Coexistence of splenic marginal zone lymphoma with hepatocellular carcinoma is rare. Although some reports have suggested the possible pathogenic role of HBV, HCV, chronic and persistent antigenic stimulation in lymphoma, their role in causing lymphomas is still unclear.We describe a hepatocellular carcinoma with concomitant splenic marginal zone lymphoma in a 64-year-old Chinese man with cirrhosis. Serum hepatitis B virus surface antigen was positive and antihepatitis C virus antibody was negative. The resected liver mass measuring 4 × 3 × 3 cm was grey and soft with a small area of bleeding, necrosis and intact capsule. Cut surface of the spleen was red-purple and had a diffuse reticulonodular appearance indicative of prominent white pulp. On histologic sections, the liver mass was well and moderately differentiated hepatocellular carcinoma, and the splenic tumor was a specific low-grade small B-cell lymphoma. Immunohistochemical staining and gene rearrangement studies supported that the splenic tumor represents a clonal B-cell lymphoma. Therefore, the diagnosis of SMZL was made from the splenic specimen.To our knowledge, this is the second case report describing coexistence of hepatocellular carcinoma and splenic marginal zone lymphoma in the course of chronic HBV infection. However, we cannot assert at present that hepatitis B virus is directly involved in splenic lymphomagenesis until more information is collected from more cases in the future. The frequency of primary lymphoma of the spleen is relatively rare, although malignant lymphoma commonly involves the spleen . SplenicA 64-year-old man was diagnosed 6 years earlier with cirrhosis secondary to chronic hepatitis B. He was noted to have splenomegaly in a routine follow up. Laboratory data were as follows: aspartate aminotransferase, 126 U/litre ; alanine aminotransferase, 66 U/litre ; alkaline phosphatase, 123 U/litre ; and γ-glutamyltransferase, 178 IU/litre . Serum fetoprotein was 256 μg/litre . Serum hepatitis B virus surface antigen was positive and antihepatitis C virus antibody was negative.Magnetic resonance imaging of the abdomen showed an approximately 3.5 cm in diameter with a heterogeneous, hypervascular enhancing mass in the left lateral segment of the liver. A multiple, small-to-moderate nodules composed of a low-density area throughout the parenchyma was present in the enlarged spleen. There were no tumor thrombi within the portal veins and lymphadenopathy Figure .Positron emission tomography findings revealed the ringed dense uptake of 18F-fluorodeoxyglucose in the spleen. It was not absorbed in the lymph nodes of the splenic hilus and behind the pancreatic head; there were no abnormal hot areas in the liver.After the liver tumor and spleen were resected, the serum fetoprotein concentrations returned to within the normal range. However, the patient who was treated symptomatically and not treated with chemotherapy due to poor general condition died 6 months after surgical resection.Grossly, the resected liver tissue measured 10 × 7 × 5 cm. There was a well circumscribed tumor measuring 4 × 3 × 3 cm. The mass was grey and soft with a small area of bleeding, necrosis and intact capsule. The non-tumorous portion showed obvious cirrhotic nodularity. The resected spleen measured 25 × 17 × 7.5 cm and weighed 1750 g. The outer capsule was smooth, glistening, and intact. Cut surface of the spleen was red-purple and had a diffuse reticulonodular appearance indicative of prominent white pulp. Splenic hilar and mesenteric lymph nodes were not enlarged obviously.On histologic sections, the tumor cells were growing in cords of variable thickness, which were separated by sinusoid-like blood spaces in the liver mass. Diffuse fatty change of tumor cells was found in small area Figure . The nonImmunohistochemical staining of the neoplastic cells showed positivity for CD20, CD79α Figure , Pax-5, Amplification of Immunoglobulin heavy chain genes was performed by semi-nested PCR, using primers directed to the framework 2 (FR-2) region and to the joining region (JH) as described previously ,8 Gene rTherefore, the liver mass was well and moderately differentiated HCC, the diagnosis of SMZL was made from the splenic specimen. The final pathological diagnosis was coexistent SMZL with HCC in the course of chronic HBV infection.Coexistence of splenic lymphoma with additional cancers is rare. Iannitto et al  reportedChronic hepatitis B virus (HBV) or hepatitis C virus (HCV) infection is believed to play important roles in hepatocarcinogenesis. As for primary lymphoma, its histogenesis remains obscure. Recently, some reports have suggested the possible pathogenic role of HBV and HCV -13. It hHBV, hepatitis B virus; HCC, hepatocellular carcinoma; HCV, hepatitis C virus; PCR, polymerase chain reaction; SMZL, splenic marginal zone lymphoma;The author(s) declare that they have no competing interests.S-H Z carried out routine and special histochemical stains. S-H Z, A-M X, J-M Z and M-X H participated equally in the design of the report and in drafting the manuscript. All authors read and approved the final manuscript.


The appearance of dumbbell neuroma of the first thoracic root is extremely rare. The extradural component of a T1-dumbbell neuroma may present as an apical mass. The diagnosis of hand weakness is complex and may be delayed in T1-neuroma because of absence of the palpable cervical mass. One-stage removal of a T1-root neuroma and its intrathoracic extension demanded an extended posterior midline approach in the sitting position.A 51-year old man had suffered a traumatic partial tendon rupture of his wrist flexor muscles 6 years ago. Since the incident he occasionally felt fullness and tenderness in the affected forearm with some tingling in his fingers bilaterally. During the last two years the hand weakness was continuous and hypotrophy of the medial flexor and intrinsic hand muscles had become apparent. Electrophysiological studies revealed an ulnar neuropathy in addition to mild median and radial nerve dysfunction, including a mild contralateral carpal tunnel syndrome. The diagnostic work-up for multiple mononeuropathy in the upper extremity was negative. Repeated electrophysiological studies revealed fibrillations in the C7 paravertebral muscles on the affected side. Chest x-ray revealed a large round apical mass on the affected side. A Horner's syndrome was noted at this point of diagnostic work-up. MRI of the cervical and thoracic spine revealed a dumbbell T1 neuroma enlarging the intervertebral foramen at T1-2 and a 5 cm large extradural tumor with extension into the apex of the ipsilateral lung. The patient underwent surgery in sitting position using a left dorsal midline approach. Although the T1 root could not be preserved, the patient's neurological condition was unchanged after the surgery.Extended posterior midline exposure described here using hemilaminectomy, unilateral facetectomy and costo-transversectomy is efficient and safe for one-stage removal of dumbbell tumors at the T1 level with a predominantly extraforaminal component in the apex of the lung extending up to 6–7 cm laterally. Horner's syndrome, if present and observed, may significantly narrow the differential diagnosis of hand weakness caused by T1-root tumors. Neuromas (schwannomas and neurofibromas) are benign slowly growing peripheral nerve-sheath tumors originating from Schwann cells -3. In thThree commonly used surgical approaches to the brachial plexus – supraclavicular, transaxillary and dorsal subscapular ,7 enableWe present a patient with a T1-root neuroma with significant lateral extension from the intervertebral foramen into the thoracic cavity. Apart from being a diagnostic challenge, tumors in this location also demanded a tailored single-stage surgical approach to the inferior proximal brachial plexus and spinal canal using an extended posterior midline approach in the sitting position.A 51-year-old right handed non-smoker experienced acute onset pain in his left forearm, following a traumatic episode 6 years previously, when a box fell onto his forearm. A partial rupture of the wrist flexor muscle tendons was diagnosed. Following this, he noticed ipsilateral hand weakness and tingling in his fingers a few months later. During the subsequent two years following his initial traumatic episode, his hand weakness worsened, particularly in the winter and subsided in the summer. However, during the last two years the weakness had become permanent. He experienced difficulty in buttoning his shirt and grasping a glass with his left hand. He also noticed wasting of the left hand muscles and complained of bilateral finger paraesthesia during the night, mainly on the right.Neurological examination of the left upper extremity revealed mild hypotrophy of the ulnar flexors and intrinsic hand muscles . In addition, he had moderate weakness of wrist flexion, and finger abduction (4/5 MRC). Sensory testing revealed mild hypoaesthesia of the medial arm, forearm and the little and ring fingers. His reflexes were all preserved.th and 5th fingers) and median (2nd and 4th fingers) sensory nerve action potentials were similar to the right. Concentric needle electromyography (EMG) revealed denervation activity in the flexor carpi radialis, the first dorsal interosseous muscles, and chronic reinnervation changes in the extensor indicis, flexor carpi radialis and abductor pollicis brevis muscles. Additional electrophysiological examinations revealed mild denervation in the left paravertebral muscles, and no nerve conduction or needle EMG abnormality in the lower limbs. Further diagnostic work-up was tailored to reveal the etiology of the upper extremity multiple mononeuropathy. B12, folic acid, TSH, lues, HIV, Hep-2, and boreliosis were all negative. Radiographs of the cervical spine revealed intervertebral hondrosis and dorsal osteophytes at C5-6 and C6-7. The chest x-ray revealed a round lesion in the apex of the left lung , with no F-waves (detection from the abductor pollicis brevis muscle). Furthermore, reduced median nerve sensory conduction velocities were noted across the wrist on the right. Amplitudes of the left ulnar  the pathological diagnosis of an atypical schwannoma was made. These peculiar neuropathological features in schwannoma are more often seen as a part of neurofibromatosis but the patient didn't fulfill the clinical criteria for neurofibromatosis. However, genetic analysis was not performed.A complex morphology and unique functional anatomy make a diagnostic work-up of brachial plexus lesions challenging even to the experienced. Significant inter-individual variations can further mask the clinical picture and make precise localization these lesions even more challenging . In patiIn our patient, the initial clinical findings were not sufficient to explain the ulnar motor neuropathy, in addition to the sensory loss in the forearm and arm. The situation was further masked by night paraesthesia in the fingers bilaterally. Electrodiagnostic studies confirmed denervation changes in the muscles innervated by the median nerve , and ulnar nerves (the first dorsasl interosseous). Furthermore, chronic reinnervation changes were also found in muscles (the extensor indicis) innervated by the radial nerve. These findings made a proximal lesion more likely, and this was further supported by denervation changes in the cervical paravertebral muscles. However, the particularly intriguing feature was the patients' history of symptoms abating during the summer, and reappearing during the winter. This broadened the differential diagnosis, to include the possibility of autoimmune neuropathy. This possibility was further supported by normal EMG findings in biceps brachii, triceps brachii and pectoralis major muscles. Nerve conduction studies demonstrated mild median neuropathy at the wrist compatible with carpal tunnel syndrome. Symmetric sensory nerve action potentials detected in the fingers were compatible with a preganglionic location or with nerve conduction block. The diagnosis was unexpectedly aided by the routine chest x-ray. After a repeated thorough clinical examination, it was noted that the patient had a miosis of the left pupil, suggestive of Horner's syndrome. Our patient nicely demonstrates that a Horner's syndrome, if present and observed, along with a long history and slowly progression of hand weakness due to involvement of multiple nerves or roots indicates possibility of a C8 or T1 spinal root tumor. In such patients even a simple chest x-ray may provide crucial diagnostic information.What makes tumor in our patient exceptional is not only its location in the intraspinal, foraminal and extraforaminal compartments, but even more its extension into the thoracic cavity, projecting dorsally behind the first 3 ribs and laterally to the costo-transverse joints.At the start of surgery, identification and preservation of the functioning spinal root fascicles should be performed. The functionality of the fascicles can be checked by direct electrical stimulation, and recording the response from appropriate muscles.The midline approach to the intraspinal and foraminal part of the tumor enables preservation of these fascicles by early proximal identification of the subarachnoid rootlets in the dural sac and sleeve, and then following them by intrafascicular dissection into the foraminal component of the tumor ,9,10. ThWe decided to put our patient in the sitting position and performed a single curvilinear paramedian incision Figure . Such inTechnical advancements have introduced other possibilities for removing these apical mass dumbbell neuromas of the T1 root in a combined approach using transthoracic endoscopic surgery ,12. HoweWe did not perform spinal fusion in our patient, because we feel similar to other authors that complete unilateral facetectomy in combination with hemilaminectomy does not bear a significant risk of spinal instability ,10.et al. [Most studies confirmed that for complete tumor removal the affected root needs to be sacrificed, with relatively low risk of severe permanent postoperative injury. McCormick  reportedet al.  noted miet al.  who repoet al.  and Seppet al.  reportedet al. ,9,17.The extended posterior midline exposure described here using hemilaminectomy, unilateral facetectomy and costo-transversectomy is useful for a one-stage microsurgical removal of dumbbell tumors in the T1 level with a predominant extraforaminal component extending to the apex of the lungs. It provides contiguous exposure of the intraspinal, foraminal and extraforaminal region, which extends up to 7 cm from the lateral dural margin. The key of our technique is piecemeal tumor debulking similar to the way in which intracranial tumors are resected. No second skin incision, wound extension, or repositioning are necessary with this approach. However, two-staged surgery may be beneficial in elderly or patients with carotid artery stenosis, cardiomyopathy, coronary heart disease, history of pulmonary embolism or trombembolisms, etc. where the sitting position is contraindicated.The author(s) declare that they have no competing interests.RB performed the surgery, concepted and drafted the manuscript together with UB and MB, who also clinically examined the patient and made the appropriate literature review. SP performed the conduction and electromyographic studies and helped drafting the manuscript with critical remarks.All authors read and approved the final manuscript.Postoperatively the patient demonstrated identical hand and finger function as preoperatively. This short movie shows hand and finger function after surgery.Click here for file


