This report describes a case of spontaneous fibroblastic osteosarcoma in the humerus of a lion from a private park in Perugia, Italy. The tumor had an irregular, smooth, brown surface and a generally firm, rubbery consistence with gritty to hard areas interspersed. The mass was poorly vascularized with areas of necrosis at the periphery. The cut surface showed a multilobulated mass that had breached the humeral cortex, with periosteal production of reactive bone. The mass invaded the epiphysis, the synovial membrane, the joint capsule and ligaments. A mild hemorrhagic effusion appeared in the joint space. Clinical signs, gross and histopathologic findings are described in this rare case of a malignant bone tumor. Fibroblastic osteosarcoma is an aggressive tumor, which is frequently histologically indistinguishable from the conventional fibrosarcoma . Reports of neoplasms in Panthera species are still rare as neoplasms are uncommon causes of disease and death in captive wild felids. The presence of primary tumors in large felids is rarely reported, and there are few documented cases in the literature.Osteosarcoma is the most common primary malignant bone tumor in animals and humans  in a zoological park became slightly lame in the right thoracic limb in March of 2011. The lioness was vaccinated against feline parvovirus, feline herpes virus type 1 and feline calicivirus. Clinical examination revealed a large, firm mass growing in the proximal third of the right humerus. The severity of the lameness increased over the course of several weeks and the animal was anesthetized with medetomidine (40 mg/kg) and ketamine (3 mg/kg), administered into the muscles of the hindquarters using a blowgun from a distance of about 10 meters. The animal became recumbent in about 10 minutes and was later intubated. Anesthesia was maintained with isoflurane in 100% oxygen.A16 year old female lion  for the cytological evaluation of the mass. Radiographs of the limb showed an osteolytic sclerosing lesion with irregular rim, mild periosteal reaction and irregular cortex, intramedullary extension and pathologic fracture of the metaphyseal area, severe invasion and complete detachment of the right humeral epiphysis .The radiographic examination of the thorax revealed no abnormalities. FNA from the mass yielded a moderately cellular sample composed of loosely cohesive groups or single spindle cells with basophilic cytoplasmic tails, oval nuclei with central prominent multiple nucleoli and mild anysokariosis .Based on these clinical, radiological and cytopathological findings the mass was diagnosed to be a primary malignant bone tumor, and the animal was euthanized and a complete necropsy performed. The tumor was 26 × 12 × 10 cm, with an irregular smooth, brown surface and a generally firm rubbery consistence with gritty to hard areas interspersed. The mass was poorly vascularized with peripheral necrotic areas. The cut surface showed a multilobulated mass that had breached the humeral cortex, with periosteal production of reactive bone. The mass invaded the epiphysis, the synovial membrane, the joint capsule and ligaments. A mild hemorrhagic effusion appeared in the joint space while the other organs were not affected. No metastases were detected during necropsy.Histological examination showed malignant mesenchymal cells producing osteoid and woven bone. There was extensive osteoblastic differentiation associated with the production of collagen, as well as a population of fibroblastic-like cells associated with diffuse activation of osteoclastic giant cells. Neoplastic cells were large, pleomorphic and had hyperchromatic nuclei and prominent nucleoli. Where the tumor was more intensely osteogenic and sclerotic, the cells were rarer, and were small, with slender nuclei, dense chromatin and no mitoses .There was a diffuse and moderate infiltration of inflammatory cells including lymphocytes and monocytes, with fibroblasts arranged in a storiform pattern. Clinical and radiological findings including the growth rate of the lesion, and the histopathological appearance supported the diagnosis of fibroblastic osteosarcoma. This appears to be the first reported case of fibroblastic osteosarcoma in a lion.According to WHO classification, osteosarcoma can be classified according to its site of origin in either the intramedullary, surface/juxtacortical or intracortical bone, either with epiphyseal, methapyseal or diaphyseal localization. Location may have a prognostic significance. In this case report it was not possible to identify the exact site of origin of the tumor. At the time of necropsy, it involved epiphyseal, methapyseal, and diaphyseal areas of the humerus and extended from the periostium and cortical bone to the medullary cavity.The oncogenic mechanism of malignant bone tumors in domestic animals is controversial, and the biomolecular modifications involved in the development of the tumors are poorly understood. Rapid bone growth appears to predispose the animals to osteosarcoma, as suggested by the increased incidence during the adolescent growth spurt, the high incidence among large-breed dogs and osteosarcoma’s typical location in the metaphyseal area adjacent to the growth plate (physis) of long bones.et al., 1987; Helm and Morris, 2012), especially in dogs, the risk of osteosarcoma increases with age and with increasing body weight . The highest risk of osteosarcoma is found in large and giant breed dogs, and the incidence is lower in smaller breeds .The only known environmental risk factor is exposure to radiation . There was no history of exposure to radiation in the animal investigated here, nor was there any indication of a genetic predisposition like bone dysplasias, fibrous dysplasia, enchondromatosis, multiple exostoses or mutations to RB gene or to p53. In domestic animals (Bitetto Neoplasms of the bone are rare in wild felids, and the unusual subtype of the tumor makes this case even rarer. This case also confirms that the diagnosis of the specific subtype of osteosarcoma can only be reached by a multidisciplinary approach including radiology, pathology and clinical examinations.


Pancreatic paragangliomas are rare tumors, with only 16 reported cases to date. One of these cases demonstrates metastasis to lymph node, while another case was functional, however, none of these cases showed malignant and large, pancreatic paraganglioma with marked invasion. Also another unique feature was the age of our patient compared to the average reported ages in published literature (42–85 years).131-metaiodobenzylguanidine (MIBG). Follow-ups showed the absence of recurrence, and she has remained disease free.A 19-year-old woman presented with a one-year history of intermittent abdominal pain. Physical examination showed a palpable mass in the right upper abdomen, but initial laboratory results were within normal ranges; tumor markers  were negative. An abdominal and pelvic computed tomography (CT) scan showed a well-defined retroperitoneal para-aortic mass. The CT scan revealed that the surrounding lymph nodes were not enlarged, but the liver showed evidence of parenchymal infiltration. Intraoperatively, a large, firm tumor originating from the head of pancreas was found pushing on the caudate hepatic lobe and the inferior vena cava (IVC). The tumor was resected through a pancreaticoduodenectomy, involving segment VI of the liver and a small segment of the IVC. The blood pressure spiked (>220 mm Hg) when the tumor was manipulated during the operation. The final pathology report showed a 9-cm tumor with lymphovascular invasions; immunohistochemistry was positive for synaptophysin and chromogranin. All resection margins were negative and 1/15 lymph nodes was positive for metastasis. Post-operative recovery was unremarkable. One month after discharge, the patient was re-admitted with abdominal pain and found to have an abdominal collection at the resection site, which was drained under CT guidance. She received a therapeutic dose of IThis patient was an extraordinary example of a rare tumor. Even more remarkable was that the tumor was malignant with lymph node invasion. To our knowledge, a case similar to that presented here has not been previously reported in the literature. Paragangliomas are rare tumors of neural crest origin, with a malignancy rate of approximately 10% and a 5-year survival rate of <50%. However, in the absence of metastasis, the tumors are considered benign. The aveA 19-year-old woman presented in March 2010 with vague, recurrent upper abdominal pain. The pain had been occurring for approximately one year and had increased in frequency and severity. A physical examination revealed the presence of a large, palpable firm mass, which was slightly mobile, with an irregular outline, in the right upper abdomen. There were no other remarkable findings. An initial laboratory investigation returned results that were within normal ranges, while the tumor markers CEA, AFP, and CA19-9 were not elevated. An abdomino-pelvic computed tomography (CT) scan  and it included segment VI of the liver and a small segment of the IVC. During the operation, the patient’s blood pressure spiked (>220 mm Hg) when the tumor was manipulated. The increase in blood pressure was not transient and it could only be controlled through a continuous infusion of a combination of alpha and beta blockers, in addition to nitroglycerine, until the tumor was resected.The final pathology report described a 9-cm tumor, composed of well-defined nests of cuboidal cells separated by vascular fibrous septa, having a classic zellballen appearance, with lymphovascular invasion; immunohistochemistry was positive for synaptophysin and chromogranin. All the resection margins were negative, and only one of the 15 lymph nodes showing evidence of metastasis Figure A-D. Hist131-metaiodobenzylguanidine (MIBG). A subsequent total body scan revealed the absence of other associated neuroendocrine tumors and no evidence of multicentricity. Repeated follow-up MIBG scans were done, without evidence of recurrence. The patient has been disease free for >36 months.Although the patient’s post-operative recovery was unremarkable, she was re-admitted with recurrent abdominal pain one month after being discharged. Further examination revealed an abdominal collection at the site of the resection. The site was drained under CT guidance and the patient received a therapeutic dose of IWe report here a case of pancreatic paraganglioma, was identified through CT scan and histological evidences. We ruled out that the tumor was retroperitoneal because it was not attached to any structure and it fully mobilized with the head of the pancreas on kocherization, as a single mass. In addition, pathological findings showed that the tumor was very infiltrative and involved the entire pancreatic tissue. On the contrary, the tumor did not seem to originate from the liver because it covered only a small part of the liver as seen on the CT scan. It did not infiltrate into the liver tissue and was only pushing onto the boundary of the liver as seen on the pathology slide, which most likely means that it did not originate from the liver.Pancreatic paragangliomas are extremely rare, with only 16 cases having been reported in the literature. Whether this tumor type is an extension of a retroperitoneal tumor of true visceral origin, derived from ectopic paraganglia, remains unknown. The meaThe CT findings of pancreatic paragangliomas are different from those of pancreatic carcinomas. The latter appear as poorly enhanced masses with dilation of the bile and pancreatic ducts, as well as retroperitoneal involvement. In contrast, pancreatic paragangliomas, even when located at the head of pancreas, are not usually associated with