was found shortly after surgery. The patient died of Fournier’s gangrene with septic shock on the 59We report a case of rapidly progressive stage IV adrenocortical carcinoma with initial presentations of hypokaelmia and hypertension, mimicking hyperaldosteronism. Adrenocortical carcinoma (ACC) is a rare malignancy, with an incidence of 0.7-2.0/million population/year . ApproxiThe clinical presentations of ACC are heterogeneous and the prognosis is generally poor. Although ACCs are often hormonally active with glucocorticoid over-secretion, it is a rare cause of Cushing’s syndrome . Here, wth day after diagnosis.A 65-year-old seemingly well male was referred to our clinic due to hypertension and hypokalemia. He denied any discomfort in the past few months. However, vague symptoms of muscle weakness with cramping, polyuria, and nocturia were noted upon further interview. During his annual health check-up six months ago, he was diagnosed with hypertension and type 2 diabetes mellitus. On physical examination, blood pressure was 148/72 mmHg. There were no purple striae, central obesity, buffalo hump, proximal muscle weakness, or skin hyperpigmentation. His serum potassium level was 2.2 mmol/l. Hormonal studies demonstrated excess of serum cortisol with loss of diurnal pattern, elevated urine free cortisol and a non-suppressible dexamethasone test. The aldosterone level and plasma renin activity were in normal limits. The laboratory results of hormonal studies are summarized in Table The patient was initially referred to our clinic under the suspicion of hyperaldosteronism due to hypokalemia and new onset hypertension. Nevertheless, his serum aldosterone and plasma renin activity were within normal limits. Instead, Cushing’s syndrome was confirmed by loss of diurnal cortisol and adrenocorticotrophic hormone (ACTH) patterns, markedly elevated urine free cortisol level and a non-suppressible dexamethasone test. Excessive cortisol overwhelms the capacity of 11β-hydroxysteroid dehydrogenase type 2 in the proximal tubule to inactivate cortisol to cortisone, thus allowing cortisol to saturate the mineralocorticoid receptor, causing severe hypokalemia in our patient .The image features suggestive of ACC in our case included large tumor size > 4 cm) with irregular margins, heterogeneous appearance, and local invasion of the liver  cm with . MRI alsDifferentiating ACC from benign adenoma microscopically is a challenging issue even for experienced pathologists. The Weiss System has been the standard method for decades. Fulfilling more than three of the nine following histologic features is regarded as malignancy: (1) high nuclear grade 3 or 4; (2) mitotic rate greater than 5/50 high-power fields (HPF); (3) atypical mitoses; (4) tumors with 25% or less clear cells; (5) diffuse architecture more than 1/3; (6) microscopic necrosis; and (7) venous, (8) sinusoidal, and 9) capsular invasion  capsular. Our patManagement for ACC requires a multidisciplinary approach. The principal considerations are surgical, which is the only curative option for ACC. For cortisol-secreting tumors with a urine free cortisol level more than 700–800 μg/day, short-term use of etomidate for rapid control of cortisol level before operation was performed in our Endocrine Unit to minimize surgical risk such as post-operation infection and poor wound healing. Etomidate as a steroidogenesis inhibitor, is an imidazole derivative that was initially used for rapid hypnosis induction . Its useThe treatment of non-resectable or recurrent tumor relies on mitotane-based chemotherapy, albeit a low response rate . Our patWe report a case of rapidly progressive stage IV ACC with liver metastasis and cortisol excess in a seemingly well individual with hypertension and hypokalemia. Poor prognosis was anticipated in our patient with old age, high stage, and cortisol hypersecretion.Written informed consent was obtained from the patient’s next-of-kin 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.ACC: Adrenal cortical carcinoma; CT: Computed tomography; ACTH: Adrenocorticotrophic hormone; MRI: Magnetic resonance imaging; FDG-PET: Fluorodeoxyglucose-positron emission tomography.The authors declare that they have no competing interests.CJH and THW analyzed and interpreted the patient’s data and wrote the manuscript. CSK revised the manuscript. THW, YHL, KTH, GSW, TSJ, CSK managed the patient clinically. All authors read and approved the final manuscript.