Catecholamine-producing tumors mimic paroxysmal conditions with hypertension and/or cardiac rhythm disorders, including panic attacks, in which sympathetic activation linked to anxiety reproduces the same signs and symptoms. These tumors may be sporadic or part of any of several genetic diseases: familial pheochromocytoma-paraganglioma syndromes, multiple endocrine neoplasia type 2, neurofibromatosis 1 and von Hippel-Lindau disease. Familial cases are diagnosed earlier and are more frequently bilateral and recurring than sporadic cases. The most specific and sensitive diagnostic test for the tumor is the determination of plasma or urinary metanephrines. The tumor can be located by computed tomography, magnetic resonance imaging and metaiodobenzylguanidine scintigraphy. Treatment requires resection of the tumor, generally by laparoscopic surgery. About 10% of tumors are malignant either at first operation or during follow-up, malignancy being diagnosed by the presence of lymph node, visceral or bone metastases. Recurrences and malignancy are more frequent in cases with large or extraadrenal tumors. Patients, especially those with familial or extraadrenal tumors, should be followed-up indefinitely.Catecholamine-producing tumors may arise in the adrenal medulla (pheochromocytomas) or in extraadrenal chromaffin cells (secreting paragangliomas). Their prevalence is about 0.1% in patients with hypertension and 4% in patients with a fortuitously discovered adrenal mass. An increase in the production of catecholamines causes symptoms  and signs  reflecting the effects of epinephrine and norepinephrine on  Pheochromocytomas (PH) are neoplasms of chromaffin tissue which synthesize catecholamines. Most of these tumors appear in the adrenal medulla. Ten percent of catecholamine-producing tumors arise from extraadrenal chromaffin tissue and are called extraadrenal PH or secreting paragangliomas (PGL). In decreasing order of frequency, secreting PGL may develop in the Zuckerkandl body, a vestigial chromaffin ganglion located at the root of the upper mesenteric artery, in the sympathetic plexus of the urinary bladder, the kidneys and the heart, or in sympathetic ganglia in the mediastinum, the head or the neck. Most head and neck PGL are non-secreting. Patients with von Hippel Lindau (VHL) disease or familial PGL (see below) may have uni- or bilateral PH, or PH plus secreting or non-secreting PGL ,2.PH and secreting PGL are defined by the synthesis and/or secretion of catecholamines: dopamine, norepinephrine and/or epinephrine. Catecholamines are partly or totally converted within the tumor by catechol-O-methyltransferase into inactive metabolites, metanephrine and normetanephrine. Consequently, the release of active catecholamines into the circulation may be modest, absent or paroxysmal. The presence of a catecholamine-producing tumor is nevertheless established by the presence of high concentrations of metanephrine or normetanephrine in the plasma or in the urine ,3.SDHB and SDHD gene mutation carriers  metaiodobenzylguanidine (MIBG) should be performed where available, [131I]MIBG scintigraphy having a lower sensitivity. Labetalol and antipsychotic drugs should be withdrawn for several days before the investigations as they reduce [123I]MIBG and [131I]MIBG uptake. Where these imaging methods give negative (mostly in non-secreting PGL) or discordant results, the investigation should be completed by scintigraphy with nonspecific ligands such as somatostatin receptor scintigraphy or [18F]fluorodeoxyglucose PET, fluoro-DOPA or fluoro-dopamine PET. In addition to the primary tumor, CT, MRI or scintigraphy may disclose lymph node, bone, liver or pulmonary metastases, thereby establishing the presence of a malignant PH or PGL.The purpose of pre-operative imaging tests is to locate the tumor, ascertain whether it is single or multiple, adrenal or ectopic, benign or malignant, and isolated or present with other neoplasms in the context of familial syndromes. Of 192 patients referred for the preoperative work-up of a first PH or PGL, 20 (10%) had bilateral PH and 25 had PGL (13%). Eleven PH and three PGL were malignant . Tumors arose in the context of a familial disease in 34 patients (18%), most of whom had associated non-chromaffin tumors . CT is tCatecholamine-secreting tumors mimic paroxysmal conditions with hypertension and/or cardiac rhythm disorders, particularly panic attacks in which sympathetic activation linked to anxiety reproduces the signs and symptoms of PH. Plasma and urinary metanephrine concentrations are usually normal in these conditions . Acute cRET proto-oncogene; VHL disease due to mutations in the tumor suppressor gene VHL; and NF1 caused by mutations in the NF1 gene. More recently, the identification of mutations in the SDH  genes in hereditary PGL and PH MIBG scintigraphy should be performed and may disclose distant metastases whose MIBG uptake was masked pre-operatively by the primary tumor's higher metabolic activity.Biochemical evidence for the cure of the tumor cannot be obtained immediately: the output of catecholamine metabolites remains high, albeit decreasing, during the first week following tumor resection. This output represents the emptying of extratumoral pools of catecholamines and should not be interpreted as only partial response to surgery. The normalization of plasma or urinary metanephrine concentration should be checked 10 days after surgery. If the metanephrine concentrations remain high, MIBG and chemotherapy may provide clinical, hormonal and sometimes tumoral improvement [Malignant PH/PGL is compatible with prolonged survival with symptom-free intervals varying from months to decades. Factors associated with a longer survival seem to be an early diagnosis and excision of the primary tumor, and, whenever possible, aggressive excision of any recurrence or soft-tissue metastases. When recurrences are small with an accessible vascular pedicle, surgical excision may be preceded, or replaced, by therapeutic embolization. In cases where soft-tissue or skeletal metastases are too widespread to make surgery or embolization feasible, several palliative therapies may be considered. Pharmacologic treatment aimed at the long-term blockade of catecholamine synthesis with rovement . Such thCurrent research aims to unravel the etiology of sporadic PH and PGL; to develop tumor-mass markers for PGL with little or no catecholamine synthesis; to assess genotype-phenotype relationships and disease penetrance in rare familial diseases; and to design trials of adjuvant therapies or alternatives to surgery for aggressive tumors.


