The benign tumors of nasopharynx are least encountered tumors in otolaryngology, as nasopharynx is considered one of notorious anatomical site for the malignant tumors. Pleomorphic adenoma of the minor salivary gland of nasopharynx and parapharyngeal space is rare. We present a pleomorphic adenoma of minor salivary gland which was mismanaged.An adult male presented with left nostril obstruction for five months. The examination found big mass extending from nasopharynx to oropharynx. On CT scan, this tumor was quite big and extending to the parapharyngeal space. The FNAB found it a carcinoma but it did not respond to radiotherapy. The excision biopsy of tumor revealed it as pleomorphic adenoma. We found only five published reports on this tumor arising from nasopharynx.Although, in this case report exact origin of the tumor could not be ascertained as it also appeared to be a parapharyngeal tumor but we kept the possibility of a nasopharyngeal tumor on the basis of clinical features. The pleomorphic adenoma of nasopharynx is rare. It can be misdiagnosed as malignant epithelial tumor on histopathology. The differentiation from its malignant variant is also difficult. A possibility of benign tumor should always be kept in nasopharyngeal growth with no evidence of metastasis, and histopathological diagnosis of growth should be available before any definitive treatment. Benign tumors of nasopharynx are rare since nasopharynx is a common site for malignant tumors. Pleomorphic adenoma is the most common benign tumor of the major and minor salivary glands, but rarely found in the nasopharynx. In this case report, we present a case of a large pleomorphic adenoma arising in the nasopharynx and extending to oropharynx that was diagnosed and managed as squamous cell carcinoma.In Sep 2007, a 35-year-old male presented with a progressive nasal obstruction associated with left aural fullness, hearing loss and hyponasal speech for the past five months. Later, the patient saw a mass pushing the soft palate downwards and began having difficulty in swallowing solid food. On examination, there was a mass behind and above the soft palate, which appeared to be arising from nasopharynx. Neck examination was found normal. Past history included a pyelolithotomy and gastrojujenostomy for duodenal ulcer.th Sep 2007, computed tomographic (CT) scan of the neck revealed a large mass of heterogeneous density involving the fossa of Rosenmuller, and extending to the valleculla through parapharyngeal space. The mass had heterogeneous contrast enhancement with central necrosis  of the mass was done intra-orally and microscopic examination revealed a poor smear with scanty cells. The pathologist suggested the possibility of a squamous cell carcinoma and advised a direct biopsy for definitive diagnosis. On 19th March 2008, a fresh CT scan  -9. PleomCT scan does not provide significant differentiation between carcinoma ex pleomorphic adenoma (CXPA) and benign pleomorphic adenoma as CXPA can have malignant appearance or benign features similar to pleomorphic adenoma. On MRI, low T1 and T2-weighted signal intensity has been suggested as an indicator for carcinoma ex pleomorphic adenoma .et al. [et al. [An extensive literature review revealed only five cases of pleomorphic adenoma arising in the nasopharynx ,18-21. Ret al.  excised Although, radiotherapy is indicated in recurrent pleomorphic adenoma but in our case, diagnosis of carcinoma was made on cytology alone. The patient received chemo-radiation but tumor increased in size, and then tumor was found to be a benign pleomorphic adenoma. Possibly, myoepithelial or epithelial atypia with keratin debris in pleomorphic adenoma Fig  led to cThe pleomorphic adenoma was undergoing malignant changes or it was a misdiagnosis, we could not ascertain the exact pathology, as we had no pre-radiotherapy histopathological evidence. However, who was at fault: clinician by not following the advice of pathologist for a direct biopsy, or oncologist who started chemo-radiation without any histopathological proof? We cannot blame anyone rather than ourselves as the clinician are the most accountable.In this case report, many questions remained unanswered but a lesson learnt was:1. The possibility of benign tumor should be kept in the differential diagnosis of nasopharyngeal tumor without adenopathy.2. Clinician should follow pathologist's advice, carefully interpret the cytological diagnosis, and consult the pathologist, whenever in doubt.3. An adequate tissue specimen should be obtained especially for cytological diagnosis as pleomorphic adenoma can simulate as a carcinoma.4. No patient should be taken for chemo-radiation without any histopathological evidence.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.FNAB: Fine needle aspiration biopsy; CT: Computed tomography; MRI: Magnetic resonance image; CXPA: Carcinoma ex pleomorphic adenoma; H & E: Hemotoxylin and EosinNoneThe authors declare that they have no competing interests.JST was the principal investigator and involved with management of the patient, design, concept and writing of the paper. He takes the responsibility for the integrity of the article. NKM was involved with management of the case, revision and final approval of the paper. SM was responsible for the histopathological analysis, critical revision and final approval of the paper. DRS was involved with management of the case, revision and final approval of the paper. AT was involved in design, literature review, data collection, drafting and final approval of the paper.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1472-6815/10/2/prepub


We report two cases of children who presented with acute abdomen due to gall bladder perforation and biliary peritonitis. Cholecystectomy with peritoneal lavage proved curative. Gall bladder perforations (GBP) after cholecystitis are usually seen in elderly patients (>60 years) and are rare in children, So far, An 11-year-old male child without any known medical comorbidity presented with a 3-day history of sudden-onset severe abdominal pain and distension. It was initially localized to the right upper quadrant and right lumbar region, but had evolved to a more generalized distribution at the time of presentation. It was associated with moderate-grade fever and nonbilious vomiting for 3 days.His vital signs were stable, except for tachycardia. His abdomen was distended, with generalized tenderness, guarding on palpation and minimal movement with respiration. His bowel sounds were sluggish. The laboratory tests revealed leukocytosis and neutrophilia. His serum amylase, lipase and serum electrolytes were all within normal limits. Abdominal radiographs showed no signs of intestinal obstruction or pneumoperitoneum. Ultrasound of the abdomen showed mild ascites and rest of the small and large bowel loops, visualized pancreatic parenchyma, liver, biliary tree were reported as normal. With a clinical diagnosis of perforative peritonitis, probably enteric ileal or an appendicular perforation, an emergency laparotomy was performed.At laparotomy, about 1/2 L of bilious fluid was drained. The gall bladder was found densely adherent to the duodenum. On adhesiolysis, a necrotic patch on the body of the gall bladder with a perforation was found. The anatomy of the Calot’s triangle was obscure and hence a near-total cholecystectomy, including the perforated area, was performed. Histopathology of the specimen showed a gall bladder lined by mucosa with focal ulceration and hemorrhage and the wall showing necrosis. A pathological diagnosis of acute on chronic gangrenous cholecystitis with perforation was made. Postoperatively, the Widal test was negative and the blood culture had shown no growth. The patient had an uneventful recovery.An 11-year-old male presented with generalized abdominal pain for 1 day that had initiated in the right lumbar and suprapubic region. He also complained of fever and multiple episodes of nonbilious vomiting. There was no other significant past or present history. There was generalized tenderness and guarding on abdominal examination.th postop day and is doing well on follow-up.All blood investigations  and radiograph of the abdomen were normal. The ultrasound abdomen showed free fluid in the abdomen with internal echoes. The bowel loops were dilated with sluggish peristalsis and pancreas, liver and biliary tree were reported as normal. With a clinical diagnosis of perforative peritonitis (appendicular or Meckel’s diverticular perforation) in mind, an emergency laparotomy was performed. Intraoperatively, frank bile was noted in the peritoneal cavity. The omentum was loosely adherent to the gall bladder, which had a perforation in the body. A fundus first cholecystectomy and peritoneal lavage was performed. Postoperatively, the Widal test was negative and the blood culture showed no growth. Histopathology revealed acute on chronic cholecystitis. The patient was discharged on the 7Only 5–10% of the patients with acute cholecystitis are associated with acalculous cholecystitis. GBP occu5Perforation results from occlusion of the cystic duct , which causes a rise of the intraluminal pressure due to retained intraluminal secretion. Our patients probably developed spontaneous GBP due to ischaemia of the gall bladder wall with inflammation and acalculous cholecystitis. Infections, malignancy, trauma and drugs  and systemic diseases such as diabetes mellitus and atherosclerotic heart disease are common predisposing factors. Fundus, 2et al. reported a case in a 14-year-old, preoperatively correctly diagnosed as acalculous cholecystitis with GBP, which was confirmed on laparotomy.[Acute uncomplicated cholecystitis is more common among females, with a female to male ratio of 2:1. However, GBP is more frequent in the male gender. GBPs are7parotomy. Both ourPerforation of the gall bladder can occur as early as 2 days after the onset of acute cholecystitis, or after a few weeks.[Abdominal X-rays may not always show pneumoperitoneum, as seen in our patients, and hence they are not always helpful. Ultrasonography and computerized tomography (CT) may demonstrate abdominal fluid but lack specificity to diagnose GBP. SignificEarly surgical intervention is an important step in the management of GBP. Although, clinically, it is difficult to predict the diagnosis of GBP, it is commonly assumed as bowel perforation when a patient presents with features suggestive of perforative peritonitis.46 Cholec4Earlier reported Indian series of cholecystitis in children did not have any GBPs. Our caseEarly diagnosis of GBP and immediate surgical intervention are of prime importance in decreasing the morbidity and mortality associated with this condition. Similarly, given the clinical scenario, we believe that interval cholecystectomy in all diagnosed cases of acalculous cholecystitis should be a viable option to consider preventing future complications.