Immunoglobulin inclusions are found in B-cell neoplasms as well as in crystal-storing histiocytosis associated with B-cell lymphoproliferative disorders. At times, the deposits may be so profound as to obscure the diagnosis and may even lead to misdiagnosis. We report one case of low-grade extranodal lymphoplasmacytic lymphoma with abundant immunoglobulin inclusions and emphasize the need for immunophenotyping and molecular assay to make the right decision in diagnosis. To the best of our knowledge, this is the first report of extranodal B-cell lymphoma with abundant intracellular immunoglobulin accumulation.A 62-year-old Asian man from China presented with a 13-year history of a right shoulder mass with recent ongoing pain. A desmoplastic fibroma located in the posterior muscles of the neck was suggested by magnetic resonance imaging, and extended local excision was performed. A biopsy, however, revealed large, isolated rhabdoid cells in a diffuse pattern with mild atypia and eosinophilic cytoplasm. Clustered lymphoid cells were interspersed among these cells. The diagnosis was initially suggested to be adult rhabdomyoma. The final diagnosis of lymphoma was made after immunohistochemical, ultrastructural and molecular studies.We emphasize this histopathologic and immunohistochemical finding because of the potential for confusion with other tumors or disorders, such as adult rhabdomyoma or crystal-storing histiocytosis. Well-developed immunoglobulin (Ig) inclusions, such as Russell or Dutcher bodies, are very important diagnostic clues for some B-cell lymphomas, but diffuse and abundant intracellular Ig inclusions are unusual. Another condition in which unusual intracytoplasmic Ig inclusions can be found is crystal-storing histiocytosis associated with B-cell lymphoproliferative disorders. All of these factors may lead to a diagnostic dilemma ,2. We deA 62-year-old Asian man from China with a 13-year history of an enlarging right shoulder mass and recent ongoing pain was admitted to our hospital. His cervical lymph nodes were not palpable, and a review of all other systems was negative. A chest radiograph was normal. A desmoplastic fibroma located in the posterior muscles of the neck was suspected on the basis of computed tomography (CT) and magnetic resonance imaging (MRI) Figure , and extCharacteristically, the excised tumor was composed of large, isolated rhabdoid cells in a diffuse pattern with mild atypia and eosinophilic cytoplasm Figure . These cOther postoperative examinations showed that levels of IgE were elevated (332IU/mL) and levels of IgA, IgM and IgG were normal. Bence-Jones protein was not found in the patient's urine. The final histopathologic diagnosis was consistent with a low-grade lymphoplasmacytic lymphoma with abundant Ig inclusions.Igs are reliable markers for the recognition of B lymphocytes and their neoplasms. In B-cell neoplasms, monotypic Ig is usually detected . Igs mayThe case reported here was initially considered rhabdomyoma because of the morphologic features, the patient's age and the location of the tumor. The initial diagnosis was ruled out by immunohistochemical analysis that showed that tumor cells were negative for desmin, HHF35, α-SMA and myoglobin and positive for PAS-D. Then we checked the slides carefully and found some Russell body-like intercellular eosinophilic bodies, which have the same characteristics as those in the cytoplasm, as well as the transition from small lymphoplasmacytic cells to those unique, large rhabdoid cells. Some other associated diseases were included in the differential diagnosis, including crystal-storing histiocytosis, extramedullary myeloma, and other Ig deposition diseases ,7. FinalWe report a rare case of low-grade B-cell lymphoma with abundant intracellular Ig accumulation. The patient's clinical presentation and MRI suggested a soft-tissue tumor, and histopathologic studies showed special rhabdoid cells with interspersed clustered lymphoid cells, thus providing a diagnostic dilemma. The final diagnosis was made with immunohistochemical, ultrastructural and molecular studies. It is important for pathologists to be aware of this rare extranodal Ig-rich lymphoma because of the potential for confusion with other tumors, such as rhabdomyoma and crystal-storing histiocytosis.The authors declare that they have no competing interests.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 the journal.ZSL and PFL contributed equally to this work as first authors by providing data collection, data analysis and interpretation and manuscript writing. ZW and GSH contributed to the conception and design of the report. All authors read and approved the final manuscript.Supplementary immunohistochemical results. Immunohistochemical detection of LCA, PAX-5, CD5, CD20, CD43 and Ki-67 .Click here for file