Spontaneous regression of metastatic renal cell carcinoma is rarely observed.Metastatic renal cell carcinoma was identified in a 70-year-old male using computed tomography-guided percutaneous needle biopsy. Two months after the diagnosis, a partial resection of the sternal bone was performed. Pathological examination revealed granulated tissue with bleeding and necrosis but no carcinogenic cells.We report a pathologically identified case in which a sternal bone metastasis that was noticed two years after radical nephrectomy regressed completely and spontaneously. Since the first reported case of spontaneous regression of metastatic renal cell carcinoma in 1928 by Bumpus [A 70-year-old male was referred to our hospital with anterior chest pain and a precordial mass in September 2004. He had undergone right radical nephrectomy in July 2002, and renal cell carcinoma, T1aN0M0, stage I, had been diagnosed. Medical follow-ups had been conducted routinely without any treatment. In May 2004, after he experienced anterior chest pain, computed tomography (CT) was performed. This revealed in the sternum a low density mass that gradually increased in size during the following three months. In September 2004, there was an 8 × 7 cm mass, and destruction of some of the cortical bone of the manubrium sterni had occurred, so a CT-guided percutaneous needle biopsy was performed. Fig.  We judgeSince Bumpus reported the first case of spontaneous regression of metastatic renal cell carcinoma , the incThe sites of regression observed in the reported cases included the brain, bone and liver, but the highest incidence was in the lung ,5. In moIn the present case, early-stage renal cell carcinoma was diagnosed. The patient underwent no tumor-specific treatment for about two years after the nephrectomy. The size of the sternal tumor gradually increased until the percutaneous needle biopsy was performed, but it regressed completely within a mere two months. During these two months, the patient had no chemotherapy or immunotherapy, or any kind of interventional treatment. Complete regression of a metastatic tumor is quite rare especially two years after the resection of the primary tumor. One of the possible explanations of this phenomenon may in this case have been induced by the needle biopsy performed in order to diagnose the metastasis; or bleeding caused by the biopsy may have triggered a chain reaction of host immune responses. The patient is now receiving interferon therapy in our outpatient clinic on individual basis rather than supported by literature data to prevent recurrence of the tumor -9. This The author(s) declare that they have no competing interests.TN conceived of the case, participated in the sequence alignment, drafted and revised the manuscript before and following the peer review. MS was the main operator in charge of the case and gave the ideas of this manuscript. SA, TI and TF made substantial contributions to the conception and interpretation of clinical data and case-related studies. AA was the pathologist. HK, the corresponding author, was the operator in charge of this case and made clinical decision, the ideas of this manuscript. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:


To describe immunohistochemical features encountered in ovarian fibrothecoma with correlation to clinical presentation and surgical management.A female age 75 presented for evaluation of melena. The patient reported total abdominal hysterectomy and removal of both ovaries 40 years earlier.CA-125 was normal and there was no evidence of hyperestrogen effect. Pelvic CT revealed a partially calcified 7 cm pelvic mass without adenopathy or ascites; ultrasound was confirmatory. Endoscopy identified three benign intestinal tubular adenomas. Following laparoscopic excision of the pelvic tumor immunohistochemichal analysis of the mass showed negative staining for keratin, S100 protein, inhibin, calretinin, melan A, smooth muscle actin, CD34, CD117, and desmin. The tissue was positive for vimentin, however.Ovarian fibrothecomas represent an ovarian stromal neoplasm developing in a wide spectrum of clinical settings. Particularly if oophorectomy is stated to have been performed remote from the time of index presentation, the status of the ovaries must be considered whenever pelvic pathology is encountered. We describe a calcified ovarian fibrothecoma identified during gastroenterology investigation and confirmed immunohistochemically via high amplitude vimentin signal. Stromal tumors of the ovary include thecoma and fibroma, yet as differentiation between these two types may be difficult the term fibrothecoma has emerged in recognition of the similar immunohistochemical features present in both. The exact incidence of fibrothecoma is unknown, although they have been described as rare ovarian neoplasms . Here weA non-smoking 75 year old Caucasian female presented for gastroenterology evaluation due to rectal bleeding. She had mild essential hypertension well controlled on diltiazem, did not smoke, and had no other medical complaint. She reported intermittent oral use of aspirin, 81 mg/d. In 1966, she underwent total abdominal hysterectomy "when both ovaries were removed". At the time of our evaluation, the patient used supplemental estrogen occasionally but was uncertain when this commenced. Her only other surgery was an uncomplicated laparoscopic cholecystectomy performed in 1996.The patient's BMI was 26. Vital signs and physical exam were normal although a heme positive stool was noted. Abdominal CT showed no significant abnormality, although pelvic CT revealed a 7 cm partially calcified mass at the upper pelvic inlet, indistinguishable from bowel Figure . UltrasoSerum electrolytes were unremarkable except for BUN and Cr at 20 mg/dl and 1.1 mg/dl, respectively. Hemoglobin was 11.8 g/dl. Serum hCG, CEA, and CA-125 were all normal.A 12 mm umbilical port was placed along with two accessory ports , one each at the left upper and lower quadrants. Extensive abdominal adhesions were encountered and lysed with blunt dissection and harmonic scalpel. Eventually an approximately 7 cm smooth, white glistening mass could be seen in the right aspect of the pelvis [Figure Formalin-fixed tumor sections embedded in paraffin were exposed to 10 mmol/L citrate buffer (pH 6.0), followed by incubation with 1:50 dilution of monoclonal mouse anti-vimentin  using 20 min heat-induced epitope retrieval in DakoCytomation Target Retrieval solution S3308) × 30 min incubation at room temperature with primary antibody. Negative control was 1:50 dilution mouse IgG1 (X0931) which was run simultaneously. Complexes were visualized via DAKO LSAB+/HRP kit (K0679) and automated stainer platform. The specimen demonstrated positive vimentin staining for mesenchymal spindle and round cells, consistent with benign ovarian stromal neoplasm [Figure 308 × 30 We report an unusual case of partially calcified ovarian fibrothecoma without ascites in an elderly female reporting previous oophorectomy and complaining of melena. For this patient, her gastrointestinal bleeding was determined to be secondary to benign polyps, although it was during the work-up associated with this lesion that suspicion was raised regarding a 7 cm calcified right pelvic mass. While this mass did appear benign intraoperatively, metal endosurgical clips were deployed to assist subsequent radiographic localization of tumor site in the event adjuvant radiotherapy was indicated. Excision of the tumor at laparoscopy confirmed its ovarian origin, and immunohistochemical labeling was performed to characterize it more fully as a benign fibrothecoma.6. However, tumor calcification and low serum CA-125 are infrequent findings.Other investigators have commented on the protean nature of ovarian tumors in this group that may occur in the setting of edema , elevateOne finding that proved useful in finalizing the diagnosis for our patient was the high affinity for vimentin staining, characteristic of ovarian fibrothecoma . Vimentiin situ ovary should still be considered whenever pelvic pathology is encountered.The impact of patient recall error while obtaining the surgical history also warrants comment. A variant of residual ovary syndrome, the problem begins when the patient is unaware of an ovary intentionally conserved at surgery. The ovary subsequently develops a pathologic process or causes symptoms necessitating its removal in a second operation . Not sur


The skeletal muscle is an unusual site for metastasis from renal cell carcinoma (RCC). Metastatic RCC must be differentiated from benign primary soft-tissue tumors because aggressive surgical resection is necessary.We present the case of a 65-year-old man with metastatic RCC in the gluteus maximus muscle (3.8 cm in diameter) found on enhanced computed tomography (CT) 6 years after nephrectomy. Retrospectively, the small mass (1 cm in diameter) was overlooked 5 years earlier on enhanced CT. Because the growth of the lesion was slow, benign tumor was a differential diagnosis. However, magnetic resonance imaging (MRI) showed that the mass had high-signal intensity on T1- and T2-weighted images (WIs) compared to that of skeletal muscle, with mild enhancement by Gadolinium. The MRI features were unusual for most soft-tissue tumors having low-signal intensity on T1-WI and high-signal intensity on T2-WI. Therefore, under a diagnosis of metastatic RCC, the lesion was resected together with the surrounding skeletal muscle. The histology was confirmed to be metastatic RCC.MRI features of metastatic RCC may be beneficial in differentiating it from primary soft-tissue tumor. Renal cell carcinoma (RCC) has widespread and unpredictable metastatic potential , even afMaking a diagnosis of metastatic RCC to the skeletal muscle is challenging, because the site is unpredictable, in addition to it being rare. Furthermore, cases of metastasis arising long after nephrectomy have been reported ,5. The dGenerally, either open or needle biopsy is necessary in order to make a diagnosis, in cases of soft-tissue tumor, because the surgical procedure is different depending upon the histological diagnosis. In the current paper, we present a case of RCC with metastasis to the gluteus muscle. The lesion was treated without biopsy, because the MRI features suggested metastatic RCC. We wish to emphasize the MRI features of metastatic RCC to the skeletal muscle, which could be beneficial in differentiating metastatic RCC from primary soft-tissue tumor.A 59-year-old man underwent right radical nephrectomy for RCC of 3.8 centimeters in diameter stage pT1a N0 M0). One year after the nephrectomy (when he was 60 years old), recurrence was seen in the adrenal gland. Consequently, resection of the adrenal gland was undertaken. Six years after the nephrectomy (when he was 65 years old), a routine checkup using computed tomography (CT) of the chest and abdomen detected a small mass in the gluteus maximus muscle. The tumor was located in the gluteus maximus muscle adjacent to the fascia, and this was enhanced by contrast medium  showed that the lesion adjacent to the fascia had higher signal intensity than that of skeletal muscle on T1- and T2-weighted, and T2-STIR images. The lesion had a rather regular border to the surrounding gluteus maximus muscle, and was capsulated by a thin area with low-signal intensity on both T1- and T2-weighted images Figure . Edema oPrimary soft-tissue tumor was a differential diagnosis. Because the growth of the lesion was slow over 5 years, benign soft-tissue tumor was suspected. The fact that the lesion was capsulated by a thin area as seen on MRI also supported the diagnosis. However, the MRI features were unusual for most soft-tissue tumors having low-signal intensity on T1-weighted imaging and high-signal intensity on T2-weighted imaging. Therefore a diagnosis of metastatic RCC was suspected rather than primary soft-tissue tumor. Because metastatic RCC was strongly suspected, wide resection including the surrounding skeletal muscle was undertaken. Biopsy was not performed in order to avoid unnecessary contamination from the procedure. The resected tumor was composed of alveolar proliferation of carcinoma cells with clear cytoplasm and small round-shaped nucleoli (Fuhrman grade 2), resembling the previously resected RCC, suggesting metastatic RCC Figure .Surgical resection of metastatic RCC reportedly improves the outcome of patients with metastatic RCC, and five-year survival rates are between 35% and 50% after surgical therapy for solitary metastasis . In the Another factor which makes a correct diagnosis difficult is that metastasis sometimes occurs long after primary surgical treatment. It has been reported that 11% of metastatic RCC cases occurred more than 10 years after initial diagnosis  and metaMetastatic RCC to the skeletal muscle must be differentiated from primary soft-tissue tumors. Primary soft-tissue tumors are more common than metastatic tumors to the skeletal muscle . GeneralIn the current case, the characteristic features of MRI for metastatic RCC were beneficial in differentiating it from primary soft-tissue tumor, because most cases of primary soft-tissue tumor have low- to iso-signal intensity relative to the skeletal muscle on T1-weighted images and high-signal intensity on T2-weighted images. Although lipoma, hemangioma , clear cThe high intensity of hemangioma seems to be related with the fat component, as is the case with lipoma. Higher signal intensity on T1-weighted images of clear cell sarcoma  is reported to be correlated with melanocytic differentiation . We haveIt has been reported that angiography shows that RCC metastatic to the skeletal muscle usually appears as hypervascular lesions ,8. The GIn conclusion, we present the case of a 65-year-old man with metastatic RCC to the gluteus maximus muscle. The nodule was slowly progressive over 5 years. MRI of metastatic RCC is characterized by high-signal intensity on T1- and T2-weighted images which may be beneficial in the differential diagnosis from primary soft-tissue tumors. Maintaining a high degree of suspicion of metastatic RCC is required for patients with a history of RCC.The author(s) declare that they have no competing interests.AS drafted the manuscript. TY, KT and ShM participated in the design of the study and performed. SuM, YO and YH and conceived of the study. AY and YI participated in its design and coordination and helped to draft the manuscript. All authors read and approved the final manuscript. All authors read and approved the final manuscript.