Subject. A 27-year-old Hispanic male with clinical manifestation of            neurofibromatosis type 1 developed chronic constipation and urination difficulty along with            recently increased abdominal bloating and anorexia. He also noted 40 lbs weight loss           over period of 1 year. Physical and radiographic examinations revealed a large mass in the           right pelvic fossa.                    Results. The surgically removed tumor was demonstrated,           histologically, immunohistochemically, and ultrastructurally, to be a malignant           hemangiopericytoma.                    Discussion.Although non-neurogenic tumors associated with neurofibromatosis            have been reported in these patients, only one hemangiopericytoma case has been found in the English literature. We report here another case of this rare malignant            hemangiopericytoma in a patient with neurofibromatosis.


Some skin lesions may accompany malignancies. Acanthosis nigricans, one such lesion, is a paraneoplastic dermatosis characterized by hyperpigmented and velvety verrucose plaques observed as symetric eruptions. With this report, we aim to present a rare case of concomitant lung cancer and acanthosis nigricans. Malignant acanthosis nigricans is most commonly associated with intra-abdominal malignancies. A 65-year-old patient who had hyperpigmented, hypertrophic and symmetric verrucose lesions at the flexor surfaces of the lower and upper extremities, face, palms and the axillary region. Thoracic computed tomography demonstrated a hypodense mass lesion with a dimension of 5 × 5.5 cm at the center of basal segment bronchi of the left pulmonary lobe. Fiberoptic bronchoscopy showed that the access to the lower left lobe was almost completely obstructed by the endobronchial lesion. The result of the histopathologic examination of the endobronchial tissue biopsy was reported as non-small cell (adenocarcinoma) lung cancer. Result of the histopathologic analysis of the punch biopsy of the skin lesions was reported as acanthosis nigricans .There are no pathognomonic dermatological findings for lung cancer. In conclusion, there are skin lesions that accompany lung cancer and we believe that these should be considered for differential diagnosis. Some skin lesions may accompany malignancies. Acanthosis nigricans, one such lesion, is a paraneoplastic dermatosis characterized by hyperpigmented and velvety verrucose plaques observed as symmetric eruptions.  It is often localized on flexor surfaces such as the axilla, posterior neck fold, anterior umbilical, and popliteal and inguinal areas where skin folds. It may be idiopathic but may also be associated with endocrinal disorders, malignancies, medicines, and genetic syndromes. Besides, acanthosis nigricans may also present autosomal dominant involvement –3.Malignant acanthosis nigricans is most commonly associated with intra-abdominal malignancies. There are very few reports in the literature of malignant acanthosis nigricans associated with lung cancer. With this report, we aim to present a rare case of concomitant lung cancer and acanthosis nigricans.A 65-year-old male patient who presented to our clinic had the complaints of cough and phlegm for the past 10 years which particularly exacerbated in winter and shortness of breath exacerbating with effort for the past one year. An informed consent was taken from the patient. Patient had no comorbid disease including diabetes mellitus.The patient also had dark-colored lesions that he first observed one month ago and spread to the body thereafter, and the additional complaints of worsened shortness of breath and weight loss. His history involved smoking, 60 boxes/year. His familial history had no relevant findings. Physical examination revealed the following values: BP: 120/80 mmHg, RR: 16/min, and temperature 36.5°C. The patient had body weight: 64 kg and body length: 170 cm, BMI: 22.14 kg/m².The patient had hyperpigmented, hypertrophic and symmetric verrucose lesions at the flexor surfaces of the lower and upper extremities, face, palms, and the axillary region. An examination of the respiratory tract with auscultation revealed bilateral infrequent expiratory rhoncus. Findings of other examinations were normal. Laboratory findings showed an ESR value of 64 m/h, fasting blood glucose as 74 mg/dl. Compensated respiratory acidosis was observed during blood gas analysis. Other laboratory findings were within normal ranges. Thoracic computed tomography demonstrated a hypodense mass lesion with a dimension of 5 × 5.5 cm at the center of basal segment bronchi of the left pulmonary lobe, which did not have clear borders distinguishable from the peripheral consolidation region . Fiberoptic bronchoscopy showed that the access to the lower left lobe was almost completely obstructed by the endobronchial lesion. Abdominal tomography, esophagoduodenoscopic, colonoscopic examination revealed no malignancies, only erosive gastritis was found. Primary lesion was considered as the primary tumor as there was no malignancy at any organ. The result of the histopathologic examination of the endobronchial tissue biopsy was reported as nonsmall cell (adenocarcinoma) lung cancer .Result of the histopathologic analysis of the punch biopsy of the skin lesions was reported as acanthosis nigricans . idiopathic acanthosis nigricans” from the literatures, but we consider that the most of the cases can be associated with a disease. There are no pathognomonic dermatological findings for lung cancer. However, some skin lesions should suggest lung cancer in differential diagnosis. Acanthosis nigricans is one such lesion. Acanthosis nigricans may both be benign or malign. Its benign form may be observed in association with some endocrinal disorders, obesity, some genetic syndromes, and drug use  , 5. We c Malign acanthosis nigricans accounts for 20% of all acanthosis nigricans cases. It develops as a result of expression of the transforming growth factor alpha  of tumor cells, melanocyte stimulating hormone alpha, and peptides causing cellular proliferation including insulin-like growth factor 1 .Of the malignancies accompanying malign acanthosis nigricans in the adult population, 45–69% are gastric adenocarcinomas. Malign acanthosis nigricans is observed with a frequency of 70–92% of all abdominal neoplasms  lymphomaAfter diagnosing as acanthosis nigricans, benign diseases were investigated . After that, possibility malign disease was thougt. The most common form of malignancy with MAN, the abdominal malignancy was investigated by abdominal tomography, esophagoduodenoscopy, colonoscopy, and rectoscopy which were all normal. The edoscopic investigations should be done in every patient with MAN. They all revealed normal findings in this patient. Brain computerised tomography and bone scintigraphy revealed no lesions. If there were no lung cancer in our case, the other rare malignancies with MAN listed above would have been searched. First described by Pollitzer in 1891, acanthosis nigricans is a key indicator of insulin resistance independent from the body mass index –15. It hCo-occurrence of acanthosis nigricans and lung cancers is rare. The association with bronchial carcinoma was first reported in 1909 . Curth eNo masses were detected during the scans of the other organs. Being assessed as advanced phase nonsmall cell lung cancer, the patient was examined by the oncology department. Chemotherapy and radiotherapy were initiated and regressions were noted in the patient's skin lesions, consistent with the literature. The patient refused to go through thorasic surgery and died three months after presentation due to respiratory failure.In conclusion, there are skin lesions that accompany lung cancer and we believe that these should be considered for differential diagnosis.


Congenital epulis is a rare lesion found on the alveolar process of a newborn child, diagnosed soon after birth. The lesion has a site predilection for the anterior maxillary alveolar process and a 9:1 sex predilection for females. Once diagnosed the traditional management of the lesion has been surgical excision under general anesthesia.The purpose of this case report is to describe spontaneous regression of congenital epulis in a three week old healthy African American female child. She presented with a 1.5 cm bilobed sessile nodular lesion in the region of the right maxillary cuspid. The clinical impression was congenital epulis. Since the lesion was not interfering with feeding and respiration, a conservative approach was taken. The child was followed-up for 18 months, during which the lesion progressively regressed.Conservative management prevented unnecessary surgery and anesthesia exposure in a neonate. The congenital granular cell epulis (CE) is a benign tumor arising from the alveolar ridges of newborns and composed of nests of cells with granular cytoplasm set in a prominent vasculature . NeumannA three-week-old African American female child was referred to our clinic for the "evaluation and treatment of cysts in her gums". The child was born at full term via vaginal delivery. She had no other medical problems. Her mother reported that pre-natal history was unremarkable, and that the child was born with a lesion in her mouth. In her mother's opinion the lesion had reduced in size over the three-week duration. Intra-oral examination revealed a bilobed sessile nodular lesion approximately 1.5 cm size in its greatest dimension on the right maxillary alveolar process, in the region of the unerupted canine Figure . The priin utero by 3D ultrasound and magnetic resonance imaging (MRI) examinations [CE is also known as CE of the newborn , congeniinations ,6. The linations . It has inations . Clinicainations . Usuallyinations ,8 and oninations . The lesinations ,10.The traditional management of the lesion has been complete surgical excision under either general anesthesia  or localHistopathologically, CE comprises of large round cells with granular eosinophilic cytoplasm in a fibrous connective tissue stroma. The overlying surface epithelium exhibits atrophy of the rete ridges . There eCE is usually an isolated finding and it has not been found to be pathognomic of any other medical condition or syndrome. However, a thorough review of case reports of CE revealed CE occurring in infants with polydactyly , goiter ex utero intra-partum treatment may be planned in cases with obstructive lesions diagnosed in the fetus in the pre-natal period [Recent advances in pre-natal imaging have enabled assessment of fetal swallowing and airway patency with the use of 3D ultrasound ,23. Thisl period ,23. Folll period .Of the more than 200 cases of CE of the new born reported in the English literature, there have been eight case reports -16,24-27Our patient presented with a relatively small lesion (1.5 cm in its greatest dimension) on the maxillary alveolar process, which was not causing any problems with feeding or respiration. With radiographic and urinary investigations we ruled out neuroectodermal tumor of infancy which also has a maxillary anterior site predilection . ParentaIt may be concluded that if a CE lesion is less than 2 cm in its largest dimensions and the lesion does not interfere with respiration or feeding, non-surgical management of the lesions ought to be considered. The advantage of conservative management of such cases is to avoid exposure of general anesthesia in a neonate for a lesion which is known to be benign and will not recur. Clinical judgment should be exercised in deciding which cases of CE to monitor for regression and which ones to consider for surgical excision.CE: congenital epulis.The authors declare that they have no competing interests.Written informed consent was obtained from the mother of the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.PR examined and treated the patient. RBB provided oral and maxillofacial pathology consultation. RJM provided pediatric dentistry consultation. All authors have read and approved the final manuscript.