The solid variant of aneurysmal bone cyst is rare, and only 13 cases involving the spine have been reported to date, including seven in the thoracic vertebrae. The diagnosis is difficult to secure radiographically before biopsy or surgery.An 18-year-old Hispanic man presented to our facility with a one-year history of left chest pain without any significant neurological deficits. An MRI scan demonstrated a 6 cm diameter enhancing multi-cystic mass centered at the T6 vertebral body with involvement of the left proximal sixth rib and extension into the pleural cavity; the spinal cord was severely compressed with evidence of abnormal T2 signal changes. Our patient was taken to the operating room for a total spondylectomy of T6 with resection of the left sixth rib from a single-stage posterior-only approach. The vertebral column was reconstructed in a 360° manner with an expandable titanium cage and pedicle screw fixation. Histologically, the resected specimen showed predominant solid fibroblastic proliferation, with minor foci of reactive osteoid formation, an area of osteoclastic-like giant cells, and cyst-like areas filled with erythrocytes and focal hemorrhage, consistent with a predominantly solid variant of aneurysmal bone cyst. At 16 months after surgery, our patient remains neurologically intact with resolution of his chest and back pain.Because of its rarity, location, and radical treatment approach, we considered this case worthy of reporting. The solid variant of aneurysmal bone cyst is difficult to diagnose radiologically before biopsy or surgery, and we hope to remind other physicians that it should be included in the differential diagnosis of any lytic expansile destructive lesion of the spine. Histologically, aneurysmal bone cyst is typically characterized by cavernous channels surrounded by a spindle cell stroma with osteoclast-like giant cells and osteoid production . There iet al.  in 1983;et al.  occurrinWe report a case of the solid variant of aneurysmal bone cyst occurring in the T6 vertebra with extensive involvement of the left sixth rib and pleural cavity in an 18-year-old Hispanic man. We review the 13 prior cases that have been reported in the literature and discuss the unique features of these unusual tumor-like lesions of the vertebral column.An 18-year-old, previously healthy Hispanic man presented to our institution with a one-year history of left paraspinal tenderness and radiation into the left chest. Our patient denied weakness or numbness of the legs and bowel or bladder incontinence. He had no difficulties with ambulation or balance.On physical examination, tenderness could be elicited on palpation of the spinous processes of the mid-thoracic spine. No motor or sensory deficits were observed. There were no signs of myelopathy. A rectal examination showed good volitional rectal tone and no perineal anesthesia. The post-void residual volume of urine was negligible.A computed tomography (CT) scan of the thoracic spine Figure  demonstrConsideration was given to pre-operative spinal angiography and possible embolization of large arterial feeders to the mass. However, the risk of spinal cord infarction with embolization was deemed to be too high by the experienced interventional radiologists at our institution, and subsequently, this was not performed.in situ with no further attempts at correction of the kyphosis. Morselized bone graft from the osteotomized laminae and cancellous morselized allograft were used as graft material.Our patient was taken to the operating room where a single stage posterior-only approach for total T6 spondylectomy with left sixth rib removal and circumferential reconstruction of vertebral column was planned Figure . Spinal Post-operatively, our patient was neurologically intact. However, he did develop a pleural effusion on the left side on the first post-operative day that necessitated chest tube placement. The effusion was likely from irritation of the pleura and post-operative oozing of fluid/blood from the operative bed directly into the pleural cavity. There was no hematoma, and there were no signs of infection. After the chest tube was removed two days later, our patient progressed quickly in his recovery from surgery and was subsequently discharged home. Imaging revealed gross total resection of the mass lesion, adequate screw placement and moderate kyphosis of 34°  remains to be seen in longer-term follow-up, and is the subject of future studies.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.The authors declare that they have no competing interests.GA was responsible for the concept and design of the manuscript and for writing and editing of the manuscript. KR aided in the illustration of the manuscript. AA analyzed and interpreted the pathological data for our patient. WEW aided in the editing of the manuscript. DJC aided in the editing of the manuscript. TGL aided in the editing of the manuscript. AJ was responsible for the concept and design of the manuscript and for writing and/or editing the manuscript. All authors read and approved the final manuscript.