biliary duct dilation and the patients are not usually jaundiced, which aids in differentiating pancreatic paraganglioma from pancreatic carcinoma,7. In thPancreatic paragangliomas have been reported in multiple locations throughout the pancreas, including the head (about two thirds of the reported cases), body, and tail,2,8. In The pathology of these tumors is based on careful gross examinations of the specimens and histopathological studies based on hematoxylin-eosin sections. In addition, immunohistochemistry usually reveals these tumors to be positive for synaptophysin and chromogranin,11, as iKimura et al. introduced a scoring system to predict the prognosis of pheochromocytomas and other extra-adrenal sympathetic paragangliomas. This scoring system depends on the tumor characteristics, including the tumor pattern , increased cellularity vascular invasion, coagulation necrosis, high proliferation index, and the type of catecholamine secretion. The sco131-MIBG radiotherapy being the treatment of choice. Chemotherapy is reserved for more advanced disease stages. Even in advanced cases, recent genetic studies have suggested that the use of targeted therapy may be useful, but additional validation is still required[131-MIBG one month after the operation, and remains disease-free after more than 36 months.Although most paragangliomas are solitary and typically arise sporadically, they can be multicentric or hereditary. Therefore, a screen for additional tumors is necessary since they may be considered as part of multiple endocrine neoplasms,2,13. Inrequired. In the In conclusion, pancreatic paragangliomas are rare tumors, with only 16 cases reported to date. The patient in the present case was the youngest among the reported cases and the tumor was the largest reported. In addition, this is the second case with lymph node metastasis ever to be reported.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.CT: Computed tomography; IVC: Inferior vena cava; MIBG: Metaiodobenzylguanidine.The authors declared that they have no competing interests.BA: The treating physician who supervised the entire case and wrote the paper. YA: The resident responsible for following up with the patient and collecting the data and images. In addition, he contributed substantially in reviewing published cases and writing the paper. SK: Assisted in the surgery and followed up the patient pre- and post-operatively. She also contributed in writing the paper and doing literature review. MH: The pathologist who worked on the slides and generated the diagnosis. MH: Wrote the paper. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2407/13/486/prepub


The incidence of brain metastases for neuroendocrine tumor (NET) is reportedly 1.5~5%, and the origin is usually pulmonary. A 77-year-old man presented to our hospital with headache and disturbance of specific skilled motor activities. Computed tomography (CT) showed a massive neoplastic lesion originating in the left temporal and parietal lobes that caused a mass edematous effect. Grossly, total resection of the tumor was achieved. Histological examination revealed much nuclear atypia and mitotic figures. Staining for CD56, chromogranin A, and synaptophysin was positive, indicating NET. The MIB-1 index was 37%. Histopathologically, the tumor was diagnosed as NET. After surgery, gastroscopy and colonoscopy were performed, but the origin was not seen. After discharge, CT and FDG-PET (fluoro-2-deoxy-d-glucose positron emission tomography) were performed every 3 months. Two years later we have not determined the origin of the tumor. It is possible that the brain is the primary site of this NET. To our knowledge, this is the first reported case of this phenomenon. Neuroendocrine tumors (NETs) are neoplasms derived from nerve and endocrine cells. NETs have the ability to produce hormones and have similarities with nerve cells, such as cytoplasmic granules and exocytotic machinery. NETs were called “carcinoid” 100 years ago and were considered benign neoplasms. Currently they are considered to be malignant, and the WHO histopathological classification eliminated the “carcinoid” label in 2000. In the WHO classification of 2010, NETs were defined as neuroendocrine neoplasms and were classified as NET G1, NET G2, NEC , mixed adenoneuroendocrine carcinoma (MANEC), hyperplastic, and preneoplastic lesions, by the Ki-67 index . NETs arβE12 and E-cadherin were negative. Considering the pathology and immunostaining results, a high-grade endocrine carcinoma was thought feasible. Grossly, total resection of the tumor was achieved; this was confirmed by postoperative enhanced CT. The mass effect had decreased, and CT showed a small amount of hemorrhage caused by the operation. After surgery, imaging by gastroscopy and colonoscopy was performed, but a tumor origin was not detected. Also, ultrasonography could not point out the thyroid lesions. Postoperative whole brain radiation therapy (30 Gy) was performed from Day 26 to Day 42. The patient had just a little motor aphasia after surgery and was discharged on Day 57. After discharge from the hospital, CT and FDG-PET (fluoro-2-deoxy-d-glucose positron emission tomography) were performed every 3 months, but a tumor origin outside the brain was not identified. On Day 270, an MRI revealed a small local recurrence with ring enhancement in the left parietal lobe near the operative site. Therefore a second surgical procedure was performed with neuronavigation and use of 5-ALA. We resected broadly, as is done for a gyrectomy. But on Day 570, MRI again revealed the small local recurrence, so gamma knife therapy was performed. From then on, until the writing of this paper (Day 720), the patient has remained free from relapse.A 77-year-old man presented with headache and disturbance of skilled motor activities. The past medical history included hypertension, diabetes, and chronic idiopathic pericardial effusion. On physical examination, the Glasgow Coma Scale (GCS) score was 14 (E4V4M6), and finger agnosia and left-right disorientation were noted, like in the Gerstmann syndrome. Blood and CSF analyses were normal. Levels of tumor markers, including CEA, CA19-9, NSE, AFP, and IL-2R, were normal. Head computed tomography (CT) showed a massive neoplastic lesion of 6.6 cm in diameter originating in the left temporal and parietal lobes that caused a mass edematous effect. That lesion included a high-density area in the dorsal aspect that indicated hemorrhage . EnhanceGenerally, the majority of NET metastases occur in the liver, lungs, and bone, and involvement of other sites is much rarer. NETs are considered to be the origin of brain metastases in 1.5~5% of all patients with brain metastases, and in 45–71% of these patients, the primary tumor was located in the bronchi or lungs. If brain metastases are present, lymph node metastases are found in 75% and liver metastases are found in 50% of these patients . PrimaryTo our knowledge, there is only one previous report of a possible primary brain NET lesion . The patThis is the first reported case of a primary NET arising in brain.It is possible that aberrant ectopic epithelial cells are found in the brain.


Paraesophageal hernia with intrathoracic mesentericoaxial type of gastric volvulus is a rare clinical entity. The rotation occurs because of the idiopathic relaxation of the gastric ligaments and ascent of the stomach adjacent to the oesophagus through the hiatus defect, while the gastroesophageal junction remains in the abdomen. The open approach remains the gold standard therapy for most patients. Here we report the case of a patient with such a condition who underwent a successful laparoscopic surgery. A literature search revealed that this is the first case report from Southeast Asia.A 55-year-old Chinese woman presented to us with symptoms suggestive of gastric outlet obstruction for one year. A chest radiograph showed an air bubble with air-fluid level in her left thoracic cavity, where a diaphragmatic hernia was initially suspected. A computed tomography scan and barium swallow study demonstrated the presence of a type III paraesophageal hernia with intrathoracic upside-down stomach. A laparoscopy was performed and the herniated stomach was successfully reduced into the abdomen. The mediastinal part of the hernial sac was excised. Adequate intraabdominal length of oesophagus was achieved after resection of the sac and circumferential oesophageal dissection. A lateral releasing incision was made adjacent to the right crus to facilitate crural closure. The diaphragmatic defect and the hiatal closure were covered with a composite mesh. A Toupet fundoplication was performed to recreate the antireflux valve. She had an uneventful recovery. She had no relapse of previous symptoms at her six-month follow-up assessment.Laparoscopic repair of such a condition can be accomplished successfully and safely when it is performed with meticulous attention to the details of the surgical technique. Gastric volvulus is classified into three types according to the axis of rotation: organoaxial (rotation around the long axis connecting the cardia and the pylorus); mesentericoaxial (rotation around the short axis connecting the lesser and greater curvatures) and combined (rotation around both the short and long axis). The mesentericoaxial type, as in our case, is a less common variant, occurring in 29% of cases . The comA 55-year-old Chinese woman presented with a history of left hypochondrial pain for a duration of one year, associated with early satiety and postprandial vomiting. She did not report any reflux symptoms. Her physical examination did not reveal any significant abnormality. An upper endoscopy did not show any evidence of reflux oesophagitis, except for a severely deformed stomach with difficult duodenal intubation. However, a chest radiograph showed an air bubble with air-fluid level in her left thoracic cavity, and a diaphragmatic hernia was initially suspected. A computed tomography (CT) scan of her abdomen and a barium swallow study Figure a confirmThe surgery was performed with her in a modified lithotomy position, under general anaesthesia. The surgeon stood between her legs (the French position), with the camera surgeon at her right side and the assistant at her left side. Five trocars were used Figure : one supInitially, the herniated stomach was reduced using atraumatic graspers  Figure a. GentleThe redundant portion of the sac was excised with an ultrasonic scalpel  taking care to avoid injury to the oesophageal and stomach wall. A wide hiatus was visualized after the dissection Figure d, makingPostoperatively, she was started on oral liquids on the evening of the surgery and she rapidly progressed to a soft diet, remaining on this diet for four weeks. She was discharged on postoperative day one. A barium swallow study at one month after surgery showed a normally located stomach Figure b. At a fPEH is an uncommon condition, with a reported incidence of 5% out of all hiatal hernias. It is classified as one of three types: type II (true PEH), type III  or type IV (contains viscera other than the stomach). Approximately 90% are type III and the other 10% are type II or IV . Type IIOur patient’s diagnosis was suspected from the finding of a retrocardiac air bubble with or without air-fluid level on the lateral view of a chest radiograph. The differential diagnoses to be considered included diaphragmatic and PEH. A CT scan of the chest and abdomen can provide information on the type of hernia. A barium swallow test can confirm the type of PEH, and provides information on rotation of the stomach, as in our case. An upper endoscopy is mandatory to exclude the presence of oesophagitis or Barrett’s oesophagus that may have resulted