Burkitt's lymphoma is a highly malignant, aggressive and rapidly growing B-cell neoplasm, which has low long-term survival rates. The abdomen is the most frequent onset site of non endemic Burkitt's lymphoma. Symptoms are often misleading and make diagnosis difficult. Ileum intussusception as acute abdomen caused by Burkitt lymphoma is rare.We are presenting a case of a 16 year-old male with acute abdomen, which three weeks prior initially has been surgically treated for acute appendicitis and Meckel diverticulitis. Following this was a second urgent operation of ileoileal intusussception caused by Burkitt lymphoma. Right extended haemicolectomy was performed.Affected terminal ileum by Burkitt's lymphoma may mimic clinically acute appendicitis and investigation tools sometimes may not provide proper diagnosis.Complete resection results in improved survival. First described by Dennis Burkitt in 1958, Burkitt lymphoma (BL) is a highly aggressive non-Hodgkin lymphoma (NHL) often presenting in extra nodal sites or as an acute leukemia [Three variants have been described: endemic (largely found in Africa), sporadic (non-endemic) subsequently described outside Africa, affecting mainly abdominal viscera and third variant, immunodeficient patients. Burkitt's lymphoma is usually diagnosed in children and young adults, and to a lesser extent in middle aged adults. BL is a B-cell lymphoma genetically characterized by a chromosomal translocation that results in deregulation of the c-MYC oncogene -4.In endemic areas, usually involves the facial bones, particularly the jaw, maxilla, and orbit, especially in young children, associated with Epstein-Barr virus (EBV) infection, as well as frequent concomitant malaria infection ,6.In comparison, the sporadic form tends to present in the lymphoid tissues of the gut, often presenting as masses in the Waldeyer ring or the terminal ileum, or even with involvement of abdominal organs with the most involvement of the distal ileum, caecum or mesentery. Bone marrow involvement is commonly seen in progressive disease -8.We are presenting a case with acute abdomen, i.e. ileoileal intusussception, caused by Burkitt lymphoma. A sixteen-year-old Caucasian Kosovar boy, presented in the surgical emergency clinic as acute abdomen, with abdominal pain, nausea, vomiting, and problems with defecation and flatulence. The symptoms started three days ago, worsening on admission day.The patient's height was 178 cm, and his weight was 70 kg. His vital signs were: blood pressure 120/80 mmHg; pulse rate: 76 beats/min; respiratory rate 20/min; body temperature 37.1°C.During physical examination, a distended, diffusely tender and painful abdomen with lower abdomen rebound was revealed. The patient's skin was pale with sweats.Plane abdominal radiography showed mechanical obstruction. Fig. 9/L; white blood cells (WBC): 7.3 × 109/L; Hemoglobin (Hgb): 128 g/L; Hematocrit (Htc): 0.42; blood sugar: 5.42 mmol/L; blood urea nitrogen (BUN): 2.6 mmol/L; serum creatinine: 88 μmol/L; BUN/Creat ratio: 8.5; total protein: 72 g/L; albumin: 36 g/l; ALP: 60 IU/L; ALT: 26 IU/L; AST:40 IU/L; direct bilirubin: 5.8 μmol/L; total bilirubin: 20.5 μmol/L; indirect bilirubin: 14.7 μmol/L; Gama GT: 26 IU/L; C-reactive protein: 15.6 mg/L; serum amylase: 30 U/L: electrolytes: Na: 138 mmol/L; K: 3.75 mmol/L; Cl: 102 mmol/L; urinalysis: 2-4 Leucocytes, some bacteria and some uric acid crystals. Blood group: O Rh(D) poz.The emergency laboratory tests presented as follows: Red blood cells (RBC): 4.61 × 10Three weeks prior he had been surgically treated as acute abdomen caused by acute appendicitis and Mckelly Diverticulitis. Appendectomy and short resection of the ileum with diverticulum, and end-to-end anastomosis was performed. The immediate post operative period went well. After his discharge, his second hospital admission was two weeks after operation with abdominal pain and constipation problems which released spontaneously after two days.At his third admission as acute abdomen, urgent laparotomy was performed in general endo tracheal anesthesia on the day after admission. Intra operative findings revealed small bowel dilatation, with intussusceptions of the terminal ileum, 2 cm from ileocecac valve. There were multiple enlarged mesenteric lymph nodes at the meso of the terminal ileum, ascending and the transverse colon, without palpable liver metastases.We have done des intussusceptions caused by tumor from the wall of the ileum. Fig. , 3, 4th postoperative days, with good bowel movements. He was oriented to the Hematology Department for further treatment, for Burkitt Lymphoma with chemotherapy.Adhesions at the anastomosis, and 60 cm above that, were released. The right extended haemicolectomy with end to end ileum-transverse anastomosis was performed. Post operative period went well; blood pressure was 120/80 mmHg, pulse rate beats: 65/min; temp.: 36.7°C. The wound healed per primam. He was treated with frozen fresh plasma , red blood cells (one dose); antibiotic, analgesics, H2 blockers, vitamins, amino acids, human albumins. He was discharged on the 10HP opinion: Giemsa staining demonstrated neoplastic lymphocytes infiltration. Immunochemical testing was positive for Burkitt lymphoma  and c-myc translocation determined by FISH analysis (fusion and split). Fig. , 9Sporadic Burkitt's lymphoma accounts for 1%-2% of lymphoma in adults and up to 40% of lymphoma in children in the United States and Western Europe [Estimated incidence ranges from one case per million for children less than ten years old, 0.7 per million from 10 to 20 and 0.6 per million from 20 to 30, and rare in patients older than 30 ,10.Burkitt's lymphoma is an aggressive, highly malignant and rapidly growing B-cell neoplasm frequently presenting onset in the abdomen in non-endemic Burkitt's lymphoma regions. Intussusception caused by Burkitt lymphoma, as a cause of acute abdomen, is rare, with symptoms which often mislead and make diagnosis more difficult .The rapidity of volumetric doubling of this neoplasm frequently justifies an acute abdomen presentation that may mime other less-rare diseases ,11.The clinical presentation on undiagnosed Burkitt lymphoma is very non-specific, which makes this a difficult condition to diagnose.The sporadic form commonly presents with abdominal swelling as a large mesenteric, retroperitoneal or pelvic mass, tenderness, pain or fullness. Some patients present with symptoms of bowel obstruction secondary to ileo-caecal intussusception caused by tumor growth, obstruction or bleeding, mimicking acute appendicitis ,10.Our patient had a clinical presentation of acute abdomen, with abdominal pain, distended abdomen, vomiting, and no flatulence with intestinal obstruction.Burkitt's lymphoma is more common in males than females ,12-15.In reviews that includes significant numbers of adult patients with abdominal Burkitt's lymphoma, about 33% to 50% of the patients were older than 20 ,15.Magrath's review included 97% patients younger than 15 .Burkitt's lymphoma occurring in adults, involving the terminal ileum with ileal or ileocolic intussusception's has been reported as acute abdomen required an emergency surgery reported as case or as small numbers in series, treated with small bowel resection or right haemicolectomy ,15,17-19Our case presentation is the first case of Burkitt's lymphoma with abdominal involvement, causing ileo-ileal intussusception, diagnosed and treated with extensive surgical resection here in Kosova.Surgery is required in the treatment of Burkitt's lymphoma to confirm the diagnosis and to relieve the common presenting symptoms of intestinal obstruction, abdominal mass, intussusception, or acute abdomen. Complete resection is associated with improved survival.Some reports demonstrated higher survival rate (58%-89%) in patients having extensive surgical resection versus for patients having only partial or incomplete resection (40%-45%) at 2-5 years ,15, and , and 15,However, this may merely reflect the fact that less disease is more easily completely resected .These authors broadened the role of surgery by suggesting that, even in patients with disseminated disease, the surgeon plays an important role in the secondary management of the disease.Affected terminal ileum by Burkitt's lymphoma may mimic clinically acute appendicitis and investigative tools sometimes may not provide proper diagnosis.Complete resection results in an improved patient survival.Symptoms like abdominal pain, nausea, vomiting, and problems with defecation and flatulence they started three days ago, worsening on admission day in Hospital. My skin was pale and sweats. During physical examination, my belly was distended, diffusely tender and painful. Plane abdominal radiography showed mechanical obstruction.Three weeks prior I had been surgically treated as acute abdomen caused by acute appendicitis and Mckelly Diverticulitis. Appendectomy and short resection of the ileum with diverticulum, and end-to-end anastomosis was performed. The immediate post operative period went well. After discharge, my second hospital admission was two weeks after operation with abdominal pain and constipation problems which released spontaneously after two days.At my third admission as acute abdomen, urgent laparotomy was performed in general endo tracheal anesthesia on the day after admission.th postoperative days, with good bowel movements, and oriented to the Hematology Department for further treatment, for Burkitt Lymphoma with chemotherapy.The right extended haemicolectomy with end to end ileum-transverse anastomosis was performed. Post operative period went well; the wound healed per primam. I was discharged on the 10Burkitt's lymphoma as sporadic form, and very rare in our country affecting abdomen in children and young adults, with extensive surgery and chemotherapy gives a good hope for the future of this patients.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.The authors declare that they have no competing interests.FTH, SHIH, DSK and SHIH performed the surgery and the general anesthesia.FIK has made Histopathology. FTH designed the work, the other authors contributed equally to this work.


