Although the usefulness of susceptibility-weighted imaging (SWI) for detecting basal ganglia germinoma has been reported, the technique is not widely used. We recently encountered an unusual case of primary cerebellar germinoma, presenting with progressive ataxia and cranial nerve palsy, characterized by gradually enlarging low-intensity lesions visible with both T2*-weighted imaging (T2*WI), which were the key to the diagnosis.A 30-year-old man was referred to our hospital because of slowly progressive dizziness and mild ataxia. Magnetic resonance imaging (MRI) revealed a small, low-intensity spot in the left cerebellar peduncle on the T2*WI and SWI without enhancement. Cerebral angiography revealed no vascular abnormality. The serum α-fetoprotein value was normal. A steroid-pulse was administered as a therapeutic and diagnostic trial, but the symptoms improved little. The patient was discharged from the hospital but soon developed brainstem dysfunction, characterized by dyspnea or hiccups, and he was readmitted. T2*WI imaging revealed expanded and extended spotty lesions in the cerebellum and brainstem, which had not enhanced with contrast agent previously. Targeted stereotactic biopsy of the newly enhanced cerebellar lesion was performed; histopathological examination of the tissue revealed pure germinoma. Serum and cerebral spinal fluid values of beta-human chorionic gonadotropin were not significantly elevated. Chemotherapy with carboplatin and etoposide was initiated. The enhanced lesion disappeared promptly, but the patient continued to require assisted automatic ventilation because of paralysis of respiratory muscles.We conclude that enlarging low-intensity lesions on T2*WI and SWI may be a reliable clue to the diagnosis of germinomas, irrespective of their location, even without enhancement. Biopsy of the tumor at an early stage is the only way to make the diagnosis conclusively and enable prompt start of treatment.The online version of this article (doi:10.1186/s12883-015-0516-9) contains supplementary material, which is available to authorized users. Intracranial germ cell tumors (GCT) are rare, comprising only 0.6–2.8 % of all intracranial tumors –3. AboutA 30-year-old male was referred to our hospital because of symptoms of slowly progressive dizziness and mild ataxia. He had been in good health until three years earlier except for the past medical history of hepatitis B, when he presented with transient dizziness without any abnormal findings on MRI  and etoposide . The enhanced lesions disappeared promptly, but low-intensity lesions remained visible by T2*WI and SWI. Radiation therapy was difficult to perform because of the patient’s poor physical condition. With rehabilitation, he made slight progress, and he now lives in his own house, but he remains almost bedridden and requires tracheostomy and ventilator assist because of the damage of the brainstem and cerebellum.Two months later, the patient was re-admitted with persistent hiccups, gait disturbance, and dysphagia. He became dyspneic and was intubated and ventilated. Alpha-fetoprotein and human chorionic gonadotropin β-subunit values were normal in serum and cerebral spinal fluid. MRI studies revealed mildly enhanced lesions surrounding the 4th ventricle and the left cerebellar hemisphere. With the use of T2*WI and SWI, low-intensity lesions were observed to be increased and expanded in bilateral cerebella and the dorsal brainstem. Fig. , k, l. AGerminomas typically arise in regions adjacent to the third ventricle, such as the pineal body and neurohypophysis, but they may be present also in the basal ganglia. Germinomas originating in the cerebellum are quite rare –11 (see 11C-methionine positron emission topography provides valuable information for making the diagnosis of germinoma, and it is useful also for evaluating treatment effects and monitoring for tumor recurrence [In the present case, low-intensity spots were present in the left cerebellar hemisphere with both T2*WI and SWI at early stages of tumor growth, when the tumor showed no enhancement after gadolinium administration. To our knowledge, this is the first report describing low-signal intensity lesions on T2*WI without any enhancement. Interestingly, low-signal lesions on T2*WI and SWI were found to concomitantly expand with tumor progression. Although having not been performed in our case, currence , 15.Since intracranial GCTs are extremely sensitive to radiation, the possibility remains that our patient’s tumor became temporarily invisible by conventional MRI techniques due to the radiation exposure of the CT scan. Since diagnostic CT scans are required in clinical practice, the patient had received a CT scan prior to the initiation of the MRI studies. Although conventional MRI showed no unremarkable findings, low-signal lesions were observed with T2*WI and SWI, suggesting that low-signal lesions may not be affected by radiation and may be useful in detecting intracranial GCTs after radiation exposure associated with CT. This view is supported by the fact that the low-intensity lesions remained visible after chemotherapy, while gadolinium-enhanced regions disappeared.The symptoms of intracranial GCTs depend on tumor location, and typical symptoms include hydrocephalus, diabetes insipidus, hypopituitarism, hemiparesis, or higher cognitive dysfunction . IntracrEnlarging, low-intensity lesions on T2*WI and SWI may be a reliable clue to the diagnosis of germinomas, irrespective of their location. Biopsy at the early stage is the only way to conclusively make the diagnosis and lead to early treatment.Written informed consent was obtained from the patient’s mother for publication of this case report and accompanying images. A copy of the written consent is available for review by the editor of this journal.


Hepatocellular carcinoma (HCC) is an aggressive tumor that frequently occurs in the setting of chronic liver disease and cirrhosis. Herein, we describe a case where a patient presented with acute onset cauda equina syndrome due to an intradural and extramedullary metastatic tumor bleeding from hepatocellular carcinoma (HCC). The patient had lower back pain that had radiated to the bilateral lower legs for 3 weeks. Then, the patient had experienced an acute onset of bilateral lower leg weakness as well as bladder-urinary dysfunction 2 days before going to the ER. The patient received a laminectomy from the L1 to L4 vertebra, removing the intradural spinal tumor and hematoma. To the best of our knowledge, this is the first reported case of HCC metastasized to the cauda equina with tumor bleeding causing cauda equina syndrome. Indeed, metastases from outside of the central nervous system in this region are very rare [The majority of ery rare . The symery rare , 3. Up tery rare . Herein,A 57-year-old man presented with a 3-week history of worsening lower back pain radiating to the posterior aspect of the bilateral legs. As well, he had an acute onset of bilateral lower leg weakness for 2 days. The patient’s medical history was significant because he had undergone a liver echo guide biopsy that revealed cholangiocarcinoma in August of 2012. Furthermore, he had hepatitis B and liver cirrhosis for many years. During the physical examination, it was noted that there was a significant reduction in the range of motion in the lumbar area due to severe back pain. The pain was worsened by coughing and sneezing. The patient had poor lower muscle strength (Muscle Power: 2/2) and a decreasing sensation to pinpricks over the right thigh (L3). Both plantar responses yielded no response. Patella and Achilles tendon reflexes were diminished in both lower extremities. In addition, he had bladder-urinary dysfunction and saddle anesthesia. Tumor marker showed AFP(EIA): 13.45 ng/mL CEA(EIA): 13.76 ng/mL, PSA(EIA): 0.195 ng/mL, CA19-9(EIA): 540.2 U/mL.A lumbosacral magnetic resonance image (MRI) indicated infiltrative hypointensity in the dural sac from T12 to S1 level. compressing the spinal cord and cauda equinal nerves was noticed on both the T1WI and T2WI, leading to suspicion of an intradural hematoma. There was an ill-defined intradural extramedullary lesion at the L2 level, showing hyperintensity on T2WI and heterogeneous contrast enhancement, leading to suspicion of a metastatic tumor.  is an aggressive tumor that frequently occurs in the setting of chronic liver disease and cirrhosis. The incidence of extrahepatic metastasis reportedly ranges from 13.5% to 36.7%, and the lungs, adrenal glands, and bone marrow are the sites where HCC frequently metastasises . Metastavia the arterial route, (2) through the extra-intraspinal anastomosing venous network (Batson’s plexus) [et al. [Several routes have been hypothesized by which malignant tumour cells might reach the cauda equina : (1) hae plexus) , (3) cen plexus) , (4) by  plexus) , and (5) plexus) . In our  [et al.  reportedTreatment options for compressive space-occupying deposits have not been clearly defined, and a nonsurgical approach is recommended by several authors. Nevertheless, surgical treatment of compressive intradural metastases of the cauda equina seems to be a feasible treatment option with low operative risk and with the potential benefit of an immediate relief of pain and improvement in motor function and thus an increase in quality of life .cauda equina is a rare occurrence. Our case presented with bleeding producing further neurological deterioration. Hemorrhagic presentation has only once been previously reported [In conclusion, intradural metastasis to reported . Therefo