T. solium. It has a worldwide distribution and is potentially harmful with variable clinical manifestations. The patient most commonly presents with subcutaneous and muscle involvement in the form of nodular lesions. The other most commonly involved sites include eye, brain, bladder wall, and heart. Cysticercosis can be diagnosed on serology, and radiologically but confirmatory diagnosis is based on histopathological examination of the involved tissue biopsy specimen. Fine needle aspiration cytology is a useful low-cost outpatient procedure tool for preoperative diagnosis of cysticercosis and is absolutely essential for diagnosis of the parasitic lesions in a peripheral hospital, one like ours. Cysticercosis is a systemic parasitic disease caused by the larval form of cestode  T. solium. The diagnostic role of  FNAC in cysticercosis was first emphasized by Kung et al. in 1989 [Human cysticercosis is the larval infestation of the cestode  in 1989 . It alsoA 4-year-old male patient presented with a superficial lump on left side of the abdominal wall since 1 month. The differential diagnosis of a lipoma, an abscess, or a tuberculous abscess was considered by the clinicians. The patient was sent to the pathology department for FNAC, it being the primary investigation in such cases. On examination, the lump measured 3.5 × 3 cm in size, slightly tender and soft to firm in consistency. Fine needle aspiration was done using 22G needle and 10 mL syringe without aspiration technique and yielded straw colored slightly turbid fluid. The smears were air dried and stained with May Grunwald Giemsa, haematoxylin, and eosin stains. Modified Ziehl Neelson stain was performed on one smear to rule out tuberculosis. On microscopic examination, fair number of neutrophils, lymphocytes, palisading histiocytes, eosinophils, and degenerated cells were noted. No granuloma or atypical cells were seen. Presence of eosinophils warranted closer look and on careful examination of five smears made from the aspirate, a solitary detached hooklet of cysticercus  was noteHuman cysticercosis is the larval infestation of the cestode Taenia solium. Though cysticercus can be found in any organ, it is especially common in skeletal muscle and subcutaneous tissue as nodular lesions and is difficult to differentiate from benign mesenchymal tumors and lymphadenitis on clinical grounds alone . Fine needle aspiration cytology (FNAC) in cysticercosis is low-cost outpatient procedure for preoperative diagnosis and may even obviate the need for open biopsy . The cytCysticercosis cellulose is more common than usually thought. In endemic areas, cysticercosis cellulose should be included in the differential diagnosis of nodular lesions including echinococcus, granulomatous lesions, and soft tissue swelling. Various diagnostic modalities employed to detect cysticercosis apart from FNAC include radiology, serology, and pathological examination . CT and


Mucinous tubular and spindle cell carcinoma of kidney (MTSCC-K) is a rare variant of renal tumor. The current data show most of MTSCCs are of low malignant potential and rare cases metastatic to lymph nodes have been reported; however, the recorded computed tomography (CT) and follow up data are limited.In the present study, we retrospectively analyzed CT and clinicopathological data of eight patients with renal MTSCC-K.A total of eight cases, including six females and two males, were included in this analysis with a mean age of 48.4 (range 25 to 81) years. Mean tumor size was 4.2 (range 2.5 to 10.0) cm. Preoperative CT demonstrated that all tumors were slightly enhanced on both corticomedullary and nephrographic phase, which was different from many other renal cell carcinomas. Three of them were treated with open radical nephrectomy, three with laparoscopic radical nephrectomy and the other two with laparoscopic partial nephrectomy. No postoperative therapy was applied. Patients were followed up for 15 to 64 months and there was no evidence of recurrence and metastasis.The MTSCC-K has special clinicopathological characteristics, low degree of malignancy and relative good prognosis. The diagnosis mainly depends on the histopathological examination and CT may help to differentiate with papillary renal cell carcinoma. Surgical treatment is recommended and additional therapies are not necessary.http://www.diagnosticpathology.diagnomx.eu/vs/8435581771088249.The