Injuries to the pancreas by blunt trauma are uncommon. The association of pancreatic injury with acute portal vein thrombosis secondary to blunt trauma abdomen is furthermore rare. The early diagnosis of the pancreas with injury to the portal vein is challenging and difficult. These injuries are associated with high morbidity and mortality, particularly if the diagnosis is delayed. Accurate and early diagnosis is therefore imperative and computed tomography plays a key role in detection. We present a case of child with a rare combination of pancreatic laceration and acute portal vein thrombosis following a blunt trauma to the abdomen. With extensive literature search we found no such cases has been described previously. Pancreatic injury occurs in less than 5% of the major abdominal injuries either as a result of penetrating or blunt trauma. Many blunt injuries to the pancreas including injuries to the main pancreatic duct are diagnosed late, thereby ending with a high morbidity and mortality.Abdominal trauma is a rare and poorly documented cause of portal vein thrombosis. This diagnosis is made when all the other causes have been ruled out. Other causes include cirrhosis, tumors and inflammation of the abdomen, coagulation disorders and hematological diseases, including latent myeloproliferative syndrome.We report a case in which the child suffered a blunt trauma to the abdomen and developed pancreatic laceration and acute portal vein thrombosis and underwent proper management due to the prompt diagnosis by preoperative CT.An 11-year-old male child presented with acute epigastric pain following a fall on the handle of the bicycle. The clinical examination revealed signs of hypotension. His past and family history was unremarkable. Laboratory tests revealed increased ESR and increased serum amylase. There was no evidence of any myeloproliferative/hypercoagulable disorders. The X-ray chest and abdomen was unremarkable, and there was no free peritoneal air. Abdominal ultrasonography revealed doubtful laceration of pancreas in the neck region with minimal peripancreatic fluid. The rest of the abdomen appeared normal with no free peritoneal fluid. Emergency (within 6 h of the injury) contrast enhanced CT abdomen revealed a complete transection of the neck of the pancreas with peripancreatic fluid and soft tissue stranding suggestive of early pancreatitis . The addBased on the clinicoradiologic and biochemical findings, the diagnosis of pancreatic laceration with ductal injury and acute portal vein thrombosis (PVT) was made. The diagnosis was confirmed at surgery and distal pancreatectomy with external drainage was performed. The patient was kept on anticoagulant therapy for acute PVT with constant monitoring. The postoperative period and follow-up to a period of 3 months was uneventful with complete resolution of portal vein thrombus.Pancreatic injuries due to blunt trauma abdomen are relatively uncommon. In adults, they occur most commonly during vehicular accidents; however, in children, such injuries occur during bicycle accidents. Ultrasound detects focal or diffuse pancreatitis or pseudocyst, but generally it does not depict the pancreatic fracture. CT is thIn most pancreatic injuries, the attention is focused on the main pancreatic duct injury. The pres2et al, only 8 cases have been reported in the literature.[Abdominal trauma is a rare and poorly documented cause of PVT. According to Beaufort Ultrasound of the abdomen in an acute trauma patient may, in fact, miss acute PVT as hyperacute thrombus is anechoic, which is similar to the vessel lumen. The important criterion for diagnosis is thus clinical suspicion and performing the Duplex Doppler, which may reveal obstruction or alteration in the color and flow pattern. Contrast-enhanced CT is, however, diagnostic in acute thrombosis as is observed in our case. If unobserved, acute PVT may progress to chronic PVT with portal cavernoma and collateral formation with consequent portal hypertension.10 Acute To summarize, in a case of suspected or evident pancreatic injury, it is very important to rule out acute PVT to decrease the morbidity and mortality. CT provides the single, most important, noninvasive and quick tool to detect the pancreatic and related vascular injuries, particulary PVT, thereby assisting in better management.


Endometriosis is a frequent benign disorder. Malignancy arising in extraovarian endometriosis is a rare event. A 49-year-old woman is presented with a large painful abdominal wall mass. She underwent a myomectomy, 20 years before, for uterus leiomyoma. Computed tomography suggested that this was a desmoid tumor and she underwent surgery. Histological examination showed a clear cell adenocarcinoma associated with endometriosis foci. Pelvic ultrasound, computed tomography, and endometrial curettage did not show any malignancy or endometriosis in the uterus and ovaries. Adjuvant chemotherapy was recommended, but the patient was lost to follow up. Six months later, she returned with a recurrence of the abdominal wall mass. She was given chemotherapy and then she was reoperated. Endometriosis isa frequent benign disorder. Several observations of the coexistence of endometriosisand cancer have been published , 2. MaliA 49 year-old woman,gravida 3, para 0, underwent a myomectomy, 20 years before, for uterusleiomyoma through a midline incision. She is presented with a painful large abdominal wallmass. On physical examination, a firm indurated mass was palpated in the lowerabdominal wall. Abdominal and pelvic ultrasounds followed by computed tomography showed aheterogeneous intramuscular mass of 8.5 cm diameter without local extension . The cliThe diagnosis ofclear cell adenocarcinoma arising from abdominal endometriosis foci wasretained. Adjuvant chemotherapy was indicated, but the patient was lost tofollow up. She returned six months after. At that time, ultrasound and computedtomography showed a recurrent mass at the abdominal wall with extension to thebladder. Three cycles of combination chemotherapy with cyclophosphamide and cisplatinwere given, but the tumor did not regress. She underwent surgery again with aresection of 5 cm encapsulated nodule. At that time, uterus, ovaries, and tubesdid not show any abnormalities. Histology demonstrated the same type of tumor. Margins were free of tumor. Three cycles of chemotherapy were also given butfailed to control the disease; the chemotherapy she got is not precised. Thecomputed tomography showed again a recurrent mass with extension to the bladderand pelvic bone, and adjuvant radiotherapy was indicated.Endometriosis,defined as the presence of endometrial-like tissue outside the uterine cavity,is usually located in the ovaries and pelvic peritoneum , 3. PariCutaneouslocalization of endometriosis is unusual and appears most frequently insurgical scars from obstetric or gynecological interventions. It is importantto recognize the possibility of tumors arising from endometriosis when thepathologist is confronted to an extraovarian tumor with endometrial appearance. Examination of multiple sections is required to demonstrate endometriosis foci.


We report the case of a three-year-old male child who presented with a single painless subcutaneously located upper lid mass of 1.2 × 1.5 cm with a central depression. The mass could be easily separated from overlying skin on complete excision biopsy and showed a never described before whitish brain like appearance consisting of multiple lobes and gyri, which histopathologically proved to be molluscum contagiosum (MC). Tests to investigate underlying immunodeficiency did not show any evidence of immunocompromised state. Molluscum contagiosum (MC) is a skin infection caused by a member of the Poxvirus family, typically affecting young or immunocompromised persons. It usualA three-year-old child presented to our hospital with a single painless swelling over the left upper eyelid, which had been gradually increasing in size for the last five months. There was no history of trauma to the eye. He did not have any other swelling in his body. Fine needle aspiration cytology (FNAC) was done by a private practitioner two months before coming to this hospital and found to be inconclusive.Examination revealed a single firm, nontender, and 1.2 × 1.5 cm mass over the outer upper half of the left upper eyelid . The oveA skin incision was made along the lid crease, the skin was undermined over the mass which was unexpectedly easy in view of its adherence to mass on preoperative examination and the mass was easily separated from the underlying orbicularis. The mass was removed in toto and primary skin closure was done. The excised mass was white in color and, interestingly, had brain like appearance morphologically. It had multiple lobes and gyri like corrugations over its surface as shown in 3, platelet count of 3.5 lakhs/mm3 and the peripheral smear showed no atypical cell. He had blood urea of 26 mg/dl, serum creatinine of 0.6 mg/dl, serum bilirubin of 0.6mg/dL, SGOT and SGPT of 26 U/L and 32 U/L respectively, serum album of 5g/dl and serum globulin of 3 gm/dl. Serology for Human immunodeficiency Virus (HIV) and hepatitis B was negative in this patient, thus, excluding common causes of immunodeficiency.Following the diagnosis of MC, patient was systematically reviewed and several investigations were done to look for immunosuppressive condition. He had hemoglobin of 15 gm%, total leukocyte count of 6700/mm2 and then it is called giant MC. Although there are many case reports of giant MC in immunocompetent individuals elsewhere on body, Medline search revealed the only one case report of giant MC involving the eyelid in an immunocompetent patient.[In conditions with altered immunity such as atopic dermatitis, corticos4 patient.A solitary giant molluscum contagiosum of eyelid can mimic commonly found lid tumors like sebaceous cyst, dermoid cyst and other skin tumors. The characteristic central umblication of MC persists even in its giant variant, which is an important clue to differentiate it from other skin tumors. The central scar with a small oozing opening present in this case was probably due to a previously done fine needle aspiration cytology.Complete excision of the mass provided us with a peculiar white brain like appearance of the mass having multiple lobes and surface corrugations , which tVarious other described treatment modalities for MC include cryotherapy with liquid nitrogen, extirpation followed by cauterization of the base with electro-dessication, or chemical agents such as silver nitrate, phenol and trichloroacetic acid. Resistant cases in immunocompromised patients have been treated with topical antiviral agent cidofovir (5%) or intralesional and systemic interferon alpha, imiquimod 5% cream.Solitary giant MC, although a rare condition in immunocompetent patients, should be suspected if central umbilication is found in the lid tumor. Complete excision of the mass is an easy and effective mode of treatment and white brain like appearance of the removed tissue may further point towards the diagnosis of MC.


Gastrointestinal stromal tumours are rare (GIST). However, the incidence of GIST among neurofibromatosis type 1 (NF-1) patients is approximately 3.9-25%. GIST can present clinically in different ways such as abdominal pain, gastrointestinal bleeding and obstruction.We present 51 year female patient admitted with Background of neurofibromatosis type 1 admitted with melena. OGD has been done and showed duodenitis with large volume fresh blood in distal duodenum but no obvious bleeding point. Exploratory laparotomy revealed smooth nodular masses on the serosal surface of jejunum. Small bowel resection and side-to-side anastomosis were performed. Histopathoogical examination revealed small bowel gasrointestinal stromal tumour with low risk malignant potential.The incidence of GIST among neurofibromatosis type 1 (NF-1) patients is not uncommon and we should pay attention to gastrointestinal manifestation in such patients. Neurofibromatosis is an autosomal dominant disorder that affects all 3 germinal layers; thereby affecting any organ system. The National Institutes of Health (NIH) Consensus Development Conference has defined 2 distinct types: the commoner of the two, neurofibromatosis type 1 (NF1) affecting 85% of patients; and neurofibromatosis type 2 (NF2) . The secWe report the case of a 51 year female patient with NF-1 who presented with lower GI bleeding caused by a jejunal GIST.th part of duodenum and upper jejunum from intestinal mass. She was subjected to emergency laparotomy. At surgery, smooth nodular masses were found on the serosal surface of the first 50 cm of jejunum; these were 3-4 cm and projected into the lumen having eroded the mucosal surface. Resection of the affected segment and end to end anastomosis was performed. The rest of the bowel appeared free of any masses and after concluding surgery the patient was transferred to ITU postoperatively. She made an uncomplicated recovery and was discharged 7 days after operation. A subsequent CT scan did not reveal any other masses.A 51 year old lady with NF-1 as she has multiple neurofibromas and seven café au lait macules larger than 5 mm. Presented to A&E with a 24 hour history of malena. She was on warfarin for aortic root and valve replacement surgery she had in the previous year. Although she remained haemodynamically stable, her haemoglobin levels gradually dropped 4 units over the next 36 hours. Along with correction of her clotting function, investigations were initiated that included an upper GI endoscopy which revealed blood in the second part of duodenum. Due to a rapid deterioration in her haemodynamic status colonoscopy was cancelled and a mesenteric angiogram was done that suggested bleeding from the region between the 4Histopathology revealed an 8 cm length of jejunum involved with tumour nodules, smallest 3.4 cm and largest 4.4 cm, composed of spindle and polygonal cells. The tumour involved the full thickness of the bowel wall and there was no spread of disease to lymph nodes. Immunohistochemistry demonstrated positive staining for CD117 (cKit)  in small bowel, arising from the submucosa Figure 2- The tumour is composed of spindle cells with plump nuclei and abundant eosinophilic cytoplasm with fibrovascular stroma Figure Our patient possessed the known c-KIT mutation that does not appear typical of NF-1 associated GISTs from review of literature. It may be that our patient developed a GIST by co-incidence rather than as an association with NF-1. However the finding of a low malignant potential GIST in our patient based on mitotic count is consistent with other NF-1 associated GISTs reported, along with other features like young age and female preponderance .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.The authors declare that they have no competing interests.EA participated in the sequence alignment and drafted the manuscript. MK participated in the sequence alignment. OD participated in the design of the study. RC conceived of the study, and participated in its design and coordination. All authors have read and approved the manuscript.