Peripheral primitive neuroectodermal tumors of the urinary bladder are rare and tend to occur in an older age group than do their counterparts in bones and soft tissue.We report a case of peripheral primitive neuroectodermal tumor of the urinary bladder in a 67-year-old woman of Arab origin. She had undergone transurethral resection followed by chemotherapy because of pulmonary metastasized muscle-invasive squamous cell carcinoma of the bladder in 2005. One year later, she first presented with a history of repeated hematuria in our institution. Performing cystoscopy any tumor could be detected. Control cystoscopy two months later showed a tumor mass of 3 cm in diameter at another location than described for the first tumor. After perforating by transurethral resection partial bladder resection had to be done. Tissue specimen after pathological analysis revealed a peripheral primitive neuroectodermal tumor with tumor cells reactive to cluster of differentiation 99, neuron-specific enolase and S100 protein and stained negative for other markers such as cytokeratins, epithelial membrane antigen, desmin, smooth muscle actin, chromogranin and leucocyte common antigen. Staging computerized tomography was especially free from any hint on organ metastasis, but the patient died due to a cardiac problem only a few months later.To the best of our knowledge, we report the eighth case of bladder peripheral primitive neuroectodermal tumors in literature and the first concerning an Arab patient. It is also the first presentation of a peripheral primitive neuroectodermal tumor patient with a history of squamous cell carcinoma of the bladder. As in other cases, expression of single-chain-type 1 glycoprotein and neural markers was positive and the disease was at an advanced stage at the time of diagnosis. Peripheral primitive neuroectodermal tumors (pPNET) are malignant small round-cell tumors that occur predominantly in bones and soft tissue of children and young adults , only in2G3). Computerized tomography (CT) scan showed diffuse but irregular thickening of the bladder wall, pelvic lymphadenopathy and multiple small bilateral pulmonary metastases. Mild hydronephrosis was noted in the right kidney, other abdominal organs were essentially normal. The patient received three courses chemotherapy, to which her response was excellent, and was discharged home in good condition. In November 2005, the patient underwent a follow-up cystoscopy that showed a recurrent malignant urinary bladder tumor which was completely resected. A CT scan of thorax, abdomen and pelvis, that did not show evidence of cancer progression, and a negative bone scan completed the follow-up.We report the first case of a peripheral primitive neuroectodermal tumor of the urinary bladder in an Arab country. The Department of Surgery of Al Sabah Hospital, Kuwait admitted a 67-year-old diabetic and hypertensive Arab woman in February 2006, due to severe hematuria and fever. In January 2005, the woman had already been treated in a hospital of Great Britain for repeated hematuria for the duration of one year. During this initial admission, cystoscopy revealed an intrinsic urinary bladder mass that was resected. Histologically, it was reported as poorly differentiated squamous cell carcinoma . As an initial attempt at transurethral resection (TUR) resulted in perforation, a partial bladder resection was performed. A post-surgery CT of the thorax, abdomen and pelvis showed mild dilatation of the right renal pelvis, a thickened urinary bladder wall without an exophytic mass and no evidence of lung metastasis. The tumor specimen gained during surgery consisted of grey-brown tissue measuring 3 × 2.5 × 1 cm. Pathohistology showed features of a malignant small round-cell tumor  of the urinary bladder are rare, aggressive masses that partly tend to occur at a much older age than do their bone and soft tissue counterparts in the Ewing's sarcoma family. It is said, that concerned patients are either of young age like in other small round-cell tumors ,8,9 or oPatients in some cases already had metastases at the time of diagnosis and this suggested a poor prognosis -8], how, how8], CD: Cluster of differentiation; CT: Computerized tomography; MIC2: Single-chain type-1 glycoprotein; NSE: Neuron-specific enolase; pPNET: Peripheral primitive neuroectodermal tumor; TUR: Transurethral resection.Written informed consent was obtained from deceased patient relatives for publication of this case report and accompanying figures. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.MKA supervised treatment of the patient and drafted the manuscript, MN, TK and WO helped to draft the manuscript. KK performed the histopathological analysis and helped to draft the manuscript.


Intraosseous lipoma is an uncommon entity that presents almost exclusively as a solitary lesion. Multiple intraosseous lipomas are exceedingly rare with only a few cases being reported in the literature.We present a case of 43-year-old African American female who presented with left leg and left wrist pain. The initial radiographs revealed well-defined radiolucent lesions in multiple bones involving the left wrist and the left lower limb. The magnetic resonance demonstrated multiple lesions, which showed high signal on the T1 and low signal on the fat suppressed T2 images. This favored the diagnosis of intraosseous lipomatosis that was confirmed by biopsy.Multiple intraosseous lipomatosis is an uncommon but important differential for multiple radiolucent lesions on the plain radiographs. This condition can lead to pathological fractures. Magnetic resonance imaging can aid in providing an accurate diagnosis. The awareness of this condition can help the clinician in guiding the correct diagnosis and management. Multiple intraosseous lipomatosis is very rare and idiopathic entity. With few cases being reported in the literature -6 the etWe present a case of an unemployed 43-year-old African American female who presented with bilateral leg pain. There was no history of trauma. Her past medical and surgical history was significant only for prior hysterectomy for fibroids and hormone replacement therapy. She denied smoking or drinking alcohol. The physical exam was significant for mild point tenderness on the medial aspect of her left knee. There was no joint effusion and no limitation in the range of movement.The plain film radiographs demonstrated well-defined lytic lesions of the left distal femur and the medial plateau . The plaAdditional studies include complete blood count, liver, renal and thyroid function tests, serum lipid panel, serum electrophoresis, B+ T flow cytometry and bone marrow biopsy. The results of these tests were normal.Magnetic resonance imaging (MRI) was subsequently done to evaluate these lesions further. MRI of the left knee and left wrist revealed regions of high T1 signal and low signal on the fat suppressed T2 images Figures ,4. TheseThe biopsy of the medial tibial plateau lesion showed hyperplastic adipose tissue and confirmed the diagnosis of intraosseous lipomatosis. No malignant or atypical cells were visualized on the biopsy .Intraosseous lipoma is a rare entity and was first described in 1880 ,8. IntraIntraosseous lipomas are almost always solitary lesions. Multiple bony involvements are unusual. There have been only a few case reports in the literature of multiple intraosseous lipomas -6. CliniImaging plays a vital role in the diagnosis of intraosseous lipomas. A correct radiological diagnosis can often be regarded as an endpoint to the investigation and biopsy or surgery can be avoided in the majority of cases . On radiThe management involves curette and graft of the symptomatic and progressive lesions ,12. FollIn summary, the knowledge of this unusual entity can help the clinician in explaining the patient's pain and aid in counseling the patient about the diagnosis, future risk of pathological fractures and the various treatment options.