in a shortened oesophagus. However, the procedure may be complicated by difficult intubation of the duodenum due to the malrotation of the stomach.et al., in a pooled analysis of several studies, estimated that the annual risk of PEH developing acute symptoms requiring surgery in such group of patients is 1.1% [Elective repair is generally indicated for symptomatic PEH in otherwise fit patients with large hiatal hernias ,5. It isThe management of PEH has evolved over the last decade, from open thoracic to open abdominal and finally laparoscopic transabdominal approaches. The paradigm shift in treatment is attributed to the development and refinement of laparoscopic techniques for anti-reflux surgery and improvement in modern laparoscopic equipment and energy devices. Since the first description of the laparoscopic approach for large PEH in 1992 , it has The key issues surrounding the laparoscopic repair of PEH are: (1) shortened oesophagus, (2) the need to excise the mediastinal hernia sac, (3) the method of crural closure and (4) the need to perform a gastropexy.Shortened oesophagus is a real and important clinical entity in patients with PEH, with a reported incidence of 11% . It is tThe resection of mediastinal sac achieves several other objectives. Firstly, it allows for the descent of the oesophagus into its normal position. Secondly, it eliminates the serous membrane lining the cavity of the mediastinum, thus reducing the risk of seroma. Thirdly, it eliminates the traction on the stomach that may cause recurrence of intrathoracic hernia . Our pat2 to decrease the risk of hernial recurrence [The use of meshes in hiatal repair is controversial because of the concern of mesh-related complications. A mesh is used to reinforce the crural repair because it has been observed that failure of the crural closure, occurring in 5.7 to 11% of patients, is the main reason for postoperative intrathoracic migration of the Nissen fundic wrap following laparoscopic antireflux surgery . The riscurrence ,12. Anotcurrence .While there are concerns of mesh-related complications, most authors did not report such untoward complications ,12,13. OAn antireflux procedure was performed as part of the repair as it has been shown that most patients with a PEH have evidence of reflux on pH monitoring , and faiThe role of an anterior gastropexy to prevent intraabdominal gastric volvulus remains controversial. In our case, we did not perform gastropexy, as the Toupet fundoplication with its wrap anchored to the oesophagus and right crus will inevitably prevent any recurrent volvulus.Our case highlights a successful laparoscopic approach to PEH with intrathoracic gastric volvulus and documents the use of a lateral releasing incision to facilitate crural closure and the placement of a mesh to reinforce a diaphragmatic defect and hiatal closure. However, the routine use of these two techniques as part of a PEH repair need to be examined in a large prospective randomized study with a long-term follow-up period.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Segmental jejunum resection showed two adjacent NETs, measuring 2.0 and 0.5 cm with a peritoneal metastasis. The largest tumor expressed ACTH in 30% of cells. Following surgery, after a transient adrenal insufficiency, ACTH and cortisol levels returned to normal values and remain normal over a follow-up of 26 months. Small mid-gut NETs are difficult to localize on CT or MRI, and require metabolic imaging. Owing to low mitotic activity, NETs are generally poor candidates for FDG-PET, whereas SSRS shows poor sensitivity in EAS due to intrinsically low tumor concentration of type-2 somatostatin receptors (SST2) or to receptor down regulation by excess cortisol. However, F-DOPA-PET, which is related to amine precursor uptake by NETs, has been reported to have high positive predictive value for occult EAS despite low sensitivity, and constitutes a useful alternative to more conventional methods of tumor localization.Ectopic ACTH Cushing's syndrome (EAS) is often caused by neuroendocrine tumors (NETs) of lungs, pancreas, thymus, and other less frequent locations. Localizing the source of ACTH can be challenging. A 64-year-old man presented with rapidly progressing fatigue, muscular weakness, and dyspnea. He was in poor condition and showed facial redness, proximal amyotrophy, and bruises. Laboratory disclosed hypokalemia, metabolic alkalosis, and markedly elevated ACTH and cortisol levels. Pituitary was normal on magnetic resonance imaging (MRI), and bilateral inferior petrosal sinus blood sampling with corticotropin-releasing hormone stimulation showed no significant central-to-periphery gradient of ACTH. Head and neck, thoracic and abdominal computerized tomography (CT), MRI, somatostatin receptor scintigraphy (SSRS), and Uncontrolled high cortisol levels in EAS can be lethal if untreated.Surgical excision is the keystone of NETs treatment, thus tumor localization is crucial.Most cases of EAS are caused by NETs, which are located mainly in the lungs. However, small gut NETs are elusive to conventional imaging and require metabolic imaging for detection.FDG-PET, based on tumor high metabolic rate, may not detect NETs that have low mitotic activity. SSRS may also fail, due to absent or low concentration of SST2, which may be down regulated by excess cortisol.F-DOPA-PET, based on amine-precursor uptake, can be a useful method to localize the occult source of ACTH in EAS when other methods have failed. Ectopic ACTH Cushing's syndrome (EAS), accounting for 5–10% of all cases of Cushing's syndrome, is mainly caused by neuroendocrine tumors (NETs) of lungs, pancreas, thymus, or tumors arising in other, less frequent sites 111Indium-Pentetreotide or with 68Gallium-DOTA-TATE-PET takes advantage of the high concentration of type 2 somatostatin receptors (SST2) that is commonly observed in NETs; ii) 6-[fluoride 18]fluoro-dihydroxyphenylalanine-positron emission tomography (F-DOPA-PET), based on the ability of APUD-derived NETs to take up, accumulate, and decarboxylate amine precursors to synthesize, store, and secrete bioactive substances; and iii) 18F-deoxyglucose-PET (FDG-PET) scan that aims at imaging glycolytic activity in tumors that have an elevated metabolic rate As epithelial-derived tumors arising in different organs and displaying predominant neuroendocrine differentiation, NETs share both organ-characteristic and neuroendocrine features Notwithstanding a frequently indolent course, NETs display a marked tendency to metastasize and progress A 64-year-old man, newly diagnosed with hypertension, presented with intense fatigue, muscular weakness, and shortness of breath (NYHA Stage IV), which rapidly increased within 4 months. On examination, the patient was in poor physical condition but hemodynamically stable without respiratory insufficiency. He showed slight facial redness, marked proximal limb muscles atrophy, and mild skin thinning with bruises. There was no moon face, purple striae, supra-clavicular fat pads, or buffalo hump. Laboratory data revealed severe hypokalemia (K 1.7 mmol/l) with metabolic alkalosis (base excess 10.9 mmol/l).Random plasma cortisol was 1600 nmol/l , with ACTH 261 ng/l , and urinary free cortisol 9181 nmol/day . Plasma cortisol level on a 2-day high-dose dexamethasone suppression test (2 mg/6 h) decreased from 1221 to 721 nmol/l.Pituitary-centered MRI showed no evidence of disease especially in hypothalamus or pituitary gland. Bilateral inferior petrosal sinus blood sampling (BIPSS), including corticotropin-releasing hormone (CRH) stimulation, showed no significant central-to-periphery ACTH gradient and ruled-out a pituitary origin of ACTH excess .Head and neck, thorax, and abdomen 3 mm-sliced CT-scan and MRI were unremarkable, excepted for moderately hyperplasic, nonnodular adrenals; SSRS A and FDGAt laparotomy, a segmental resection of the mid-jejunum was performed, including adjacent mesenteric lymphadenectomy. Histopathology revealed two adjacent well-differenced NETs in the jejunum wall, displaying low proliferation index , classified as WHO and ENETS grade 1 Soon after surgery, ACTH and cortisol fell to low levels and transient adrenal insufficiency required cortisol replacement. The postoperative hospital stay was complicated on day 9 by a lower limb deep venous thrombosis and pulmonary embolism. Following anticoagulation, the patient was discharged uneventfully 5 days later.The patient fully recovered, and after a follow up of 26 months, ACTH and cortisol levels remain normal and sequential CT scans show no evidence of recurrence.Cushing's syndrome as paraneoplastic syndrome is a well-known phenomenon associated with NETs. ACTH-secreting tumors are more commonly encountered with bronchial carcinoids and small cell lung cancer Small ACTH-secreting tumors may remain undetected on anatomic imaging , despite clinical and biochemical evidence of ACTH-dependent Cushing's syndrome, and noninvasive dynamic tests are not always able to distinguish between Cushing's disease and EAS due to NETs. In our case, the intermediate cortisol suppression of 41% on high-dose dexamethasone was consistent with either possibility, and BIPSS was necessary to confirm EAS.Surgery is a key element in therapy, allowing radical resection of metastases-prone NETs and in the setting of EAS rapid control of life-threatening cortisol excess, which can avoid chemical adrenolysis or palliative surgical adrenalectomy in vitro expression of SST2-mRNA in pituitary adenoma cells SST5 mRNA in pituitary adenoma corticotrophs is five- to ten-fold higher than that of SST2 mRNA in vitro ACTH in the presence of high levels of glucocorticoid 68Gallium peptide receptor radionucleides with octeotide derivatives were superior to 111Indium-Pentetreotide for NET detection 68Ga-DOTA-TATE-PET, using octreotate, showed a high sensitivity to detect well-differentiated metastatic NETs SSRS, which was negative in our case, is expected to identify NETs expressing high concentrations of cell membrane SST2, for which octreotide displays selective high-binding affinity. However, the sensitivity of SSRS in EAS is relatively low, ranging from 25 to 60% FDG-PET may highlight tumors with high metabolic activity, but shows a low sensitivity  to identify NETs causing EAS F-DOPA-PET imaging is based on the ability of neuroendocrine cells to take up, accumulate, and decarboxylate amine precursors for the synthesis of bioactive substances. It has emerged as a diagnostic and staging tool for NETs, and was of utmost importance to solve our case. Furthermore, based from our case it can be hypothesized that the tumor with hormone secretion required more precursor uptake than the inactive one, possibly through upregulated membrane transport systems: this would explain why the ACTH-secreting tumor and its adjacent lymph-node metastases were accurately identified with F-DOPA-PET, whereas the neighboring nonsecreting NET remained silent, despite the fact that both tumors had similarly low mitotic indexes.et al. Zemskova The optimal therapy of EAS remains surgical excision of secreting tumor(s). However, small tumors causing EAS are difficult to detect on anatomical imaging and may require metabolic imaging techniques. We describe the case of a 64-year-old man presenting an occult EAS. The source of the secretion remained undisclosed after CT-scan, MRI, FDG-PET and SSRS. A small NET tumor causing EAS has been finally unveiled with FDOPA-PET. Despite its relatively low sensitivity, F-DOPA-PET should be considered as a useful alternative to locate elusive tumors in occult EAS when all other diagnostic modalities have failed.Written informed consent has been obtained from the patient for publication of the case report and accompanying images.