Low-grade gliomas are uncommon primary brain tumors, located more often in the posterior fossa, optic pathway, and brain stem and less commonly in the cerebral hemispheres.Two patients with diagnosed recurrent cystic pilocytic astrocytoma critically located within the brain  were treated with gamma knife surgery. Gamma knife surgery (GKS) did improve the patient's clinical condition very much which remained stable later on. Progressive reduction on the magnetic resonance imaging (MRI) studies of the solid part of the tumor and almost disappearance of the cystic component was achieved within the follow-up period of 36 months in the first case with the  and 22 months in the second case with the (brain stem located lesion).Gamma knife surgery represents an alternate tool in the treatment of recurrent and/or small postoperative residual pilocytic astrocytoma especially if they are critically located Low-grade gliomas are uncommon primary brain tumors classified as histological grades I or II in the World Health Organization (WHO) classification. The most common variants are pilocytic astrocytoma, low-grade astrocytomas, oligodendrogliomas, and mixed oligo-astrocytomas located more often in the posterior fossa, optic pathway, and brain stem and less commonly in the cerebral hemispheres. Prognostic factors that predict progression-free and overall survival include young age, pilocytic histology, good Karnofsky performance status, gross total resection, lack of enhancement on imaging, and small preoperative tumor volumes. Edema and vasogenic effects are typically managed with corticosteroids. The rationale for open craniotomy depends on the need for immediate palliation of symptoms by reduction of intracranial pressure or focal mass effect, and/or improved oncologic control. ,2Gross total resection of low-grade glioma is generally defined as the absence of residual enhancement on contrast-enhanced postoperative MRI scan. Most retrospective studies suggest that patients who have undergone a gross total resection of tumor have improved survival. Depending upon the proximity of the tumor to eloquent brain areas, gross total resection may or may not be possible. In these cases a stereotactic biopsy is required to provide the histological diagnosis. Adjuvant radiotherapy is recommended for patients with incompletely resected grade II tumors or for patients older than age 40 regardless of extent of resection, it may be considered for any pilocytic astrocytoma from which a biopsy has been made. Radiosurgery and/or experimental chemotherapy may provide some measure of local control in the recurrent disease setting -6Stereotactic radiotherapy provides excellent local control for small, localized low-grade glial tumors. Marginal failures have not been observed, supporting the use of limited margins radiation dose to minimize late sequelae using stereotactic leksell gamma knife technique ,8A 10 years old girl presented with right side weakness, with vomiting attacks and blurring of vision. MRI delineated a large cystic lesion at left thalamic region with solid part. Stereotactic biopsy from the solid portion and aspiration of the cyst revealed pilocytic astrocytoma. Post aspiration the child improved clinically, but three months later the symptoms recurred and MRI revealed recurrence of the cystic part of the tumor that was treated again with stereotactic aspiration of the fluid, followed with stationary course. There was no history of patient receiving radiotherapy.The child was referred to our Gamma Knife Center in October 2003; 6 months post second stereotactic aspiration with right side weakness more in the upper limb, with right facial palsy, and headache. MRI revealed large nonenhanced left thalamic cystic lesion with small two hyperintense intracystic solid parts.The child was treated with Gamma Knife with peripheral prescription dose of 12 Gy to the volume of the solid parts with maximum dose of 24 Gy at 50% isodose curve figure .In October 2006, after 36months of follow-up, MRI of the brain showed even more reduction in tumor and no solid part enhancement was seen with good clinical stable condition.A 19 year old female patient with partially cystic brain stem (pontine) tumor that was histologically diagnosed as pilocytic astrocytoma, who had undergone two previous surgeries, first an open posterior fossa surgery with partial tumor excision and cyst evacuation in January 2004, and secondly stereotactic evacuation surgery for the cystic part recollection in August 2004 was referred to us on February 2005 with recurrence of her symptoms as repeated infrequent attacks of vomiting, unsteadiness, limb ataxia, and nystagmus. The MRI showed recurrence of the large cystic tumor within the pons, its solid portion extends down to the medulla oblongata  showed marked reduction of the cystic part and even reduced in size of the solid portion  declare that they have no competing interests.RFH: conceived and prepared the manuscript.


Scrotal schwannoma is a rare neoplasm and poses a diagnostic challenge to urologists. This article describes a rare case of ancient scrotal schwannoma and reviews the current modality of investigation and treatment of this tumour.A 28 year old man presented with a 3-month history of an asymptomatic scrotal swelling. Ultrasonography and computer topography revealed an intra-scrotal and extra-testicular mass without local invasion. Surgical excision was undertaken and histology was an ancient schwannoma of the scrotum.Schwannoma is a benign encapsulating neoplasm with an overall low incidence, occurring mostly in the head and neck region and seldom in the scrotum. Histology shows two distinctive patterns, Antoni type A and B areas. Variations of schwannoma such as cellular, ancient, glandular and epithelioid are observed based on the appearances. Ancient schwannoma exhibits pleomorphism without mitosis as the result of cellular degeneration, which can lead to an erroneous diagnosis of malignancy. Imaging modalities are non-specific for schwannomas, but can define tumour size, site and extension. The mainstay treatment is complete excision, although local recurrence may occur in large and incompletely excised lesions. Malignant change is exceedingly rare. A revieA 28-year old man presented with a three month history of an asymptomatic scrotal swelling. He had previously undergone orchidopexy in childhood for a mal-descended right testis. There is otherwise no other significant medical or family history. The mid-scrotally located mass measured 4 × 7 cm and was nodular, hard and clinically separate from the testes. It was not attached to the scrotal skin or other underlying structures. Lymphadenopathy was not detected.Initial ultrasonography Fig  showed aAn initial diagnostic wedge biopsy indicated the lesion could be an ancient schwannoma. Curative surgical excision with partial scrotectomy was undertaken for removal of the mass and for definitive diagnosis. At resection, the tumour appeared to be superficial to the tunica vaginalis, testes and corpus spongiosum. Inferiorly, the tumour was attached to the bulbospongiosus requiring partial resection of the superficial muscle fibres.The resected specimen Fig  was whitcellular, glandular, epithelioid and ancient types, and all exhibit benign progression. Cellular schwannomas are almost exclusively composed of Antoni A areas but lack Verocay bodies. The glandular and epitheloid variants compose of epitheloid areas and glandular component, respectively, to acquire their descriptive names. Ancient schwannomas show bizarre hyperchromatic nuclei without mitoses. To the unwary, these features can lead to an erroneous diagnosis of malignancy, although the very low mitotic activity should allow these tumours to be distinguished from malignant nerve sheath tumours.A schwannoma is a benign encapsulated neoplasm derived from schwann cells of the nerve sheath. The exact incidence of schwannomas is unknown, but they are rare. Schwannomas are found in all age groups but are more common in the first four decades and affect both sexes equally. They may occur in association with neurofibromatosis or arise sporadically. The microscopic appearance of schwannoma is distinctive , with twA literature review showed that whilst ancient schwannomas are rare, most cases occur in the head and neck region   declare that they have no competing interests.PTC drafted the manuscript, prepared illustrations and performed the literature search. ST helped to draft the manuscript and helped to acquire ultrasound and CT images. SEL helped to draft the manuscript, paying particular attention to the pathological aspect and kindly acquired histological images for illustration. LQR conceived this study and supervised the drafting and overall structure of the manuscript. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:


Purpose. The objective of this study is to report a case of a rare, highly lethal tumor, extrarenal malignant rhabdoid tumor (EMRT) in a 43-year-old man who initially presented with a local recurrence and is now continuously disease free 14 years after aggressive surgical treatment. The case and literature are discussed. Malignant rhabdoid tumor (MRT) wasfirst described as a rhabdomyosarcomatous subtype of Wilm's tumor . Maligna2 × 5 days), VP-16 (100 mg/m2 × 5 days), and G-CSF (390 μg SQ daily × 20 days) followed by surgical excision. After two rounds of chemotherapy and three months after the initial resection, a 3 cm firm, fixed, subcutaneous mass was palpated on the medial aspect of the inguinal incision. CT confirmed the presence of a locally recurrent mass not observed on the previous CT exam performed immediately postresection  between 1987 and 1999. The chemotherapy regimen recommended consisted of vincristine 1.5 mg/mOther chemotherapy regimes for MRT have included combinations of cisplatinum, cyclophosphamide, adriamycin, and VP-16 –23. The