Cholangiocarcinoma is a dreaded complication of unresected choledochal cysts, with an incidence approaching 20-30% in early adulthood. The risk of cholangiocarcinoma remains high where an internal drainage procedure has been performed and the cyst has been partially resected or left unresected. We report a case of cholangiocarcinoma occurring in an unresected choledochal cyst following a drainage procedure in infancy and highlight the role of PET/CT in its diagnosis. Cholangiocarcinoma is a dreaded complication of unresected choledochal cysts, with an incidence approaching 20-30% in early adulthood. The risk1A 20-year-old unmarried girl reported to the surgical outpatient department with complaints of unrelenting pain in the upper abdomen, vomiting, and loss of weight for 2 months. There was a history of some surgery done for jaundice in early infancy but the details were not available. Clinical examination revealed a jaundiced, cachexic patient, with a rounded mass in the right upper quadrant that was separate from the liver. She was anemic and biochemical tests for liver function revealed a cholestatic type of jaundice.USG revealed a large cystic lesion containing air and debris in the subhepatic region and a lobulated soft tissue mass along the anterior wall of this cyst inferiorly . AnotherOn the basis of these findings, the diagnosis of a type 1 choledochal cyst, with evidence of a prior cysto-duodenal bypass surgery and the presence of an intracystic cholangiocarcinoma, was made. The patient was unwilling for image-guided fine needle aspiration cytology. A PET/CT performed to further evaluate the intracystic mass showed markedly increased tracer uptake within this soft tissue as well as in the pancreatic head mass (SUV 4.5). Additional peritoneal deposits were also detected in the left lower quadrant and pelvis . This reCholedochal cysts are more prevalent in Asia than in Western countries and approximately 33-50% of reported cases are from Japan. Todani'sType I - Dilatation of hepatic and common bile ducts (40-85%)Type II - CBD diverticulum (2-4%)Type III - Intraduodenal CBD dilatation (1.4-5.6%)Type IV - a Intrahepatic and extrahepatic bile duct dilatation (18-20%)Type IV - b Multiple extrahepatic cysts (rare)Type V - Intrahepatic bile duct dilatation (rare)The classic clinical triad of abdominal pain, jaundice, and abdominal mass is found only in a minority of patients. The clinical presentation largely depends on age - abdominal pain being the most frequent presenting symptom in adults and jaundice in infants. In most 11Complications of choledochal cysts are the result of stasis and include cholangitis, stone formation, recurrent pancreatitis, cirrhosis, and portal hypertension. Cholangiocarcinoma is a dreaded complication and is attributed to chronic mucosal irritation; it has an incidence of 10-30%. This risk is even higher after drainage procedures.1 Nodular 5Because of the high and persistent risk of cholangiocarcinoma, primary excision of extrahepatic choledochal cysts with biliary-enteric anastomosis is the treatment of choice in all cases, even in the absence of symptoms.2 Cholang


Benign fibrous histiocytoma is an interesting and challenging entity even in its most usual, cutaneous presentation. Noncutaneous presentation is extremely limited, even more so for the mucosa of the head and neck area. We herein report such a case, describing the clinical characteristics of the lesion, complete diagnostic evaluation, management, and follow-up. Diagnostic histopathological challenges are specifically illustrated. A complete review of the relevant literature is also included. The benign fibrous histiocytoma (BFH) is a mesenchymal tumour that has been described as a benign neoplasm composed of fibroblasts and histiocytes arising in the cutaneous and noncutaneous soft tissues . BecauseThe occurrence of this lesion in deep soft tissues of the head and neck has been rarely reported , 5. An eIn contrast to malignant fibrous histiocytoma (MFH), benign fibrous histiocytoma of the noncutaneous soft tissues of the head and neck has received little attention in the head and neck surgical literature.BFH is seen mainly in adults and most frequently in young and middle aged women. Patients are usually under 50 years of age and have a history of sun exposure, trauma, or chronic infection, suggesting that BFH is a reactive disease , 13.This article describes a case of BFH of the buccal mucosa and discusses its clinical and pathological characteristics and management.A 36-year-old man was referred to the Department of Oral and Maxillofacial Surgery of “Theagenio” Cancer Hospital for an asymptomatic, slowly growing nodular lesion on the right buccal mucosa that had been present for approximately one year. His medical history was noncontributory. Examination revealed a well-circumscribed, moderately mobile, nontender fibroelastic lump of approximately 3 cm in diameter. The mucosal surface appeared smooth and moderately inflammatory, possibly due to local trauma .The patient underwent an intraoral excision of the mass under general anesthesia with primary closure. On surgical excision the mass was easily dissected from the surrounding tissues .Histologic analysis of the specimen revealed a macroscopically smooth, well-circumscribed encapsulated lesion of 2.4 cm in greatest dimension. The mass was grossly round in appearance, with a white grey surface . Microscα-SMA), S-100 protein, Leu7, and CD117 (c-kit). The tumour cells showed strong immunoreactivity for vimentin, weak and focal immunoreactivity for CD34  and someThe final diagnosis was BFH. The patient was free of loco-regional disease at 12-month follow-up.Pathologic analysis and diagnosis of this type of lesions is often challenging and usually based on a combination of light microscopy and immunohistochemistry .Until recently the term fibrous histiocytoma (FH) referred to both benign and malignant neoplasms. The differential diagnosis between the two entities was often difficult . The so-α has occasionally been reported for BFH [Due to the lack of specific markers for fibrohistiocytic lesions, the diagnosis of BFH is generally based on the absence of markers for cells of other lineages . Immunoh for BFH .α-SMA (−).In the present study, the diagnosis was confirmed using immunostaining for vimentin (+), CD68 (+), CD34 (+), S-100 (−), CD117 (−), Leu7 (−), desmin (−), and Histopathologically, BFH typically shows a biphasic cell population of histiocytes and fibroblasts . In some The treatment of choice is the complete resection of tumour, with an excellent prognosis and recurrence rate of almost zero , 34. FewA review of the literature identified additional 6 cases of buccal mucosa BFH . Most leWe reported the clinical, microscopic, and immunohistochemical aspects of a case of BFH of the buccal mucosa. Although rare, BFH must be considered in the differential diagnosis of oral soft tissue tumors.The prognosis of BFH is excellent. The results of this study support local excision as definitive treatment of BFH of buccal mucosa. When pathologically clear margins are found, the incidence of local recurrence is unlikely. Incomplete excision or enucleation may result in significant percentage of recurrence . RadiatiA thorough knowledge of this lesion is important to the head and neck surgeon, who will provide the primary management of this rare lesion.


Pyloric atresia (PA) is a very rare condition. Its incidence is approximately 1 in 100,000 newborns and constitutes about 1% of all intestinal atresias. We describe the neonatal course of a peculiar case of type 1 pyloric atresia, in which the pyloric membrane was connected to a second duodenal membrane through a virtual duodenal lumen in a premature newborn. The atypical variant required an unusual side to side gastroduodenostomy. We emphasize the importance of a prompt diagnosis to avoid potentially fatal complications and to warrant a good outcome even in the presence of a strange form of PA in the neonatal period. Pyloric atresia (PA) is a very rare condition. Its incidence is approximately 1 in 100,000 newborns and constitutes about 1% of all intestinal atresias . Sometimleft). The baby was referred for a surgical opinion on day 3. Contrast study with barium meal confirmed complete obstruction at the pyloric region . At laparatomy she was found to have a moderately distended stomach and a narrowed duodenal tract. A first membrane was found at the pylorus and a second one was found 2 cm forward, with a virtual duodenal lumen between them. Partial resection of the gastric antrum and of the first duodenal tract was performed, followed by a two layers, side to side gastroduodenostomy.A girl was born by emergency cesarean section for abruptio placentae at 34 weeks' gestation and weighted 2130 grams. Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. Pregnancy was complicated by gestational diabetes and polyhydramnios. Physical examination at birth revealed a good-looking, with normal vital data and no signs of sepsis. Routine hematological examinations showed normal values. Nonprojectile and nonbilious vomiting began on the second day of life and persisted for the next day, with staunching > 4 cc/kg after feeding. Feeding was suspended but emesis persisted and the abdomen was mildly distended. A high intestinal obstruction was suspected and an abdominal roentgenogram showed a single gastric bubble with no air in the small and large intestine (Figure The child had an uneventful postoperative course. Ten days postoperatively, a contrast study documented the patency of the entire gastrointestinal tract Figure . She wasth and 12th week of intrauterine life [Pyloric atresia is a very rare condition. Its incidence is approximately 1 in 100.000 newborns and constitutes about 1% of all intestinal atresias . Calder ine life ,11. Famiine life -6 PA mayine life -9. The pAntenatal diagnosis may be difficult and in our case only suspected by the presence of polyhydramnios, which is associated with PA in more than 50% of cases, with a dilated stomach but in the absence of a double bubble . The cliDifferent operative procedures can be used, depending on the anatomic type. In the literature, the best results from operative treatment of typical type 1 and type 2 PA were obtained by excision of the membrane and pyloroplasty according to Heineke-Mikulicz or Finney . Pyloro-Our case demonstrates that a prompt diagnosis is crucial to obtain a good outcome, even in the presence of a strange form of PA in the neonatal period.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal."The authors declare that they have no competing interests.EZ took care of the general presentation and the final version of the manuscriptMC got all the information about the case and wrote the first draft of the manuscriptCP and LN were the surgeons involved, they gave the pictures and all details concerning the surgical proceduresSZ made an extensive literature review and revised the English version.We declare that all authors read and approved the final manuscript