Primary diffuse leptomeningeal gliomatosis (PDLG) is a lethal neoplasm that is characterized by glioma cells exclusively infiltrating into cerebral and spinal meninges. Intraventricular hemorrhage as an initial symptom in PDLG patient has not been reported in the literatures.A 39-year-old man initially presented with intraventricular hemorrhage. The patient had an improved outcome at the early stage of hemorrhagic course; however, the clinical condition began to a sudden turn for deterioration with intracranial hypertension and cerebral hernia on day 15 after admission. Cerebral CT and MRI showed diffuse patchy signals with enhancement in bilateral cerebellopontine angle cistern, suprasellar cistern, ambient cistern, quadrigeminal cistern, bilateral cerebellum, cerebral hemisphere, and upper cervical cord surface. Pathological examination revealed that numerous spindled cells were scant of cytoplasm with hyperchromatic nuclei and various mitotic figures. Immunohistochemistry showed that the cells were positive to glial fibrillary acidic protein (GFAP) with about 5 % Ki-67 positive labeling. The pathological findings were consistent with the diagnostic criteria of anaplastic astrocytoma (WHO grade III).We reported an interesting case that PDLG initially presented with intraventricular hemorrhage that might be caused by astrocytoma rupturing into pial vessels.The online version of this article (doi:10.1186/s12883-015-0341-1) contains supplementary material, which is available to authorized users. Primary diffuse leptomeningeal gliomatosis (PDLG) is a lethal neoplasm that is characterized by glioma cells diffusely infiltrating into cerebral and spinal meninges, while absent of tumorous lesions in the brain parenchyma –3. Non-sIn a cohort of study, 4.9 % patients discharged with diagnosis as brain neoplasm were initially considered as a stroke . About 5The patient was a 39-year-old man who was diagnosed as tuberculous pleurisy at age 30, and then was treated with standard anti-tubercular drugs for 6 months, but clinical follow-up was not carried out after alleviation of his symptoms. No history of hypertension was recorded. He had nonspecific headache one month ago, but no attentions were paid. He was admitted to our hospital with right paresis, headache and vomiting for one hour. On admission, his blood pressure was 130/80 mmHg. He had slightly slurred pronunciation; muscle strength was 4/5 grade  in the right limbs; and slight hearing loss was found on the right side. Neck stiffness and Kerning sign were also observed. Cerebral computed tomography (CT) revealed hemorrhage in the left posterior horn of lateral ventricle and subarachnoid space Fig. . At the However, on day 15 after admission, he suddenly suffered from severe headache and recurrent vomiting. No fever and bellyache was observed. To exclude the possibility of rehemorrhage, cerebral CT was performed again. No hematoma was found Fig. , but theOn day 36 after admission, second CSF examination was performed. Results showed nucleated cells count was 8 cells/uL; glucose level was 3.39 mmol/L (concomitant blood glucose 5.37 mmol/L); chlorine level was 116 mmol/L; and protein level was 1787 mg/L. Malignant cells also were not found in cytology. His situation showed no signs of improvement and gradually worsened daily. After a written consent signed by patient’s wife, a needle biopsy of the lesion in the left frontal lobe was performed on day 46 after admission. On day 3 after biopsy, he died from cerebral hernia owing to intractable intracranial hypertension.Pathological examination revealed that numerous spindled cells were scant of cytoplasm with hyperchromatic nuclei and various mitotic figures Fig. . NecrosiLeptomeningeal gliomatosis is usually divided into secondary type and primary type . The secet al. in 2009 described a case diagnosed as hemorrhagic glioblastoma of the frontal lobe adherent to the underlying dura with leptomeningeal metastases [The most interesting clinical feature was intracranial hemorrhage as the initial symptom in this PDLG patient. Burrus TM tastases . Howevertastases –3. The mtastases , 15. As The most important features of PDLG in radiological images are exclusive lesions in pia mater, which are prone to involving in posterior fossa, basilar cistern and spinal cord , 15. Ceret al. reported patients with leptomeningeal dissemination of anaplastic astrocytoma suffered from subarachnoid hemorrhage after radiochemotherapy [The pathological features in all of PDLG patients were almost consistent with high-grade gliomas (WHO III or IV) , 2. In ootherapy . The hemThis diagnosis should be differentiated from several diseases. First of all, the tubercular meningitis should be considered. Antituberculosis therapy had been administered in more than half of reported cases with PDLG . Our patIn summary, we described a case of primary diffuse leptomeningeal gliomatosis associated with anaplastic astrocytoma. It was an interesting case of PDLG initially presenting with intraventricular hemorrhage that might be caused by astrocytoma rupturing into pial vessels.Written informed consent was obtained from the patient’s wife for publication of this case report and any accompanying images. A copy of the written consent is available for review by the editor of this journal.


Three sporadic, synchronous, and separate lesions in the ampulla of Vater and the head of the pancreas presented in an 81-year-old male. One was symptomatic and two were incidental. One was detected preoperatively (the ampullary lesion) and two by examination of the resected specimen (the neuroendocrine and pancreatic carcinomas). The case is summarized and the literature and the issue of commonality are reviewed. Multiple primary neoplasms (MPNs) develop in 5 to 10% of the population. SynchroThree synchronous, sporadic, separate tumors involving the head of the pancreas are very rare <0.5%),,6], and An 81-year-old male presented with painless jaundice of 1 week duration, preceded by nausea, anorexia and 4.5 kg weight loss. There was a history of coronary artery disease and type II diabetes mellitus. Abnormal laboratory tests included a bilirubin of 11.1 mg/ dl, an alkaline phosphatase of 481 U/L, and an aspartate and alanine transaminase ratio of 303/262 U/L. Magnetic resonance imaging showed a 1.6-cm cystic neoplasm in the head of the pancreas. An endoscopy, endoscopic retrograde cholangiopancreatogram, and endoscopic sonogram revealed a bulbous ampulla suggesting a submucosal mass, which was confluent with a mass in the head of the pancreas. It was 2.6 cm, solid and irregular, and obstructed a dilated bile and pancreatic duct. The impression was a pancreatic or ampullary malignancy. The surgical impression was a pancreatic malignancy invading the ampulla and duodenum and, if health risks permitted, surgery was advised. A biliary stent was placed at endoscopy, the jaundice cleared, and after medical and cardiac clearance the patient underwent surgery. Absent of metastases, a single lesion was palpated in the head of the pancreas near the ampulla. A pylorus preserving pancreaticoduodenectomy was performed in January 2013. The patient’s postoperative course was uneventful. He was discharged on postoperative day 9. Despite chemotherapy, hepatic metastases were noted by June 2013 and had increased by August 2013. He died in November 2013, 10 months after surgery.The gross and histological images are shown in Figures In the uncinate process, a second lesion Figure  was idenThe third lesion in the anterior pancreatic head Figure  was a 1.An additional finding throughout the resected specimen was chronic pancreatitis and high grade pancreatic intraductal neoplasia (PanIN 3).A diagram depicting the location and relation of each tumor in the head of the pancreas is provided in Figure MPN are not common, with an incidence of 5 to 10%, 6. The in situ and the third was a 3-mm invasive lesion. Despite the small size of the invasive lesions, and normal markers, one metastatic node was present[Multiple reported synchronous pancreatic lesions include combinations of adenocarcinoma, NETs, and cystic lesions. Usually two lesions are identified. Synchronous pancreatic adenocarcinomas and intraductal papillary mucinous tumors (IPMNs) have been reported (most are side-branch lesions) to coexist in 9% of cases. One pub present. Many MP present, 10. In  present. The pat present. Adenoca present, 23. Whe present. Immunos present–14. In o present. AlthougAmpullary cancer accounts for 1% of all gastrointestinal malignancies, 6% of all periampullary cancers, and are one-tenth as frequent as pancreatic cancer. AmpullaThe second lesion, a 3.1-cm adenocarcinoma of the pancreas was an incidental lesion, not identified by endoscopic sonogram or magnetic resonance imaging. The dismal prognosis of pancreatic adenocarcinoma is well known. Even when incidental to other lesions or detected by serial screening the prognosis and cure rates are poor.The third lesion was a small (1.2 cm) pancreatic NET with cystic degeneration. As body imaging has become common, small incidental NETs are frequently discovered. AlthougAn 81-year-old male who presented with jaundice was found to have an ampullary cancer and an incidental pancreatic adenocarcinoma and a neuroendocrine cystic lesion. After stenting of the bile duct and medical clearance, a pancreaticoduodenal resection was performed. The postoperative course was uneventful. The pathology report noted an obstructive ampullary adenosquamous cancer - a rare, highly malignant and dismal tumor. Two synchronous lesions, a 3-cm adenocarcinoma of the pancreas, and a 1.2-cm NET were identified as separate lesions in the resected specimen. Metastases were evident at 5 months and the patient died at 10 months.Informed consent was obtained from the patient’s next of kin for publication of this case report and any accompanying images.AS and MI are surgical residents at Mount Sinai Beth Israel. MW, JS, and AMC are attending surgical oncologists at Mount Sinai Beth Israel. SY is an attending pathologist at Mount Sinai Beth Israel.


Vascular neoplasms of the pancreas are extremely rare and usually manifest as symptomatic, cystic lesions. This study presents a case that includes the clinicopathologic information used to discriminate pancreatic hemangioma from other types of cystic lesion of the pancreas. A 40-year-old female visited hospital with a chief complaint of abdominal pain. The serum CEA and CA19-9 levels of the patient were within the normal limits. An abdominal computed tomography scan and magnetic resonance imaging showed a 100-mm mass lesion in the body and tail of the pancreas, and the tumor extended toward the retroperitoneum and surrounded the splenic vein. The lesion was subsequently resected. Macroscopically, it was a multiloculated cyst with intracystic hemorrhage. Microscopically, the lesion was composed of numerous, heterogeneous cysts lined by a flattened single layer of cells without significant atypia. Notably, numerous neoplastic vessels extended into the interlobular septa of the pancreas and surrounded the main pancreatic duct. Immunohistochemical analysis showed that the lining cells expressed CD31 and CD34. The lesion was diagnosed as adult pancreatic hemangioma. Surgical treatment may be required when a direct contact between the lesion and the pancreatic tissue is demonstrated using imaging. Vascular neoplasms usually occur in the visceral organs including the liver, spleen and gastrointestinal tract and occsionaly in the pancreas. A pancreatic vascular neoplasm is an uncommon type of the primary cystic neoplasm . The occA 40-year-old female visited St. Mary’s Hospital  with a chief complaint of abdominal pain. The results of the physical examination were within the normal limits, as were the levels of the two tumor markers, serum CEA and CA19-9. An abdominal computed tomography scan detected a well-defined 100-mm mass with low and high attenuation areas in the body and tail of the pancreas. The tumor extended into the retroperitoneum and surrounded the splenic vein . MagnetiMacroscopically, the lesion was multiloculated with intracystic hemorrhage and no mucinous component . MicroscFollowing full explanation of the findings, the patient accepted the proposed surgical removal of the tumor. Surgical exploration revealed an 85-mm mass in the pancreatic body and tail that was inseparable from the pancreas. Consequently, a pancreatectomy was performed. The postoperative course was uneventful and six years later, the patient had experienced no recurrence. Patient provided written informed consent.et al  The et al in 2009 (A few adult pancreatic hemangiomas have been reported. As reviewed by Mundinger  in 2009 –12 and f in 2009 –12. VascThe two most common types of cystic neoplasm of the pancreas are the intraductal papillary mucinous neoplasms and mucinous cystic neoplasms of the pancreas, and these neoplasms have malignant potential ,13,14. HGenerally, adult pancreatic hemangiomas are composed of cysts lined by a single layer of uniform endothelial cells that express CD31 and CD34 . The cas