Intraosseous cavernous hemangioma is a rare cause of osteolytic lesions of the skull, and its multifocal type is even more infrequent. This tumor is difficult to accurately diagnose by imaging and can be confused with osteolytic Langerhan’s cell histiocytosis or other neoplasms. Here we present a case of multifocal intraosseous cavernous hemangioma of the skull treated with surgical intervention in our hospital five years ago. A review of related literatures and case reports is also provided to help clarify the diagnosis and devise treatment regimens. In light of the difficulties of early diagnosis, early en bloc surgical removal is recommended. Primary cavernous hemangiomas are rare skeletal tumors accounting for 0.7-1% of all bone neoplasms . These tA 29-year-old female came to our neurology outpatient clinic because of a painful skull defect found incidentally over the right parietal area. The lesion was soft and with mild dimpling. Intermittent pain had started at least 3 weeks before the initial visit. She denied any history of head injury or systemic disease. Cranial computed tomography (CT) and magnetic resonance imaging (MRI) identified 2 individual osteolytic lesions with contrast enhancement Figures , 2 over Primary intraosseous cavernous hemangioma is a rare, benign, and slow-growing tumor formed by blood vessels separating fibrous tissue and accounting for 0.2% of all benign tumors of the skull . In the Differential diagnoses of intraosseous neoplasm include LCH, osteoma, sarcoma, and fibrous dysplasia. LCH is caused by abnormal proliferation of dendritic cells, which often occurs in young patients. It is commonly located in the skull , especially in the parietal and frontal bones . CompareOur literature search failed to identify an ideal noninvasive method that can differentiate cavernous hemangioma from other diseases such as LCH or malignant cell invasions. Therefore, in the majority of cases, the final diagnosis is established only after surgery. For the above reasons, early en bloc surgical removal of intraosseous tumors is recommended irrespectively of whether the osteolytic lesion is benign or malignant , and unifocal or multifocal. Correction of cosmetic changes and treatment of local pain are other indication for the surgery .


Abdominal cocoon is a complete or partial encasement of intestines and rarely viscera by a fibrocollagenous sac which is usually formed by a nonspecific chronic inflammatory reaction. We report a case of abdominal cocoon in a 2-day-old neonate presenting with intestinal obstruction. Abdominal cocoon is a complete or partial encasement of intestines and rarely viscera by a fibrocollagenous cocoon like sac which is usually formed by a nonspecific chronic inflammatory reaction [1-3].The condition has frequently been described in females of tropical and subtropical regions around their puberty [2]. The etiology is pointed towards chronic peritonitis due to tuberculosis or ascending infection in females . Meconium peritonitis has never been reported as an etiology of abdominal cocoon. Our case adds another cause to the list of etiologies and favors secondary causes instead of congenital causes which lead to formation of abdominal cocoon.A 2-day-old male neonate, born by spontaneous vaginal delivery at home, presented with failure to pass meconium, vomiting and abdominal distention since birth. The history of polyhydramnios was present. Antenatal anomaly scan was reported normal. On general physical examination, patient had weak cry, feeble peripheral pulses, and respiratory rate of 42 per minute. The birth weight was only 2.5 kg. On examination, abdomen was symmetrically distended with tenderness. Routine lab tests were normal. The abdominal x-ray  showed bowel gas shadows gathered in the center of abdomen and seemed to be surrounded by homogenous opacity. The abdominal ultrasound showed free fluid present in peritoneal cavity. We suspected complicated meconium ileus preoperatively.On exploration, there was about 300 ml of meconium stained fluid in the peritoneal cavity. Entire small bowel, large bowel, and liver were encased in a transparent membrane . The distal ileum had a perforation which was pouting like a stoma through the cocoon . The membrane was dissected off the intestines. Moreover, inter-loop adhesionolysis was also performed . Distal patency of bowel was confirmed and ileoileal anastomosis performed after refreshing the margins of the perforation. The immediate postoperative recovery was uneventful. The nasogastric tube was removed on 4th postoperative day. Patient was allowed orally on the same day and discharged on establishing full feeds on 8th POD. The patient again presented on 10th postoperative day with anastomotic leakage. He underwent re-exploration and divided ileostomy was formed. However, the neonate succumbed to sepsis on 13th postoperative. The histopathology of the membrane showed proliferation of fibrocytes with enrichment of collagen fibers having non-specific inflammatory reaction.Abdominal cocoon may be idiopathic or acquired. Chronic inflammations due to tuberculosis, systemic lupus erythrematosis and sarcoidosis, echo virus peritonitis, retrograde infection in females, peritoneal dialysis , following placement of LeVeen shunt and ventriculoperitoneal shunt, drugs like beta blocker practolol, and tumors like leiomyoma, endometrial cyst or ovarian tumors are the proposed acquired etiologies of abdominal cocoon . Congenital etiology has also been emphasized and it is considered that cocoon like membrane may be derived from yolk sac during 12th week of gestation or omentum plays a role in the formation of abdominal cocoon [5]. We believe that meconium peritonitis led to the formation of abdominal cocoon in our case. There are three varieties of abdominal cocoon. In type I, membrane covers a part of intestine, in type II entire intestine, and in type III other viscera along with entire intestine are encased [7]. Our case was a type III abdominal cocoon.The clinical presentation is often non contributory to the clinical suspicion, as in our case, where the patient presented with neonatal intestinal obstruction. Older patients may have abdominal pain, with or without constipation or abdominal mass. Intestinal obstruction with peritonitis may develop secondary to myriad of etiologies and a high index of suspicion is required to detect this condition as to extreme scarcity of abdominal cocoon especially in neonates and infants. The disease has been reported to remain dormant till the old age and found at operation done for other reasons [6]. The definitive treatment is dissection and excision of the membrane [1-6]. Some authors also recommend appendectomy as part of operation fearing a difficult dissection on account of adhesions should the patient has appendicitis in future [6]. To conclude, abdominal cocoon is extremely rare in neonates and infants. Crowding of bowel gas shadows with peripheral homogenous opacity on plain radiograph may raise a suspicion of abdominal cocoon. Meconium peritonitis may be enlisted in the etiology of abdominal cocoon.Source of Support: NilConflict of Interest: None


Congenital granular cell epulis is an uncommon tumor which is apparent at birth.Here we report an unusual case of congenital granular cell epulis present in the mouth of a 4-day-old Asian Chinese female newborn. She had six round, soft, multiple, pedunculated swelling masses, of which two were on her upper anterior ridge and four on her lower anterior ridge. The size of the largest lesion was 3.5×3cm, which was causing difficulty in feeding.The case of a patient with congenital granular cell epulis was reported here because of its rarity. The lesions were surgically removed and satisfactory results were achieved. Congenital granular cell epulis (CGCE) is an uncommon tumor seen only in the newborn. The gingival overgrowth was first described by Neumann in 1871 . New casA 4-day-old Asian Chinese female newborn was admitted to our department because she presented with six round, soft, multiple, pedunculated swelling masses with two on her upper anterior ridge and four on her lower anterior ridge. The size of the largest mass was 3.5×3cm Figure . No famiAll lesions were well defined, firm, round, smooth and pink in color on the cut surface. All alveolar masses were excised surgically without complications under general anesthesia on the sixth day after the birth of the baby, who was discharged on the third postoperative day. Postoperative recovery and surgical site healing were satisfactory.The excised masses were fixed in 10% neutral buffered formalin. The tissue was submitted for histopathological examination. Immunohistochemical analyses were also carried out using a panel of antibodies, including vimentin, Ki-67, smooth muscle actin (SMA), synuclein (Syn), neuron-specific enolase (NSE) and S-100. Corresponding positive and negative controls were performed in parallel for all the antibodies tested.Microscopic examination showed a benign tumor composed of sheets of closely packed, large, rounded polygonal cells with abundant granular, eosinophilic cytoplasm and round to oval and lightly basophilic nuclei Figure . The oveFollow-up was conducted for the next 2 months of the baby’s life; no signs of recurrence were found Figure .Figure 2CGCE is an uncommon lesion arising from the mucosa of the gingiva, typically from the anterior part of the maxillary alveolar ridge, but some cases have also been reported in the mandibular gingiva where the lesion was localized (with the 3:1 ratio of maxillary to mandibular predilection) –10. It hThe lesions were excised under general anesthesia because they were initially thought to be malignant neoplasm. Reported cases have been excised under either local anesthesia or general anesthesia , 13. CGCThe diagnosis of CGCE should essentially be clinical, but it could pose some difficulties as seen in this case because of the low level of suspicion. While CGCE is uncommon, it is important that dentists should be able to recognize it as they may be asked to consult and provide crucial information on patient management as well as allay the anxiety of parents. Polyhydramnios is a common symptom in patients with CGCE due to swallowing difficulties as a result of the large size of the mass growth, but the mother did not attend antenatal clinics and gave birth at home.On histological examination, CGCE is very similar to granular cell tumor (GCT) but different from GCT epidemiologically as well as in clinical behaviors. CGCE is seen exclusively on neonatal gingivae, presents at birth and has a marked predilection for females, whereas GCT is rarely seen in the first decade of life, it is most frequently diagnosed between the third and the sixth decades of life, affects a wide variety of visceral and cutaneous sites, and also has a predilection for the female gender .However, a particularly interesting finding made in this study is the demonstration of a strongly positive immune staining with NSE and a negative staining for S-100 protein. In contrast to GCT, the overlying epithelium never shows pseudoepitheliomatous hyperplasia but typically demonstrates atrophy of the rete ridges. In addition, in contrast to GCT, immunohistochemical analysis showed that the tumor cells were negative for S-100 protein, whereas GCT usually demonstrates a strong staining for S-100.The histogenesis of CGCE is not clear, and proposed derivations include odontogenic epithelium, undifferentiated mesenchymal cells, pericytes, fibroblasts, smooth muscle cells, nerve-related cells, and histiocytes , 9. HoweRecommended management for epulis is surgical excision, although some opt to wait for spontaneous regression if the mass is small and does not interfere with respiration or feeding. In addition to simple excision, the techniques of undermining and advancing gingivoperiosteal flaps, and suturing them over the bony defect, and thus extrapolating for cleft alveolar management have also been reported by Millard and Latham .Written informed consent was obtained from the patient’s legal guardian 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.