A case of renal adenosquamous carcinoma is presented. The fact that the urothelium has no glandular or squamous structures makes the pathogenesis of this tumor unique. The process is assumed to begin with urothelial metaplasia resulting from chronic irritation leading to dysplasia and subsequently squamous and glandular differentiation. Primary adenosquamous carcinoma of the kidney is a rare phenomenon. We describe a case of a 62-year-old diabetic male with previous bilateral staghorn calculi who presented to our unit with anemia, painless left hypochondrial mass, microhematuria and leukocytosis.A 62-year-old male, with a past history of diabetes mellitus, hypertension and chronic renal impairment had bilateral staghorn calculi for which he underwent bilateral percutaneous nephrolithotripsy (PCNL) 6 years ago. He also underwent left kidney double-J stenting 1 year ago for left hydronephrosis.He was initially admitted to the gastroenterology unit for diarrhoea of 2 weeks duration. There was no fever, loss of weight or loss of appetite. On clinical examination, he was afebrile but was noted to have mild pallor. Abdominal examination revealed a nontender left hypochondrial mass. Per rectal examination was normal.9 units/l. The hemoglobin level was 9.7 g/dl with a microcytic hypochromic picture. Septic workup which included blood cultures, urine culture and a chest radiograph was normal. Urine microscopy showed microhematuria and pyuria.Blood investigations showed leukocytosis with raised total white blood cells of 31×10Gastroscopy and colonoscopy demonstrated antral gastritis and ascending colon diverticula, respectively. An ultrasound of the abdomen revealed a left renomegaly secondary to a large complex septated cyst with internal debris and left renal lower pole calculi. A computed tomographic (CT) scan of the kidneys demonstrated a large 7.8-cm heterogenous lesion in the lower pole of the left kidney with perirenal fat stranding, and adjacent enlarged para-aortic lymph nodes .The patient underwent an open left radical nephrectomy, and intraoperatively was found to have large left renal lower pole tumor adherent to, but not involving, the posterior peritoneum with edematous perirenal fat.Histologic finding was that of a poorly differentiated adenosquamous carcinoma involving the renal pelvis. There was perineural and perirenal fat invasion. The remaining specimen showed hydronephrosis, chronic pyelonephritis and focal atypical squamous metaplasia of the pelvis. Surgical margins were cleared of tumor.In the postoperative period, the patient developed enterococcus wound infection which resolved with intravenous antibiotics. He was discharged well and was subsequently referred to the oncologist for chemotherapy.Three months after surgery, the patient was admitted for complains of shortness of breath. A chest radiograph done showed multiple cannon ball lung lesions and a moderately sized left pleural effusion  The effuPrimary adenosquamous carcinoma of the kidney is rare with only a handful of cases reported in the medical literature.2 SecondaPrimary adenosquamous tumors are associated with calculi, hydronephrosis and pyelonephritis. Hence, chronic irritation due to recurrent infections is assumed to be an etiologic factor.–6 Chroni1Key histologic features noted in this case were that of sheets of pleiomorphic cells with foci of squamous differentiation (keratinized squamous pearls)  and glanRenal adenosquamous carcinoma follows an aggressive course. It can metastasize to locoregional areas or to diWe learnt that from this case that although primary renal adenosquamous carcinoma of the renal pelvis is a rare entity, it should be considered as one of the differential diagnosis in the evaluation of patients with microhematuria. This is because clinical presentation is atypical, and hence, could lead to a delay in diagnosis and treatment of this aggressive disease.


Thymic large cell neuroendocrine carcinomas (LCNECs) are very rare. We here describe a case in which the tumor could be completely resected. A 55-year-old male was admitted to our hospital for treatment of an anterior mediastinal tumor found at a regular health check-up. The patient underwent an extended thymectomy of an invasive thymoma of Masaoka's stage II that had been suspected preoperatively. The tumor was located in the right lobe of the thymus and was completely resected. Final pathological diagnosis of the surgical specimen was thymic LCNEC. The patient underwent adjuvant chemotherapy with irinotecan and cisplatin in accordance with the diagnosis of a lung LCNEC, and is alive without recurrence or metastasis 16 months after surgery. Primary thymic neuroendocrine carcinomas (NECs) were categorized under the rubric of 'thymomas' until 1972, when Rosai and Higa suggested that these tumors were sufficiently distinctive to warrant classification as carcinoid tumors . In partA 55-year-old Japanese male was admitted to the Kitasato University Hospital for further examination and treatment for an abnormal shadow on the chest x-ray found at a regular health check-up. He had smoked 35 packs per year for 20 years. Chest x-ray films showed a solid mass with a clear border at the right hilum and a negative silhouette sign for the right first arch Figure . EnhanceUnder the diagnosis of invasive thymoma or thymic carcinoid, the patient underwent an extended thymectomy. The tumor was intraoperatively revealed in the right lobe of the thymus without any invasion to the adjacent organs:the aorta, superior vena cava, pericardium, bilateral phrenic nerve, or the right lung. Because the tumor had invaded the right parietal pleura, we also resected the right parietal pleura with a sufficient surgical margin.Macroscopically, the elastic soft tumor surrounded by thymic fat tissue was 40 × 35 × 28 mm in size. The cut surface was mainly whitish-yellow in color and showed focal necrosis and red bloody spots.Microscopically, the tumor manifested morphologic features of a carcinoid. The tumor cells were arranged in wide trabeculae with irregular nests separated by thin fibrovascular stroma, and scattered abortive rosette-like structures Figure . The tum2 and irinotecan at 60 mg/m2 for three courses, and is alive without recurrence or metastasis at 16 months after surgery.The patient underwent adjuvant chemotherapy based on a platinum doublet containing cisplatin at 60 mg/mThe neuroendocrine subtype of thymus tumors is defined on the basis of histopathological features and immunophenotypes. In recent studies ,5, NECs Although chest CT and MRI revealed no invasion to the superior vena cava or the innominate vein in the present case, and T1- and T2-weighted images demonstrated isointensity, the tumor was highly suspected of having invaded the normal thymic tissue due to its unclear rim. Therefore, our preoperative diagnosis was an invasive thymoma or a carcinoid.For optimal treatment, an accurate pretherapeutic diagnosis is important. However, as a thymic tumor is not always morphologically homogeneous, this may be difficult with a standard needle biopsy. Surgery offers the best chance for a definitive diagnosis and curative treatment of thymic tumors. The differential diagnosis for the anterior mediastinum includes other primary mediastinal tumors, mainly thymoma, paraganglioma, lymphoma, parathyroid adenoma or carcinoma, as well as medullary carcinoma of the thyroid. The most difficult but most important differential diagnosis in this setting is with thymoma, particularly of the spindle cell type. This latter can often show areas displaying a prominent neuroendocrine appearance with abundant epithelial cells disposed radially around an empty space closely simulating the microacinar growth pattern sometimes observed in carcinoids. To make a successful differential diagnosis, immunohistochemical staining can be helpful. Even though both types of lesions share strong CAM 5.2 positivity, thymomas are negative for neuroendocrine markers  and may be useful for NECs [Thymic LCNEC is very rare. A search of the PubMed database revealed only a few case reports in the literature -11. MegaCurrently, there is no evidence to support the use of postoperative therapy for Thymic LCNECs. Recent studies -14 of LCBecause primary thymic LCNECs are very rare, and the patients'prognoses are very poor, along with the lack of experience, a standardized treatment protocol, and the limited literature, all these contributing factors make it a difficult tumor to treat. Additional studies area warranted to determine the optimal treatment of thymic LCNECs.The authors declare that they have no competing interests.FO carried out the manuscript and collected references. YS coordinated all authors. FO and YS underwent this operation, and AI, HA, and KN helped for clinical support with them. SJ and IO reported pathological findings and took the pathologic pictures. AI and YS helped to draft the manuscript.All authors read and approved the final manuscript.Written informed consent was obtained from patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Primary localized laryngeal amyloidosis is an extremely rare condition. It usually presents with hoarseness, pain and/or difficulty in breathing.We present the case of a 23-year-old woman with primary localized laryngeal amyloidosis who presented with hoarseness and dysphagia.2 laser technology method.A search of PubMed shows that dysphagia in patients with laryngeal amyloidosis has been reported only once, although this symptom is relatively common in other conditions presenting with laryngeal mass. There were no signs of any systemic disease in our patient and diagnosis was established histopathologically. She was treated surgically by microlaryngoscopy under general anesthesia and the mass was excised using a CO Amyloidosis is a benign, slowly progressive condition that is characterized by the presence of extracellular fibrillar proteins in a variety of organs and tissues. Localized deposition of amyloid may be observed in individual organs without any systemic involvement. The progressive accumulation of amyloid deposits interferes with the normal structure of affected tissues resulting eventually in impairment of their function. Localized deposition of amyloid protein is regarded to be the result of local synthesis rather than the deposition of light chains produced elsewhere in the human body.Primary laryngeal amyloidosis is a rare lesion representing 1% of all benign laryngeal tumors  describeWe present a case that underlines the role of birefringence from red Congo stain to confirm the diagnosis. Another important feature of our case was that dysphagia was the main presenting symptom.A 23-year-old Caucasian Greek woman presented with a history of progressively worsening hoarseness during the previous 9 months and recent onset of dysphagia. She reported no weight loss and she denied the use of tobacco or excessive alcohol consumption. She had a history of skin atopy, reporting sensitivity to wool, and a history of an episode of anaphylactic reaction of unknown cause 2 years previously.Physical examination of the neck was normal. Indirect laryngoscopy and endoscopy with a flexible endoscope revealed a smooth, red-yellow, cystic formation localized in the left hemilarynx between the false vocal cord and the aryepiglottic fold. Chest X-ray was normal. A tracheal computed tomography (CT) scan in 5 mm sections confirmed the existence of the mass Figure . The thy2 laser. Pathological examination revealed a mass with a smooth surface. On macroscopic inspection the cut sections were yellow-grey and solid with a soft and elastic consistency. On microscopy, it was not clear whether its structure was bundled or micronodular, consisting of bundles of woven eosinophilic tissue, with sparse fibroblastic cells, several small vessels and rare atrophic glandular regions. There were also a few collections of lymphocytes and granulocytes. Staining with Congo red stain, under polarized light, revealed blue-green birefringence throughout the mass due to the presence of amyloid. No signs of malignancy were seen.We proceeded to perform microlaryngoscopy under general anaesthesia and removal of the mass (measuring 4 × 2.5 × 2 cm) using a COFurther examinations were done to rule out systemic amyloidosis. The patient's complete blood count, erythrocyte sedimentation rate, basic metabolic and biochemical panel and liver function tests were within normal limits. Serum calcium was also normal. Serum and urine electrophoresis were normal. Rectal biopsy was negative. Based on these findings systemic amyloidosis and multiple myeloma were excluded from the differential diagnosis. The amyloid light chain was lambda (λ) type. The amyloid mass was removed by microlaryngoscopy.Amyloidosis is a metabolic protein disturbance in which extracellular protein fibrils are deposited in various tissues. Amyloidosis is classified as systemic or local. According to the type of amyloid, classification of amyloidosis appears as follows for systemic and localized amyloidoses respectively: hereditary amyloidosis, for example, amyloidosis in familial Mediterranean fever with types AA, AF amyloid; idiopathic systemic amyloidosis with AL amyloid; secondary systemic amyloidoses (reactive amyloidosis) and chronic infections, neoplastic diseases with AA amyloid and localized amyloidoses with AL, AA, AK types of amyloid. Table The ventricles and the false and true vocal cords are the most common sites for localized amyloidosis in the respiratory tree . Other set al. [Regarding the requested laboratory control, in cases of localized laryngeal amyloidosis, Lewis et al.  studied The most commonly used method to detect the amyloid protein is the histological staining of biopsy samples excised with Congo red stain. Amyloid is birefringent in polarized light and appears apple-green in color  in Congo red stained sections. This should be distinguished from pseudoamyloid, which is often found in vocal cord nodules. This is of fibrous consistency and represents an amorphous granular degeneration of collagen fibers with sparse disseminations between the fibroblasts . PotassiMagnetic resonance imaging (MRI) is the technique of choice to detect the most specific features, since amyloid deposits present an intermediate T1-weighted signal intensity and low T2-weighted signal intensity, and MRI is thus considered to be a more specific technique than CT scanning . UnfortuIn our case, the excised mass was oval shaped, uncapsulated, tumor-like yellow-gray nodule. Microscopically, the mass was composed of micronodules consisting of amorphous, acellular, eosinophilic, glassy material with variable inflammatory reactions including lymphocytes Figure . Foreign2 laser excision of the mass should be the first line of therapy. The course of the condition under discussion is slow but sudden relapse is possible [et al. [et al. [There are indications that immunological mechanisms are involved in the pathogenesis of human amyloidosis and that the latter could be a complication in immunodeficient conditions. Factors that affect the human immune response, such as steroids, immunosuppressive and ionizing radiation, may accelerate the appearance of the disease. Some types of amyloid deposits may be the result of an immunocyte dyscrasia or mucosa associated lymphoid tissue neoplasms . Althougpossible . Howeverpossible . In this [et al.  in a mea [et al. , 17 out 2 laser technology method was the therapy of choice.Our case report is the second one in which dysphagia is referred to as a disease symptom in primary localized amyloidosis. The diagnosis of the disease is always established histologically; surgical excision of the mass by microlaryngoscopy using a COWritten informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.IY, LM, AG and AC analyzed the patient's medical data regarding the disease and performed the treatment. They were also involved in drafting the manuscript, making substantial contributions to the conception and design of the manuscript. CG and KC analyzed and interpreted the patient's clinical and laboratory data and were major contributors in writing the manuscript and revising it critically for important intellectual content. PH performed the histological examination of the mass and interpreted the results. He also contributed in drafting the manuscript. All authors read and approved the final manuscript.