Ciliated foregut cysts are rare anomalies arising from remnants of aberrant embryological development. Around 100 reports on the presence of these congenital masses in the tracheobronchial tree, mediastinum, liver, pancreas and, rarely, the gallbladder have been described. In this article, the case of a 33-year-old woman, who was operated for a laparoscopic cholecystectomy, is presented. During the dissection of the triangle of Calot, a cystic mass, attached to the common hepatic duct, was discovered incidentally. This cyst was dissected off the hepatic duct, and no communication between both structures was found. The histopathological diagnosis was consistent with a ciliated foregut cyst. The postoperative course was uneventful. After reviewing the literature on this pathological entity, we found that this is the first report of a ciliated foregut cyst that is located in the triangle of Calot and found separate from the biliary structures, the gallbladder and the liver. We present a review of the literature on this entity, discussing diagnostic measures and therapeutic options. The presence of cysts originating from the embryonic primitive foregut and containing a respiratory-type epithelial lining has rarely been described in medical literature. These cysts result from aberrant embryological development and are usually located above the diaphragm. Their presence in relation to abdominal organs has been described in around 100 reports. We present the first report of a ciliated foregut cyst in the triangle of Calot, in relation to the common hepatic duct. The diagnostic and therapeutic implications and the malignant potential of these lesions are further discussed.A 33-year-old woman presented to the emergency department at our hospital with recurrent episodes of right-upper-quadrant pain associated with multiple episodes of vomiting. She had an ultrasonographic scan of the abdomen that showed multiple mobile stones in the gallbladder with normal intra- and extrahepatic bile ducts with no other abnormality. The blood workup was normal including liver function tests. She was operated for a laparoscopic cholecystectomy on an ambulatory surgery basis. The operation was uneventful except for the finding of a 1.5-cm nodule within the triangle of Calot Fig.  was perfCiliated foregut cysts (CFC) are extremely rare, benign, congenital cystic lesions that arise from the embryonic primitive foregut . These cDuring embryonic development, the differentiation of the primitive foregut cells gives rise to the oropharynx, oesophagus, larynx, tracheobronchial tree, lungs, stomach, proximal duodenum, pancreas, liver and biliary system –5. The pConsequently, the association of CFC with the biliary system is quite exceptional. The first description of these lesions in the gallbladder was by Kakitsubata et al. in . A ciliaThe differential diagnosis of hepatic CFC would include biliary cyst, parasitic cyst, mucinous cystic neoplasm and various cystic metastases, such as cystic neuroendocrine tumour or necrotic metastases. When identified in the gallbladder fossa, a pancreatic pseudocyst, choledochal cyst or gallbladder duplication should also be considered .To our knowledge, this is the first description of a ciliated foregut cyst in the triangle of Calot presenting as a unilocular cyst that is attached by fibrous tissue to the common hepatic duct and is totally extramural and without any communication to the gallbladder or the hepatic ducts .This case highlights the diagnostic challenges and management options posed by this pathological rarity. In this case, the pre-operative ultrasound scan could not specifically identify the presence of the cyst, and an intra-operative cholangiogram could not prove a communication between the cyst and the biliary tracts. Furthermore, there were reports on the transformation of hepatic ciliated foregut cysts into primary squamous cell carcinoma , 10. TheCiliated foregut cysts in relation to abdominal organs are a pathological rarity. This is the first report of a foregut cyst in the triangle of Calot. Due to this incidental presentation, no firm conclusions can be drawn with regard to the diagnostic workup and management plan. However, sharing histological similarities with the more common hepatic ciliated foregut cysts, which are known to have neoplastic potential, the excision of these tumours would be a rational approach mainly in young and symptomatic patients.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.


Hemangioblastomas are tumors of the central nervous system, and the cerebellum is the most common site of occurrence. Cerebellar hemangioblastoma with enhanced wall thickness is rare and often misdiagnosed preoperatively. At present, no unified radiological classification system based on magnetic resonance imaging (MRI) findings exists for cerebellar hemangioblastoma, and this tumor type can be solid or cystic mass, according to the MRI findings. The most common presentation of cerebellar hemangioblastoma observed radiologically is a large sac with small nodules, where the wall of the large cyst is not enhanced. A tumor with enhanced large cysts and tumor nodules is extremely rare. The most effective treatment is complete resection of the cyst and the solid growth. The present case reports the successful treatment of two cases of cerebellar hemangioblastoma with enhanced wall thickness, including the MRI findings for the differential diagnoses and the surgical experiences. Hemangioblastomas are tumors of the central nervous system that most frequently arise from the vascular system; they are classed as is WHO grade I tumors . In adulFor cerebellar hemangioblastoma with an enhanced cystic wall, surgical resection is the most effective treatment. The tumor is unlikely to recur following complete resection, therefore chemotherapy or radiotherapy is not usually required. Cerebellar hemangioblastoma exhibits a good prognosis following complete resection, with a five-year survival rate of >50% . The curA 66-year-old male, presented to the Department of Neurosurgery, Tiantan Hospital, Capital Medical University  with the primary complaint of an intermittent headache for four months, with two months of ataxia. Physical examination showed abnormal ataxia in the right extremities. MRI revealed a cystic mass in the right cerebellar hemisphere. The mass was hypointense on T1-weighted images, hyperintense on T2-weighted images, and showed an enhanced solid portion on the wall of the mass following the injection of gadolinium-diethylenetriaminepentaacetate . The patA 60-year-old male was admitted to the Department of Neurosurgery, Tiantan Hospital, Capital Medical University, with gait ataxia, headaches, nausea and vomiting for one month. Physical examination showed abnormal ataxia in the left extremities with gait ataxia. Subsequently, MRI revealed irregular long T1/T2 signals in the left cerebellar hemisphere and vermis, and enhanced irregular nodular lesions without obvious edema . SurgeryCerebellar hemangioblastoma is the most common form of hemangioblastoma ,4. BasedSurgical resection is the most effective treatment for cerebellar hemangioblastomas with an enhanced cystic wall . HoweverAccording to our experience regarding surgical procedures, in order to achieve separation of the lesion from the surrounding tissue in the present cases, initially the feeding artery was occluded until the surface tension decreased, and subsequently the draining veins were blocked . The tumIn the present cases the tumors were resected successfully. No subsequent treatment was required following surgery, and a full recovery was achieved. After three years of follow-up, the patients were asymptomatic and at the time of writing, no recurrences had been identified. Continued follow-up of the two patients has been planned. The limitations of the current study included the small number of cases presented and the short follow-up periods.


Testicular metastases from pancreatic carcinomas are extremely rare and are usually seen in the late phase of the disease. Fewer than 10 cases have been reported in the literature to date, all of which occurred in patients more than 50 years old. Herein we describe the first case, to our knowledge, of testicular metastasis as the first clinical manifestation of pancreatic carcinoma in a young adult.A 36-year-old Caucasian man presented to our institution with an acutely developed severe pain in his right testis. His clinical examination and scrotal ultrasounds were consistent with a tumor involving the entire right testis. The patient underwent radical orchiectomy. His pathologic examination revealed the tumor to be a metastasis from a pancreatic cancer that was confirmed by an abdominal computed tomographic scan.The present case shows that testicular metastasis could be the presenting sign of metastatic pancreatic cancer and that testicular metastases from solid organ tumors, though typical of elderly people, may occasionally be seen in young adults as well. Testicular metastases from solid non-lymphoid tumors are extremely rare, accounting for only 0.02% to 3.6% of all testicular neoplastic lesions –3. They In a review of the literature, we found fewer than 10 cases of testicular metastases arising from a pancreatic neoplasm, all of which occurred in patients more than 50 years old –7. HereiA 36-year-old Caucasian man presented to our institution with an acutely developed severe pain in his right testis. One year before this presentation, owing to azoospermia, he had undergone bilateral testicular biopsy and sperm retrieval that led to pregnancy. Clinical examination disclosed bilateral hypogonadism with a hard and nodular right testis and absence of palpable inguinal and pelvic nodes. Scrotal ultrasounds showed marked structural changes of the entire right testis Fig. . A pre-oTaken together, such findings allowed us to rule out a primitive testicular cancer as well as metastasis from hepatocellular carcinoma, and they were consistent with metastatic adenocarcinoma of the pancreas. Chest and abdominal computed tomography demonstrated the presence of a tumor of the tail of the pancreas Fig. , along wThe low incidence of secondary tumors to the testis is apparently due to the lower temperature of the scrotum, which could impair the metastatic ability of tumor cells from solid organs , 9.Various routes of spread of metastatic tumors to the testes have been suggested, including arterial embolization, retrograde venous spread, retrograde lymphatic spread, direct spread along the vas deferens to the epididymis, and transperitoneal seeding through a patent tunica vaginalis. The dissemination mechanism seems to vary with type and location and the primary tumor, and one does not exclude the other , 10. In Testicular metastases usually are a final manifestation of widely disseminated tumors, and therefore they often go unnoticed . Rarely,et al. [Clinically, there are no specific features that differentiate primary from secondary testicular tumors, as both may present with local pain, tenderness, nodular lesions, or an indurated testis; painless swelling, however, points toward the diagnosis of a testicular tumor. Also, serum tumor markers such as AFP and β-hCG are not helpful in distinguishing primary from secondary testicular tumors. On the one hand, normal values may be consistent with primary as well as secondary malignancies; on the other hand, elevated values may be related to gonadal as well as extragonadal tumors . Intereset al. , two arcet al. , anotheret al. . In our Immunohistochemistry is nowadays regarded as the most sensitive and specific way of determining the origin of the tumor. Recently, new markers have been developed and are being used in diagnosis. These include homeobox protein NANOG, SOX2, and Oct-3/4 , 18. In The present case shows that testicular metastasis can be the presenting sign of metastatic pancreatic cancer and that testicular metastases from solid organ tumors, though typical of elderly people, may occasionally be seen in young adults as well.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.