virtual slides for this article can be found here:  Mucinous tubular and spindle cell carcinoma of the kidney (MTSCC-K) is a rare pathological entity and has been described as a specific subtype of renal cell carcinoma (RCC) in the 2004 World Health Organization classification . It is cEight cases of MTSCC-K were identified from a review of approximately 1430 cases of RCC from the Department of Urology, Ren Ji Hospital between January 2006 and December 2010. Detailed clinical information of the 8 patients was listed in the Table Hypovascular renal masses were noted in all cases on ultrasonography, and subsequent computed tomography was performed initially to obtain baseline attenuation values of lesions and to identify calcification; it demonstrated that all tumors had well-defined margins, and were slightly enhanced on both corticomedullary phase (CMP) and nephrographic phase (NP) Figure a,b,c. ThRadical nephrectomy was applied to six cases, among which three were treated via laparoscopic approach. Laparoscopic partial nephrectomy was applied to another two cases as the tumors’ sizes were less than 4 cm. No postoperative therapy was given to any of the eight patients.Grossly, these tumors were well-circumscribe solid, tan-yellow or grayish yellow in appearance Figure A, with oMucinous tubular and spindle cell carcinoma is a rare, malignant renal epithelial tumor which showed a female predominance and favorable prognosis and had been recognized as a new entity of RCC, and usually behaved in a low-grade fashion . More thAs described before, our results are similar to the previous studies. They, histologically, are characterized by small elongated tubules lined by cuboidal, spindle cells and variable amounts of myxoid stroma.We further described the clinicopathological findings about the MTSCCs and pay particular attention to the CT features. On unenhanced scan, the MTSCC-K, as many of the other subtypes of solid RCCs, are with attenuation values ranged from 31 to 40 HU. And the attenuation values were ranged from 38 to 50 HU on CMP, 45 to 67 HU on NP, respectively. Many previous studies suggest that MTSCCs may represent a variant of papillary RCC ,6, but tIn addition, MTSCC-K lack the gains of chromosomes 7 and 17 and losses of chromosome Y which are typical of papillary RCC , and usiTill now, the origin of MTSCC-K remains uncertain. In the literature, epithelial markers, especially AMACR, CK7, EMA and Vimentin, have been reported to be positive in 80-100% of cases , and theIn our series, prognosis seems to be favorable and there was no recurrence or metastasis, with a follow-up of between 15 and 64 months. However, occasional cases with local recurrence, distant metastasis and regional lymph node involvement have been reported . In receAccording to the literature and our series, the clinicopathological features of MTSCC are indicated as follows. Firstly, the tumor is characteristically consisted of large eosinophilic spindle or cuboidal cells arranged in tubular patterns embeded in mucinous or myxoid stroma . SecondlThe MTSCC-K has special clinicopathological characteristics, low degree of malignancy and relative good prognosis. Our reports are benifitial supplement for better understanding the clinicopathological features of MTSCC. Meanwhile, the CT findings, treatment and follow-up data are valuable information for guiding future clinical practice but further studies are required to confirm our computed tomography findings.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.MTSCC-K: Mucinous tubular and spindle cell carcinoma of the kidney; RCC: Renal cell carcinoma; CT: Computed tomography; CMP: Corticomedullary phase; NP: Nephrographic phase; HU: Hounsfield units.The authors declare that they have no competing interests.X-RW and Y-HC designed the study, performed the histological and immunohistochemical evaluation, drafted the manuscript and performed the literature review; J-JS participated the literature review and collected the patient’s clinical information; LZ and J-WH carried out the immunohistochemical staining; J-JB and Y-RH participated in pathological investigations and helped to draft the manuscript .D-ML participated in pathological investigations, revised manuscript for important intellectual content and had given final approval of the version to be published. All authors have read and approved the final manuscript.