Deep penetrating nevus (DPN) is a distinct variant of melanocytic nevus and remains a histopathologic challenge to pathologists because of its resemblance to blue nevus, malignant melanoma, pigmented Spitz nevus, and congenital melanocytic nevus. It often goes unrecognized due to its relative rarity.Here we report a case of DPN of the left anterior leg in a 51-year old female. A brief review of the literature shows that these lesions have a distinct growth pattern and cellular morphology that can differentiate these lesions from other entities including malignant melanoma.It is important to recognize these features because DPN carries a better prognosis than malignant melanoma. Deep penetrating nevus is a distinct variant of melanocytic nevus. It is characterized by its dark pigmentation, occurring mostly on the face, neck, or shoulder, and usually measures less than 1 cm in diameter . Here, wA 51-year old Caucasian female presented to an outside institution in 1997 with a left shin lesion and was diagnosed with melanoma. A wide excision was performed with two subsequent re-excisions performed in 2001. At that time she received a split-thickness skin graft and a left inguinal sentinel node biopsy which was negative for malignancy. Punch biopsies were also obtained from multiple tan-brown lesions on both legs. These biopsies showed both typical and atypical melanocytic hyperplasia along with compound and dermal nevi. In 2002, more biopsies of the left lower leg were performed, revealing squamous cell hyperplasia. In February of 2005, she presented to us with a left lower leg skin lesion in the same area as the prior melanoma. The lesion was excised and submitted for pathologic evaluation.The specimen was a skin ellipse measuring 1.5 × 0.7 cm with an excisional depth of 0.7 cm. Centrally located on the external surface of the skin was a dark brown, discolored macular area with irregular borders measuring 0.1 × 0.1 cm. The lesion did not appear to involve the margins of the specimen, which was entirely submitted for formalin processing.5-um sections from the formalin-fixed, paraffin-embedded tissue were used for routine light microscopic study. The sections showed deep dermal and subcutaneous nested proliferations of spindle and epithelioid cells arranged in a wedge-shaped pattern with the base in parallel with the reticular dermis andthe apex oriented toward the epidermis Fig. . These nDPN was first described by Seab in 1989. Since then, 169 cases have been reported in the English literature see . This leClinically, DPN usually presents as a darkly-pigmented papule or nodule that generally appears dark, ranging from pink to black in color -6. The iDPN has distinct morphologic and histologic features. It is usually characterized by a sharply-demarcated, wedge-shaped lesion with its base in parallel with the epidermis andits apex oriented toward the subcutaneous fat or deep reticular dermis ,3-6. OddAt a higher magnification, this lesion demonstrates clustering of pigmented nevus cells with pleomorphic, vacuolated nuclei with smudged chromatin, rare or no mitoses, and scattered melanophages. Nuclear vacuoles and large pseudoinclusions are often observed -3,5. If Even while DPN displays such characteristics as a unique architectural pattern and a minimal mitotic rate, such characteristics as cellular atypia, pleomorphism and an infiltrating growth pattern leave little to wonder as to why DPN can be so difficult to differentiate from malignant melanoma. However, Ball and Golitz provide a comprehensive, tabulated review of the differences between malignant melanoma and DPN .Barnhill described a case of plexiform spindle cell nevus as a plexiform pattern of fascicles composed of spindle cells extending through the reticular dermis. It was also intimately associated with adnexal structures, nerves and blood vessels with intervening normal dermis. Since these features are similar to DPN, the plexiform spindle cell nevus has now been considered as DPN .Immunohistochemical analysis usually shows positivity for both S-100 and HMB-45 while stains for keratin are universally negative , therebyExcision has been the unanimous choice of treatment -6. AfterOf all the reported cases, there were no recurrences after follow-ups of one to twenty-three years other than that featured in a report by Robson in 2003 , in whicDeep penetrating nevus is a distinct entity that can mimic various other skin lesions, most notably malignant melanoma. However, distinct architectural and cytologic characteristics should allow the pathologist to arrive at the correct diagnosis. These lesions have shown zero metastatic potential, and recurrences are exceptional. Therefore, accurately diagnosing this neoplasm is important because of its favorable prognosis and less burdensome treatment strategy, in stark contrast to that of malignant melanoma. Patients may thereby avoid unnecessary, often grueling, treatment courses as well as the emotional and psychological impact a misdiagnosis can have on them and their families.Table 1: Reported Cases of Deep Penetrating Nevus. Herein we detail the existing reported cases of deep penetrating nevus including the literature source, demographics, clinical presentation, histopathology, treatment, and follow-up.Click here for file


Duodenal metastasis is rare in renal cell carcinoma (RCC) and early detection, especially in case of a solitary mass, helps in planning further therapy.We present the case report of a 55 year old male with duodenal metastasis of RCC. This patient presented with jaundice and abdominal lump one year after nephrectomy. On upper gastrointestinal endoscopy a submucosal mass lesion was noted in the duodenum, the biopsy of which revealed metastasis.In a nephrectomized patient presenting with jaundice and an abdominal mass, the possibility of metastasis should be suspected and a complete evaluation, especially endoscopic examination followed by biopsy, should be carried out. Renal cell carcinoma (RCC) has a potential to metastasize to almost any site. In descending order of frequency, the most common sites of metastasis are the lung, lymph nodes, liver, bone, adrenal glands, kidney, brain, heart, spleen, intestine, and skin . It can The patient was a 55 years old male who came to gastroenterology out patient department with complaints of jaundice and an abdominal mass. He had a history of RCC in the left kidney and had undergone left radical nephrectomy one year ago in our institute. The tumour was present in the lower pole and measured 7 × 5 × 6 cm. Microscopically, it was a conventional clear cell carcinoma (Furhman grade III) involving the renal sinus with tumour emboli in the renal vein. The adrenal gland and ureter were free. This time the patient had jaundice and an abdominal lump. An upper gastrointestinal endoscopy (UGIE) followed by duodenal biopsy was performed. Endoscopy showed a 4 × 4 cm submucosal mass lesion Fig.  in the sThe biopsy consisted of three fragments which revealed duodenal mucosa with normal villi, however many of the vascular channels in the lamina propria showed tumour emboli. The tumour cells were mildly pleomorphic, had abundant pink cytoplasm and low nuclear:cytoplasmic ratio Fig. . These wnd part of duodenum. The patients commonly present with gastrointestinal bleeding or intestinal obstruction [Small bowel involvement by metastatic tumors is rare and has been reported in only 2% of autopsy cases . Metastatruction ,17, howeThe majority of patients are found to have metastasis within a year after nephrectomy though it can be seen even after several years . The rouTreatment options in a case of RCC metastasis depend upon the extent and location of the lesion. In the majority of reported cases of duodenal metastasis, metastatectomy was done. However for disseminated malignancy like in our case treatment is in the form of palliative (non-curative) surgery, radiotherapy, chemotherapy (Sunitinib) or immune stimulating agents (Interleukin-2). However even after treatment the patients with metastatic disease have poor survival. The average survival is about 4 months and only 10% of these survive for one year .The report therefore highlights the importance of investigating patients of RCC presenting with any gastro-intestinal tract manifestations for metastasis. A complete evaluation, especially endoscopic examination and biopsy, should be carried out in such patients. Awareness of this entity and a high index of suspicion on the part of the treating physician and pathologist would help in proper diagnosis and treatment.


Spontaneous perforation of the uterus is rare, its incidence beingabout 0.01% − 0.05%. We report a rare case of diffuse peritonitis caused by spontaneously perforated pyometra. A 63-year-old woman with severe abdominal pain was admitted to our hospital. Laparotomy was performed because of the suspicion ofgastrointestinal perforation with generalized peritonitis. At laparotomy, about 900 mL of pus was found in the peritoneal cavity. There were no abnormal findings in the alimentary tract, liver, or gallbladder. A total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed. Pathological investigation of the surgical specimen revealed endometritis and myometritis of the uterus; but there was no evidence ofmalignancy, and the cervical canal was patent. Although spontaneously perforated pyometra is rare, a perforated pyometra should therefore also be considered when elderly women present with acute abdominal pain. An uncommon condition known as pyometra occurs when the natural drainage of the uterine cavity is comprised and pus accumulates within the cavity . It has We report a patient that was admitted to our hospital for diffuse peritonitis caused by spontaneously perforated pyometra who was successfully treated by surgery.A 63-year-old Iranian woman was admitted to our hospital in December 2005 with the chief complaint of abdominal pain, vomiting, and fever that had developed one day earlier. She was a diabetic patient and she had no history of sexually transmitteddisease. She had 3 pregnancies and all of her deliveries were normal vaginal deliveries. On physical examination, she looked acutely ill: her body temperature was 38.1°C, pulse rate was 126 beats/min, and blood pressure was 140/70 mmHg. Herabdomen was soft and was not distended. General tenderness with muscular guarding was recognized.The results of laboratory studies on admission were as follows: white blood cell count: 18700/mL; red blood cell count: 3.4 × 106 /mL; hemoglobin: 10 g/dL; albumin: 2.4 mg/dL. Ultrasonography demonstrated a large amount of ascites in Morisonpouch. Computed tomography (CT) with an intravenous injection of contrast material visualized a calcified uterine body and fluid and ascites without free air. She was diagnosed as having diffuse peritonitis due to a perforated gastrointestinal tract. Afterobtaining adequate informed consent, laparotomy was performed on the basis of a diagnosis of perforation of the gastrointestinal tract but revealed a perforated pyometra. At laparotomy, about 900 mL of pus was found in the peritoneal cavity. There were no abnormal findings in the alimentary tract, liver, or gallbladder. A total hysterectomy and bilateral salpingo-oophorectomy were carried out. A culture of the pus was negative. A histological examination of the resected specimen revealed pyometra. There was no evidence of malignancy. Postoperatively, intensive antibiotic therapy was performed. She recovered well and was discharged on the 15th postoperative day.Pyometra is common in postmenopausal women, and more than 50% of all patients of nonruptured pyometra are asymptomatic . The incA spontaneous perforation of pyometra leading to diffuse peritonitis only rarely occurs. Once ruptured, the symptoms become severe and acute abdomen often develops; it is therefore important to make a differential diagnosis from other causes of acutesurgical abdomens .In conclusion, the diagnosis of spontaneous perforation of pyometra is rarely made preoperatively and the possibility of a perforated pyometra should therefore be considered when elderly women suffer from acute abdominal pain . Their m