Introduction. Spinal schwannomas are benign nerve sheath tumors. Completely extradural schwannomas of the lumbar spine are extremely rare lesions, accounting for only 0,7–4,2% of all spinal NSTs. Standard open approaches have been used to treat these tumors, requiring extensive muscle dissection, laminectomy, radical foraminotomy, and facetectomy. In this paper the authors present the case of a minimally invasive resection of a completely extradural schwannoma. Operative technique literature review is presented. Material & Methods. A 50-year-old woman presented with progressive complains of chronic right leg pain and paresthesia. The magnetic resonance imaging revealed a giant well-encapsulated dumbbell-shaped extradural lesion at the L3-L4 level. The patient underwent a minimally invasive gross total resection of the tumor using a tubular expandable retractor system. Results. The patient had complete resolution of radiculopathy in the immediate postoperative period and she was discharged home, neurologically intact, on the second postoperative day. Postoperative MRI demonstrated no evidence of residual tumor. At latest follow-up (18 months) the patient remains asymptomatic. Conclusion. Although challenging, this minimally invasive procedure is safe and effective, being an appropriate alternative, with many potential advantages, to the open approach. Schwannomas are nerve sheath tumors (NSTs) that originate from neural crest-derived Schwann cells mainly located along the dorsal sensory spinal roots and only sporadically arising from the ventral motor roots. They represent around 85% of all NSTs, which are the most common form of spinal cord tumor, corresponding approximately to 30% of all primary spinal neoplasms and having roughly the same incidence as meningiomas .Schwannomas are considered slow growing, benign lesions, although malignant subtypes exist. These tumors are uniformly distributed throughout the spine and topographically, they may be intradural-extramedullary 72%), intra- and extradural (13%), completely extradural (13%), or, exceptionally, intramedullary (1%) %, intra-. Purely Being unusual, completely extradural spinal schwannomas that are located in the lumbar spine form an even rarer subgroup of spinal NSTs, representing only 0,7 to 4,2% of all extradural schwannomas , 4. ThisIn current literature, completely extradural schwannomas have not been methodically addressed nor given much attention due to their rarity, making it difficult to establish optimal surgical strategies for their treatment , 7. AccoA minority subgroup of these tumors exhibits an invasive pattern . AccordiIn the literature, the term giant spinal schwannoma is not well defined . To simpPresentation is usually between the third and fifth decades of life, with no particular gender predilection . The golIn this paper we present a minimally invasive resection of an extradural lumbar schwannoma with contiguous intraspinal, foraminal, and extraforaminal components. Operative technique and surgical nuances are described. A revision of current literature is discussed.A 50-year-old woman, without known history of neurofibromatosis, was admitted with progressive complains of right distal anterior thigh and medial lower leg pain and paresthesias for 2 years and recently proximal ipsilateral leg weakness. These symptoms were refractory to medical therapy, exacerbated in the night and by movements, causing severe functional disability with progressing shortening of walking performance. Neurological examination revealed an abolished right patellar reflex and a 4+/5 strength in the ipsilateral psoas muscle. There were no other sensory-motor deficits or sphincter (bowel/bladder) dysfunction.On MRI, an expansive, well-circumscribed, encapsulated dumbbell-shaped extradural lesion, 36 × 26 × 33 mm , was seen, at the right L3-L4 level . This glThe patient was electively operated by one of the senior authors (Bruno Santiago) using a minimally invasive approach with an expandable tubular retractor system .Anesthesia and Positioning. Under general anesthesia the patient was intubated. Arterial line was placed for blood pressure monitoring. Intravenous dexamethasone and antibiotic prophylaxis (cefazolin 1 g) were administered preoperatively. The patient was positioned prone   . The patThe postoperative MRI (6 months after surgery) showed no evidence of residual tumor . At 18-mPostoperative bone window computed tomography (CT) scan confirmed pars interarticularis and facet joint integrity . At 18-mCompletely extradural spinal schwannomas constitute a rare subgroup of spinal NSTs. In current literature, they have not been methodically addressed, making it difficult to establish optimal surgical strategies for their treatment . Most ofAs described by Celli et al. in their retrospective series of 24 extradural schwannomas surgically treated, these tumors rarely localize in the lumbosacral region and generally extend for more than one vertebral segment and occupy a lateral position in the spinal canal, nearly always with an intraspinal-extraspinal hourglass shape [Although different classifications for dumbbell tumors of the spine have been proposed, none is sufficient to determine optimal surgical planning in view of recent advances in minimally invasive spine surgery techniques . The treThe optimal surgical approach may depend on many factors. Tumor related factors include location , extension , affected neural compartments , size, and “modified Sridhar classification of benign NSTs” . Patient related factors include the preoperative neurological status (presence or not of neurologic deficit), age of the patient, and the duration of symptoms. Surgeon's related factors combine an understanding of the underlying anatomy, surgeon's experience, and individual preference.The mainstay of treatment for extradural schwannomas is, like in other NSTs, GTR which is usually curative. According to published surgical series, conventional open posterior midline approaches have typically been used to treat extradural schwannomas, requiring a dorsal midline incision, wide-ranging bilateral disruption to the paraspinal muscles and ligaments (major contributors to the maintenance of proper spine biomechanics), extensive laminectomy (from one or two levels above and below the tumor), radical foraminotomy, and complete unilateral facetectomy , 13, 14.To avoid these iatrogenic complications, many reports describe the resection of spinal tumors  through a more restricted hemilaminectomy and medial facetectomy  , 24. WitFew reports exist stressing the potential advantages of minimally invasive techniques in the treatment of extradural schwannomas . Good su de novo neurological deficit and/or deafferentation pain. Fusion was achieved in all patients with no perioperative complications. All the patients had short hospitalization (even after reoperation) and operative time [Lu et al. presented a retrospective analysis of three patients with extradural lumbar schwannomas of the lumbar spine who successfully underwent miniopen resection and fusion using expandable tubular retractors. Two of the three tumors were giant according to Sridhar classification. Two patients had undergone prior operations . In these cases the main advantage was to approach the tumor lateral to the scar tissue, probably reducing time required for surgical dissection and risk of complications. GTR was achieved in all patients but one in whom a subtotal resection (STR) was intentionally done to preserve a functional nerve root avoidingive time .Haji and colleagues recently published their retrospective case series for the treatment of extradural, intradural-extramedullary, and intramedullary spinal tumors using minimally invasive techniques (with expandable tubular retractors). Six patients had completely extradural schwannomas (5 in the lumbar spine and 1 thoracic) that were resected with good results. The size of the tumors was not referred to. GTR was achieved in all patients but one , with comparable results in terms of blood loss, operative time, hospitalization days, and narcotic usage, when compared to prior reported data with standard open techniques , 28, 29.Weil et al. reported the case of a minimally invasive removal of a giant extradural foraminal schwannoma through a nonexpandable tubular retractor. According to authors' point of view, this may be associated with even less tissue destruction than miniopen techniques using expandable retractors, translating into less blood loss and a quicker functional recovery. GTR was achieved and no complications were found .Nzokou and colleagues recently presented a retrospective series of consecutive patients who underwent minimally invasive resection of spinal tumors using nonexpandable tubular retractors. Seven extradural schwannomas were removed, 5 in the thoracic and 2 in the lumbar spine. In all patients a GTR was achieved except in one thoracic tumor, in which the tumor capsule was adherent to the diaphragm. A STR  was performed in this patient. Authors did not mention the dimensions of the tumors .To our knowledge, the case we present is the 19th completely extradural schwannoma resected through a minimally invasive approach, the 13th in the lumbar region and, considering its size and Sridhar modified classification, the 4th giant tumor.For this kind of pathology, consensus regarding the best treatment approach does not exist. This minimally invasive approach which technically is not extrademanding allows achieving results equivalent or superior to traditional open technique. Completely resecting extradural schwannomas can be both technically challenging and rewarding because these tumors are most often benign lesions for which curative resections can be safely performed through minimally invasive approaches. Patient outcomes are usually very good. Minimally invasive approaches provide adequate exposure to safely remove giant dumbbell type extradural schwannomas, without the need for potentially destabilizing facet or pedicle resection. Tubular retractors provide a direct surgical corridor to the tumor minimizing soft tissue damage and allowing decreased neural structures retraction or manipulation. To assure optimal postoperative outcome and patient satisfaction, proper patient selection is paramount.This minimally invasive procedure is safe and successful, being an appropriate alternative, with many potential advantages, to the open approach for the treatment of giant lumbar extradural schwannomas. Further studies are needed to completely validate this.


EWSR1 and members of the ETS family in most of the cases. Neuronal maturation after chemotherapy is a rare phenomenon and we herein describe such an exceptional case.Ewing sarcoma is the second most common bone tumor, occurring mainly in children and young adults. It shows a typical primitive, small round cell morphology and a characteristic fusion oncogene involving EWSR1-FLI1 gene fusion. He underwent neo-adjuvant chemotherapy and resection of the tumor. On microscopic evaluation, part of the tumor showed ganglioneuroblastoma-like differentiation with expression of neuronal markers. The continued presence of EWSR1 rearrangement in both the blue round cell component and the ganglioneuroblastoma-like component was shown by FISH analysis.An 8-year old boy was diagnosed with a Ewing sarcoma in the left femur. On biopsy the morphology was typical and there was an In conclusion, this case describes the possibility of a Ewing sarcoma to differentiate into a ganglioneuroblastoma-like lesion after neo-adjuvant chemotherapy treatment; the prognostic value of this phenomenon remains questionable.The online version of this article (doi:10.1186/s13000-016-0516-0) contains supplementary material, which is available to authorized users. EWS-ETS fusion genes as a driver mutation [Ewing sarcoma, first described by James Ewing in 1921, is the second most common malignant bone tumor after osteosarcoma. Occurring mainly in children and young adults; it accounts for 3 % of childhood malignancies. During the last decades, the 5 year overall survival has improved up to 85 % for patients with localized disease but is still only 30 % for patients with metastatic disease [mutation .Neural differentiation in Ewing sarcoma is a rare phenomenon and has been described before in a few cases , 5. HereAn 8-year old boy with no previous relevant medical history, presented with pain and a palpable mass in the left upper thigh. MRI showed a ca. 10 cm large lesion in the femur with soft tissue extension. There were no signs of metastasis elsewhere. The patient received neo-adjuvant chemotherapy by the Ewing 2008 protocol. After 4 cycles of VIDE  chemotherapy, a staging FDG-PET/CT-scan was performed, showing tumor regression, however no normalization of metabolic activity at the primary tumor site. After 6 cycles of VIDE, resection of the femur diaphysis was performed with reconstruction with a vascularised fibula transplant. An optimal recovery has been seen so far, with no sign of recurrence or metastatic disease at 21 months after surgery.EWSR1-FLI1 fusion gene confirmed this diagnosis.The pretreatment biopsy  revealed microscopically a small blue round cell tumor with necrosis. A panel of antibodies was applied according to standard protocol  background Fig. . To confThe cellular origin of Ewing sarcomas remains both elusive and controversial. However, the immature neural phenotype of many tumors, formerly known as PNET , 6, alonDelattre et al. and Burchill et al. described neuroblastoma-like features in a few untreated Ewing sarcoma cases with genetic confirmation , 9, whicOn the other hand, our case and the reported cases by Maeda et al. and Collini et al. show ganglioneuroblastoma-like maturation after chemotherapy probably as treatment effect .EWSR1-FLI1 fusion gene in both pre- and post-treatment specimens, as we did in our case [Whereas Maeda et al. confirmed their diagnosis molecularly only on biopsy material (5), Collini et al. demonstrated the presence of the our case .However, such cases are very rare, we didn’t find another one when reviewing 26 cases from our archives. This is in line with the results of Delattre et al. investigating a series of more than 90 cases with two neuroblastoma-like instances .Maturation during chemotherapeutic treatment is a more common finding in other primitive pediatric tumors such as rhabdomyosarcoma and neuroblastoma –13. SincIn conclusion, this case describes the possibility of a Ewing sarcoma to differentiate into neural tissue after neo-adjuvant chemotherapy treatment, the prognostic value of this phenomenon remains questionable.CT, computed tomography; FDG, fluordeoxyglucose; GFAP, glial fibrillary acidic protein; HE, hematoxylin-eosin; MRI, magnetic resonance imaging; Neu-N, neuronal nuclei; NF, neurofilament; PET/CT, positron emission tomography–computed tomography; PNET, primitive neuroectodermal tumor