Thymoma is an epithelial neoplasm of the thymus, which commonly lies in the anterior mediastinum and, therefore, an intrathoracic origin is considered to be rare. This report presents a case of giant thymoma arising in the thoracic cavity. A 61-year-old male presented with a chronic cough and breathlessness. Chest CT revealed a well enhanced giant mass approximately 18 cm in diameter in the right thoracic cavity. FDG-PET showed that the SUVmax of the tumor was 5.0 in the center and almost 2.5 in the surrounding area. A trans- bronchial needle biopsy was performed to find atypical cells. Surgery was scheduled based on the radiological and histological findings. A well-defined giant mass in the thoracic cavity, measuring 18 × 14.5 × 11 cm had undergone expansive growth without apparent invasion. The tumor was completely resected without combined resection of the other organs. The weight of the tumor was 1350 g. The tumor was histologically diagnosed to be type AB thymoma according to the World Health Organization classification and Masaoka stage IIB. Thymoma is an epithelial neoplasm of the thymus, which commonly lies in the anterior mediastinum and ectopic thymomas account for only 4% of all thymomas . Most ecA 61-year-old male presented with chronic cough and breathlessness. Chest CT revealed a well enhanced giant mass, approximately 18 cm in diameter in the right thoracic cavity Figure . A microThymomas develop mainly in the thymus and are usually located in the anterior mediastinum, only 4% of the tumors are ectopic tumors . EctopicFDG-PET showing increased uptake of FDG is suggestive of malignant potential and SUVmax correlates with malignancy in mesenchymal tumors. Several authors reported a significant correlation between the histological subtype of thymicepitherial tumors and FDG-PET accumulation ,5. Who tAlthough thymomas can present as huge masses, the symptoms and stage may not always correlate with tumor size. A large tumor size is a significantly poor prognostic factor of thymomas . Limmer This report presented a very rare case of an intrathoracic giant thymoma, histologically diagnosed as type AB Masaoka stage IIB. Surgical resection must be considered regardless of tumor size if curative resection is possible. FDG-PET is useful modality for preoperatively evaluating the grade of thymomas.Our Institution and the patient provided Clinical Consent for the publication of this case.All the authors declare that they have not competing interest.TT: concept and design, writing the article. YH, ST, HM made a critical review of the manuscript. TI: final approval of the article. All authors read and approved the final manuscript.


Pleomorphic rhabdomyosarcoma (RMS) is a rare tumor with an aggressive behavior, described mainly in adulthood. Herein we present two cases of paratesticular pleomorphic RMS in 71- and 16-year-old patients with metastases at initial diagnosis. Histological, immunohistochemical, and ultrastructural findings were essential to confirm diagnosis. Few months after radical orchiectomy, both patients died before or just after starting adjuvant chemotherapy. Rhabdomyosarcoma (RMS) is a highly malignant mesenchymal tumor thought to originate from immature striated muscle. It is characterized by the presence of cells having an identifiable striated muscular differentiation with rhabdomyoblasts cells . Twenty Herein, we report two cases of pleomorphic RMS diagnosed in an adolescent and adult patient with multiple metastases at initial diagnosis.A 71-year-old man presented to our urology department for a 2-year history of testicular swelling and a painless mass in the left scrotum. The patient felt uncomfortable with that mass with a feeling of heaviness. His past medical history was unremarkable. The physical examination showed a 20 cm solid mass occupying the left scrotal region with a buried penis .The mass seemed to be independent from the testis and ultrasonography confirmed that it was an extratesticular solid mass. A contrast enhanced computed tomography (CT) revealed multiple pulmonary lesions and retroperitoneal lymph nodes. The diagnosis of paratesticular tumour with multiple metastases was made and surgical excision with adjuvant chemotherapy was decided. The patient underwent left high inguinal orchiectomy. The testis was macroscopically normal and two tumours of 15 and 4 cm were localized in paratesticular region . The totTwo months after surgery the patient died from cachexia before starting any