We describe a 16-year-old boy with rhabdomyosarcoma, consistent with embryonal subtype, of the lower extremity who received systemic neoadjuvant chemotherapy and subsequent excision. Microscopic sections of the postchemotherapy excision demonstrated diffuse, prominent, and immature adipocyte-like differentiation, in addition to skeletal muscle differentiation. Adipocyte-like differentiation was confirmed by a combination of positive Oil Red O and adipophilin immunohistochemical staining. To our knowledge, this represents the first report of an unusual phenomenon of differentiation of a soft tissue rhabdomyosarcoma into adipocyte-like cells after chemotherapy. Rhabdomyosarcoma is an aggressive mesenchymal neoplasm, most common in children, that frequently requires multimodality therapy consisting of surgery and chemotherapy . So-callOne year prior to presentation, a 16-year-old boy with no significant medical history noticed a lump, which progressively enlarged over the course of several months, at his posterior left knee. Magnetic resonance imaging demonstrated a 13-centimeter mass in the left posteroinferior thigh, centered in the region of the semitendinosus muscle and extending to the long head of the biceps femoris muscle. FOXO1 13q14 rearrangement. Based on the cytomorphology and lack of FOXO1 rearrangement, an embryonal subtype was favored. He received chemotherapy per intermediate risk protocol ARST0531 with vincristine/irinotecan for 10 weeks.A fine-needle aspiration of the tumor demonstrated a heterogeneous population of ovoid to tapered spindled cells with variable amounts of densely eosinophilic cytoplasm and occasional multinucleated cells . No lipoFollowing chemotherapy, the tumor was completely excised. Grossly, the mass was mixed, cystic and solid, and separated from the surrounding skeletal muscle by a thin white capsule . RoutineRhabdomyosarcoma is the most common soft tissue malignancy in children and adolescents and consists of a biologically and genetically diverse group of sarcoma . StandarSeveral studies have described the range of microscopic treatment related changes in rhabdomyosarcoma. Nonspecific treatment effect, such as necrosis, macrophage infiltrate, fibrosis, and skeletal muscle atrophy are commonly observed. A decrease in proliferative activity is also often present . RhabdomIn addition to the phenomenon of cytodifferentiation, vacuolated cells have been previously noted in cases of treated rhabdomyosarcoma. Coffin et al. described the presence of vacuolated cells in a rhabdomyosarcoma . LikewisIn our differential diagnosis, we also considered the possibility of clear cell rhabdomyosarcoma, or glycogen-rich rhabdomyosarcoma . HoweverRhabdomyoblastic differentiation is well described in a variety of mesenchymal tumors, including malignant mixed Mullerian tumors of the uterus and carcinosarcomas, as well as neuroectodermal neoplasms such as malignant Triton tumor or medulloblastomas. Intriguingly, rhabdomyoblastic differentiation has been reported in liposarcoma , 18, butTo our knowledge, this is the first reported case of a soft tissue rhabdomyosarcoma with evidence to support adipocyte-like cells as a posttreatment phenomenon and expand the spectrum of cytodifferentiation that can be observed in treated rhabdomyosarcoma.


Mesenchymal hamartoma and hepatoblastoma are common causes of hepatic masses in pediatric population; they have similar radiologic and pathologic features. Herein, we present a case of mesenchymal hamartoma that was preoperatively diagnosed as hepatoblastoma. The mass was completely resected instead of being treated with preoperative chemotherapy. Postoperative pathological evaluation revealed mesenchymal hamartoma with free margins; the patient incidentally received the standard treatment. If we would have measured serum AFP in our patient, we could make the correct diagnosis preoperatively, because AFP increases largely in hepatoblastoma. When suspicious exists, serum AFP is a good guide in differentiating hepatoblastoma from mesenchymal hamartoma. Mesenchymal hamartoma is the second most common benign tumor of liver in childhood. Most of these tumors are large multicystic masses that usually diagnosed before the third year of life mostly in boys. Mesenchymal hamartoma may present with various signs and symptoms like anorexia, vomiting, abdominal distension, and poor weight gain, but pain is rarely a dominant feature. Laboratory studies usually reveal normal liver function tests with moderately elevated serum α-fetoprotein (AFP). Ultrasound, CT, and MRI demonstrate a multiloculated cystic tumor with variable amount of solid tissue.Hepatoblastoma is the most common malignant tumor of childhood which is more frequent in boys. It is usually diagnosed in young children with a peak incidence in newborns . It usuaIn most patients, diagnosis of mesenchymal hamartoma is suggested by imaging and confirmed with histologic examination. Aspiration cytology rarely enables a definite diagnosis and due to its inability to ruling out hepatoblastoma or other malignant mesenchymal tumors, it has limited diagnostic value .The main treatment for mesenchymal hamartoma is complete excision of the mass, whereas the first line for the treatment of hepatoblastoma may be chemotherapy , thus, mHerein, we present a case of liver tumor that was primarily diagnosed as hepatoblastoma, but it was found to be really mesenchymal hamartoma, postoperatively. A 13-month-old boy, a known case of thalassemia major, was referred to our center from Jiroft, South-east of Iran, because of an abdominal mass diagnosed two months before. He had history of jaundice and diarrhea during this period. Abdominal ultrasonography prior to his admission, showed a 114-mm hyperechoic mass in the right liver lobe. Abdominal MRI showed a large well-defined heterogeneous mass 10×12 cm in diameter in the right liver lobe; the mass displaced the right kidney and compressed the adjacent vessels . Both raOpen liver biopsy, done for the patient 20 days before his referral, was also reported as hepatoblastoma. Patient was referred to Pediatric Oncology service for receiving preoperative chemotherapy, however, because of some personal problems, parents of the patient did not with the preoperative chemotherapy. Therefore, we decided to operate the patient because the mass seemed to be resectable in MRI.The abdominal cavity was entered via a Chevron incision. The standard abdominal exploration was done. No sign of peritoneal seeding or involvement of adjacent organs was observed. After releasing the falciform, right triangular, and right coronary ligaments, the right liver lobe was exposed. Right hepatic artery, and middle and right hepatic veins were ligated. Bleeders were controlled. After the extension of tumor was evaluated, right extended hepatectomy was performed.Gross pathology report described tumor as a well-defined creamy green mass with measured at 12×11×8 cm; the margins were grossly free of tumor. Microscopic evaluation was in favor of mesenchymal hamartoma. The patient was discharged from hospital in good condition.Our patient was found to have liver mesenchymal hamartoma that resembled hepatoblastoma preoperatively. A similar report was also published from Turkey in 2008 . The patIn the differential list of a pediatric liver mass with various amount of solid tissue and cystic components in radiologic evaluation, are hepatoblastoma, embryonal sarcoma, hemangioma, infantile hemangioendothelioma, hepatic hydatid cyst, and mesenchymal hamartoma . There aThe treatment strategy for hepatoblastoma is combined chemotherapy and surgery. However,If we would have measured serum AFP in our patient, we could make the correct diagnosis preoperatively, because AFP increases largely in hepatoblastoma. When suspicious exists, serum AFP is a good guide in differentiating hepatoblastoma from mesenchymal hamartoma.