A 19-year-old Caucasian male presented with complaints of headaches and syncope. Suspicion of hydrocephalus prompted computed tomography (CT) and magnetic resonance imaging (MRI), which revealed pineal and suprasellar prominences with diffuse, thick, nodular subependymal enhancement of the lateral and third ventricles. Based on imaging, the differential diagnosis consisted primarily of malignancy, such as lymphoma, with inflammatory and infectious etiologies not excluded. Cerebrospinal fluid (CSF) samples were non-specific, and neuroendoscopic tissue biopsy histologically confirmed the diagnosis of pure germinoma. The patient was treated with radiation, and follow-up MRIs at one, three, six, and 12 months demonstrated progressive resolution of tumor burden with marked clinical improvement.Germinomas are rare germ cell tumors that are more frequently diagnosed in Asian countries. They uncommonly seed into the lateral ventricles, and only two other cases have been described with diffuse subependymal involvement. Unlike other malignant germ cell tumors, germinomas have marker negative CSF samples that are important in the normal diagnostic workup of diffuse subependymal lesions. Histopathologic correlation is required for definitive diagnosis in the United States and can be achieved with endoscopic tissue sampling. Germinomas are highly radio- and chemotherapy sensitive and have a fair prognosis with modern therapeutic techniques. Germinoma should be considered with simultaneous midline and diffuse ventricular lesions. The World Health Organization (WHO) categorizes central nervous system (CNS) germ cell tumors (GCTs) as non-germinomas and germinomas . While rClinical presentation is variable and dictated by tumor location. A careful history and physical exam detailing any endocrine, circadian rhythm, and cranial nerve abnormalities, as well as symptoms of increased intracranial pressure, is helpful. Germinomas typically present in the pineal 38 - 57%) or suprasellar regions (34 - 49%), less frequently as a double lesion in the pineal and suprasellar regions synchronously (5-10%), and rarely in other locations (3-5%), including the ventricles 8 - 57% o, 23-25. A 19-year-old Caucasian male presented to an outside emergency department with a variety of non-specific symptoms, including severe headaches with resultant emesis and syncopal episodes. A review of systems was otherwise essentially negative for constitutional, neurological, and endocrine symptoms. There was no history of prior meningitis, frequent infections, familial cancers, or high-risk behaviors, such as illicit drug usage. The patient's vitals were stable and extensive lab workup was normal, but fundoscopic examination showed optic pallor bilaterally. A computed tomography scan (CT) was ordered for suspected hydrocephalus, which demonstrated a transependymal fluid shift with enlargement of the lateral and third ventricles with nodular densities prominent in the occipital and anterior horns bilaterally  samples were sent for cytology with the suspicion of an underlying malignancy, such as lymphoma, inflammatory, or infectious etiology. CSF samples showed a non-specific reactive T-cell lymphocytosis but were otherwise unremarkable for tumor and inflammatory markers, including for alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (β-hCG).The patient was then evaluated at a tertiary center where a right frontal endoscopic biopsy was undertaken for definitive diagnosis. Preoperative magnetic resonance imaging (MRI) confirmed extensive subependymal enhancement in a thick, nodular pattern diffusely throughout both lateral ventricles and the pineal region as well as the diffuse involvement of the third ventricle, including the foramen of Monro, suprasellar region, and Sylvian aqueduct, with moderate ventricular and aqueduct dilatation Figure .Tissue samples taken from the right frontal horn of the lateral ventricle were once again negative for AFP and β-hCG but positive for cytoplasmic placental-like alkaline phosphatase (PLAP), confirming the diagnosis of pure CNS germinoma.The patient was treated with 25.4 Gy radiation to the cranial-spinal axis due to the significant intracranial spread, 36 Gy total whole brain radiation, and 50.4 Gy (in 1.8 Gy or fewer fractions) boosts to the prominent midline and ventricular regions. Follow-up MRIs at one, three, six, and 12 months demonstrated diminishing tumor burden with mild residual lateral ventriculomegaly (Figure At his last visit, the patient was asymptomatic with a resolution of his syncopal episodes and headaches.This patient’s imaging characteristics were not typical of any particular lesion; therefore, neoplastic, inflammatory, and infectious etiologies all needed to be considered. For the patient’s age and diffuse subependymal involvement of the lateral and third ventricles, lymphoma and metastasis were the main concern. The differential for masses of the ventricular system is vast, and when including the pineal and suprasellar regions as well, the possibilities are immense -32. GermCNS GCTs are categorized based on secreted tumor markers that are most sensitive and reliably measured in CSF , 35. YolIntracranial germinomas are highly curable tumors with a fair prognosis despite being WHO Grade IV lesions and rapidly fatal, if untreated . RadiatiA review of the English literature demonstrated only 17 other reported cases of a germinoma in the lateral ventricles, with a majority of these cases occurring in Asian countries. However, many of these cases had involvement of the ventricles only Table  23, 41--41-44, aIn conclusion, intracranial germinomas are uncommon tumors that primarily affect adolescent males in Asian countries. We present a rare case in a Western country of a pure germinoma in the pineal and suprasellar regions with the atypical radiographic appearance of diffuse spread to the lateral and third ventricles. Unlike other malignant germ cell tumors, germinomas have marker-negative CSF samples that are important in the normal diagnostic workup of diffuse subependymal lesions. Histopathologic correlation is required for definitive diagnosis in the United States and can be achieved with endoscopic tissue sampling. Germinomas are highly radio- and chemotherapy-sensitive and have a fair prognosis with modern therapeutic techniques. A germinoma should be considered with simultaneous midline and diffuse ventricular lesions.