This case report highlights two unusual surgical phenomena: lipoma-like well-differentiated liposarcomas and sciatic hernias. It illustrates the need to be aware that hernias may not always simply contain intra-abdominal viscera.A 36 year old woman presented with an expanding, yet reducible, right gluteal mass, indicative of a sciatic hernia. However, magnetic resonance imaging demonstrated a large intra- and extra-pelvic fatty mass traversing the greater sciatic foramen. The tumour was surgically removed through an abdomino-perineal approach. Subsequent pathological examination revealed an atypical lipomatous tumour (synonym: lipoma-like well-differentiated liposarcoma). The patient remains free from recurrence two years following her surgery.The presence of a gluteal mass should always suggest the possibility of a sciatic hernia. However, in this case, the hernia consisted of an atypical lipoma spanning the greater sciatic foramen. Although lipoma-like well-differentiated liposarcomas have only a low potential for recurrence, the variable nature of fatty tumours demands that patients require regular clinical and radiological review. We report the case of a young woman in whom a lipoma-like well differentiated liposarcoma presents as a sciatic hernia. This case therefore highlights two unusual surgical phenomena, and illustrates the need for clinicians to be aware that hernias may not always simply contain intra-abdominal viscera. We also review the medical literature surrounding lipoma-like well differentiated liposarcoma and sciatic hernias.A 36 year old woman was referred by her family physicians with a 3 year history of a large lump on her right buttock Fig. . She hadOn examination, she had a large swelling at the ischial area of her right buttock that, in most respects, was consistent with a typical subcutaneous lipoma. However, it had the unusual feature that it was reducible in a similar way to a sciatic hernia.An MRI scan demonstrated that the lesion was not simply a subcutaneous lipoma.It showed a well-defined 14 cm × 8 cm × 8 cm maximum diameter mass that traversed the greater sciatic foramen through the infra-piriformis area. It lay partly within the pelvis and partly within the right ischiorectal fossa and upper thigh.The tumour was homogenously iso-intense with fat except for a small focus of low signal intensity at its superior margin. There was no abnormal signal on the STIR sequence, which demonstrated normal fat suppression.The size and situation of the mass displaced the pelvic organs, resulting in the bladder being pushed anteriorly, the uterus superiorly and the rectum to the left. Inferiorly, the mass extended through the ischio-rectal fossa, medial to the ischial tuberosity, to come to lie in the subcutaneous fat medial to sartorius and the hip adductors Fig.  and 3. EAfter discussion with the local sarcoma multi-disciplinary team, the plastic and general surgeons removed the tumour in a joint procedure. To fully reduce the tumour back into the pelvic cavity (via the greater sciatic foramen) and maintain its position there, an assistant was employed to exert external manual pressure on the right buttock throughout the procedure. Tumour dissection was then performed through an abdomino-perineal approach Fig.  and 5. AThe tumour was a lobulated mass of adipose tissue measuring 230 mm × 180 mm × 40 mm Fig. . HistopaThese features were in keeping with an atypical lipomatous tumour (synonym: lipoma-like well-differentiated liposarcoma) with low potential for recurrence.The patient remains under the care of the plastic surgeons. She has remained well for 2 years following her surgery and follow-up CT scans have not demonstrated recurrence.Sciatic hernia is an extremely rare variety of hernia. Following its initial description by Papen in 1750, there have been less than a hundred case reports in the medical literature . There aSciatic hernias may present as a gluteal mass, as a rare cause of sciatica (following compression of the sciatic nerve) , or withSymptomatic hernias should be surgically repaired as soon as possible , through either a transabdominal or transgluteal approach ,11.Well-differentiated liposarcoma accounts for about 40% to 45% of all liposarcomas , and theWell-differentiated liposarcoma is further subdivided into the lipoma-like (adipocytic), sclerosing, inflammatory and spindle cell subtypes, of which the first two are by far the commoner. Our case was an example of the lipoma-like variant of well-differentiated liposarcoma. These tumours, as they sound, are composed mainly of univacuolated adipocytes showing some variation in size and shape, associated with only scattered multivacuolated lipoblasts (the presence of which are an absolute prerequisite for the diagnosis of liposarcoma) and occasional hyperchromatic spindle or stellate cells, which are often found within dense fibrous septa . It has Cytogenetically, well-differentiated liposarcomas appear to be relatively homogenous, exhibiting characteristic ring, as well as giant marker chromosomes, containing amplified genetic material derived from the 12q 13–15 chromosome region .It is important that all well-differentiated liposarcomas are extensively sampled, both to ensure identification of lipoblasts , and to exclude the presence of a dedifferentiated element. Such an area of dedifferentiation confers a much worse prognosis, with up to a 50% chance of metastasis . DediffeThe classification of these tumours has been a source of debate for pathologists in recent years, but the current trend is to classify all pure well-differentiated cases arising in a limb as atypical lipoma, since, although they commonly recur, they never metastasise and therefore wide excision should be curative. Liposuction alone has been advocated as a possible treatment for these tumours . HoweverIn this particular case, we have employed CT as the radiological modality of follow-up. However, this decision was largely based on the availability of local resources and we would otherwise usually recommend MRI. As lipoma-like well-differentiated liposarcoma have only a low potential for recurrence, we would suggest that follow-up MRI need only be performed annually or when new symptoms evolve.The author(s) declare that they have no competing interests.RJES is a surgical trainee who was involved in this patient's management. He was also involved in drafting and revising the manuscript.GHMS is a surgical trainee who was involved in this patient's management. He was also involved in drafting and revising the manuscript.KJS was the Plastic Surgery Consultant in charge of this patient's surgical management. He was also involved in drafting and revising the manuscript.DNA was the General Surgery Consultant in charge of this patient's surgical management. He was also involved in drafting and revising the manuscript.All authors read and approved the final manuscript.


A case of a local recurrence and metastases of hemangiopericytoma is described.Hemangiopericytoma occurs with increasing frequency in 5Recurrence of hemangiopericytoma in the greater omentum and the jejunal mesentery as well as metastases in the retroperitoneal space were diagnosed in a 61-year-old patient who had a hemangiopericytoma of the greater omentum excised 18 years before.Because of the rarity of this disease and its typical clinical course associated with late recurrence and metastases, the authors decided to present this case emphasizing the necessity of systematic oncological follow-up after the end of treatment. Hemangiopericytoma is a tumor originating from the vascular pericytes of Zimmermann. Most frequently it occurs in patients aged 40 and above with its main localization in the retroperitoneum, pelvis and lower extremities -3, howevWe report the case of a local recurrence and metastases in the retroperitoneum infiltrating the liver and diaphragm in a 61-year-old patient operated on due to a hemangiopericytoma of the greater omentum 18 years before.A 61-year-old patient was admitted to the Department of Transplantation and General Surgery with a tumor diagnosed in the left lumbar and retroperitoneal space. Eighteen years before he had been operated for a tumor of the greater omentum with histopathological diagnosis haemangiopericytoma omenti maioris, until now with no recurrence nor distant metastases were found.On admission the patient was in good general condition with left lumbar pain and a mobile tumor in this area. Abdominal ultrasonography (US) and computed tomography (CT) revealed a retroperitoneum space tumor with suspected infiltration of the segment VII of the liver and diaphragm.en bloc with the infiltrated part of the diaphragm and the segment VII of the liver  malignant tumors, 6 of 16 (37.5%) borderline lesions and no benign tumors metastasised [Hemangiopericytomas represent less than 1% of all vascular neoplasms . They aret al., reveal that metastases became apparent in 11% of patients with malignant tumors and 7% with borderline tumors after 5 years' remission [et al., it occurred after 4 months in one patient and 22 years in another [Metastases occur by hematogenous and lymphogenous routes affecting mainly the lung, liver, bones and regional lymph nodes ,3,9,10. emission . Local remission . In two  another . Hyperce another .et al., reported 70% 10-year survival, whereas it was 50% 5-year survival according to other authors [Surgical resection with subsequent radiotherapy remains the treatment of choice for hemangiopericytoma. The role of preoperative angiography and tumor embolization is also emphasized in literature ,5,6. Suret al., in primary cutaneous melanoma the mean disease-free interval after surgery was 182 months [et al., report seven patients in whom local or regional recurrence occured 11 to 23 years after first treatment of melanoma [Late recurrence is common in other malignancies as well. According to Shen 2 months  whereas melanoma . Anothermelanoma ,17. The melanoma ,17,19.Every patient treated due to a malignant or borderline hemangiopericytoma should be under long-term oncological follow-up as the risk of recurrence and distant metastases even after many years is very high.The authors declare that they have no competing interests.MS conceived the idea for the study, participated in its design and coordination, analyzed the data, wrote the first draft of the manuscript.IP conducted the literature review, was involved in data collection, analyzed the data, helped to draft the manuscript.ZW participated in the study design and coordination, helped to draft the manuscript.All authors read and approved the final version of the manuscript.