The purpose was to highlight the diagnosis and treatment of extra-adrenal para-gangliomas, which often causes catecholamine hypersecretion and hypertension.67 cases of extra-adrenal paraganglioma of retroperitoneum proven pathologically from 1999 to 2009 were reviewed and studied after operation. Endocrine secretion examinations, B-US, CT, MRI, 131-MIBG, octreotide and hands microcirculation inspection were used to diagnose the disease.All patients underwent successful surgical resection of the tumors, which proved to be paragangliomas. They were from 3 cm to 25 cm in size. Almost all of them were diffusely positive for cgA, syn, NSE and s-100 by immunohitochemical staining. There were nine cases assayed malignant paraganglioma by the follow-up.131-MIBG and octreotide have high sensitivity and accuracy in diagosing extra-adrenal paraganglioma. Surgical treatment should be carried out on the basis of correct drug preparation of α-receptor blocker, such as prazosin and phenoxybenzamine. Complete surgical excision is the treatment of choice for extra-adrenal paragangliomas as well as recurrent or metastatic disease, which could be resected laparoscopically. Intimate lifelong follow-up is necessary and important. Extra-adrenal retroperitoneal paragangliomas (PGLs) arise from dispersed paraganglia that tend to be symmetrically distributed in close relation to the aorta and sympathetic nervous system. They are rarely encountered in every day surgical practice. Meanwhile, Extra-adrenal retroperitoneal paragangliomas are rare tumors causing considerable difficulty in both, diagnosis and treatment. They can be unicentric or multicentric, tend to be locally invasive and, therefore have a high incidence of local recurrence. Histological and immunological phenotype of silent extra-adrenal retroperitoneal paragangliomas are no significant difference with functional ones. In this article, we reviewed the experience in our hospital of this uncommon tumors to highlight the diagnosis and treatment of extra-adrenal paragangliomas.This was a retrospective single-institutional study. We identified 67 adult patients  with extra-adrenal PGLs of retroperitoneum between 1999 and 2009 in PeKing Union Medical College Hospital (PUMCH). The number of silent extra-adrenal paraganglioma of retroperitoneum was 18 % of all 67 retroperitoneal paraganglioma resected by operation during this period of time in PUMCH. Patients with silent extra-adrenal retroperitoneal PGLs were older than those of fuctional tumors with more tumor diameters . The cliEndocrine secretion examinations, B-US, CT, MRI, 131I-MIBG, and octreotide were performed for preoperative diagnosis. US can be a simple and effective diagnostic method. The diameter of adrenal masses as measured by US correlates highly with mass diameter measured by CT. FDG-PET has been suggested for the characterization of adrenal masses in patients. At the same time urine UFC, serum F, blood ACTH, size of the dose dexamethasone suppression test, blood renin - blood vessels angiotensin - aldosterone, and other tests were used to rule out other diseases. Hands microcirculation inspection was used to understand systemic microcirculation. Preoperative management is aimed at prevention of possible catecholamine-induced complications like hypertensive crisis, cardiac arrhythmias, pulmonary oedema and cardiac ischemia due to manipulation of the tumor, and especially to prevent hypotensive crises after tumor removal. We prefer to use an adrenoceptor blocking agent for 2–4 weeks.The medical case records were also reviewed. Pathological evaluation was performed with HE staining and S-P immunohistochemical test by two pathologists. Positive CgA, Syn, NSE staining was diffuse cytoplasmic brown granules, while positive S-100 staining was in the nucleus and the cytoplasm.[Hypertension were observed in 55 functional extra-adrenal retroperitoneal paragangliomas before operation, who were admitted to hospital because of headache, palpitations and diaphoresis.While silent tumors were admitted to hospital by routine physical examination or tumor-related pain, no abdominal mass was palpable in all patients. Six patients had been found with familial history.The tumors were from 3 cm to 25 cm in size. MSCT shows a round or similarly round retroperitoneal tumor with inner central hemorrhage and necrosis. Tumors show obvious intensification after injection of contrast medium. Figures –B. All pThe cut surface of the tumor showed integrated amicula and abundant blood supply. There was a large area remote hemorrhage and necrosis inside of tumor Figures –B. UnderExtra-adrenal PGLs of retroperitoneum are neoplasias of the chromaffin cells which are located in the para-aortic sympathetic chain, aortic bifurcation. They oftAlmost all the patients in our study were found PGLs of retroperitoneum by US in physical examination according to medical records. However, the US depends to a large extent on operator skills. Furthermore, obesity and overlying gas are obstacles for the visualization. Unsurprisingly, the US does not detect tumors with the same sensitivity as CT or MRI. The tumors usually appear as rounded or oval masses with a similar density to the liver on unenhanced CT. Larger lesions may show a cystic component due to central necrosis or hemorrhage. Calcification is present in some cases. Owing to their hyper-vascularization, the tumors usually exhibit intense enhancement. The tumors are generally characterized by low T1 and bright T2 signal intensities. Central necrosis is frequently observed. There are no signal changes from out-of-phase to in-phase images. For the localization and identification of PGLs of retroperitoneum, the radio-pharmaceuticals 131I-MIBG and 111in-octreotide have been most commonly used. The sensitivity of 131I-MIBG for detecting PGLs ranges between 80 and 90%, with a specificity of 90–100%.Most malignant tumors show an enhanced glycolytic metabolism with increased up-take of deoxyglucose that can be visualized by PET using18F-2-fluoro-d-deoxyglucose (FDG). However to date, there are insufficient data to justify the use of PET to diagnose clinically silent extra-adrenal PGLs of retroperitoneum outside clinical studies. We are engaged in the relevant research with help of PET center of our hospital.Hypertension and urinary catecholamine was usually elevated in extra-adrenal PGL of retroperitoneum. The measurement of plasma catecholamines is not recommended because this method has poor sensitivity and specificity, which often leading to false-positive results. Urine metanephrines have a higher specificity, receiver operating characteristic curves revealed a better test performance than other biochemical tests. Special Almost all our patients were performed a complete surgical resection without hypertensive crisis, who were took α-receptor blocker for 2-4 weeks preoperatively. Among 12 silent extra-adrenal PGLs of retroperitoneum, four cases were operated without pharmacological preparation, two of whose blood pressure were observed rising close up to 27/16 kpa transiently. This shows that silent extra-adrenal retroperitoneal paragangliomas is nonfunctional relatively, In the practical clinical work, it is possible to result in severe consequences if the preoperative awareness is not enough. Randomized controlled trials for preoperative drug treatment are lacking temporarily.Surgery remains the mainstay of treatment of extra-adrenal PGLs of retroperitoneum. However, it must be born in mind that the "silent" extra-adrenal PGLs of retroperitoneum is silent ones relatively in the handling of these tumors. Carrying out of physical maneuvers on the tumor and the employment of drugs that free catecholamines can induce hypertensive crises. Once the diagnosis and drug preparation are accomplished, attempt should be made to perform a complete surgical resection. Resection is often challenging as these highly vascular tumors are located near multiple vital blood vessels.Initially, Almost all PGLs of retroperitoneum (10/11) were performed via the open trans-abdominal route in our hospital, especially before 2003. With the development of laparoscopic technique, we applied the technique to resect PGLs of retroperitoneum (32/56) successfully. Initial indications were limited to small tumors due to concerns about bleeding, the safety of removing tumors . As we gained experience, indications for laparoscopic resection expanded to include large tumors more than 8 cm Figures –B. We alExtra-adrenal retroperitoneal PGLs have a more aggressive course than their adrenal counterparts, which are called pheochromocytomas. The prognosis of PGLs can be very difficult to predict. However, their anatomical location may influence their biological behavior: carotid body PGLs have a relatively low rate of malignant behavior while retroperitoneal PGLs tend to have a higher incidence of malignant transformation. Approximately 20–42% of PGLs metastasize, compared to only 2–10% of adrenal pheochromocytomas. Dissemination occurs both lymphatically and hematogenously, with the most common sites of metastasis being the regional lymph nodes, bone, liver, and lung. Distant metastasis to bones, lymph nodes and lungs has been reported as a unique feature of the metastatic spread of extra-adrenal retroperitoneal PGLs. DiagnosiUp to 36% of the extra-adrenal sympathetic PGLs were malignant. Consequently, not only metastasis but also local invasion should be carefully evaluated when making a diagnosis of sympathetic PGL. There were nine cases which assayed malignant PGL by follow-up. A total of six cases with paraganglioma infiltrating the surrounding lymph nodes were diagnosed as malignant tumors, two were found relapsed 2–4 years after operation; one case of bone metastasis occurred five years after operation. These may indicate the disease has a tendency to relapse and may be multi-center results. Therefore, intimate follow-up is necessary and very important. Treatment with therapeutic doses of 131I-MIBG or combination chemotherapy  may induce  responses to malignant PGLs.Taken together, A heterogeneous, hypervascular, retroperitoneal mass with areas of necrosis with typical clinical setting of headaches, hypertension and tremor, is highly predictive of the presence of an extra-adrenal PGLs. 131I-MIBG and octreotide have high sensitivity and accuracy in diagnosing silent extra-adrenal PGL. Once the correct diagnosis is acquired, surgical treatment should be carry out after using alpha-adrenergic blockade for 2–4 weeks in order to prevent and treat a syndrome of possible intra-operative catecholamine release. Tumors should be resected laparoscopically, if possible. Future efforts should be directed toward obtaining a larger database to define the true natural history of extra-adrenal retroperitoneal PGLs. More prospective clinical trials are needed to provide the most reliable evidence regarding the management of patients.