Extradural spinal meningiomas are rare. Our understanding of purely extradural spinal meningiomas is still incomplete and they may be easily confused with malignant neoplasms, much more common in this location. We report a rare case of a purely extradural thoracic spine meningioma in a 70-year-old man, with an unusual progression. In addition we discuss the pathogenesis of these tumors and the potential pitfalls in differential diagnosis and review the relevant literature concerning their treatment and outcome. Meningiomas account for 25 to 46% of primary spinal neoplasms  and spinWe report a rare case of a purely extradural thoracic spine meningioma in a 70-year-old man with an uncommon progression. In addition we discuss the pathogenesis of such tumors and the potential pitfalls in differential diagnosis and review the relevant literature concerning their treatment and outcome.A 70-year-old man with no significant medical history was admitted to our hospital in January 2005 complaining of back pain and sensory changes of both lower limbs for the past 08 months and difficulty in walking for the past two days. He did not present with sphincter disturbances and seemed to be healthy before the onset of the symptoms.On admission, the patient's neurological examination revealed paraparesis with with motor strength of 3/5, a T6 sensory level and exaggerated knee and ankle reflexes along with extensor plantar response in both sides. There was no bowel or bladder dysfunction.MRI revealed a space occupying anterior lateral extradural lesion at T5 level resulting in marked compression of the thecal sac. The lesion was hypointense to the spinal cord on T1-weighted images, T2 hyperintense, and displayed important enhancement after Gadolinium injection. Vertebral body of T5 was in T2 hypersignal too  meningioma as the tumor demonstrated small cell differentiation and increased mitotic activity on light microscopy Figures  and 4.The patient's immediate postoperative course was uneventful, and he recovered quickly. His neurological condition improved continuously, and follow-up examinations showed that the motor weakness had disappeared completely. An adjunctive 50 Gy radiation therapy was administered and six-month serials MRIs were made during postoperative follow-up .Two years after surgery the patient was lost to view. He presented once again in February 2012 with severe symptoms of spinal cord compression. MRI showed a local relapse of the disease with mainly anterior lateral progression including T5 vertebral body and posterior mediastinum . A posteSpinal meningiomas can still cause a great deal of morbidity. They are typically intradural extramedullary and extradural space is usually the site of malignant neoplasms such as metastases or lymphoma especially in the elderly.Exclusively extradural meningiomas are very rare , 4–7. FuPurely extradural meningiomas do not essentially differ from the common intradural types . They ofThere is typically a delay between the onset of symptoms and diagnosis. Patients with spinal meningioma often present with pain, sensory motor changes, and sphincter disturbances. Generally back pain precedes the weakness and sensory changes. The sphincter dysfunction is always a late finding . The cliMRI is the best imaging technique for diagnosing spinal meningiomas. It clearly delineates the level of the tumor and its relations to the cord, which is useful in planning surgery , 8. The Cushing and Eisenhardt defined the successful removal of a spinal meningioma as one of the most gratifying operative procedures. Posterior laminectomies are indicated for posterior locations. If the tumor is located anteriorly, the laminectomy can be extended laterally towards the articular process to provide sufficient exposure and cause minimal displacement of the spinal cord, or an anterior approach via posterolateral thoracotomy can be indicated. In the present case, a 70-year-old man presented with a rapid neurologic deterioration due to an extradural mass at T5 level. After a brief radiological investigation we decided to achieve a posterolateral approach in emergency with total removal of the extradural mass and cauterization of the dural basement. For the relapse a more aggressive surgical treatment was opted with good surgical results.Pathologically, spinal meningiomas tend to be well-defined discrete lesions with a dural attachment. Microscopically, the common patterns are meningothelial, fibroblastic, transitional, and psammomatous and most case reports describe features of a meningothelial or psammomatous meningioma , 9. The In literature review of major spinal meningioma series, including intra- and extradural subtypes, results of surgical excision are reported to be good to excellent with recurrence rates of 3 to 7% . MorbidiFor the present case, the diagnostic of a malignant lesion was erroneously suspected before surgery and no intraoperative histological examination was performed. We believe it would have been better to completely remove the dural basement of the tumor, or at least one layer of it, to decrease recurrences during the first surgery. Although literature did not provide any guidelines and regard of the dismal progression of our patient's tumor, a second operation would have been necessary after the final pathology results were obtained. Therefore, we want to stress on the importance of intraoperative histological examination for adequate surgical decision making.Another standpoint is that it is better to reserve adjuvant radiation therapy for recurrent, difficult to reach, high grade cases than after first surgery due to the risk of irradiating a functional spinal cord with no proven benefit, especially that the lesion was anterior and difficult to reach without spinal cord radiation exposure.Finally, the fact that this patient demonstrated neurologic improvement following his second surgery makes us suggest the possibility of favourable outcome after surgery for recurrent extradural meningiomas.


We report a case of pathologically confirmed tumefactive multiple sclerosis (MS) followed shortly thereafter by the diagnosis of an oligoastrocytoma. The complexity of diagnosis and management of concomitant presence of tumefactive MS and glial cell tumors is discussed. A 69-year-old man presented to our Neurosurgery Service with a 2-month history of trouble writing, poor coordination of his right hand, and word finding difficulties. Initial neurological examination was unremarkable. Medical history was remarkable for hypertension, dyslipidemia, coarctation of aorta (diagnosed at age 18 and corrected with surgery), and L4–L5 laminectomies due to significant pain. There was a strong family of cancer including a brother (deceased age 77) with glioblastoma (GBM) and a daughter (deceased age 34) with osteosarcoma (unknown location).Routine blood chemistries and CBC were unremarkable. MRI of the brain, with and without contrast, showed a left frontal, ring-enhancing mass measuring 2 cm × 2.3 cm with substantial edema Figure . He was He was weaned off steroids. He experienced considerable improvement of his dexterity and ability to concentrate.About 10 months later, an MRI of the brain was taken at follow-up, showing no evidence of local recurrence of the left frontal lesion. There were patchy hyperintensities in the right occipital and left frontal areas. At that time, he was asymptomatic, and his clinical examination was unremarkable.Three months later, he presented with difficulty concentrating and poor short-term memory. MRI of the brain now showed increased size of the right occipital and parietal lobe lesions, with a new area of occipitoparietal ring enhancement Figure A.A 5-day course of high-dose IV methylprednisolone was started for a possible recurrent demyelinating process. Cerebrospinal fluid (CSF) analysis showed normal protein levels with no white blood cells (WBC), glucose 63 mg/dl (60%), and 0.77 IgG index (with serum IgG 622 mg/dl). Myelin basic protein (MBP) and serum neuromyelitis optica (NMO) antibodies were negative. CSF sampling for oligoclonal bands (OGB) was positive. Somatosensory evoked potentials (SSEPs) were normal and visual evoked potentials (VEPs) were uninterpretable.He was discharged on a tapering dose of oral steroids. He was subjectively better, but became symptomatic again with generalized weakness, more prominent on the left side. The weakness slowly progressed until an initial dose of natalizumab was given. The patient then experienced abrupt worsening of symptoms. Neurological examination showed left-sided hemiparesis, left hemibody neglect, and left inferior homonymous quadrantanopia. MRI of the brain revealed an increased area of enhancement in the deep, right parietal lobe with mass effect Figure B. The paOccurrence of multiple sclerosis (MS) and brain tumors has been reported <50 times in the literature, since first described by Bosch in 1912 . WhereasTumefactive demyelinating lesions (TDLs) are defined as large (>2 cm) demyelinating lesions occurring either as solitary lesions or as a few separated lesions with little vasogenic edema, and incomplete or open ring-enhancing lesions , 11. LikN-acetylaspartate peaks . Tumors also show decreases in N-acetylaspartate but have typical increased choline peaks (indicating rapid mitotic activity) and increased lactate peaks  (MRI is the most sensitive technique for depicting demyelinating disease. A study by Kim and colleagues  showed tcolysis) . When lecolysis) .Management of high-grade gliomas often requires cytoreductive therapy followed by adjuvant therapy with radiotherapy (XRT) and/or chemotherapy . AdditioThis case submission met all of the requirements for publication under the rules of the Loyola University Stritch School of Medicine Institutional Review Board.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Dear Sir,Lipoblastoma is a rare benign soft tissue tumor occurring mostly in infancy and early childhood. It arises from white embryonic fat. Adipose tissue tumors constitute about 6% of all soft tissue neoplasms in children and lipoblastoma accounts for only 5 to 30% of adipose tissue tumors.[1-3] Many patients present with a painless mass, which lead to misdiagnosis. A 2-year old girl was admitted with an asymptomatic progressively enlarging swelling at the right inguinal region for 4 months. There were no obstructive bowel symptoms. She had a history of right inguinal hernia repair 8 months ago (operative notes were not available). On physical examination, a non-tender, mobile and irreducible (2x3 cm) swelling was noted. Inguinal ultrasonography was normal. Patient underwent surgical exploration of the swelling through the old scar with the initial diagnosis of recurrent sliding inguinal hernia of ovary. At operation, there was no recurrence of inguinal hernia and the inguinal swelling was more like a mass. The tumor was completely excised, with the resulting specimen of 4 x 2.5 x 2cm, with no invasion into the surrounding tissues. The histopathological examination revealed lobulated tissue composed of immature fat cells separated by fibro-vascular septa and areas with a myxoid matrix. The lobules contained lipoblasts in different stages of differentiation, ranging from primitive, spindle-shaped cells to lipoblasts simulating mature fat cells . She has been followed up every 3 months for 2 years and no recurrence has occurred.Lipoblastomatous tumors are rare, benign adipose tissue tumors of infancy and childhood. Ninety percent of reported cases are under the age of three years and 55% less than 1 year of age.[3] Histopathologically, these tumors are classified into two forms as lipoblastoma (circumscribed type) and lipoblastomatosis .[4] The inguinal region is an uncommon location for this tumor.[2] Because of the nonspecific presentation of lipoblastomas, they are often mistaken for other conditions.[5] The previous operation on the same side may point to a possible recurrence, however, the history and physical examination did not correlate with recurrence as it was a progressive swelling and was of significant size. Ultrasound which was reported as normal could have differentiated between a fatty mass and a firm structure like ovary, however, this modality could not help us. Patients with lipoblastoma usually have a good prognosis despite the tumor's potential to invade locally and grow rapidly.[2] Post-operative follow-up is important to detect relapses but appropriate length of follow-up is controversial.Source of Support: NilConflict of Interest: None declared