Staphylococcus aureus in a 48-year-old woman complicated by systemic embolism and septic shock.Myxoma is the most common primary tumor of the heart. The rarity of infected cardiac myxomas leads to numerous diagnostic and therapeutic difficulties. We present a case of infected left atrial myxoma caused by methicillin-sensible  Myxomas are the most common primary tumors of the heart in adults, which have an estimated incidence of 0.5 per million population per year . The most common clinical presentation is symptoms of mitral valve stenosis or peripheral embolism. The rarity of infected cardiac myxomas leads to numerous diagnostic and therapeutic difficulties. We report a case of infected left atrial myxoma in a 48-year-old woman complicated by systemic embolism and septic shock.A 46-year-old woman, presented with subacute dyspnea, maintained fevers for 3 weeks of unkoun origin and fatigue. She had diabetes mellitus and no history of recent surgical or dental intervention or drug abuse.In the physical examination, the patient appeared ill, cachectic, and tachypneic. She presented a blood pressure of 80/50 mmHg, a heart rate of 120 beats/min and a temperature of 38.2°C. She had jugular venous distention with positive hepatojugular reflux and normal heart sounds without murmurs. Examination of the lungs revealed coarse bilateral breath sounds with inspiratory basal crackles. Abdominal examination revealed no organomegaly; extremities had mild bilateral edema. The remaining examination showed no abnormalities.3, an erythrocyte sedimentation rate (ESR) of 100 mm/h, CRP was 64 mg/L, and urine analysis showed no abnormalities.Electrocardiogram showed sinus tachycardia with an incomplete right bundle-branch block. Chest radiography was normal. Hematologic laboratory values revealed anemia (haemoglobin: 9 g/dL), leukocytosis white blood cell count: 23 000/mmTransthoracic echocardiography and transesophageal echocardiography  revealedStaphylococcus aureus. There is no other source of infection; therefore, the atrial mass was suspected to be the cause of sepsis. The patient's hospital course was complicated on day 3 by thrombo embolism cerebral attacks, and then urgent surgical removal was scheduled on the seventh day of hospitalization.Empirical antibiotic therapy with vancomycine and gentamicin was started, surgical treatment was postponed, and blood was drawn for cultures, which were positive for methicillin-sensible Staphylococcus aureus. She died 10 days after surgical intervention by disseminated intravascular coagulation.The patient underwent surgical excision of the mass, which was found to be a myxoma infiltrated with abundant inflammatory cells, the cultures of the myxoma which were positive for methicillin-sensible Primary cardiac tumors represent less than 0.2% of all neoplasms, three quarters of the tumors are benign, and half of these are myxomas , 4.The most common manifestations are dyspnea and central nervous system embolization; whereas Dias et al.  reported Although there have been several reports of infected myxomas in the recent years, they remain a rare entity –10; a reThey can be challenging to diagnose because of their rare occurrence and varied clinical presentation in fact infected and uninfected myxomas; endocardiac thrombus and endocarditis may exhibit the same symptoms  making the correct diagnosis difficult. These systemic symptoms in myxoma, anemia, and raised ESR could be due to the systemic effects of interleukin 6, the cytokine implicated in generating a generalized inflammatory response in patients with myxomas, with levels decreasing after tumor excision , 12, 13,Streptococcus viridians (44%) and Staphylococcus aureus (15%), a microbiological spectrum similar to that of native valve endocarditis [In our case, the documented myxoma as evidenced by clinical examination, pathology, and positive blood cultures qualifies this case as a “definite” infected cardiac myxoma. The microorganisms involved were s aureus %, a micrSystemic embolization occurs in 30% to 40% of patients with myxomas; infected cardiac myxomas are more dangerous than noninfected myxomas and their incidence of embolization is increased two- to threefold , 16. Little is known of the correct strategy for treatment of an infected myxoma; surgical excision of cardiac myxoma carries a low operative risk and gives excellent short-term and long-term results. Surgical excision of the tumor appears to be curative, with few recurrences at long-term followup. After diagnosis, surgery should be performed urgently, in order to prevent complications such as embolic events or obstruction of the mitral orifice. But there is a dilemma between the urgency to prevent the embolic complications and the need for a surgery on sterile tumor and it seems advisable to recommend surgery despite active infection in an attempt to prevent a catastrophic embolization , 9, 17. We conclude that infective left atrial mxoma is an extremely rare condition. The diagnosis is difficult, and because it has such a high incidence of embolization, emergency surgery should be done to remove this type of myxoma once it is diagnosed.