Abscess of the spleen is a rather clinical rarity with reported mortality rate up to 47%. The timely and widespread use of imaging methods facilitates early diagnosis and guides treatment, thus improving the prognosis. Most of patients were with recognized risk factors including conditions that compromise the immune system, trauma and intravenous drug abuse in addicts. The surgical treatment by splenectomy is usually the first choice of treatment.A healthy 45-year-old woman presented to the outpatient clinic with fever, 39°C together with persistent upper-left-quadrant abdominal pain. Her past medical history was free from any chronic debilitating diseases or other predisposing factors. Imaging included chest and abdominal X-ray, followed by a CT scan of the upper abdomen.Splenic abscess is an unusual and potentially life-threatening disease with a diagnostic challenge due to the nonspecific clinical picture and diagnosis confirmed based mostly on imaging studies. Multiple splenic abscesses are very rarely encountered in surgical practice with a reported high mortality rate in neglected and untreated cases. Splenectomy is a safe procedure for patients with splenic abscess. Abscess of the spleen is a rather clinical rarity as only about 600 cases have been described so far in the international literature ,2. Most The surgical treatment by splenectomy is usually the first choice of treatment, but image-guided percutaneous drainage is also a therapeutic option in selected cases .A healthy and otherwise normal 45-year-old woman from the rural middle Delta in Egypt presented to the outpatient clinic with fever, 39°C together with persistent upper-left-quadrant abdominal pain. She was a heavy-duty worker as a farmer, helping her husband nearly all the day time.Her past medical history was free from any chronic debilitating diseases such as diabetes, liver or renal diseases. Searching for other predisposing factors known to cause splenic abscesses was negative such as metastatic and distant hematogenous infection and a contiguous site of infection. Moreover, the patient recent medical history was free from any surgical operation, recent hospital admission or trauma.Clinical examination reproduced localized pain and tenderness in the left hypochondrium together with coated tongue and toxic face. The patient radial pulse was 110 beats/minute. Constitutional symptoms as nausea and anorexia were present.Laboratory testing for hematological and biochemical controls was performed and revealed leukocytosis while other tests were otherwise within the normal ranges.Imaging study included chest and abdominal X-ray, followed by abdominopelvic ultrasonogam and CT scan of the upper abdomen. Both chest X-ray and CT scan of chest denoted clear lung fields and free costophrenic angles. Both ultrasonography and CT scan showed mild enlarged spleen with multiple well-defined cystic focal lesions; the largest measure 2 × 1.5 cm , 2. LiveThe patient underwent laparotomy for splenectomy under general anesthesia. Spleen was found mildly enlarged with three small abscesses pointing through the serosal surface. Surgery was completed with smooth operative course and the patient has been found healthy with complete relief from all disease-associated symptoms in the early postoperative period. Operative and post-operative photographic pictures were taken showing the enlarged spleen in situ and the abscesses on splenic surfaces as well as the cavity of the abscesses when laid open , 5, 6.The histopathological study of the spleen revealed that the cause of these multiple splenic abscesses was nonspecific inflammatory process and the bacteriological study denoted that pathogens detected include staphylococcus and streptococcus.In the early postoperative period, within 2 weeks, the patient was referred to our internist and hematologist for proper immunization program and antibiotic prophylaxis.Splenic abscess is a rare entity, with a reported frequency of 0.14-0.7% in autopsy series. Its reported mortality rate is still high, up to 47%, and can potentially reach 100% among patients who do not receive antibiotic treatment. Appropriate management can decrease the mortality to 14% ,8,9.The timely and widespread use of imaging methods facilitates early diagnosis and guides treatment, thus improving the prognosis. Ultrasound is used as a preliminary diagnostic modality, which is often followed by CT scan . HoweverIn the present case, the author found that abdominal ultrasonography could diagnose the presence of multiple splenic focal cystic lesions and the same finding was confirmed by C T abdominal scan.The finding of this imaging study was suggestive specially when correlated with clinical data including left upper abdominal pain, fever and other constitutional symptoms ,6,8.There is often overlap in the imaging appearance alone, so the clinical setting is very helpful in differential diagnosis. Common symptoms in patients with splenic abscess are fever, abdominal pain and nausea and vomiting ,12.Solitary splenic lesions are unusual  and multAn interesting recent study published in 2008 stated that abscess of the spleen is a rare discovery and put a hint about this rarity as only 600 cases have been described so far in the international literature [To clarify the rarity of splenic abscess in clinical aspect, the medical records of one institution were retrospectively reviewed and only six cases of splenic abscesses seen within ten years between 1989 and 1999 were identified . Also, rMost of these patients, if not all, according to these studies and others of same interest, are with recognized risk factors ,5-8. TheOn the other hand multiple splenic abscesses of unclear aetiology are a very rare finding in clinical practice .The treatment modalities of splenic abscess are antibiotic therapy whether in conjunction with splenectomy, percutaneous drainage or aspiration or antibiotic therapy alone ,8. But bThe patient of this case gained benefit from splenectomy and passed a very smooth postoperative course where the fever declined dramatically and all the constitutional symptoms disappeared earlier within the first 48 hours after surgery.Although overwhelming postsplenectomy sepsis is a rare event, it is devastating and often lethal . GuideliSplenic abscess is an unusual and potentially life-threatening disease with a diagnostic challenge due to the nonspecific clinical picture. Splenic abscess should be suspected in febrile patients with left upper quadrant tenderness and leukocytosis, and diagnosis confirmed based mostly on imaging studies.Multiple splenic abscesses are very rarely encountered in surgical practice with a reported high mortality rate in neglected and untreated cases. High index of suspicion and liberal use of imaging studies is essential for timely diagnosis. Splenectomy is a safe procedure for patients with splenic abscess.


Ollier's disease is a rare nonhereditary disorder characterized by multiple enchondromas with a predilection for unilateral distribution. Malignant changes in Ollier's disease may occur in adult patients. Radionuclide bone scanning is one method used to assess lesions depicted on radiographs or magnetic resonance images that are presumed to be enchondromas. Also, a bone scan may give a clue to the multifocal nature of the disease and it has been recommended that scintigraphy is useful in the monitoring of lesions and the development of any malignant transformation.99mTc-MDP) scan showed multiple foci of increased activity involving the proximal and distal right femur and tibia, proximal right humerus, distal right ulna, right metacarpals, metatarsals and phalyngeal tubular bones, consistent with unilateral distribution of the lesions. The long bones of the left hemi-skeleton were unremarkable, representing unilateral involvement of the skeleton. In this case, the intensity of uptake in the lesions of the lower extremity was high, raising the possibility of malignant degeneration of the enchondromas. Hence, the patient underwent surgical excision of the suspected lesions. Pathology analysis revealed their benign nature.A 21-year-old man with a history of pathologic fractures of the right tibia and multiple limb surgeries related to Ollier's disease was referred to our nuclear medicine department. Radiographic assessment showed multiple radiolucent expansile lesions, suggestive of multiple enchondromas. A whole-body bone (Although the malignant transformation of enchondromas is a well known phenomenon, it should be kept in mind that other etiologies can be considered as the cause of intensely increased uptake. Retrospective assessment of our patient revealed that the etiology of increased uptake in the lower limb lesions was due to previous insufficiency fractures and the possibility of malignant transformation was ruled out based on the pathology findings. Ollier's disease, a rare nonhereditary disorder characterized by multiple enchondromas with a predilection for unilateral distribution , was iniThe disease is usually detected during early childhood . NotableRadionuclide bone scanning is one method used to assess lesions depicted on radiographs or magnetic resonance images that are presumed to be enchondromas . Also, aA 21-year-old man presented with a history of pathological fractures of the right tibia and multiple incidences of limb surgery related to this rare dysplasia. Radiography assessment showed multiple radiolucent, expansile, homogenous lesions with an oval or elongated shape and well defined, slightly thickened bony margins, suggestive of multiple enchondromas  In this case, the intensity of uptake in lesions of the lower extremity was so high that the possibility of malignant degeneration of the enchondromas was raised . Hence, Chondromas are benign tumors of hyaline cartilage. They may arise within the medullary cavity, where they are known as enchondromas. In fact, enchondromas are the most common cause of intraosseous cartilage tumors. They are most frequent from the 20th to the 40th year of age. The cartilage tumors in enchondromatosis are asymptomatic and are detected as incidental findings. However, the cartilage tumors in enchondromatosis may be numerous and large, producing severe deformities. The radiography features are characteristic, as the unmineralized nodules of the cartilage produce well-circumscribed oval lucencies that are surrounded by a thin rim of radiodense bone (O-ring sign). If the matrix calcifies, it is detected as irregular opacities.It has been emphasized that Ollier's disease usually stops spontaneously with skeletal maturity; therefore, any lesion showing activity or increased uptake after termination of the growth period requires thorough examination ,5. ScintAs was mentioned by Silve and Juppner, the histopathological criteria for malignancy, which are used for conventional chondrosarcomas, cannot be applied for Ollier's disease because of the increased cellularity; hence, distinguishing enchondromas from grade-I chondrosarcomas in the context of enchondromatosis is extremely difficult or even impossible . The diaRetrospective assessment of our patient revealed that the etiology of increased uptake in the lower limb lesions was due to previous insufficiency fractures and the possibility of malignant transformation was ruled out based on the pathology findings.Although the malignant transformation of enchondromas is a well known phenomenon, it should be kept in mind that other etiologies should also be considered as the cause of intensely increased uptake.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.BF revised the article for intellectual content details and helped to draft the manuscript. AG participated in writing of the manuscript and interpretation of the scintigraphic figures. KA, MB and DB supervised the acquisition process and interpreted the scintigraphic and radiological images. All authors read and approved the final manuscript.