Congenital cystic eye is an exceedingly rare ocular malformative disease, originated from the failure in the invagination of the optic vesicle during the fetal period and it can be associated with other ocular and non-ocular abnormalities. Diagnosis is based on clinical, radiological and histological features.We report a case of a congenital cystic eye associated with a cerebellar lesion accidentally detected at magnetic resonance imaging. Biopsy of the mass has not been performed due to parental rejection. Based on radiologic features and absence of clinical signs, a low-grade glioma diagnosis was hypothesized, but histological characterization was not obtained. Follow-up neuro-imaging 6 months after diagnosis showed that intracranial lesion spontaneously regressed without any treatment.Our report stresses the importance of early MRI in children with ocular malformations, in order to detect associated intracranial defects, also of non-malformative origin. Additionally, we debate the clinic-radiological features of the intracranial lesions that could allow a wait-and-see policy. We also recommend a strict clinical and neuro-imaging follow-up for these lesions. Finally, biological mechanisms at the base of spontaneous regression of the brain lesions are discussed. Congenital cystic eye  is a verWe here report a rare case of a congenital cystic eye associated with a glial cerebellar lesion accidentally detected at magnetic resonance, that spontaneously regressed during follow-up. Finally, we also debated the biological mechanisms at the base of spontaneous regression of the brain lesions.A 6-month-old child was referred to our Ophthalmology Department for a congenital unilateral cystic orbital mass, noticed at birth. Ophthalmologic examination revealed a left empty socket and a cystic lesion protruding from the left orbit, stretching upper eyelids. No ocular structures could be identified. The child didn’t present cafè-au-lait spots, specific cutaneous lesions or Lisch nodules in the iris. Patient was tested by array comparative genomic hybridization, without evidence of genomic abnormalities: in particular, SOX2/PAX6 mutations were excluded and karyotype was normal. On the ultrasonography, no globe was disclosed in the left cavity. Brain and orbit MRI suggested a non-infiltrative extraconal superior lesion Figure  that varTo our knowledge, less than 40 cases of congenital cystic eye, also termed “anophthalmos with cysts”, have been published to date. Duke-Elder  reviewedThis rare disembriogenetic disorder results from a defect in the invagination of the primitive optic nerve vesicle, causing the incomplete evolution of the ectodermal elements into the mature eye tissues between the 2-mm and 7-mm stages of fetal development. The etiology of the cystic eye remains unclear; genetic investigations performed in few cases have not yielded any peculiar defects ,15, as iDiagnosis is based on clinical, imaging and histopathological features. Newer imaging modalities, as prenatal magnetic resonance imaging, could reveal characteristic findings of this condition at or even prior to birth, as suggested by Singer et al. .The hardest histological differential diagnosis includes orbital teratoma, in which derivatives of all three germ lines are present . TeratomThere is no standardized protocol for management of the congenital cystic eye. Surgical intervention is strongly advised in order to obtain an optimal cosmesis. Regarding timing of surgery, cystic globes have been excised within a week to several years after the birth. After excision, Chaudry et al.  achievedAt our knowledge, cerebellar lesion of supposed glial-origin has not been previously reported in the contest of the congenital cystic eye. Furthermore, spontaneous regression of low-grade glial lesions  is a rarBrain tumors are the most common solid tumor diagnosed in childhood that account for significant morbidity and mortality. Children affected by low-grade gliomas are known to have an excellent 10-year survival rate. Bandopadhayay et al.  identifiTherefore, in our case, the glial origin of the lesion could be anyhow suspected considering several elements. In pediatric population, the detection of incidental intracranial abnormalities can occur due to the advancing diagnostic imaging. Identified radiological features suggestive for low-grade gliomas are as follows : non-enhLow-grade glioma was also considered the most likely diagnosis in all cases, based on the consistent pattern of T1 hypointense, T2 and T2 fluid-attenuated inversion recovery hyperintense, and non-enhancing pattern of the lesions. Therefore, as in our case, in which the informed consent to the biopsy was denied, a “watchful waiting” management could be supported by the radiologic features consisting with a low-grade glial lesion and the absence of neurological deficits . The intBiological mechanisms which induce spontaneous regression may only be speculated . SeveralIn conclusion, congenital cystic eye should be suspected in infants with an unrecognizable ocular globe. We present the first report of a low-grade lesion of suspected glial origin in the cerebellum, with unusual biological behavioral, disclosed in association with congenital cystic eye. Currently, we have no convincing explanation, supported by “ad hoc” literature and documentation of larger series, to explain the link between these two clinical entities; so it seems to be an accidental but very interesting association, in which two rare conditions are overlapped. We could also confirm that the well-known practice of early brain MRI in children with ocular malformations is very useful, in order to investigate the possible association with other intracranial alterations, also of non-malformative origin. This case presentation also highlights the radiologic features of the accidentally disclosed intracranial lesions that could justify a wait-and-see policy. Long-term follow-up is required to closely survey the tumor growth rate; in case of enhancement modifications, increase of size and/or edema appearance, biopsy and resection are then considered.Parental written informed consent was obtained 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.All the authors disclose all potential, commercial, financial or other conflicting interests that could in any way affect the realization of this manuscript.MGC wrote the first draft and cured the manuscript preparation. AR and AM cared for the patient. GSC selected the neuro-imaging and realized figure and legends. RDV selected and described the histopathological sections. AM has been crucial in ideating the work and supervising the draft revision. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2415/14/80/prepub


The diagnosis of pancreatic cystic neoplasms has become more accurate recently. In some cases, however, doubt remains regarding the lesion’s malignant potential. CA 19–9 has long been identified as a reliable biomarker in differentiating pancreatic benign and malignant lesions, especially in non-jaundiced patients.We report a case of a young female who presented with a mucinous lesion in the tail of the pancreas and a serum CA 19–9 over 1,000,000 U/mL. She was taken to surgery and had a distal pancreatectomy and splenectomy. Pathology reports showed only a mucinous cystadenoma. After 1 year of follow-up, her serum CA 19–9 was normal. Following that, the work-up in these lesions, the role of the biomarker in pancreatic adenocarcinoma and in the differentiation between benign and malignant lesions is discussed. Pancreatic cystic neoplasms (PCNs) still constitute a challenge in everyday clinical practice. Current imaging diagnostic methods, increasingly more sophisticated and associated with an ever-increasing spectrum of serum biomarkers or cystic content analysis, are still not able to differentiate reliably enough the benign lesions, amenable to follow-up, from those malignant or premalignant lesions that require surgical treatment.A female patient, 39 years old, is complaining of diffuse abdominal pain, more prominent in the flank and left hypochondrium, initially bearable and with response to simple analgesics, but with progressive and significant worsening in the last 24 h. The patient reported bilious vomiting and denied fever or other similar episodes in the past. She was admitted in good general condition, moderately dehydrated and discolored. On abdominal palpation, there was evidence of diffuse pain without peritonitis. The patient was given IV hydration with crystalloids and underwent an abdominal/pelvic CT scan, which identified a voluminous expansive formation in the pancreatic tail, with compression, but without direct invasion of the splenic vein Figure . LaboratUpper gastrointestinal (UGI) endoscopy did not show any extrinsic compression or gastric/duodenal wall invasion.An MRI of the upper abdomen was performed in order to better characterize the lesion, with evidence from projection imaging of a mass in the pancreatic tail, predominantly with cystic aspect, multiloculated, with thick septations and regular margins, showing heterogeneous contrast enhancement and areas of T1 signal hyperintensity, possibly corresponding to hematic content. Its size was 63 × 60 × 61 mm Figure .Figure 2No endoscopic ultrasound was indicated, since the main diagnostic hypothesis was a mucinous lesion with malignant transformation, and its treatment included surgery, provided that no metastatic disease was found.Clinical stabilization and preoperative evaluation was performed, with no additional significant findings. The patient was the taken to the operating room. At first, she underwent a diagnostic laparoscopy, with no signs of peritoneal and liver metastasis. Laparotomy was then performed, and a distal pancreatectomy with splenectomy was conducted. The patient had an uneventful postoperative outcome and was discharged on the fifth postoperative day.The final pathology report provided the diagnosis of a mucinous cystic neoplasm with moderate dysplasia and no signs of invasion.Its macroscopic exam described a 6-cm well-delineated cystic tumor surrounded by a thick, fibrotic capsule. In its composition, multiple cysts with trabecular and thickened septa were