adjuvant therapy.A 16-year-old boy presented to our department for scrotal swelling evolving for 6 months. He complained of scrotum enlargement and of uncomfortable heavy sensation. He denied any scrotal trauma and his history was unremarkable. Physical examination revealed an enlarged, tender, right scrotum measuring 18 cm with unrecognizable testis.Ultrasonography revealed that the mass originates from the paratesticular region. CT scan showed a solid process in the right scrotum compressing the two testicles with large retroperitoneal lymph nodes. The diagnosis of paratesticular tumour was considered and a right radical inguinal orchiectomy was performed.hematoxylinophilic eosin staining, vacuolar cytoplasm, and with ovoid circular core provided with vesicular chromatin. There were also many zones of necroses. Mitoses were very numerous. This sarcomatoid proliferation invaded muscular and fat fabric of vicinity. The testicular parenchyma was normal. The immunohistochemical study using the muscular markers and the PS100 showed a positivity of the tumoral cells for desmin, actin smooth muscle, and myogenin attesting the striated muscular nature with this proliferation. The final pathologic diagnosis was pleomorphic RMS involving paratesticular soft tissues.Pathologic microscopic examination revealed a malignant mesenchymal proliferation made out of fusocellular pleomorphic architecture arranged with spindle-shaped cells. Elsewhere, architecture was diffuse; made of polygonal, racket-shaped cells, and large multinucleate cells with strong An adjuvant chemotherapy protocol using vincristine, actinomycin and ifosfamide was started one month postoperatively and radiotherapy to retroperitoneal lymph nodes was planned thereafter. Unfortunately, the patient died 4 months after surgery.Malignant tumours of the paratesticular region are uncommon and are mainly sarcomas. The most common histological subtypes of sarcomas are leiomyosarcoma 32%), rhabdo-myosarcoma , liposarcoma (20%), and malignant fibrous histiocytoma  [2%, rhabdRMS is a highly malignant tumour arising from striated muscle cells and is associated with early and wide spread metastasis , 3. CommRMS is more frequent in males than in females with a ratio of 3 : 1  and occuClinically paratesticular tumour presents as an intrascrotal mass, usually large, sometimes reaching up to the external inguinal ring and compressing the testis and epididymis. In large tumours it may be clinically indistinguishable from testicular tumours. The usual clinical presentation is a painless testicular enlargement. Pain has been reported only in 7% of cases , sensatiExtension of paratesticular tumours to retroperitoneal lymph nodes is common and occurs in up to 30% of patients and lung metastases are present at diagnosis in 14% of cases . UsuallyDiagnosis of pleomorphic RMS is histopathological. Diagnosis of this tumour is based on morphologic, immunohistochemical, and ultrastructural findings that identify the skeletal muscle phenotype . NormallTreatment of pleomorphic RMS paratesticular tumour consists mainly of surgical excision and adjuvant chemotherapy and/or radiotherapy. As paratesticular sarcomas are rare there is no standard treatment; however, there is a general consensus that a radical orchiectomy including high ligation of the spermatic cord should be done first , 14. HemAdjuvant chemotherapy is added after surgery in case of distant metastases to improve prognosis. Many protocols of chemotherapy have been tried. VAC, IVA, and VIE protocols  were mainly used and better results were observed with VAC protocol , 10, 15.According to Intergroup Rhabdomyosarcoma Study (IRS) classification, patients with metastases at diagnosis are classified as stage IV (14%) . They reIn fact survival rates vary with the initial site and histological subtype of RMS. Pleomorphic RMS is known to have the worst prognosis . Our twoParatesticular pleomorphic RMS is a rare and aggressive tumour. The diagnosis is made after histological examination. Immunohistochemical and ultrastructural findings could be necessary in some cases. Retroperitoneal lymph node dissection causes a big morbidity and is not recommended. Computed tomography is effective in staging the disease.The first step of treatment of paratesticular RMS consists of a high inguinal orchidectomy. Adjuvant chemotherapy should be added, with or without radiotherapy, for distant metastases diseases.Metastatic paratesticular pleomorphic RMS has a poor prognosis.