Vascular leiomyosarcoma are rare tumors typically originating from the inferior vena cava (IVC). Due to nonspecific clinical signs most tumors are diagnosed at advanced stages. Complete surgical resection remains the only potential curative therapeutic option. Surgical strategy is particularly influenced by the level of the IVC affected. Due to the topographic relation to the renal veins level-II involvement of the IVC raises special surgical challenges with respect to the maintenance of venous outflow.We herein report two cases of leiomyosarcoma of the IVC with successful en bloc resection and individualized caval reconstruction. One patient presented with a large intramural and intraluminal mass and received a complete circumferential resection. Reconstruction was performed by graft replacement of the caval segment affected. The other patient displayed a predominantly extraluminal tumor growth and underwent semicircumferential resection of the IVC including the confluence of the left renal vein. In this case vascular reconstruction was performed by cavoplasty and reinsertion of the left renal vein into the proximal portion of the IVC. Resection margins of both patients were tumor free and no clinical signs of venous insufficiency of the lower extremity occurred.This paper presents two cases of successfully managed leiomyosarcomas of the vena cava and exemplifies two different options for vascular reconstruction in level II sarcomas and includes a thorough review of the literature. Primary vascular leiomyosarcoma is a rare tumor with less than 300 cases reported. It originates from the smooth muscle cells of the media and predominantly arises within the inferior V. cava (IVC) . While iDue to the absence of early symptoms, retroperitoneal tumors are often not diagnosed until the disease is at an advanced stage with large tumor growth and involvement of surrounding structures. Clinical symptoms are unspecific, and most patients present with abdominal or flank pain , which iBy reason of the poor long-term prognosis and the surgical risk, the involvement of large vessels has traditionally been considered a limiting factor for resection of retroperitoneal tumors . Yet advHowever, the surgery that is required to accomplish complete tumor resection is challenging. The goals of surgical management of these tumors include the achievement of local tumor control, maintenance of caval flow, and the prevention of recurrence. The surgical strategy, however, is not only influenced by the level of the caval segment that is affected, but also by the extent of retroperitoneal collateral circulation, and by the topographic involvement of neighboring structures. In particular, the involvement of renal or hepatic veins dictates the strategy for vascular reconstruction.The surgical management of partial resections of the IVC is a matter of current debate and includes ligation, primary repair/cavoplasty, or replacement with a graft. Reconstruction of the IVC is not always required, because gradual occlusion of the IVC allows the development of venous collaterals. However, when pararenal leiomyosarcoma of the IVC is present, reconstruction of the IVC and the renal vein is necessary to prevent transient or permanent renal dysfunction .We herein report two cases of leiomyosarcoma of the IVC with emphasis on the surgical procedure and reconstruction of caval continuity.For more than seven years a 34-year old male patient had been complaining about recurrent discomfort of the upper abdomen and pain emanating to his back. Due to an increase of the preexisting disorders an abdominal CT-scan was conducted, which showed a cystic retroperitoneal tumor figure .The patient was referred to us with the suspicion of a pancreatic neoplasia. After reviewing the CT scans we suspected a retroperitoneal neoplasia and completed staging which showed no distant metastases. An exploratory laparotomy was performed. However, no pancreatic mass was palpable intraoperatively. After mobilization of the right hepatic lobe and the right colic flexure, the tumor was located between the inferior vena cava and the left renal vein compressing and infiltrating those vessels. To obtain cranial and caudal control, the IVC was longitudinally exposed and secured with vessel loops. Subsequently, the IVC was gradually clamped. Prior to exclusion of the venous segment the patient received heparin intravenously. Due to the predominantly extravascular tumor growth figure  partial Intraoperative assessment of the surgical margins by frozen section confirmed that a complete R-0 resection had been achieved. Vascular reconstruction was performed by a running suture of the caval resection margin and by reinsertion of the left renal vein into the proximal portion of the IVC at the site of the suture, since the length of the remaining left renal vein was sufficient after mobilization for a tension-free anastomosis. Intraoperative duplex ultrasound of the cavoplasty presented a good venous flow in the reconstruated vessels.The postoperative course was uneventful, without impairment of the renal function or swelling of the lower limbs. Due to the vascular surgical procedure, the patient was therapeutically anticoagulated with heparin. Histopathology revealed a lowly differentiated leiomyosarcoma with an extension of approximately 7 cm in diameter; the resection margins were tumor-free. After interdisciplinary discussion of the case adjuvant radiotherapy was conducted with 57.4 Gy in particular due to the advanced tumor size.During the first follow-up after three months, the patient presented in good general condition. A CT-scan of the thorax and an abdominal MRI showed no indication for tumor recurrence. Under sustained anticoagulation with warfarin, the reconstructed vessels constituted without any pathological findings. Yet after six months the patient was still free of local recurrence, however, CT-scan showed a central hepatic lesion. A subsequently performed explorative laparotomy revealed inoperable disseminated hepatic metastases. According to interdisciplinary consent, the patient received regional hyperthermia as well as chemotherapy including ifosfamide, adriamycine, and etoposid during the following months.We furthermore report on the case of a 58-year-old female patient who was referred to our department with a retroperitoneal tumor which was diagnosed in an external hospital. This finding had resulted from an abdominal ultrasound, which was carried out because of unspecific abdominal pain. A CT-scan described a tumor that likely originated from the IVC, however not being clearly distinguishable from the right adrenal gland.adrenalecomy en bloc was required. In contrast to patient 1, this tumor presented with a larger proportion of intravascular growth. Therefore, adequate oncologic resection required a complete circumferential resection of the IVC in addition to a right adrenalectomy en bloc, which was performed. Because the caval confluences of the renal veins were unaffected by the tumor on both sides, approximately 3 cm of the entire circumference of the infrahepatic IVC were resected as far as slightly above the renal confluences. Due to the low pressure in the IVC, the vascular continuity was reconstructed using a ring-enforced PTFE prosthesis 19 mm in diameter to optimize caval flow.After mobilization of the liver, including segment one, the subsequent intraoperative finding revealed a large inferior caval tumor with an infrahepatic suprarenal localization that was consistent with the radiological statement. The tumor could not reliably be separated from the right adrenal gland. Thus, apart from a partial resection of the IVC, a right Because of the exogenous material implanted, the patient also received effective anticoagulation with heparin. On the second postoperative day, a relaparotomy was required under clinical suspicion of a secondary hemorrhage. Whereas this was confirmed, and hematomas in the right upper abdomen as well as within the omental bursa were detected, exploration of the entire abdomen could not reveal an origin of the bleeding. In particular, the exposure of the prothesis, including the anastomosis showed proper conditions. Thus, besides the removal of hematoma with abdominal lavage, no further intervention was performed. During the following course the patient recovered without complications.Histopathological examination showed a moderately differentiated caval leiomyosarcoma. with a maximum extension of 4 cm in diameter, and almost complete luminal obliteration, as well as tumor-free resection margins. In compliance with interdisciplinary consent, no adjuvant therapy was indicated. The follow-up examinations currently lasting up to a year after surgery showed no signs of tumor recurrence.Curative surgical resection remains the current treatment of choice for primary leiomyosarcoma of the IVC. A major surgical issue is the need for venous reconstruction. Basically, ligation of the IVC, cavoplasty, and graft replacement represent the major therapeutic options. However, surgical strategy is particularly influenced by the level of the IVC affected. When dealing with the infrarenal level 3 of the IVC, simple ligation has been found to yield good functional results with the assumption that the slowly growing tumor allows sufficient collaterals to develop. After extensive curative resection with disruption of collaterals or if only few collaterals have developed before surgery, however, ligation of the IVC may cause lower limb edema with significant functional impairment . We consActually, there is considerable controversy about the type of caval reconstruction. Several authors recommend prosthetic replacement, but others often perform cavoplasty or ligation of the IVC . In leveIf reconstruction of the renal vessels is possible and complete resection of the tumor is achieved, the surgeon should try to maintain renal function.Prosthetic replacement, which enables circumferential resections is favored because of a more radical approach. This technique is predominantly applied in patients with large intramural and intraluminal tumors. However, this procedure, at least theoretically, carries an increased risk of pulmonary embolism as well as further graft-related major complications such as sepsis, graft occlusion and graft-enteric fistulas ,7. PartiSo far there is neither evidence for an increased risk of thromboembolic complications after prosthetic replacement nor for a protective impact by effective anticoagulation. Whether long term anticoagulation is truly required is still under debate since it also carries the potential of hemorrhage. Yet cavoplasty and in particular foreign material in low flow venous segments predispose to thrombosis and potential embolization. Several cases of postoperative graft occlusions were reported ,12. ThusThe use of arterio-venous-fistulas represents another possibility to optimize caval patency. However, the effect of this procedure is yet controversial, since the potential benefits might not outweigh the risks. Complications such as cardiac insufficiency or local complications should be considered. Besides, we share the view of several authors stating that arterio-venous-fistulas are not required if the suprarenal IVC is reconstructed because of the high-volume blood flow at this level ,12.Leiomyosarcoma is reported to have a poor prognosis. Over half of patients who underwent radical resection develop tumor recurrence, and the 5-year survival rate ranges between 31 and 62% [To improve the outcome of patients with leiomyosarcoma of the IVC, adjuvant and neoadjuvant treatment protocols are applied with promising results . HoweverSurgery, whether performed alone or in combination with adjuvant therapy constitutes the only hope of prolonged survival. For this reason, we recommend aggressive surgical management by using modern vascular surgical and oncological techniques.Written consent was obtained from the patients for publication of these case reports. A copy of the consent is available with Editor in Chief.The authors declare that they have no competing interests.AA was involved in the conception, design and preparation of manuscript, literature review, substantial intellectual contribution. ARe was involved in manuscript preparation and thorough review of manuscript, literature review and substantial intellectual contribution. Ara was involved in the initiation of report, critical revision of manuscript. CP was involved in the histopathological work up and contribution to pathology part of manuscript. WTK was involved in the initiation of report, performed surgery, through review and drafting of manuscript, substantial intellectual contribution. CFE was involved in the initiation of the report, important intellectual contribution drafting and review of manuscript. All authors read and approved the final manuscript.