Endometriosis is ectopic development of endometrial glands and stroma in extra-uterine sites and if the lesions occur as a well-defined mass is referred to as endometrioma. In the literature, endometrioma has been reported in only women and there are no reports of endometrioma in animals, including non-human primates.A rare case of endometrioma is reported in an 11-year-old female German Shepherd with clinical signs of dehydration, anemia and prostration. Necropsy revealed ascites, generalized pallor, and a well-demarcated reddish mass adjacent to the left ovary and uterus and adherent to the retroperitoneum. The mass measured 25.0 × 20.0 cm with intermingled soft and firm areas. Upon incision, the mass was found to be solid with variable sized cystic cavities filled with coagulated blood. Microscopically, the mass was composed of cuboidal or prismatic epithelial cells arranged in tubules or acini. The epithelium of the mass had similar characteristics to the normal endometrium with PAS-positive secretions. The stroma was prominent and formed by loose connective tissue and smooth muscle fibers as confirmed by Masson trichrome. Extensive multifocal areas of hemorrhage were also observed in the stroma of the mass and in the interior of some epithelium-lined, cystic structures. Most of the epithelial cells had strong and diffuse cytokeratin expression, and some had vimentin expression. Epithelial and stromal cells also showed ERβ, AR, VEGF and COX2 expression. The stroma showed areas with strong and diffuse vimentin expression. Factor VIII expression was observed only in the endothelium of blood vessels in the stroma.The macroscopic, microscopic and immunohistochemical findings are consistent with an endometrioma. Endometriosis is ectopic development of endometrial glands and stroma in extra-uterine sites , 2. EndoEndometriosis is a common gynecological disease in women, affecting approximately 10 % of the female population in the reproductive age. In rare cases, endometriosis has been diagnosed in men , 5. In aTwo theories have been proposed to explain the development of endometriosis in women and non-human primates. The retrograde menstruation theory suggests that viable endometrial cells, which are shed during menstruation, are fixed outside of the uterus . The coeThe occurrence of endometriosis in animal species with an estrous cycle is rare. In addition to the lack of shedding of the endometrium, which prevents the development of endometriosis by implantation, these species have minimum levels of estradiol, with only one peak along the estrous cycle . TherefoThe objective of this study is to report the macroscopic, microscopic and immunohistochemical features of an endometrioma in a bitch.An 11-years-old female German Shepherd was admitted to the Veterinary Teaching Hospital of the Universidade Federal de Minas Gerais, Brazil with a history of prostration one month ago. According to the owner, the animal had multiple normal deliveries, never exposed to contraceptives and experienced estrus regularly twice a year. Clinical examination showed mild dehydration, prostration and intensely pale mucosa. However, no other changes were observed as the dog suddenly died during the clinical examination, before laboratory testing was possible.Necropsy revealed intensely pale mucous membranes, generalized atrophy of the skeletal muscles and absence of subcutaneous and visceral adipose tissue. In the abdominal cavity, there were approximately 600 ml of reddish and translucent fluid. Adjacent to the left ovary and uterus and adherent to the retroperitoneum, there was a well-demarcated reddish mass, measuring 25.0 × 20.0 cm Fig.  with a mThe entire genital tract and specimens of the mass and other abdominal and thoracic organs were fixed in 10 % neutral phosphate-buffered formalin, sectioned, processed, embedded in paraffin, and stained with hematoxylin-eosin for histopathological examination. To better characterize the mass, staining with Masson trichrome, Periodic Acid Schiff (PAS) and immunohistochemistry were performed. Antibodies used for immunohistochemistry and their dilutions were as follows: anti-Factor VIII (1:300) , anti-vimentin (1:200) , anti-cytokeratin (1:200) , anti-VEGF (1:100) , anti-COX2 (1:40) , anti-estrogen receptor (ER) β (1:25) , and anti-androgen receptor (AR) (1:500) . The streptavidin-biotin-peroxidase technique was used and antigen retrieval was performed in a water bath at 98 °C using Retrieval solution for anti-Factor VIII, anti-cytokeratin and anti-vimentin antibodies. For anti-ERβ and anti-androgen receptor (AR), antigen retrieval was achieved by microwave. The slides were incubated overnight in a humid chamber with the primary antibody and for 30 min for each of the following steps: blocking endogenous peroxidase, blocking serum  and streptavidin peroxidase . Incubation with the secondary antibody  was performed for 45 min. The chromogen used was diaminobenzidine . Sections were counterstained with Harris hematoxylin. As a positive control for each antibody, the ovary of the animal was used because it expresses all the markers assessed. A negative control was performed by replacing the primary antibodies by IgG.Microscopically, the mass was composed of cuboidal or prismatic epithelial cells arranged in tubules, acini or cystic structures, which were sometimes filled with erythrocytes. The epithelium had a round basal nucleus with loose chromatin and the apex of the cell had a finely particulate PAS-positive material Fig. and b. ThEpithelial cells, especially prismatic cells, formed structures such as endometrial glands and demonstrated strong and diffuse cytokeratin expression Fig.  althoughModerate cystic endometrial hyperplasia with the presence of endometrial glands in the myometrium was observed in the uterus (adenomyosis). The right and left ovaries showed corpus luteum in regression and some follicles at different stages of development. The other organs showed no significant microscopic changes.Clinical and macroscopic findings of anemia may have been derived from extensive bleeding in the endometrial tissue. Cases of ascites associated with endometriosis have also been reported. It is suggested that the occurrence of ascites may be due to the rupture of cysts in the endometrial tissue with extravasation of fluid and red blood cells into the abdominal cavity. Moreover, when the formation of large masses occurs, as in this dog, lymphatic drainage may be impaired with extravasation of fluid from the endometrial tissue or from adjacent organs and tissue , 21–23. Despite the endometrioma, the dog had showed no disturbance of the estrous cycle or fertility as it had delivered normal viable full-term offspring previously. Unlike endometrioma, deep endometriosis is the main presentation that causes pelvic pain and infertility in women , 26. In The occurrence of endometriosis caused by cell implantation is unlikely in dogs because dogs lack the endometrial desquamation observed in women during menses and in non-human primates in the diestrus phase. In dogs, endometrial shedding does not occur and the bleeding during estrus comes from red cell diapedesis from blood vessels. In addition, female dogs have 3–10 months of anestrus characterized by the permanence of serum sex hormones at basal levels. Therefore, we suggest that these differences might favor, at least in part, the occurrence of endometriosis in women . HoweverStudies have demonstrated that estradiol plays an important role in the appearance, perpetuation and proliferative potential of the endometrial tissue , 14, 16.rete ovarii because such tumors have cysts averaged 8.7 cm, a flat, cuboidal, or columnar, usually nonciliated-lining epithelium and can sometimes present a fibromuscular wall [Despite the macroscopic characteristics of the mass described in some cases of endometrioma in women , 32, baslar wall , 34. Howlar wall , 34. Thelar wall , 36. Thelar wall . Howeverlar wall . Moreovelar wall –42.The endometrial tissue found in this bitch was restricted to a mass located adjacent to the uterus and ovary and fixed to the retroperitoneum. The association of the epithelium with a richly vascularized fibrous stroma observed in the present dog has also been described in endometrioma in women , 7, 43. The immunohistochemical characteristics of endometriosis have been widely studied. Similar to what was observed herein, ectopic endometrial cells are positive for cytokeratin  and may Immunohistochemical expression of COX2 and VEGF by endometrial epithelium and stromal cells was also observed in cases of endometriosis in women , 50. It Based on the macroscopic, microscopic and immunohistochemical findings, the diagnosis of endometrioma was confirmed. The occurrence of endometrioma in animal species with an estrous cycle is rare, and this is the first case described in dogs.


An otherwise healthy 55-year-old female, nonsmoker, was seen in pulmonary consultation for progressively worsening shortness of breath. She had undergone a complete hysterectomy 7 years prior for bleeding leiomyomas. On presentation, her initial chest X-ray showed a large right-sided pleural effusion with multiple pulmonary nodules. Two thoracenteses failed to reveal any cytologic abnormalities. Bronchoscopy revealed smooth, round, endobronchial lesions. Histologic examination showed features consistent with leiomyosarcoma. We present a rare case of a patient that initially had possible leiomyomas of the uterus surgically removed and years later presented with bronchopulmonary leiomyosarcoma. We present a case of a 55-year-old female that presented with a 3-week history of shortness of breath, cough, and right-sided pleuritic chest pain. The patient was a nonsmoker and had a significant past medical history of complete hysterectomy 7 years prior for bleeding uterine fibroids. Pathology at that time had shown the fibroids to be leiomyomas. On current presentation, her chest X-ray showed multiple pulmonary nodules with a large right-sided pleural effusion. Subsequently, a computed tomography scan of the chest, abdomen, and pelvis revealed numerous bilateral pulmonary nodules with a large pleural effusion, and near complete collapse of the right middle and right lower lobes . The siz1 Both primary tumors and metastatic disease have been reported.2 If present in the uterus, these lesions can cause excessive uterine bleeding and surgical intervention is needed. Bronchopulmonary leiomyosarcoma can have a very unpredictable clinical course as a primary tumor in the lung, or as a metastatic focus to the lung from another source. Even when surgically removed, metastatic seeding can occur and remain dormant for many years before declaring itself. In even rarer cases, leiomyosarcoma can coexist with benign fibroid tumors called leiomyomas.3 These benign fibroid tumors occur in reproductive-aged females and can cause excessive uterine bleeding sometimes requiring surgical intervention. Metastatic seeding of these tumors is also rare but has been reported in case reports.3 There is some suggestion in the literature that leiomyomas may be a type of low-grade leiomyosarcoma but this point is of great debate.4Bronchopulmonary leiomyosarcoma is a rare entity that can occur in males and females of varying age groups.5 They are characterized by prominent cellular atypia, greater than 10 mitoses per 10 high power fields, and areas of tumor cell necrosis. These 3 characteristics have been deemed the Stanford criteria. The presence of 2 or more of these criteria represents a greater than 10% risk of metastatic spread.6Leiomyosarcoma is a rare entity. Out of all the pulmonary tumors, pulmonary sarcomas comprise 0.5% of lung neoplasms. Leiomyosarcomas are the most common sarcomas of the lung.2 In a case report from 1957, twelve documented cases were quoted.1 In a 2002 case report, 92 cases worldwide were quoted.2 These tumors are initially detected on radiography and commonly arise in the left lower lobe,7 metastasize very late in their course, and spread hematogenously. When leiomyosarcoma metastasizes to the lung, the tumor is usually centrally located and can be often seen in the major bronchi. Grossly, this lesion has been described as a finger-like, fungating projection with little invasion of local lung parenchyma. The tumor itself is white to red in appearance, very friable, and bleeds easily. Depending on the tumor size, it can fill part or all of the bronchial opening.1 According to Naik et al,5 these tumors, unlike epithelial tumors, do not exhibit any exfoliating tendencies and therefore bronchoscopy and washings or even pleural fluid collection may not yield much diagnostic information.Pulmonary leiomyosarcoma case reports are rare in the literature. For every 3000 cases of pulmonary carcinomas, there is 1 case of pulmonary leiomyosarcoma.4 By 2001, there were about 75 documented cases.3 Leiomyosarcomas and leiomyomas are separate entities. Both processes can coexist in the same patient but they exhibit different pathologic and cytogenetic properties.6 In benign metastasizing leiomyoma, pulmonary nodules can be seen 3 months to 20 years after hysterectomy due to its slow and indolent course.3 Histologically, leiomyomas consist of well-differentiated stroma, with a benign appearance of smooth muscle morphology. Some authors have suggested that these lesions represent metastatic low-grade leiomyosarcoma but that point is still of great debate in the literature.4A similar entity, benign metastasizing leiomyoma, was first described in 1939.For this case, it was determined by the tumor board that the lung involvement was part of a metastatic process. This assessment is based on the clinical history, the patient’s previous fibroid history, and the comparison of the pathology slides from 7 years ago and present time. The reason this case was reported is the fact that this particular malignancy can lie dormant, even when surgically removed.In conclusion, bronchopulmonary leiomyosarcoma is very rare, very unpredictable and can clinically declare itself within a few months to many years after surgical resection. In rare cases, it can be present in patients that have been diagnosed with benign leiomyomas of the uterus.The patient’s previous histopathology slides were reviewed and compared with the more recent slides, and although it is not official, there is a possibility that the initial lesion 7 years ago was a low-grade leiomyosarcoma. In review of the original slides 7 years prior, it was decided that the original diagnosis of uterine fibroids was actually leiomyosarcoma. However, there the original tissue samples to confirm this were not available but tissue from the present-day lung was compared with the original slides. The pathologist, oncologist, and rest of the tumor board concluded that the primary was likely the uterine fibroids from 7 years ago due to the similarities seen between them.The patient had a follow-up PET-CT scan that showed stable-sized pulmonary nodules with decreased metabolic activity. The large, dominant mass showed increased necrosis with decreased metabolic activity. There was a smaller, residual pleural effusion with no metabolic uptake and nonspecific mediastinal and retroperitoneal lymphadenopathy also with no metabolic uptake. As of August 2011, the patient continues to have weekly chemotherapy and is slated to have serial PET-CT scans. However, patient did not respond to chemotherapy and subsequently passed away in 2012.