Gastrointestinal autonomic nerve tumours are uncommon stromal tumours of the intestinal tract. They can involve any part of the gastrointestinal system, but are very rarely seen in the rectum.We report a unique case of rectal schwannoma with associated synchronous adenocarcinoma of the splenic flexure and adenoma of the descending colon. A 70-year-old patient was admitted with complaint of bleeding per rectum and investigations revealed the presence of a large submucosal rectal lesion in addition to the colonic pathologies. Following panproctocolectomy with permanent spout ileostomy, histopathology and immunohistochemistry confirmed the rectal lesion to be a schwannoma.Literature review of the few reported cases has suggested radical surgical excision to be the best approach. Prognosis tends to be favourable after resection. It is a in 1984 . Schwann in 1984 .Without immunohistochemical studies, Schwannomas are often misdiagnosed as leiomyomas or leiomyosarcomas and generally present as an asymptomatic mass and/or with non-specific symptoms of fatigue and prolonged pain as well as signs of low grade pyrexia, anaemia and haemorrhage. Conventional pathological techniques are usually not diagnostic ; electroCommon sites for GANTs include the stomach, duodenum, jejunum, ileum  and to dA 70-year-old gentleman presented with a two-month history of bleeding per rectum and altered bowel habits. There was no history of tenesmus but sensation of incomplete evacuation following defecation as well as a feeling of a lump in the anal canal was present. His appetite and weight had been stable.Clinically he looked well and abdominal examination revealed no abnormality. Per rectal examination revealed a mass in the posterior wall of the rectum with intact mucosa, which had significantly narrowed the lumen. Colonoscopy revealed the presence of a mass in the posterolateral wall of the rectum extending from 4 to 14 cm from the anal verge, with normal overlying mucosa. In addition, there was also a large sessile polyp in the descending colon and an ulceroproliferative growth at the splenic flexure; histopathology confirmed adenomatous polyp and adenocarcinoma respectively.Computerised tomography (CT) of the pelvis showed thickening of the distal rectum with an obvious low density poorly enhancing lesion with possible calcification in its wall. Tissue plane between this lesion and prostate was not clearly seen but there was no infiltration of the ischiorectal fat Figure . MagnetiThe case was fully discussed at the colorectal multidisciplinary team meeting and all the surgical options including left hemicolectomy combined with rectosigmoidectomy plus transverse colostomy were fully evaluated. However, the consensus was that the additional presence of an adenocarcinoma as well as an adenoma higher up in the colon combined with the size of rectal schwannoma (12 cms) made panproctocolectomy with permanent ileostomy the best viable option. Furthermore, this was also the preferred choice of the patient as he opted to have an ileostomy rather than a colostomy. Surgery was performed without complications and the postoperative recovery was uneventful. At 6 months follow-up, the patient remains asymptomatic.Macroscopic examination of the resected specimen confirmed the malignant tumour in the splenic flexure, the polyp in the descending colon and a 10 × 12 cm tumour with a well-defined pseudocapsule in the postero-lateral wall of the mid and low rectum Figure . The surHistopathology of the splenic flexure lesion showed an invasive moderately differentiated adenocarcinoma . The polyp further down the colon was a tubulovillous adenoma with moderate focal dysplasia. The rectal tumour showed irregularly running fascicles of spindly to plump mesenchymal cells. The tumour was fairly vascularised with presence of a number of dilated and engorged vessels. The mitotic count was 4 per high power field. Some parts of the tumour were quite cellular while few areas were hypocellular reminiscent of the Antoni A and Antoni B patterns respectively Figures  and 4b. Gastrointestinal autonomic nerve tumours represent a distinct but rare subcategory of gastrointestinal stromal tumours accounting for 1% of all malignant gastrointestinal tumours. Initially described as plexosarcomas, these tumours have been reported to be more common in males and have a wide spectrum of age range . Literatet al, [Conventional imaging modalities such as barium enema, colonoscopy, computerised tomography and MRI have been used to investigate these patients but there are no definite radiological criteria to differentiate benign from malignant stromal tumour . As showet al,  have repet al, . Howeveret al, . Endoscoet al,  but in oet al, [Although GANTs do exhibit a variety of specific histological features, Lauwers et al,  believe et al, . Furtheret al, , others et al, . Such paImmunohistochemical studies of GANTs have usually demonstrated positivity to vimentin, CD34 and CD117 ,10,21. PWhilst immunohistochemistry provides important diagnostic differentiation of GANTs from other stromal tumours, definitive diagnosis can only be based on ultrastructural studies . The ultet al, reported that 30% of these patients developed local recurrence [The exact biological behaviour of GANTs is not yet fully elucidated due to the limited number of reported cases and as a result, determination of malignancy poses a difficult challenge, which cannot always be resolved by conventional histopathology. Various parameters have been studied in relation to tumour behaviour and to date; no single one is fully predictive of malignancy. Mitotic activity (counts >5 mitoses per high power fields) and tumour size (> 5 cm) tend to be associated with a high risk of metastasis or recurrence . Althougcurrence  and as aThe recent finding of CD117 receptors in these tumours combined with technological advances has led to the development of anti-tumour agents. Treatment of such CD117 positive GANTs with tyrosine kinase inhibitors have been shown to be beneficial  and coulFurther studies are required not only to fully characterise the molecular biology of these tumours but also their aggressiveness as there is an inherent difficulty in making a benign diagnosis in GANTs. This is clearly important since it may result in a poor cross over in the assessment of the responsiveness of GANTs to anti-CD117 treatment.The author(s) declare that they have no competing interest.MMH collected the information, did literature search and wrote the manuscript.DC assisted in writing the manuscript.OJO assisted in pathological view and the microscopic pictures.JBV helped in preparing the manuscript and edited the final version.All authors have read and approved the final version.


Herein we present a case of appendiceal adenocarcinoma presenting as left-sided large bowel obstruction, we also review the literature of unusual presentations of appendiceal tumours.we report a case of left sided large bowel obstruction found to be secondary to an appendiceal adenocarcinoma. The patient presented with abdominal pain, distension and constipation, CT scan showed large bowel obstruction thought to be due to a sigmoid tumour, on laparotomy the appendix was also noted to be abnormal. A low Hartman's was performed with en-bloc total hysterectomy and bilateral salpigo-oophorectomy. A separate ileocaecal resection with end ileostomy was also performed, pathology specimens showed that the primary neoplasm was the appendix with metastasis to the distal sigmoid.appendiceal tumours are rare, they usually present as acute appendicitis, other presentations are far less common. Neoplasms of the vermiform appendix are rare, they account for 0.4 – 1% of all gastrointestinal malignancies . They arMost symptomatic appendiceal tumours present as appendicitis, other presentations have also been described, but are far less common, these tumours may also be encountered unexpectedly in any acute or elective abdominal operation and the correct diagnosis is rarely made pre or intraoperatively, most of these tumours are identified only after histopathological examination of an appendicectomy specimen  was raised at 12.6, with CRP of 54 & bilirubin of 34. A CT scan of the abdomen showed large bowel obstruction thought to be secondary to a sigmoid carcinoma. A solid mass in the right adnexa was also noted and thought to be either a primary ovarian tumour or metastatic deposit. No distant disease was seen.After a period of resuscitation, a laparotomy was performed. The large bowel obstruction was found to be secondary to a "sigmoid tumour". The uterus and right ovary were abnormal and were "fixed" to the upper rectum distal to the tumour. The appendix was noted to be abnormal with palpable ileocaecal lymph nodes. A low Hartman's was performed with en-bloc total hysterectomy and bilateral salpigo-oophorectomy. A separate Ileocaecal resection with end ileostomy was also performed. Postoperatively the patient made an uneventful recovery and she was discharged home two weeks later.Macroscopic examination of the specimen showed an appendix measuring 40 mm in length and up to 15 mm in diameter. Microscopic examination showed invasive high grade adenocarcinoma of appendiceal origin showing diffuse infiltration of the wall of the appendix and the caecum with serosal extension. Metastatic tumour was seen in 2 out of 9 ileocaecal lymph nodes. Distant metastatic carcinoma was also shown to involve the wall of the distal sigmoid colon with stricture formation and infiltration of the adherent uterus, cervix, both ovaries and right fallopian tube.Immunohistochemistry showed that in all areas of tumour infiltration identical staining was identified. There was strong CK20 positivity, patchy chromogranin positivity and negative staining for CK7. There was similarity in appearance to a malignant goblet cell carcinoid although the tumour was classified as a high-grade aggressive adenocarcinoma of the appendix with neuroendocrine differentiation , anotheAppendiceal neoplasms could be found at any acute or elective abdominal surgery, management plan should then be based on the intraoperative findings. Murphy et al  suggesteDepending on the stage and the grade of the tumour, the overall 5-year survival reported for adenocarcinoma of the appendix is in the region of 55 percent . RadiothAppendiceal neoplasms are also noted to be associated with a significant incidence of synchronous and metachronous colorectal neoplasms . PatientAppendiceal tumours are rare, they usually present as acute appendicitis but can be encountered unexpectedly in any acute or elective abdominal operation. An unexpected clinical presentation should be considered by clinicians.As part of the formal exploratory laparotomy, in addition to the routine thorough examination of the abdominal viscera, looking for deposits and lymphadenopathy, we suggest the inspection and palpation of the appendix and mesoappendix as part of the intraoperative examination of any gastrointestinal malignancy surgery.The author(s) declare that they have no competing interests.MA: Collected data, wrote the provisional manuscriptNB: Helped with the design of the manuscript, revised the contents of the paperJW: General supervision, given final approval of the version to be submittedAll authors read and approved the final manuscript.


Colostomy site carcinomas are rare with only eight cases reported in the world literature. Various etiological factors like adenoma-cancer sequence, bile acids, recurrent and persistent physical damage at the colostomy site by faecal matter due to associated stomal stenosis have been considered responsible. Two such cases are being reported and in both cases there was no evidence of any local recurrence in the pelvis or liver and distant metastasis. Both patients had received adjuvant chemotherapy following surgery.First case was a 30-year-old male that had reported with large bowel obstruction due to an obstructing ulcero-proliferative growth (poorly differentiated adenocarcinoma) at the colostomy site after abdomino-perineal resection, performed for low rectal cancer six years previously. Wide local excision with microscopically free margins was performed with a satisfactory outcome. Four years later he presented with massive malignant ascites, cachexia and multiple liver metastasis and succumbed to his disease.Second case was a 47-year-old male that presented with acute large bowel obstruction due to an annular growth (well differentiated adenocarcinoma) in the upper rectum. He was managed by Hartmann's operation and the sigmoid colostomy was closed six months later. Five years following closure of colostomy, he presented with two parietal masses at the previous colostomy site scar, which, on fine needle aspiration cytology were found to be well-differentiated adenocarcinomas of colorectal type. Surgery in the form of wide local resection with free margins was performed. He presented again after five years with recurrence along the previous surgery scar and an incisional hernia and was managed by wide local excision along with hernioplasty. Follow-up of nine years following first surgery is satisfactory.Colostomy site/scar recurrence of rectal carcinoma is rare and could be due to various etiological factors, although the exact causative mechanism is not known. Surgery with microscopically free margins is recommended in the absence of metastatic disease. Stenosis of the stoma is considered as one of the most important contributory factors and should be followed carefully. Metachronous carcinomas rarely occur at the colostomy site and only eight cases have been reported previously. Various factors like adenoma-cancer sequence, stenosing stoma or bile acids have been implicated. Colon cancer presenting as cutaneous metastasis in an old operative scar has also been reported -6. Possird of the transverse colon en bloc with the involved para and pre-aortic lymph nodes. The left ureter, which was encased within the lymph node mass, was dissected free and right-sided transverse colostomy was done  in the form of classical abdomino-perineal resection with microscopically free (R0) margins and satisfactory outcome. He was on a regular follow-up for three years and was subsequently lost to follow-up. In April 2002 he presented with acute large bowel obstruction due to complete stenosis and infiltration of the stoma site by an ulcero-proliferative growth, which was extending in to the surrounding skin and the abdominal muscles . He was again lost to follow-up and presented subsequently in August 2006 with cachexia, massive malignant ascites, and liver metastasis and succumbed to his disease.A-45-year old male presented to the surgical emergency in Aug 1997 with features of acute large bowel obstruction of three days duration due to an annular growth in the upper rectum without any evidence of liver or peritoneal metastasis. Due to poor general condition  and the unprepared bowel, he was managed by Hartmann's operation. The histopathological examination of the resected specimen revealed well-differentiated adenocarcinoma of the rectum (T4N1M0). The colostomy was closed after eight months and the regular follow-up was satisfactory. He received adjuvant chemotherapy in the form of Levamisol and 5 fluorouracil. He was subsequently lost to follow-up and reported back five years later in 2002 with two parietal lumps in the left lumbar region and the fine needle aspiration cytology showed them to be well-differentiated adenocarcinoma of colorectal variety. The Ultrasound and contrast enhanced computed tomographic scan (CECT) showed infiltration in to the muscles of the anterior abdominal muscles and the peritoneum. A wide local excision including the abdominal muscles was done and the wound was closed in layers. He reported back in May 2005 with a recurrent 2 × 3 cm parietal nodule along the scar of the previous colostomy and an incisional hernia. The scar recurrence was excised with an R0 margin and the hernia was repaired using an onlay prolene mesh. The histopathological examination of the specimen revealed well-differentiated adenocarcinoma of colorectal type  is associated with a good outcome if there are no liver, peritoneal or distant metastases.The author(s) declare that they have no competing interests.CM was the chief surgeon in charge of the case and VS was the first surgical assistant; AB and SS were the pathologists responsible for the histopathological examination, DB assisted in the preparation of the manuscript. All authors read and approved the manuscript.