A peri-menopausal woman presented with symptoms and signs suggestive of fibroids. She was fit and healthy with no significant past medical history. She consented to having a hysterectomy but her surgery was performed prior to any diagnostic imaging being done.At surgery there was an unexpected finding of disseminated carcinoma, diffusely infiltrating the uterus, fallopian tubes and ovaries. There was an omental cake that was biopsied. Frozen section showed signet ring cells, suggesting bowel carcinoma. Further intra-operative examination revealed a caecal tumour. After surgery she was investigated further and eventually referred for palliation, due to her advanced disease. A 46-year-old Caucasian woman presented to the gynaecology outpatient's department complaining of menorrhagia, bladder pressure and a feeling of heaviness in the pelvis. She had recently also started experiencing deep dyspareunia. She reported no bowel or constitutional symptoms.She was a housewife and was a teetotal, non-smoker. She had one child, born by vaginal delivery and her previous cervical smears were normal. She was healthy and had no significant medical, surgical or family history. Examination revealed an enlarged, fourteen-week size uterus. There were no adnexal masses felt. In view of her symptoms, she was counselled about the various treatment options for menorrhagia. She declined all conservative measures and was very keen on having a total abdominal hysterectomy. An ultrasound scan was requested and her operation was booked.Before diagnostic imaging could be performed, she attended hospital for her hysterectomy. Surgery was done through a Pfannenstiel incision, which revealed some haemorrhagic fluid in the abdomen. This was sent for cytology. The uterus was found to be uniformally enlarged, the fallopian tubes looked thickened and the ovaries appeared to have carcinomatous deposits on their surface.The upper abdomen was explored. The omentum was caked with tumour, a biopsy was taken and frozen section performed. This showed deposits of mucin with some signet ring cells – suspicious of carcinoma of the bowel. The surgical consultant was called, at which time examination demonstrated a palpable tumour in the caecum. A decision was taken to proceed with total abdominal hysterectomy and bilateral salpingo-oophorectomy and to defer hemi-colectomy to a later date, until further investigations could be performed.Subsequent gastroscopy was normal but her colonoscopy was limited due to looping of the bowel. A computerized tomography scan of the chest, abdomen and pelvis revealed no mediastinal nodes or lung parenchymal deposits. Her upper abdomen was normal. However there was thickening of the right para-colic area and caecal pole consistent with local infiltration.Histology of her pelvic organs showed extensive infiltration of the cervix, basal endometrium, myometrium, ovaries and tubal tissues by poorly differentiated adenocarcinoma, with a signet ring pattern showing mucin production. This was identical to the omental biopsy. The impression was that these appeared to be metastases rather than intrinsic lesions.The patient was later referred for palliative chemotherapy as the tumour was felt to be incurable. She subsequently died of her disease some twelve months later.Bowel cancers invade adjacent structures dependent on their site. Ascending colon tumours tend to invade the duodenum and liver, whilst rectal tumours are more likely to invade the genital organs . In geneOur case report re-iterates the importance of considering bowel pathology in the differential diagnosis of all patients presenting with gynaecological symptoms. These days, it is vitally important to bear in mind that diagnostic imaging must be obtained prior to any surgical intervention, as we run the risk of missing serious pathology is we omit this step.In this case imaging prior to surgery may not have changed the eventual outcome, but it may have triggered further investigation, which could have led to the correct diagnosis prior to surgery.Written informed consent was obtained from the patient's next of kin for publication of this case report. Unfortunately the patient has died as a result of her condition. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.Both SD's and HS were integrally involved in the patient's management and diagnosis. All also contributed to writing of the case report. LI performed the literature search and final copy-editing the paper. All authors read and approved the final manuscript.


Fibrous histiocytoma of larynx is a rare entity. We herein present a 35-year-old femaleof benign fibrous histiocytoma oflarynx with severe airway obstruction that requiring urgent endotracheal intubation followed by tracheostomy. We also report the good long-term survival of this case after such a critical condition. Fibrous histiocytoma is uncommon in the head and neck region and rare in the larynx. Herein w2 40.3 mm Hg, PaCO 2 85.4 mmHg and oxygen saturation of 64.7%. Endotracheal intubation was done with great difficulty because of huge supraglottic lump and very narrow glottic opening, with endotracheal tube size 6 mm and she was put on mechanical ventilation. Fluid resuscitation was given followed by nor-adrenaline infusion along with antibiotics and other supportive treatment.A 35-year-old female was admitted to the ICU of Era’s Lucknow medical college, in a gasping condition. On examination, she was cyanosed with cold peripheries. Her blood pressure was not recordable and on palpation, carotid pulse was very feeble. Arterial blood gas analysis revealed PaO On detailed evaluation of previous records, she was having fever (low grade), cough, change in voice and breathing difficulties for last two months. She had consulted a local physician and he diagnosed her as a case of laryngeal tuberculosis based on history and positive mantoux test (indurations 12 mm). Previous chest radiograph revealed no abnormality. ComputeShe gradually improved and weaned off from the mechanical ventilation and was referred to Sanjay Gandhi Institute of Medical Sciences for further management where biopsy was taken from the mass. The histopathological diagnosis of the biopsy was benign fibrous histiocytoma (BFH) of larynx. The patient underwent resection of the tumor by lateral pharyngeal approach. The general condition of the patient improved after the operation and she was discharged subsequently. For more than two years postoperatively, there has been no evidence of local recurrence.Fibrous histiocytoma usually occurs in the soft tissues, tendons and joints of the upper and lower extremities. It is un


Cystic lesions of the upper abdomen normally develop from pancreatic tissue. The differential diagnoses include neoplastic and non-neoplastic lesions. Pseudocystic tumors that secondarily involve the pancreas are very rare and may lead to diagnostic pitfalls.PDGFRalpha gene, exon 18 proved the diagnosis of gastrointestinal stromal tumor. The tumors were resected and both patients are still alive and disease-free.A 51-year-old woman and a 65-year-old man, both German, presented with abdominal cystic lesions suspected to be pancreatic pseudocysts. Both tumors were classified as gastrointestinal stromal tumors, epithelioid subtype. In one case, tumor origin in the gastric wall was confirmed by relaparotomy. In the other case, a point mutation in PDGFRalpha mutation are typical diagnostic parameters of gastric gastrointestinal stromal tumors.The differential diagnosis of cystic lesions in the upper abdomen must include gastrointestinal stromal tumors with pseudocystic change. The origin of a large cystic gastrointestinal stromal tumor may be difficult to determine. Epithelioid tumor pattern, weak or absent KIT expression and detection of  Cystic lesions of the pancreas are usually pseudocysts, related to chronic pancreatitis. Some are inflammatory pseudotumors with regressive cystic change; others are solid-pseudopapillary tumors or serous or mucinous cystic neoplasms of the pancreas.The diagnostic spectrum of pancreatic cystic lesions has been well investigated. Recently, we introduced a new entity entitled solid and cystic pancreatic hamartomas with a review of the literature . The diac-kit exons 9 and 11 and PDGFRalpha exons 12 and 18 were carried out. A mutation was not detected.A 51-year-old woman presented with a palpable abdominal mass on physical examination. From a computed tomography (CT) scan, a cystic tumor, 20 cm in diameter, was notes to be present between the stomach and the pancreas. With endosonography, the pancreas was inhomogeneous like chronic pancreatitis. Suspected of being a pancreatic pseudocyst, the mass was resected. Necrotic material and multiple solid nodules up to 3 cm in diameter were present. Histological examination revealed an epithelioid neoplasm, resembling a neuroendocrine tumor. Immunohistochemical analysis indicated the characteristic antigen expression pattern of mesenchymal tumors (positivity of vimentin). A postoperative control CT detected a residual tumor. Relaparotomy was performed with complete tumor resection and wedge resection of the gastric wall. The resected specimen demonstrated multiple 0.5 cm gray tumor nodules within a 4 cm in diameter hemorrhagic mouldered area. Histologically, the solid tumor component was well circumscribed and multilobated with fibrous septae, extensive sclerosis, diffuse hemorrhage and pseudocystic degeneration. The histological growth pattern was predominantly epithelioid . These results confirmed the diagnosis of GIST.A 65-year-old man with slight abdominal discomfort was examined by ultrasonography, which revealed a well-demarcated pancreatic cystic tumor, 10 cm in diameter. Three nodules were resected for histological examination. The specimens were solid, gray-brown and up to 3 cm in diameter. The tumor presented an epithelioid growth pattern Figure  with necFive months later, control CT found a 5.3 cm diameter well demarcated inhomogeneous cystic mass projecting into the lesser sac. Relaparotomy located the residual tumor between the stomach and pancreas. Infiltration of the digestive tract was not observed. The tumor was resected completely. There was a multipart mouldered gray-brown mass 3 cm in diameter. Histological diagnosis confirmed residual epithelioid GIST with pseudocystic change.The two cases presented here were clinically diagnosed as pancreatic cystic lesions. They were suspected of being pancreatic pseudocysts, due to chronic pancreatitis. Histologically, they showed a solid epithelioid growth pattern resembling a pancreatic neuroendocrine tumor, but lacked epithelial and neuroendocrine markers in the immunohistochemistry. A pancreatic carcinoma was excluded. Instead, the tumors revealed mesenchymal differentiation, but well known mesenchymal entities of the pancreas, that is, leiomyosarcoma or malignant peripheral nerve sheath tumor, were not shown on immunohistochemistry . MoreovePDGFRalpha exon 18 strengthened the diagnosis of GIST, evolving in the stomach, since this mutation was detected predominantly in gastric GISTs with epithelioid growth pattern [In our two cases, epithelioid growth pattern, weak KIT expression and low proliferative activity was suspicious for gastric GIST. In case one, the tumor origin in the stomach was confirmed at relaparotomy. In case two, a point mutation in  pattern . Moreove pattern ,8, as se pattern . The proThe differential diagnoses of pancreatic cystic lesions must include GISTs with pseudocystic changes. The origin of large GISTs in the upper abdomen may be difficult to determine, especially when located between the stomach, duodenum and pancreas. A weak or absent expression of KIT should not exclude the diagnosis of GIST, since this is characteristic for gastric GISTs with epithelioid differentiation. Mutation analyses are helpful in such cases.Written informed consent was obtained from the patients for publication of these case reports and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. The retrospective study was undertaken in accordance with institutional guidelines. Informed consent for scientific evaluation had been obtained from patients at their primary clinical treatment.The authors declare that they have no competing interests.UP drafted the manuscript, carried out the immunohistochemical staining, the molecular analysis, researched the literature and took the photographs. SH contributed the clinical data. HM and AF revised the manuscript critically for important intellectual content. All authors read and approved the final manuscript.