A 22-year-old man presented for orthodontic surgery because of mandibular prognathism. Clinical symptoms suggested acromegaly, and diagnosis was verified by an endocrinologist as well as by radiograph. Bilateral mandibular prognathism often represents the first and most striking physical characteristic of acromegaly; usually, it is also the main reason why patients seek help from orthodontists or maxillo-facial surgeons. This case report recapitulates the clinical and histopathological findings in pituitary growth hormone (GH) adenomas and emphasises their importance in surgical orthodontic planning. Mandibular prognatism, macroglossia and abnormal growth of hands and feet represent strong indicators for the diagnosis of acromegaly. This disease and its complications not only affect the entire body but increase mortality if the pituitary gland tumour remains untreated. Pituitary adenomas have a variety of clinical manifestations that are related to excessive hormone secretion by the tumour, hormone deficits by normal pituitary gland tissue and the expansion of tumour mass. Most patients have a 5- to 10-year history of changes in facial features before acromegaly is diagnosed . Our casA 22-year-old man was referred to our hospital for orthognathic surgery after 12 months of presurgical orthodontic treatment fig. .Lateral cephalometric radiograph examination showed massive mandibular prognathism, prominent supraorbital ridges and an enlarged sella turcica fig. . AdditioEndocrinological examination showed increased levels of the insulin-like growth factor-I (IGF-I) , increased prolactin  and depressed testosterone levels . Ultrasonography showed hepatosplenomegaly and an enlarged left kidney. In addition, colonoscopy showed dilatation of the colonic lumen. Ophthalomological screening showed a large bi-temporal visual field defect due to tumour compression of the optic chiasm. The patient underwent trans-sphenoidal surgery with complete tumour resection.Histological examination showed a highly vascularised pituitary adenoma with a diffuse (solid) growth pattern. Higher magnification showed uniform cells with broad eosinophilic cytoplasm and round to oval nuclei fig. . The proReestablishment of endocrine balance would be followed by mandibular osteotomies to reestablish facial harmony and Angle class I occlusion.Since symmetrical mandibular prognathism can be part of syndromal growth abnormality, this disease is usually noticeable shortly after birth or in early childhood. Non-syndromal growth abnormalities of the jaws are mainly due to genetic or unknown factors. The third group are acquired growth abnormalities of the jaws. When symmetrical bilateral mandibular prognathism is noticed, the diagnosis of acromegaly should always be taken into consideration .Acromegaly is a rare disease with a prevalence of 40 to 70 cases per million people in the population and an annual incidence of 3 to 4 new patients per million . Owing tIn our patient, the diagnosis of acromegaly was considered because of the patient's manifestations and symptoms at first presentation. Mandibular prognathism is amongst the most commonly found oral manifestations of acromegaly and it was the main reason for the patient to seek orthodontic treatment. Enlargement of the lips and the tongue, such as in our patient, may impair swallowing, chewing and speaking. Frontal bossing, prognathism, macroglossia and an increased size of hands and feet have often been described in the literature as cosmetic changes associated with GH adenomas ,8-10. AlDecreased energy, osteoarthritis and somnolence occur in about 50% of patients ,9. PatieAdenomas that grow upwards or expand massively, such as in our patient, may compress the optic chiasm. Such a compression may lead to visual field defects, which begin in the superior temporal sectors and then progress to bitemporal hemianopsia. Persistent compression may lead to blindness. Routine assessment of visual fields and acuity is therefore essential.Measurement of GH response to glucose load is the standard diagnostic test. An increase in the serum concentration of IGF-I, the main GH dependent growth factor, confirms this diagnosis ,10. MRI Available treatment options include surgery, medication and radiotherapy . Trans-sAfter a permanent reduction of GH and IGF-I levels, the enlarged soft tissue regresses gradually . HoweverAcromegaly is a rare disease that is responsible for bilateral mandibular prognathism in adults. Excess of growth hormone and local tumour growth of the pituitary gland affect the entire body and increase mortality. In most patients, this disease is diagnosed far too late. Orthodontic and maxillo-facial surgeons dealing with orthognatic surgery should be well aware of this disease as patients usually present with them first because of striking mandibular growth. In case of mandibular prognatism in combination with enlargement of lips, nose, tongue, hands and feet followed by visual field defects, sleep apnea, decreased energy and osteoarthritis, every clinician should consider the diagnosis of acromegaly. Although acromegaly is a rare disease, its symptoms are striking and can hardly be misconstrued.The authors declare that they have no competing interests.GM and MA analysed the patient's history, reviewed all patient data and drafted the manuscript. VC carried out the histological analysis, wrote the histological part of the paper and contributed to the writing of the final version. DO, PP and KJ were involved in revising the article. Each author reviewed the paper for content and contributed to the writing of the manuscript. All authors approved the final report.


Arteriovenous malformations of the lower urinary tract are uncommon lesions, usually presenting as scrotal masses. A case of recurrent acute scrotal pain mimicking testicular torsion that was attributed to the presence of an arteriovenous malformation of the spermatic cord is described. To our knowledge this is the first reported case of an arteriovenous malformation of the spermatic cord presenting with acute scrotal pain. Arteriovenous malformations (AVMs) occur mainly in the central nervous system, although they have been described in other organs as well. AVMs rarely involve the testis or the scrotal components, presenting mainly in the form of para-testicular or intra-testicular masses. This case reveals an uncommon clinical presentation of a non-palpable AVM of the spermatic cord. To our knowledge we present the first reported case of an arteriovenous malformation of the spermatic cord being the cause of recurrent acute scrotal pain.An otherwise healthy 22-year-old man presented with acute pain in his right hemiscrotum. He recalled having similar episodes of self-limited scrotal pain since his adolescence. The pain had been attributed by attending physicians to episodes of intermittent testicular torsion. He denied any history of trauma, urinary tract infection, sexually transmitted diseases, or voiding symptoms.Clinically both testes were intrascrotal, there was no swelling or signs of inflammation and the cremasteric reflex was intact. The right testis was, however, very tender in palpation. Both epidydimis were also normal and non-tender in palpation. Routine laboratory test and urinalysis were normal, and urine culture was sterile.Grey scale scrotal ultrasonography and Color Doppler Ultrasound (CDU) were performed. Sonography revealed no evidence of testicular tumor, varicocele or other pathology of the testis or epidydimis. Figure The patient underwent a diagnostic right scrotal exploration due to his persistent scrotal pain. Scrotal exploration revealed a macroscopically normal testicle, epididymis and spermatic cord. There was no evidence suggestive of intermittent testicular torsion, e.x "bell clapper" deformity of the testis, or torsion of testicular appendages.Four months later the patient presented with the same intractable right scrotal pain. Clinical examination and ultrasound investigation were insignificant. Orchiectomy was suggested this time and was performed through an inguinal incision after obtaining patient's consent. The surgical specimen consisted of a 16 ml testicle with its spermatic cord structures. The testis and spermatic cord were macroscopically unremarkable. Detailed pathology examination of the testicle and vas deferens revealed insignificant pathological changes, except for a small, smooth vascular mass (0.5 cm maximum diameter) in the lower part of the spermatic cord with the characteristics of an arteriovenous malformation Figure . The patArteriovenous malformations (AVMs) represent defects of the circulatory system that are generally believed to arise during embryonic or fetal development or soon after birth. The characteristic of AVMs is that arteries and veins are tangled and not connected by capillaries. The lack of capillaries allows blood traveling through these abnormal vessels to flow rapidly and under high pressure, thus preventing arterial blood from reaching the tissues leading to various degrees of ischemia and resulting pain. Histologically, the irregular vascular spaces are lined by nonproliferating and quiescent endothelial cells and are separated by fibrous stroma . The abnAVMs involving the lower urinary tract are uncommon as opposed to AVMs located in the Central Nervous System (CNS). Even more rare is the case of an AVM of the urinary tract presenting with pain. There is a report of ejaculatory pain caused by an AVM located between the prostate and seminal vesicles , while tArteriovenous malformations of the spermatic cord and testis are benign lesions consisting of complex tangles of enlarged dilated arteries and veins without intervening capillaries. In the cases published so far AVMs of the scrotal compartments present as either painless paratesticular masses ,5-7 or aScrotal AVMs appearing as masses can be detected by pelvic arteriography and managed by subsequent superselective embolization, however success is not always guaranted , necessiHowever in our case the small size of the lesion would make it impossible to detected by means of arteriography and to embolize successfully. The decision for orchiectomy was justified by the patient's intractable pain, suggesting some degree of testicular torsion considering his age, and the absence of findings on imaging studies.The case presented highlights a rare and unique cause of recurrent acute scrotal pain, attributed to the presence of an arteriovenous malformation of the spermatic cord. Therefore there is reason to believe that AVMs of the scrotum should be considered in the aetiology of otherwise inexplicable, recurrent scrotal pain. In that case consideration should be given to a trial of superselective angio-embolization of the lesion before one resorts to orchiectomy.The author(s) declare that they have no competing interests.PS was the treating urologist of the patient and performed together with AB both surgical procedures. PS has also drafted the manuscript. IR was the anaesthesiologist in charge in both operations performed. HA was the pathologist performing the stains and making the final pathology report for the case.All authors have have read and approved the final manuscript.