Neoplasms of the urinary bladder following augmentation ileocystoplasty are rare. We present the case of a 39-year-old male with a tubular adenoma with high-grade dysplasia in the ileal segment 34 years after augmentation ileocystoplasty to enlarge a post-chemoradiation-induced shrunken bladder. He presented with gross hematuria. Cystoscopy revealed a papillary tumor at the site of ileovesical anastomosis, and transurethral resection was performed. Histologic examination revealed a tubular adenoma with high-grade dysplasia. There are only two previous reports of tubulovillous adenoma in ileal segment after ileocystoplasty, both without high-grade dysplasia. Our observation supports the hypothesis that an ileal neobladder may undergo all the morphologic and molecular changes observed in the development of gastrointestinal adenocarcinoma. Therefore, patients who had an ileal neobladder created should be closely followed. Currently, the use of intestinal segments is the main option both for augmentation and replacement of the urinary bladder, and for diversion of urine from the normal outlet in patients following cystectomy or post-therapy shrunken bladder. These surgical procedures are separated into two main groups, namely bladder substitution or augmentation with transposed intestinal segments and ureterosigmoidostomy. Several types of orthotopic bladder substitution or augmentation have been developed, of which ileocystoplasty with the creation of an ileal neobladder is one of the most common procedures. An ileal neobladder is easily constructed and provides unchanged voiding habits with good continence and upper urinary tract preservation, with relatively low rates of complication . UreteroThere is now a general consensus from long-term follow-up studies that ureterosigmoidostomy results in an increased absolute risk of adenocarcinoma arising from the ureterocolic anastomosis -5. ThereA 39-year-old man underwent augmentation ileocystoplasty 34 years previously to enlarge his shrunken bladder following chemotherapy and radiation therapy to his bladder and prostate for embryonal rhabdomyosarcoma at the age of 5. He was completely continent and was voiding normally after the augmentation ileocystoplasty. He had no family history of gastrointestinal malignancy. He presented with gross hematuria. A voiding cystogram showed a polypoid mass at the level of the ileovesical anastomosis. An intravenous pyelogram showed no evidence of a mass or obstruction in the upper urinary tract. Computerized tomography scans showed normal kidneys without obstruction. Flexible cystoscopy revealed a solitary 5 cm papillary tumor of the ileal segment in close proximity of the site of ileovesical anastomosis. Transurethral loop resection was used to completely excise the tumor to the underlying muscle. Histopathologic examination revealed a tubular adenoma with high-grade dysplasia Figures  &1B, andNeoplastic changes after ureterosigmoidostomy as urinary diversion are not uncommon, and these neoplasms are generally attributed to the mixture of the urine and fecal stream . SeveralNeoplasms developing after ileocystoplasty are indeed rare; only 10 cases of carcinomas developing after ileocystoplasty have been reported in the English language medical literature -9. HowevEscherichia coli. Nitrites and urinary amines react to form N-nitrosamines. Experimental studies using animal models have reported both supportive [Two main hypotheses have been proposed for the increased risk of neoplastic changes following ureterosigmoidostomy and ileocystoplasty ,14,15. Tpportive  and contpportive ,18 evideUreterosigmoidostomy patients have consistently showed substantially higher levels of N-nitrosamine in their faeces or urine compared to control subjects, and was associated with chronic inflammatory changes and altered mucin secretion at the anastomotic site . HoweverThe second hypothesis, inflammatory response hypothesis, states that the inflammatory response stimulated by the anastomosis of differing mucosa lead to nuclear instability, perhaps through the generation of reactive oxygen species ,14. HoweClinical studies examining the cellular origin of tumors found at the line of anastomosis between the intestinal segment and native bladder have reported contradictory findings ,25. ThouTherefore, evidence in support of either of the two hypotheses of pathogenesis for malignancies following ureterosigmoidostomy and ileocystoplasty is incomplete and conflicting -22,26-30Immunohistochemical, the high-grade dysplastic ileal epithelial cells in the case herein presented expressed both DNA MMR proteins MSH2 and MLH1. The presence of protein expression of these 2 major human DNA MMR proteins indicates the absence of pathogenic mutations in these MMR genes and the absence of microsatellite instability (MSI) at this stage in this neoplastic process. Therefore, MSI was not contributory to the pathogenesis of the high-grade dysplastic adenomatous changes in the case herein presented, but does not exclude the possibility that MSI may contribute to the subsequent progression to invasive adenocarcinoma. Indeed, a recent report documented increased microsatellite levels in urine from a patient who developed an invasive adenocarcinoma within an augmented bladder after cystoplasty . Loss ofIt is well established that malignant transformation in colonic carcinoma involves the transition from metaplasia, through benign adenoma (without high-grade dysplasia), adenoma with high-grade dysplasia, carcinoma in situ, and finally invasive adenocarcinoma. Tubular adenoma (with or without high-grade dysplasia) is a precursor of gastrointestinal adenocarcinoma, and requires complete surgical excision when encountered in small bowel segments used for urinary tract reconstruction . TubularPatients undergoing augmentation ileocystoplasty are at increased risk of glandular malignancy in the ileal segment. Therefore, clinicians should be aware of the potential of malignant transformation developing in the neobladder when cystoplasty with any kind of intestinal segments are performed. Hence, emphasizing the importance of careful lifelong follow-up, including cystoscopy and urine cytology, of all patients who undergo urinary tract reconstruction with small bowel segments.The author(s) declare that they have no competing interests.HBA participated in the histopathological evaluation, performed the literature review, acquired photomicrographs and drafted the manuscript. AMK and AVP conceived and designed the study, gave the final histopathological diagnosis and revised the manuscript for important intellectual content. All the authors read and approved the final manuscript.


Papillary meningiomas are rare meningeal tumors and are associated with aggressive clinical behavior as compared with other meningiomas. Because of their rare occurrence, they may pose a diagnostic dilemma to the unwary pathologist. We report a case of papillary meningioma in a 16-year-old boy.A 16-year-old boy presented with complaints of headache, progressively diminishing vision and more recently generalized seizures. MRI revealed a large bifrontal meningioma which showed presence of a predominantly papillary pattern with areas of focal necrosis, frequent mitoses and bone invasion. He underwent radical excision of the tumor and is free from recurrence or metastasis at 15 months follow-up.Papillary meningiomas are rare but well recognized variants of meningioma. They need to be differentiated from other intracranial tumors with a papillary pattern. They are malignant, frequently show bone and parenchymatous invasion and have the potential for extracranial metastasis. Their timely recognition could prevent local and distant metastasis and the mortality or morbidity associated with it. Meningiomas account for approximately 13–19% of all primary intracranial tumors and their prognosis is generally favorable  and is dA 16-year-old boy presented with a history of headache of 8 months duration, progressively decreasing vision for 3 months and more recently had an episode of generalized seizure 1 month back. Neurological examination did not reveal any focal neurological deficit except bilateral papilledema on fundus examination. MRI scan revealed a large 10 × 8 cm extra-axial hyperintense contrast enhancing mass lesion on the T1-weighted image, occupying the anterior cranial compartment [Fig Gross examination of the resected tumor showed a 10 × 8 × 2 cm soft tissue with its cut-section showing grayish-white, irregular surface with focal necrosis Fig  along wiPM is a malignant variant of meningioma and was first described in 1938 by Cushing and Eisenhardt [In one of the largest series of PM reported, Ludwin et al  describePM are frequently seen in the supratentorial compartment though rare locations like posterior fossa ,9, jugulIn meningiomas of higher grade, losses of chromosome 22q and 1p have been reported apart from mutations of the NF2 gene , but thePM is exceptional variant of meningioma with high frequency in children, increased incidence of local recurrences and potential for distant metastasis. Their timely recognition may have important implications for management as well as prognostication in order to reduce the morbidity and mortality.The authors declare that they have no financial or non-financial competing interests and all the authors have read and approved the manuscriptSA gave the histopathological diagnosis, took pictures and wrote the final draft of the manuscript. SV participated in grossing of the tumor, literature review, and modified the images and contributed suggestions for drafting the manuscript. SS participated in the histopathological diagnosis and KC was involved with the clinical management of the patient.