Solid pseudopapillary tumor of the pancreas is extremely rare in children; it usually occurs in young women between 18 and 35 years of age. It comprises less than 3 % of pancreatic tumors. It is of low malignancy; however, it may be locally aggressive. Surgical resection is the treatment of choice and its prognosis is excellent.Two Caucasian girls, 15 and 12 years of age were diagnosed with tumor of the pancreas. The first patient had severe abdominal pain. In the second case the tumor was asymptomatic, detected incidentally during ultrasound. Computed tomography confirmed pancreatic mass. In the first case, apart from the tumor located in the head and the body of her pancreas, focal change in her right kidney was found, which was an indication to biopsy that confirmed solid pseudopapillary tumor. In the second patient the tumor was located in the body of her pancreas, with portal vein occlusion and well-developed collateral circulation. In the first patient a pancreatoduodenectomy (Traverso-Longmire) was performed; there was no mass in her right kidney. In the second case, distal pancreatectomy and splenectomy were performed. In both cases histopathology revealed solid pseudopapillary tumor resected radically. Our first patient’s postoperative course was uneventful. In the second case, her postoperative course was complicated by necrosis of the remaining pancreatic head that needed pancreatoduodenectomy. Follow-up at 28 and 26 months revealed no evidence of tumor recurrence or metastases on magnetic resonance imaging.Typical radiological appearance of solid pseudopapillary tumor is an indication for surgery. The treatment of choice is tumor resection with sparing of pancreatic tissue. In one of our two cases we performed a preoperative biopsy because of an uncharacteristic mass in her right kidney. In our second patient, necrosis of her spared pancreatic head meant that we could not preserve pancreatic tissue. Our whole diagnostic process, treatment and possible complications analysis should be of interest and noteworthy not only to surgeons as the treatment of choice is radical resection, but also to pediatric oncologists because of differentiation from other pancreatic tumors in children. Solid pseudopapillary tumor of the pancreas  is very rarely diagnosed in children. The entity was first described in 1959 by pathologist Virginia Kneeland Frantz and in 1996 reclassified by the World Health Organization (WHO) . It compThe aim of this study is to analyze symptoms, diagnosis, treatment and long-term outcomes of two adolescent girls treated for SPT of the pancreas.3) and C-reactive protein (5.3 mg/dl) were found. An ultrasound (USG) examination and computed tomography (CT) scans showed the presence of a tumor located in the head and the trunk of her pancreas  level (12 × 103 arising from the trunk of her pancreas (Fig. 0 esophageal varices treated endoscopically.A 12-year-old Caucasian girl was admitted to our department after a mass in her pancreas was incidentally revealed during USG. CT scans showed a cystic and solid mass 5.2 × 5.5 × 5.2 cmeas Fig. . The tumeas Fig. , as welleas Fig. . Her posSPT of the pancreas is extremely rare in children. It is usually found incidentally on radiologic examination for other reasons and there are no typical clinical signs and symptoms. In some patients the tumor is noticed because of chronic or acute abdominal pain. SPT is usually localized in the pancreatic head. Exocrine or endocrine insufficiencies have not yet been described. No specific tumor markers are known –9. In onTypical radiological appearance of SPT is an indication for surgery. The treatment of choice in SPT is tumor resection with sparing of pancreatic tissue. We performed a preoperative biopsy in one of our two cases because of suspected additional mass in the right kidney. In the other case, SPT of the trunk and tail of the pancreas associated with portal vein thrombosis and collateral circulation meant that we could not preserve the pancreatic head. Control MRI revealed a small piece of tissue characteristic of the pancreatic tail above the upper pole of the left kidney, which could explain why an insulin supply was not needed during the postoperative course.Written informed consent was obtained from the patients’ legal guardians 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.


Congenital abdominal aortic aneurysm (AAA) is distinctly rare in infants and children and carries a high mortality rate. Our objective was to summarize the experience of the diagnosis and treatment in patients with congenital AAA.Reported cases of congenital AAA published prior to November 8, 2014, were identified through PubMed, EMBASE, Web of Science, and reference lists. All selected cases were evaluated for main clinical characteristics.Twenty-six cases of congenital AAA were identified in the English language literature. Congenital AAA occurred primarily in children under three years old, but it was also found in young adults and fetuses. With regards to the localization, the great majority of congenital AAA was infrarenal AAA. The majority of the AAA patients lacked specific symptoms, and a painless pulsatile abdominal mass was the most common clinical presentation. The diagnosis of AAA was based on ultrasound scanning in twenty-five cases, multi-slice spiral computed tomography angiography (MSCTA) in sixteen cases, and magnetic resonance angiography (MRA) in nine cases. Histopathological analyses were available in seven cases. Seven patients received conservative management. Surgical treatment was performed in seventeen cases, and open repair with an artificial graft was the main surgical intervention. The mortality associated with congenital AAA was high (30.76%). Ruptured aneurysm and renal failure were the main causes of death.Good outcomes can be achieved in children with early identification of congenital AAA and individualized surgical repair with grafts. Abdominal aortic aneurysm (AAA) is the pathologic local dilation of the abdominal aorta and is defined as an aorta size of more than 30 mm or a local dilation of the abdominal aorta greater than 50% compared with another site along the aorta. Despite the evolution of the understanding and treatment of AAA over the past few decades, it continues to be a major threat to health due to the 80% overall mortality in the event of rupture.Acquired AAA is associated with various definitive predisposing factors, including infection  , congeniIn comparison with acquired AAA, congenital AAA has an unknown etiology. Congenital AAA in neonates, infants and children is extremely rare and carries a high mortality rate. To date, fewer than thirty pediatric cases have been reported. Awareness of the diagnosis and advances in diagnostic techniques may allow the diagnosis to be made earlier in the course of disease. Improved operative skills and conservative treatment may significantly decrease the mortality rate. To summarize the experience of the diagnosis and treatment in patients with congenital AAA, a systematic review of cases published in the literature was conducted.All English publications that reported cases of congenital AAA in any population were assessed. A comprehensive search of PubMed, EMBASE, and Web of Science was performed for all relevant papers published prior to November 8, 2014. The search keywords included  OR . Language limits for all searches were set to English. A manual search was performed by checking the reference lists of the original reports and review articles that were retrieved through the electronic searches to identify studies not yet included in the computerized databases.All potentially relevant papers were reviewed independently by the authors. We assessed the eligibility of each article and abstracted data with standardized data abstraction forms. Disagreements were resolved through discussion.First, all non-duplicate publications reporting cases of congenital AAA were selected for review. Altogether, 131 articles were identified from 1967 to November 8, 2014. Case reports and reviews relative to congenital AAA were screened to select eligible articles. Next, all four articles regarding acquired AAA were excluded. Finally, the reference lists of all three previous reviews concerning congenital AAA were reviewed to search for additional eligible articles. Twenty-six articles were considered eligible after this process. The results of the review process are outlined in Figure All selected cases were evaluated for the main clinical characteristics. The following data were collected from all eligible articles: authors, age at discovery, gender, clinical presentation, diagnostic methods, treatment and outcome.Twenty-six cases of congenital AAA were identified in the English language literature -31. The Congenital AAA mainly occurred in neonates and infants (57.69%), but it was also found in young adults and fetuses 42.31%). Of these twenty-six cases, seven were diagnosed prenatally, six were diagnosed as neonates, nine were diagnosed between one month and three years of age, two were diagnosed between three and twelve years of age, one was diagnosed at age nineteen, and one was diagnosed at age thirty. As shown in Table %. Of theWith regards to localization, the great majority of the congenital AAA cases were infrarenal AAA (69.23%). Of the twenty-six cases, eighteen occurred infrarenally, five occurred juxtarenally, two occurred thoracoabdominally, and the remaining case was unspecified. These aneurysms ranged from 1.6–11.0 cm in maximum diameter and extended to one or both of the iliac arteries in the pelvis. In four cases, additional aneurysms were identified in the iliac artery, renal artery, or superior mesenteric artery ,16,20.Clinical presentation varied from a complete lack of symptoms to ruptures. A pulsatile abdominal mass was observed on examination or unexpectedly. Of the twenty-six cases, six were asymptomatic, and three were discovered during surgical procedures to address other diseases. Other presentations included painless pulsatile abdominal mass in seven cases ,16,19,26Imaging examination was the main method for the diagnosis of AAA. The diagnosis of AAA was made by ultrasound scanning in twenty-five cases, MSCTA in sixteen cases, and MRA in nine cases. In one case, an intra-abdominal mass was found in an abdominal X-ray plain film, but AAA was only confirmed during the operation. Histopathological analyses were available in seven cases ,14-17,19Seven patients received conservative management because of extensive aneurysms, small vessel size, and hemodynamic instability. Seventeen patients underwent surgical treatment (65.38%). The timing of these repairs ranged from the neonatal period to twelve years of age. Aneurysmorrhaphy was used in two cases, and good results were noted three months after the operation in one case. Aortobiliac reconstruction using native vessels was performed in one case. In nine cases, the aneurysms were successfully reconstructed with an artificial graft. In three cases, they were reconstructed with an allograft. The maximum time of follow-up in these cases was fourteen months. It was still impossible to assess the value of this surgical repair because there was a limited amount of information regarding the patency of the graft, necessary reoperation, or long-term survival of the patients.The mortality of congenital AAA was high (30.76%). Ruptured aneurysm and renal failure were the main causes of death. Offering timely elective repair remains the most reliable strategy for preventing death. Of the seven patients who underwent conservative management, one patient died of acute pulmonary hypertension and cardiac dysfunction, one died of cardiac and renal failure, one died of rupture, one died of thrombosis of the aneurysm and renal dysfunction at three years, one died of renal failure, and the remaining two patients were stable after six to seven months of follow-up. Of the seventeen surgical patients, eleven underwent successful repair and had uneventful recoveries, two newborns did not survive operative intervention, three had unknown outcomes, and one died of sepsis and cardiopulmonary insufficiency.According to the Hamburg Classification of Congenital Vascular Malformations , congeniThe etiology of congenital AAA may be associated with a molecular genetic defect. The transforming growth factor-β (TGF-β) signaling pathway controls cellular proliferation, growth and differentiation and regulates several functions of the connective tissue. Disruption of genes coding for components of the TGF-β signaling pathway or its interactors, such as fibrillin-1, have recently been described in patients with aortic aneurysm. Marfan’s syndrome and Loeys-Dietz syndrome represent rare congenital connective tissue disorders that have similar patterns of cardiovascular defects ; these syndromes are caused by mutations in the genes encoding for TGF-β2 or TGF-β receptor (TGFBR) I or II -36. ThesThere is no specificity in the clinical manifestation of congenital AAA. The clinical presentation of congenital AAA varies considerably according to the vessel affected. In the present review, many cases presented with a painless pulsatile abdominal mass, which drew much attention. As an AAA expands, it may become painful. Renovascular hypertension and renal failure should be highly concerning. These presentations were found to be the result of renal aneurysms, renal artery thrombosis or renal dysplasia.Congenital AAA can usually be diagnosed with specific imaging modalities. The primary cause of misdiagnosis is doctors not considering the possibility of an AAA. Once a clinical presentation such as abdominal pulsatile mass, abdominal vascular murmur, or tremors is detected, AAA should be considered. Relevant imaging such as ultrasonography, MSCTA and MRA should be performed to identify AAA. Ultrasonography can be used to screen for aneurysms and to determine their size. MSCTA scanning has approximately 100% sensitivity for aneurysms and is useful for preoperative planning because it can detail the anatomy and the possibility of endovascular repair. In the case of suspected rupture, it can also reliably detect retroperitoneal fluid. The present review also indicates that additional aneurysms and concomitant diseases are common. These conditions are occasionally critical for determining the optimal time for surgical repair. Thus, comprehensive evaluation with multimodality imaging is recommended for investigating patients with congenital AAA.Of course, it is very important to note that various cases of acquired AAA are far more frequent than cases of congenital AAA. The secondary causes, including congenital connective tissue disorder , must be ruled out for a definitive diagnosis of congenital AAA. A recent study also suggested that some patients diagnosed with congenital AAA might be suffering from variants of Ehlers-Danlos syndrome, with the most likely variant being type IV . This coCurrent therapeutic decisions are largely extrapolated from the adult literature, and there is no universal approach to the management of congenital AAA. The treatment options include conservative management, surveillance leading to eventual repair and surgical repair.The conservative management of AAA has, to a large degree, been ignored until recently. Conservative management aims to prevent AAA progression and rupture, reduce the long-term requirement for surgery and limit cardiovascular events, and provide an important supplement to surgical treatment. Conservative management is indicated for patients for whom surgical repair carries a high risk of mortality and is unlikely to improve life expectancy . In the The conservative measures include ultrasound follow-up and internal medical therapy such as statins, beta-receptor blockers, matrix metalloproteinase inhibition, angiotensin converting enzyme inhibitors (ACEI), anti-platelet drugs, and non-steroidal anti-inflammatory drugs (NSAIDs). Hypertension must be controlled because it may exacerbate the stress on the aortic wall, and antihypertensive therapy can decrease this stress . This stThere are currently two modes of repair available for an AAA: open repair and endovascular aneurysm repair (EVAR). However, treatment of congenital AAA in a neonate, infant, or young child requires additional considerations. Although experience with EVAR in adults has been increasing rapidly , there aAneurysmorrhaphy increases the risk of recurrence because it maintains the dysplastic aortic segment. In the present review, aneurysmorrhaphy was only used in two cases. It was reported that an artificial graft was an appropriate vascular substitute in children and did not impact their growth and development or their quality of life ,46. HoweThe use of a standard cryopreserved allograft is not recommended because they induce an immunologic response that has been linked to a high incidence of fibrosis, calcification, and degradation. Recently, a new generation of decellularized cryopreserved allograft with reduced immunogenicity has been introduced for the treatment of congenital heart disorders . These gIn the present review, reconstruction of AAA was achieved using native vessels without exogenous material in a 10-day-old boy . This meThe cause of congenital AAA remains unclear, and little is known about the molecular pathways leading to AAA formation. In the future, the genetics of AAA, the mode of inheritance, the candidate gene approach and linkage analysis may illuminate the genetic background of congenital AAA.Good outcomes can be achieved in children with early identification of congenital AAA and individualized surgical repair with grafts. Once suggestive clinical presentation such as pulsatile abdominal mass are observed, multimodality imaging such as ultrasonography and MSCTA should immediately be performed to confirm the diagnosis of AAA. When AAA is encountered, an exhaustive search for the underlying factors should be conducted to exclude acquired AAA. Management strategies should be highly individualized, and early individualized surgical repair with grafts is advocated for the treatment of congenital AAA.