Paraganglioma is a rare tumor arising from undifferentiated cells of the primitive neural crest. These tumors are most commonly found in the adrenal gland, other localisations are also possible. A 58-year-old woman who presented with history of left lumbar pain, headache, hypertension, palpitation and sweating was found to have a secreting left renal hilar pheochromocytoma. Radical excision of the tumor was therefore undertaken and her hypertension was controlled. From the case report and literature review, the authors suggest a diagnostic and therapeutic strategy for the management of ectopic localization of pheochromocytoma. Pheochromocytoma is a neuroendocrine tumor that usually develops upon the chromaffin cells of the adrenal medulla. Extraadrenal retroperitoneal paragangliomas arise from paraganglia, which are collections of specialized neural crest cells symmetrically distributed along the aorta in close association with the sympathetic chain. The usual signs are lumbar pain, hypertension associated with generalized symptoms due to raised catecholamines such as headache, blurred vision, heart palpitation and flushing. However, 40% of the retroperitoneal pheochromocytomas don,’t feature any hormonal activity .Following an observation of a renal hilar pheochromocytoma together with a review of the literature, this paper aims at reminding the clinical, therapeutic and histological features of this rare tumor.The patient, a 58-year-old Moroccan housewife, suffered from a 5 year history of left lumbar pain, headache, hypertension, palpitation and sweating. During the physical examination, her blood pressure showed a 200/150 mmHg; she also had a mass in the left subcostal region. The rest of the physical examination was normal. Ultrasonography performed on the patient revealed a 6 cm size heterogeneous mass located in the region of the left renal hilum and an additional computed tomography of the abdomen demonstrated a dumbbell-shaped tumor, measuring 7.0 × 6.5 × 7.5 cm, in the left renal hilum and surrounding the left renal vein with a heterogeneously increasing density after injection of iodine-containing contrast solution. Both adrenal glands were normal with a negative extension summary . Levels Transperitoneal laparototomy has been therefore performed on the patient; a circumferential dissection of the hilar lesion demonstrated a smooth tumor surrounding the left renal vein . RadicalPheochromocytoma develops from the germinal cells coming from the neural crest, and is usually an adrenal tumor. Paragangliomas of the retroperitoneum arise from specialized neural crest cells distributed along the aorta in association with the sympathetic chain. Men are affected more frequently than women, and most patients are between the ages of 30 and 40 years ,3.Paragangliomas can be detected early if clinical findings caused by excess secretion of catecholamines are present. The literature states that extraadrenal retroperitoneal paragangliomas are functional for 60% of patients at most ,3,4. Cha60% of the retroperitoneal pheochromocytomas display signs of hormonal activity; however the hormonal measurement can sometimes be normal ,3,4.With regards to the patient mentioned above, the urinary catecholamines measurement was elevated and the radiological diagnosis performed via ultrasonography and computed tomography (CT) maintain the tissue nature of the tumor, which increase its density after iodine contrast solution injection. These two examinations give the opportunity to also seek for concomitant locations, as well as potential ganglionic or visceral metastasis. Computerized tomography is considered as the investigation of choice for demonstrating retroperitoneal structures; it might define the location, extent and nature of these tumors, and may also demonstrate infiltration into surrounding tissues, which provides essential information as to whether the tumor is respectable . CorrelaThe Magnetic Resonance Imaging (MRI) is a very reliable examination that can also detect other localizations despite their small size. Thanks to an outstanding characterization of the tissues, especially at the level of the retroperitoneum and the vascular axes, the added value of the MRI is greater than the one of the CT in the evaluation of the paragangliomas . In ordeThe treatment of the retroperitoneal pheochromocytoma is solely surgical and the patient preparation is a necessary step and covers a preoperative treatment with α- and β-blocking agents. The treatment of choice is radical excision of the tumor: Krugger-Baggesen et al. reported that out of 15 patients who had undergone a radical operation, none had died of the disease; one patient had nonetheless recurrent disease 7 years later . RadiothA long term follow up with regular biological and clinical examinations is necessary, in order to detect any recurrence or metastasis. From a histological perspective, the usual criteria of malignancy have no ground, but the appearance of a metastasis is a sign of malignancy.Paragangliomas are slow-growing tumors which metastasize late. Long-term survival is expected even with extensive local invasion. Radical excision should therefore be attempted, and if achieved, a good prognosis can be given.The renal hilar pheochromocytoma is a rare tumor. Its diagnosis is suspected in the presence of paroxysmal hyper blood pressure paired with a lumbar mass. The treatment of choice of those uncommon tumors is the radical excision that benefits from a good prognostic.


Giant cell tumor (GCT) of the spine is uncommon but most aggressive benign tumor of the spine with unpredictable outcome. We present our observation on six cases of GCT of the spine. We treated six patients with giant cell tumors (GCT) of the spine between 1993 and 2006. A total of nine surgical interventions were carried out. Four interventions were carried out in patients presented as ‘new’ cases, while five on recurrences from past GCT resections. All presented with cord compression and neurological deficits of varying grades. All patients also presented with clinical as well as radiological instability. Preoperative tissue diagnosis was available only in the five recurrences (tissue from the old resection). Posterior only (n=2), anterior only (n=4) and single-stage back and front (n=3) surgeries were carried out depending on the nature of the tumor.Overall results were satisfactory, as all patients were symptom-free postoperatively. Two out of our four new patients had tumor recurrence and both needed repeat resection. Both have been disease-free at last follow-up.Surgical intervention is mandatory. Close follow up is needed for early diagnosis of recurrences. Giant Cell Tumor (GCT) of the spine remains an intriguing and unpredictable entity. It is the most aggressive of the benign primary tumors of the spine, with a high predilection for recurrences. Most of the available literature reports small series, clearly indicating that it is not a common occurrence.Spinal GCTs however, often present with the unique problem of spinal cord compression due to extension into the spinal canal. Also, as it is often situated on either side of the neural tissues, complete resection becomes surgically challenging and most often, marginal or intralesional excision with backup therapy has to be resorted to.As with other sites, various treatment options have been described, ranging from surgical excision to adjuvant modalities like cement injection, phenol ablation, cryotherapy and radiotherapy. We report our experience with nine surgical interventions for six patients, over 13 years.We retrospectively analyzed cases of spinal GCT that had been operated between 1993 and 2006. From the approximately 200 surgeries done for spinal tumors during this period, nine were for GCTs and formed our study group.All data was extracted from hospital records, including preoperative and sequential postoperative clinical findings, radiological details and pictures and details of the status at last follow-up. Neurology was assessed by Frankel grading. X-rays and CT scans were studied for the presence or recurrence of tumor, instability and the status of the fusion and spinal implants. Quality of life questionnaires were not used. There were three males and three females in our group of six patients. Age ranged from 22 to 39 years. Mean follow-up was seven years (range - four to nine years).Four patients presented as fresh cases. Two of these had a recurrence after their first surgery with us. One of the two had a third recurrence. Two patients presented with a recurrence of a spinal GCT operated elsewhere primarily. One of these had had two surgeries before presenting to us. All patients, presented with cord compression and neurological deficit. Three cases graded Frankel C at presentation and all others were Frankel D. All had clinical as well as radiological evidence of spinal instability.Four of our six patients had GCTs in the thoracic spine , one in On X-ray, all cases had lytic, expansile lesions, with a ‘soap bubble’ appearance. All the four ‘new’ cases in our series had an magnetic resonance imaging (MRI) as well as a computerized tomography (CT) scan preoperatively. In situations where MRI non compatible metallic implants were present from the primary surgery, CT scans alone were done preoperatively. The MRI gives an excellent delineation of the soft tissue component of the tumor mass, the exact location of the neural compression and to an extent, an idea of the tumor vascularity. A CT scan, on the other hand, reconstructs the bony anatomy very well to give a preoperative idea of the nature of reconstruction needed. Chest X-rays were done in all patients, to rule out an unusual but specific probability of lung metastasis.Of the nine surgeries, the five that were done for recurrent tumors, had a histopathology report from the old surgery. Of the other four, two had had a preoperative fine needle aspiration cytology, which was inconclusive. In all these four cases without a preoperative diagnosis, an intraoperative frozen section was relied upon after making a preoperative diagnosis on radiology. In all the four cases, the radiological diagnosis of GCT matched with the frozen section and the eventual final histopathology.We planned our surgical approach based on a variety of factors including tumor location, extent, area of cord compression, degree of instability and status of previous implants. In all, of the nine surgical interventions, four were done by the anterior approach . These hFor all cases done posteriorly, we used the trans pedicular route for tumor resection and reconstruction with posterior fixation by pedicle screws or sub laminar wires. We found decompression to be adequate by this approach and it also gave enough access for reconstruction. Our earlier four cases had reconstruction with tricortical iliac strut grafts, while we used cages and bone cement in the three later cases. Two cases, done by the ‘posterior only’ approach (details as above) did not need reconstruction.All our nine interventions were preceded by digital subtraction angiography (DSA) embolization to reduce intraoperative bleeding. None of our surgeries had to be cut short due to uncontrolled bleeding.Four of our patients, all with recurrent GCTs received radiotherapy postoperatively. The doses were varied depending on the type of lesion and previous radiotherapy.Our eight interventions achieved the short-term goals of neurological spinal decompression and stabilization. All eight returned to Frankel Grade E, in the early postoperative period. The ninth intervention patient had worse neurology (Frankel C) to begin with, took longer (three months on an average) to come back to Frankel Grade E.We had no major complications, though two of the recurrent cases had superficial wound problems that healed uneventfully. In the long follow-up, two of our cases had recurrence of the GCT. One 23-year-old female (case number 1) with a back and front surgery done in 1997 returned with a recurrence in 2002, with signs of cord compression and needed anterior surgical decompression and reconstruction with a cement spacer. The other, a 36-year-old male (case number 5), was operated for a GCT of the C5 vertebral body, by anterior corpectomy and bone grafting by the senior author (SYB) in 1993. He did not receive postoperative radiation therapy and the tumor recurred after a year. This time, he had a complex back and front reconstruction with backup radiotherapy, which ensured a long tumor-free period. The ‘aggressive’ GCT, however, recurred six years postoperatively and a redo anterior reconstruction with fibular graft and plating was performed in 1999. Further radiation could not be given postoperatively due to potential danger of radiation myelopathy, as maximum possible doses had been previously received.Both these patients have remained asymptomatic till the time of submission of this paper (2006), one for five years and the other for seven years. Both have been followed up regularly and with a close watch on recurrences.Giant cell tumor in the spine, especially above the sacrum, is a relatively rare entity.We report nine surgical interventions in six patients of the GCT of the spine, from the 200 spinal tumors operated by us in the 13 years between 1993 and 2006. Though the incidence is similar to published data, there were no sacral GCTs in our series.Spinal GCTs most commonly present with pain due to the expansile lesion with or without vertebral collapse and spinal instability.Radiologically, GCTs of the spine present as cystic, expansile lesions on plain roentgenograms.For the previously cited reasons, none of our cases had a positive presurgical histology via biopsy . However, a transpedicular, CT-guided core needle biopsy is definitely recommended in all cases and is the best method of obtaining a pretreatment diagnosis. We have been able to get histological diagnosis in 80-85% cases in our clinical practice. Procedure site bleeding is not uncommon and the interventional radiologist should be aware of this.Pathologists can usually comment on the aggressiveness of the GCT, which helps planning treatment. This pertains especially to the postoperative use of radiotherapy and the frequency of postoperative CT scans. In tumors reported as ‘aggressive’ we plan radiotherapy post resection even if the resection has been adequate. Also, in these cases, CT scans are performed every six months for two years at least, to keep a watch on possible recurrences.Giant cell tumor being a highly vascular tumor, DSA aided tumor embolization within 24 hours before surgery is recommended.Complete, extralesional surgical resection would be the ideal treatment for spinal GCTs.Usually, some microscopic tumor tissue is expected to stay behind, however thorough the surgical excision is and hence postoperative radiotherapy is recommended. Though earlier literature seemed to suggest that irradiation converts benign GCTs to malignant ones, this is no longer true with modern radiotherapy techniques, especially by keeping the total radiation dose under 50Gy.et al.				Recommended method of reconstruction is by cement or metallic cages. Bone graft is avoided because the tumor is known to recur in the grafted bone. Also postoperative irradiation is frequently used, hampering graft fusion.A high index of suspicion is mandatory to diagnose recurrences in the GCTs.To summarize, spinal GCT are challenging clinical entities. Surgical intervention is mandatory and demanding and close follow-up is important to spot recurrences early.