observed. The cysts contents were mucoid and showed no areas of solid tumor. Degenerative changes, including hemorrhage and macrocystic degeneration were seen in focal areas.Microscopically, the epithelial lining was consisted of tall, columnar cells with frequent apical mucin. The tumor was mostly largely bland in appearance, containing uniform, basally oriented nuclei. Focally, it exhibited architectural complexity with pseudostratified hyperchromatic nuclei, but no invasive carcinoma was found. The subepithelial stroma was hypercellular containing spindle cells resembling the stroma of the ovary (ovarian-like stroma) Figures  and 4.FiOn the 40th postoperative day, the patient had an outpatient visit, reporting no symptoms and without any change in blood glucose levels. The CA 19–9 serum concentration at that time was 688 U/mL. Additionally, the patient brought a complete abdominal ultrasonography showing only postoperative changes.After 5 months, the patient remained without complaints and her CA 19–9 serum concentration was still above normal (56 U/mL).In a late postoperative visit, 1 year after surgery, her CA 19–9 serum concentration had returned to normal levels (35.2 U/mL). No abnormal events have been recorded since, with 3 years of follow-up.A controversy has been observed regarding the appropriate indication for surgery in some pancreatic tumors, with some authors identifying up to 30% of unnecessary resections . DiffereThe widespread use of imaging tools in differentiating these tumors has provided safe diagnosis for typical lesions; however, many cystic tumors show intermediate characteristics between benign and malignant ones. Even with the use of modern methods of diagnostic imaging, such as contrast-enhanced ultrasonography and endoscopic ultrasonography associated with fine needle aspiration (FNA), which are also important in this differentiation ,5, the dAn ‘ideal’ tumor biomarker is highly sensitive in identifying disease in an asymptomatic population. CA 19–9, a serum carbohydrate antigen produced by exocrine epithelial cells, was first described more than three decades ago . It is tP < 0.01). The authors advocated that Sendai criteria-negative patients who present elevated CA 19–9 be considered for surgery [In evaluating benign and malignant intraductal papillary mucinous neoplasms (IPMNs), CA 19–9 has a role in the differentiation invasive and benign lesions. In a study with 142 patients, 80% of those with invasive IPMNs had CA 19–9 over 37 U/ml versus only 18% of those with benign lesions. The specificity was 85.9% and accuracy 81.7% . A cance surgery .Two other common cystic neoplasms, serous and mucinous cystoadenomas, have a different pattern of CA 19–9 concentrations. In an American study with 114 patients, CA 19–9 was rarely elevated in serous lesions, but was associated with increases of up to sixfold in mucinous ones, with the presence of symptoms being the most sensitive criterion for malignancy. The cut-off value used was 35 mg/dL [In patients with resectable ductal adenocarcinoma, the increase of CA 19–9 has had an impact on staging and survival. In a large series of 424 patients, tumors that were larger and had lymph node metastasis were associated with a median CA 19–9 over 160 U/mL. Also, individuals with CA 19–9 over 1,000 U/mL had median survival time of only 1 year . SimilarIt must be reminded that some patients, while having invasive neoplasm, do not have an increase in CA 19–9, as 5% to 7% of the population has the Lewis blood group phenotype Le(a-b-) that does not synthesize the antigen detected by the antibody CA 19–9 .K-RAS gene mutation and loss of amplitude in allele expression in cyst fluid confer specificity up to 96% for the diagnosis of mucinous malignant lesions, but with specificity as low as 37% [Further tools have been investigated in order to earlier detect cysts that tend to progress. Genetic analysis of their content constitutes a horizon of particular interest. The concentrations of the CEA biomarker in the cyst can help differentiate between serous and mucinous lesions, but cannot, however, infer malignancy . Detectiw as 37% .The PAM4 marker, an antigen produced by tumor cells, shows a sensitivity of 76% with a specificity of 96% in the diagnosis of pancreatic adenocarcinoma, including in the early stages of onset. The association of CA 19–9 concentrations to PAM4 conferred 84% specificity in diagnosis, regardless of serum bilirubin concentrations .One scenario in which CA 19–9 results must be interpreted with caution is in jaundiced patients. Those who present with benign pathologies of the biliopancreatic tract coursing with jaundice have CA 19–9 concentrations above 37 U/mL at a frequency of up to 64.7% versus 7.3% in non-jaundiced patients, showing the limited potential of CA 19–9 concentrations in the differential diagnosis between benign and malignant conditions in jaundiced individuals .In the case in question, no focal point of invasion was identified and the patient remained anicteric throughout the course of the disease. The presence or absence of dysplasia, and the degree thereof, has shown no correlation with the serum concentrations of biomarkers. This just confirms the authors’ findings that in spite of the vast evolution of diagnostic methods, whether originating from imaging or the laboratory, the correlation between the preoperative diagnosis and the postoperative histological diagnosis of the surgical specimen still revolves around 78.4% .In the current report, a case is presented in which a young female patient has a single pancreatic cystic lesion in the pancreatic tail and also preoperative serum marker of 1,000,000 U/ml, whose pathology report identified just a mucinous cystoadenoma, with no invasive tumor. This further demonstrates that biomarkers should be used as a diagnostic and staging aid and should not be used for screening and/or defining treatment course.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


The classic presentation of cystic hepatobiliary lesions is usually nonspecific and often identified incidentally. Here we describe the case of a patient presenting with acute pancreatitis resulting from a large centrally located biliary cystadenoma compressing the pancreas. Determination of the origin of the cystic lesion was difficult on imaging studies. Due to the difficult location of the lesion, a complete surgical resection was achieved with mesohepatectomy and the suspected diagnosis confirmed by pathology. The patient continues to do well 2 years post-op with no signs of recurrence. Biliary cystadenoma is an uncommon benign cystic neoplasm with potential for malignant transformation . PreoperA 34-year-old female presented to the emergency room with upper abdominal pain and epigastric fullness. She had no associated nausea or vomiting and denied any alcohol consumption or cholecystectomy. Blood work showed elevated amylase and lipase levels consistent with pancreatitis. Serum beta-HCG was negative. She was admitted for acute pancreatitis and treated supportively with pain management and hydration. Her amylase and lipase levels returned to normal within 36 hours.Ultrasound revealed a normal gall bladder and a large cystic mass in the epigastrium prompting further imaging. A contrast enhanced CT confirmed the presence of a large, complex cystic mass with septations measuring 15 × 15 cm in close proximity to the pancreas and extending to the liver and stomach . No biliOn MRI of the abdomen, the locules of the cystic mass were shown to be homogeneously T2 hyperintense and T1 hypointense with the mass overall measuring up to 14 cm in greatest dimension. The mass also demonstrated multiple low T2 signal septations measuring up to 4 mm in thickness  and showGeneral surgery described a complex cystic mass on CT occupying parts of segments 3, 4a, 4b, 5, and 8 without any involvement of major vessels. Endoscopic US revealed a normal pancreas and confirmed the hepatic origin of the cystic mass. Tumor markers  were normal and the patient was sent for definitive treatment with hepatobiliary surgery.Patient consent was obtained for a planned central hepatectomy or mesohepatectomy of segments 4a, 4b, 5, and 8 in order to preserve an adequate FLR (Figures Biliary cystadenomas are rare hepatic lesions that often present with nonspecific signs and symptoms . Though Due to both the potential for the malignant transformation of a biliary cystadenoma to cystadenocarcinoma and the inability to differentiate a benign from malignant mass preoperatively, complete surgical excision is the recommended course of treatment .Biliary cystadenomas arise from the epithelium cells lining either the gall bladder or the bile ducts and are multiloculated and multiseptated. These masses typically arise from the bile ducts of the right hepatic lobe . AlthougOn Ultrasound, biliary cystadenomas are typically multiloculated and demonstrate enhanced transmission. Furthermore, if septal or wall calcifications are present, acoustic shadowing may be exhibited . The conCurrently, the imaging modality of choice in the initial evaluation of liver masses is CT . As therExtended hepatectomy is the procedure of choice for hepatic neoplasms involving central segments of the liver 4a, 4b, 5, and 8 [To date, mesohepatectomy is seldom used due to the technical challenges of the procedure and risks of vascular damage owing to the anatomical complexity of the liver . HoweverAdvances in surgical techniques, such as intraoperative imaging and hemostatic transection devices, are encouraging the use of mesohepatectomy in select patient populations . This ca


Tumors of the paratesticular region most often arise from the soft tissue surrounding the spermatic cord and the epididymis or from the soft tissue (dartos muscle) of the scrotal wall. Paratesticular tumors, despite their rarity, present a high incidence of malignancy (30%), and the therapeutic approach of choice is surgical resection with negative margin. The grade, the histology type, the presence of metastases during the diagnosis, the size of the tumor, the age of the patients, and the surgical margins are all important prognostic factors. We present a case report of a 86-year-old patient with a high grade paratesticular and scrotum sarcoma of soft tissues which was presented as a hard painful mass of the scrotum. The patient was subjected to high ligation of the spermatic cord and received no further treatment and 6 months after the operation no local or systematic recurrence was observed. Soft tissue sarcomas (STSs) are a heterogeneous group of dense tumors which originate from embryonic mesenchymal cells (mesoderm), presenting multiple clinical patterns –4. Respeβ-HCG was measured preoperatively from the surgery department. The Magnetic resonance imaging (MRI) of the abdomen and the pelvic showed a huge mass in the scrotum arising from the inguinal canal  due to myocardial infraction (MI) prior to angioplasty (2005) and a second MI in 2009. Full blood count, lever function, serum glucose, urea, creatinine, LDH, and electrolytes levels revealed no abnormalities throughout his hospitalization. Patient's biomarkers, CEA, AFP, CA-19.9, and PSA, showed also no abnormalities. Unfortunately no al canal . An explal canal . The pos0% due toThe majority of the masses occurring in the scrotal sac are within the testis and usually neoplastic . The par