Desmoplastic small round cell tumor (DSRCT) is an uncommon and highly aggressive malignancy with undetermined histogenesis and poor prognosis. To date, no case of testicular DSRCT has been reported in the literature.A 42-year-old Chinese man presented with painless swelling of his left testis and a painless palpable nodule in his left inguinal region. Computed tomography showed a solid mass in the left testis and multiple metastases in the body. Laboratory tests gave no abnormal results. Left radical orchiectomy was performed, and histopathological and molecular pathological examination showed typical features of DSRCT. Six cycles of chemotherapy were administrated after the operation, leading to partial remission. Postoperative 9-month follow-up indicated no progression. Desmoplastic small round cell tumor (DSRCT) is an extremely rare malignancy with undetermined histogenic origin. To date, about 300 cases have been reported in the literature, since it was first described as a mesenchymal entity with distinct clinicopathological features by Gerald and Rosai in 1989. DSRCT iA 42-year-old Chinese man was admitted to the Department of Urology at our hospital, with an 8-month history of painless swelling of his left testis and a 6-month history of a painless palpable nodule in his left inguinal region. He had no relevant personal or family history of malignancy.On physical examination, an egg-size solid lump was found in the left testis, and a palpable, slightly mobile, solid nodule, 2 × 3 cm in size, in the left inguinal region. Laboratory tests, including α-fetoprotein, β-human chorionic gonadotropin, and lactate dehydrogenase, gave no abnormal results. Computed tomography (CT) showed a confined solid mass, 2 × 3 cm in size with homogenous attenuation in the left testis, and multiple lesions in the right diaphragmatic crus, retroperitoneal region, and peritoneal and pelvic cavity, in the left inguinal region, and in the near-bilateral iliac vessels, suggestive of multiple metastases Figure . In addi3 N2 M1 S0).The preoperative diagnosis was left testicular tumor with multiple metastases. Left radical orchiectomy was carried out, and the cut surface of the tumor showed fish-like changes. Histopathological examination of the resected specimen revealed a malignant tumor composed of well-defined nests of small round blue cells, which were separated by abundant desmoplastic stroma Figure . The spe2, day 1) and cyclophosphamide . After six cycles of chemotherapy, the patient was appraised as being in partial remission. Postoperative 9-month follow-up indicated no progression as measured by CT assessment , adriamycin  and a palpable abdominal mass, are the main problems leading to patients visiting a doctor. If present in extraperitoneal sites, DSRCT can cause specific symptoms, mostly owing to expansion and compression of the tumor. Weight loss, acratia, ascites, and cachexia may occur when there is tumor dissemination or extensive lymphogenous and hematological metastases. In our case, the patient was in good general condition when he visited his doctor, but multiple metastases had already developed. The tumor features, such as non-specific manifestations and high aggression, meant that the patient was already experiencing late-stage disease at his first visit.et al. evaluated the role of fluorodeoxyglucose PET/CT for diagnosis of DSRCT, and found an accurate detection of 97.4% of all DSRCTs, with sensitivity, specificity, positive predictive value, and negative predictive value of 96.1%, 98.6%, 98.0%, and 97.1%, respectively[Imaging methods can help to detect DSRCT, although there are no typical characteristics. CT scans show homogenous low attenuation with modest uniform enhancement on contrast-enhanced CT in small masses, while central low attenuation with modest heterogeneous enhancement on post-contrast CT, suggestive of central hemorrhage or necrosis, may be observed in cases of larger masses. In addiectively. Recentlectively.Microscopically, DSRCT consists of well-defined nests of small round blue cells with hyperchromatic nuclei and indistinct nucleoli, which are separated by affluent desmoplastic stroma. Peripheral palisading of tumor cells and rosette-like structures may be observed,16. ImmuIn general, DSRCT must be distinguished from other small round cell tumors, such as EWS/PNET, neuroblastoma, and lymphoma. Similarly to DSRCT, EWS/PNET is also composed of small round cells in sheets or nests. Typically, EWS/PNET is positive for CD99 and vimentin, and negative for cytokeratins and myogenic markers. Different from DSRCT, which has a translocation of t , EWS/PNET has a characteristic reciprocal chromosomal translocation of tq24;q12). Neurobl;q12. NeCurrently, no consensus has been made as to the treatment protocols for DSRCT. Debulkiet al., and another study group reported that 71% of patients died within a mean of 25.2 months[Generally speaking, the prognosis of DSRCT is poor. A median progression-free survival of 2.6 years was reported by Schwarz 2 months,31. BecaDSRCT is a relatively uncommon and highly aggressive tumor, with poor prognosis. The diagnosis is usually established by immunohistochemistry. To date, no standard treatment protocol has been proposed. More investigations are warranted to define the optimal therapeutic strategy.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.in situ hybridization; HIPEC: Hyperthermic intraperitoneal chemotherapy; PET: Positron emission tomography; PNET: primitive neuroectodermal tumor; WT-1: Wilm’s tumor1.DSRCT: Desmoplastic small round cell tumor; EWS: Ewing sarcoma; CT: Computed tomography; FISH: Fluorescent The authors declare that they have no competing interests.GMZ and YZ conceived of the concept, and participated in drafting the manuscript. HLG reviewed the pathological slides and revised the manuscript. DWY supervised the project and revised the manuscript. All the authors read and approved the final version and agreed to publish the manuscript.