The occurrence of primary sex cord-stromal tumors at extraovarian sites is exceedingly rare. We report a new case of Sertoli-Leydig cell tumor in the mesentery of a 78-year-old woman who presented with occlusive syndrome and reviewed the previously reported cases of extraovarian sex cord-stromal tumors in the English literature. Theoccurrence of primary sex cord-stromal tumors at extraovarian sites isextremely rare . These t A new case of a 78-year-old woman who presented with bowelobstruction is reported.Three years previously, she underwent bilateral salpingo-oophorectomy and totalabdominal hysterectomy for bilateral mucinous cystadenoma and vaginal prolapse. Ultrasonography showed a solid mesenteric tumor; exploratory laparotomy showeda nodular solid tumor at the mesenteric border of the distal ileum, measuring11 × 8  cm. Twenty centimeters of ileum containing the isolated lesion was resected. Macroscopically, the tumor was nodular and well circumscribed measuring 11 × 8  × 4 cm. The cut surface was composed of pale yellow-grey soft tissue with foci of haemorrhage. The tumor was limited to mesentery without extension to the ileal wall. Microscopic examination revealed cellular lobules with nests and poorlydeveloped tubules of Sertoli cells  that shoThe occurrenceof primary sex cord-stromal tumor at extraovarian sites is extremely rare ,such thTo ourknowledge, this is the first case of a primary mesenteric Sertoli-Leydig celltumor. Clinically, these tumors sometimes produce estrogen , 6. HoweThe histogenesisof extraovarian sex cord-stromal tumors has been reviewed in the literature .In recentyears, several investigators have claimed that the sex cords may originate fromthe mesonephros . A dualiThe prognosisfor extraovarian sex cord-stromal tumors seems to be favorable; however, reportedcases and clinical experiences are limited.


Cardiac papillary fibroelastoma is a rare, benign cardiac tumor. It often arises from valvular endocardium, and non-valvular endocardial location is rare. Although transthoracic echocardiography is usually sufficient for the diagnosis of most cardiac tumors, small tumors such as papillary fibroelastoma may be missed. Transesophageal echocardiography is superior to transthoracic echocardiography in diagnosing these tumors. Despite their benign histology, and independent of their size, they should be resected surgically because of their high potential for embolization.In this report, we present a case of papillary fibroelastoma located on the left atrial wall, presenting with symptoms of cerebral ischemia. The patient was treated surgically for the prevention of further embolic complications. Pertinent literature is also reviewed for this rare and benign cardiac tumor. Cardiac papillary fibroelastoma (PFE) is a rare, benign cardiac tumor. Usually, it arises from valvular endocardium. Nonvalvular endocardial location is rare, and may confuse the clinician for the differential diagnosis between organized mobile thrombus, pedinculated myxoma and fibroelastoma -3. HereiA seventy-two year old man was hospitalized in the Department of Neurology for the treatment and investigation of etiology for his ischemic cerebral event. Physical examination was unremarkable except monoparesis of the right upper extremity, right fascial paralysis and a pulsatile abdominal mass. Transthoracic echocardiography (TTE) showed a 0.7 × 0.7 cm mobile mass attached to the left atrial wall at the base of the anterior mitral leaflet. A transesophageal echocardiography (TEE) was performed for the differential diagnosis, and revealed a mass measuring 1.2 × 0.8 cm, which was attached to the left atrial wall at the level of the aortic non-coronary leaflet. Pre-operative diagnosis according to TEE was pedinculated left atrial myxoma  incision for optimal surgical exposure. A 1-cm, gelatinous and solid looking mass Figure  was founHistologic examination of the resected tumor revealed a papillary proliferation including few fibroblasts and collagenous tissue, covered with endothelial cells Figure . These mThe incidence of cardiac tumors in autopsy series is estimated at 0.021%, and cardiac PFE constitutes 10% of these . It is tThe histogenesis of PFE is still unclear ,6,8. TheThe location of PFE in the heart is very important because of its potential to embolize. Left-sided tumors may cause stroke, myocardial infarction, mesenteric ischemia, renal infarction and limb ischemia. Depending on their size and mobility, PFE can also give rise to obstruction of left ventricular filling during diastole, resulting in recurrent pulmonary edema. Right-sided cardiac tumors remain predominantly asymptomatic until they become large enough to interfere with intracardiac blood flow, alter hemodynamic function or induce arrhythmias. These features can mimic the clinical picture of tricuspid valve stenosis . ChronicThe diagnosis is usually made by echocardiography. Although TTE is sufficient for the diagnosis of most cardiac tumors, small tumors such as PFE may be missed, as evidenced by the present case. TEE is superior to TTE in diagnosing these tumors ,9. EchocThere is still a debate for surgical treatment of asymptomatic patients. Despite their benign histology, surgical excision is mandated regardless of the size if the patient has recurrent embolic complications. If the patient has cerebral embolisation, the operation should be delayed at least 4 weeks in order to prevent hemorrhagic transformation of the ischemic infarct. Several atrial incisions might be used for surgical exposure. We prefer trans-septal or extended vertical trans-atrial septal (Guiraudon) incisions for complete resection of left atrial tumors with optimal exposure, if the patient does not have dilated left atrium. Differential diagnosis of PFE encompasses other heart tumors, thrombi, vegetations, valvular calcification and Lambl's excrescences. Despite its typical shape, imaging techniques may fail to differentiate PFE from other cardiac tumors, as evidenced in this case. Histological investigation after surgical resection is mandatory to confirm the diagnosis. Cardiac myxoma is a predominant left atrial tumor, and is usually attached to the atrial septum by a stalk. Histologically, myxoma differs from PFE by the presence of polygonal myxoma cells and blood vessels within the papillae. Cardiac fibroma frequently demonstrates calcification and cystic degeneration. Cardiac rhabdomyomae are predominant in infants and children. Metastic tumors of the heart are more frequent than primary tumors [In conclusion, PFE are histologically benign tumors of the heart. They have the potential for peripheral or pulmonary embolisation regardless of their size. Since it is usually small, it cannot be detected reliably by TTE, thus TEE should be considered for a patient with an unexplained neurological ischemic event. Although there is a debate for resection of the asymptomatic PFE, surgical excision is mandated regardless of the size in order to hinder future embolic and hemodynamic complications.The authors declare that they have no competing interests.MB assisted the operation and participated manuscript writing. MC assisted the operation and participated manuscript writing. EP participated manuscript writing. HM performed echocardiographic examinations. SD made morphologic and histo-pathologic examination of the resected tumor. AK is the surgeon and participated manuscript writing. All authors read and approved the final manuscript.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.


Background Malignant tumours of the duodenumare rare and often difficult to diagnose. Due to thesmall clinical experience with duodenal malignanciestheir prognosis is unknown and resection is thetreatment of choice.Case report Adding to a small series of incidentaltumours, we report the case of a 65-year-old patientwith primary extranodal (MALT-) lymphoma of theduodenum infiltrating the pancreatic head. Thepatient was admitted because of anaemia andepigastric discomfort with a history of Helicobacter-pylori associated gastric ulceration. Physicalexamination and bloodchemical values were otherwisenormal. Endoscopy revealed duodenal ulcerationbut the biopsies taken from the ulceration didnot give any evidence of malignancy or residualHelicobacter pylori infection. But MRT showed acircular intramural tumour of the duodenum. Onlaparotomy a large duodenal tumour adherent to thepancreatic head was found and a Whipple procedurewas performed.Conclusion Apart from describing the case of arare lymphoproliferative disorder of the duodenum,this report illustrates the diagnostic difficulties withuncommon neoplasm's of the duodenopancreaticregion and the value of MRT prior to resection of aduodenopancreatic mass.