Cystic epithelial-stromal tumors of the prostate are rare, with 82 cases reported in literature. These cases have been published under a variety of diagnoses, including phyllodes tumor and prostatic stromal proliferation of uncertain malignant potential as well as a malignant tumor called “prostatic stromal sarcoma”. We report a case of a 60-year-old man with the histological diagnosis of phyllodes tumor of the prostate in transurethral resection specimen. Prostatic cystic epithelial-stromal tumors, commonly described as phyllodes tumors of the prostate, are extremely rare lesions. They exhibit a spectrum of histological features similar to their better-known counterpart in the breast. Although a benign clinical course has been emphasized in some reports, cumulative evidence in literature indicates that these lesions should be considered neoplasms rather than atypical hyperplasia due to the frequent early recurrences with possible dedifferentiation, infiltrative growth, and potential for extraprostatic spread in some cases. An individualized approach to complete excision of the tumor is needed. A case report of a patient recently treated in our hospital is presented, and a review of literature was done.A 60-year-old man, who visited our hospital, complained of dysuria and bladder outlet obstruction. Digital rectal examination revealed a slightly enlarged benign-feeling prostate. Prostate-specific antigen was 1.1 ng/mL. The urine culture showed no urinary tract infection. X-ray of the kidneys, ureters, and bladder was normal, as was renal ultrasound. Transrectal ultrasound revealed an increased prostate volume (60 mL) without suspicious hypodense or hyperdense areas. Cystoscopy demonstrated enlarged prostate adenoma causing bladder outlet obstruction, which was subsequently resected.Pathological examination showed a benign prostatic hyperplasia and features of complex glandular architecture with a prominent stromal component . The strAdditional staging investigations including X-ray of the lung and abdominal magnetic resonance imaging revealed no extracapsular extension of the tumor with absence of lymph node involvement or metastasis. Sextant biopsies of the peripheral zone demonstrated normal prostatic tissue. The patient denied radical surgery, and we performed a second transurethral resection of the prostate three months later without further recurrence of the phyllodes tumor. Due to the malignant potential and the possibility of sarcomatoid differentiation of this tumor entity, we perform periodical close followup every three months in order to be able to detect and to treat any progression at an early stage by radical surgical intervention. The patient remains alive and well without recurrence of the phyllodes tumor after 18 months.Phyllodes tumor of the prostate is an uncommon prostatic neoplasm with distinctive clinical and pathological characteristics. It has been assigned numerous names during the last 20 years, including prostatic cystic epithelial-stromal tumor, phyllodes type of atypical hyperplasia, cystadenoleiomyofibroma, prostatic stromal proliferation of uncertain malignant potential, and cystosarcoma phyllodes . The lacAlthough the mean age of patients who present with phyllodes tumors is about 55 years, age range at presentation from 22 to 86 years and 40% of the patients are younger than 50 years , 4. The Histologically, phyllodes tumor of the prostate is biphasic, consisting of stromal and epithelial components arranged to form cysts lined by hyperplastic epithelium. The proliferating stroma is of variable cellularity and sometimes shows subepithelial condensation. The lining epithelium is benign, with basal cell and secretory layers that show immunoreactivity typical of prostatic epithelium, but may show various metaplastic and proliferative changes such as basal cell hyperplasia or squamous metaplasia . There iTumor recurrence after transurethral resection is common, occurring in 65% of patients overall, with recurrence rates from 50% for low-grade tumors to 100% for high-grade tumors . TargeteSince phyllodes tumor is potentially aggressive, complete tumor resection propably provides the best chance for cure. In low-grade tumors alternatively, as in our case, close cystoscopic and transrectal ultrasound surveillance after 2nd transurethral resection without further detectable tumor can achieve local control. However, careful surveillance is essential as progression to higher grades can occur, requiring more aggressive intervention.


It originates from multipotential primitive mesenchymal cells, rather than mature adipose tissue.Liposarcoma is the second most common soft tissue sarcoma in adults with a peak incidence between the 4An unusual case of a rapidly growing giant lipoma-like liposarcoma of the left gluteal and perineal areas in a young male was presented. The patient was managed by wide local excision of the lesion and coverage with split thickness skin graft. The key issues surrounding the treatment of lipoma-like liposarcoma and literature review is discussed.For such unusual case of this particular rapidly growing tumor, a longer follow-up is needed to evaluate the outcome in these cases. This tumor originates from multipotential primitive mesenchymal cells rather than mature adipose tissue . It commle gender. The autA 26 year old gentleman presented to our out-patient clinic at Al-Babtain Center for burns and plastic surgery with a 16 month history of a rapidly growing mass in the left gluteal region. Although this mass was interfering significantly with his daily normal activities, walking, anal hygiene and even with defecation, the patient did not search for treatment until that date when it became unbearable. Clinical examination revealed a huge well defined, polypoidal, cutaneous, fleshy mass of about 59 cm × 39 cm × 19 cm occupying most of the left gluteal and perianal area (Figure Contrast enhanced MRI showed a huge irregular lobulated mass at the anal region extending outwards and inferiorly preserving the anal canal. The mass was mainly of fatty signal intensity with evidence of solid component that showed moderate enhancement suggestive of liposarcoma figure . IncisioSurprisingly, the final pathology described yellow to white firm serial section with occasional necrotic foci. Histological sections showed variable sized lobules separated by fibrous septa. The lobules were composed of relatively mature adipocytic proliferation with significant variation in cell size, with occasional atypical lipoblasts exhibiting evidence of nuclear atypia and hyperchromasia. Hyperchromatic stromal cells were also, identified in the thickened fibrous bands. Monovacuolated and multivacuolated lipoblasts as well mononuclear chronic inflammatory elements were frequently seen figure . SurfacePost-operative CT scan of the abdomen and pelvis with oral and IV contrast were done as part of the metastatic workup and showed no abnormalities.The case was discussed in the tumor board meeting and the decision was made to achieve an adequate loco-regional control by wide local excise. The operation was done in a left lateral decubitus position under general anesthesia with oro-tracheal intubation. Total excision of the lesion was done with a 2 cm free margin down to a deep sub-facial plane exposing the gluteal maximus muscle which was found to be free of the disease. The resultant soft tissue defect was covered with a split thickness skin graft taken from the left thigh. Part of the graft was lost because of a localized pseudomonas infection which healed completely with daily wound care and without surgical intervention. The final histopathology diagnosis was well-differentiated and well defined completely excised lipoma-like liposarcoma, therefore adjuvant radiotherapy was not indicated. The patient was followed for about 16 months showing no evidence of recurrence.Adipocytic tumors represent the largest single group of mesenchymal neoplasms, due to the high prevalence of lipomas and their variants. Liposarcoma represents the single most common soft tissue sarcoma in adults, accounting for approximately 20% of all cases. Its prith and 6th decade of life[This male patient was quite young though the peak incidence is considered between the 4e of life. The preLiposarcomas can be divided into three basic histological categories; well-differentiated liposarcomas which morphologically subdivided into lipoma-like, sclerosing, inflammatory or spindle cell type, myxoid liposarcoma and pleomorphic liposarcoma .Well-differentiated liposarcomas account for about 40%–45% of all liposarcoma and therefore represent the larger subgroup of adipocytic malignancies. Although the recurrence rate can reach up to 30% of the cases, this tumor which is surgically amenable behaves as a benign neoplasm and is not known to metastasize, thus requiring a less aggressive treatment . The mos4.The benefit of wide local excision over marginal excision is recognized in the literature . In our An unusual case of a rapidly growing, giant gluteal lipoma-like liposarcoma was presented. Surgical excision is the main treatment for most primary soft tissue sarcomas. As such every effort should be made to achieve complete tumor resection. A longer follow-up is needed to evaluate the outcome such cases. Although not used in this case, we wonder if radiotherapy could be used in such giant tumors to improve the loco-regional control.The authors declare that they have no competing interests.HB Substantial contributions to conception, design, and in drafting the manuscript or revising it critically for important intellectual content. MS Substantial contribution in literature review and data analysis. RLB Substantial contributions in reviewing the draft and the addition of important data to the text. WE analysis of slides taken from the patient and reaching a diagnosis. ME analyzed the data related to pathology in the text with a significant contribution in drafting. All authors read and approved the final manuscript.


A 6 year old female with symptoms of small bowel obstruction underwent an exploratory laparotomy which revealed widespread evidence of inflammatory fibrotic adhesions involving the jejunal mesentery. In view of persistent growth failure, chronic anaemia, elevated acute phase reactants and imaging evidence of a diffuse progressive inflammatory process, the child was treated with corticosteroids and methotrexate with complete response. The literature on juvenile idiopathic sclerosing mesenteritis has been reviewed. Idiopathic sclerosing mesenteritis (ISM) is an uncommon disease involving the small bowel mesentery and characterised by chronic inflammatory changes ultimately progressing to fibrosis. The first reported series featured 34 cases of "retractile mesenteritis and mesenteric sclerosis"; published in 1924 .Though the exact aetiology remains obscure, proposed mechanisms include prior abdominal trauma/surgery, autoimmunity, infection and ischemia . A wide 9/l  and platelets 663 × 109 /l. Her ESR was elevated at 54 mm/hr [1-15 mm/hr) and CRP 84 mg/l (<10 mg/l). Ultrasonography of the abdomen revealed diffuse, mesenteric lymphadenopathy. A barium meal with follow through suggested a small bowel obstruction. A laparotomy showed extensive intraperitoneal adhesions, mesenteric lymphadenopathy and a tight jejunal band with obstruction. The adhesions were divided, the obstruction was relieved and the patient's bowel function recovered steadily. Tissue cultures were negative. Histopathological analysis of the peritoneal tissue revealed fibro-fatty tissue with extensive fibrosis throughout, infiltrated by many scattered lymphocytes, plasma cells and occasional neutrophils but no malignant cells. The single lymph node examined revealed dilated sub-capsular sinuses with an expanded para-cortex with mixed histiocytes and occasional immunoblast with no granulomas, Reed Sternberg cells or any evidence of lymphoma. Ziehl-Neelsen stains were negative for tuberculosis organisms as was subsequent culture. The clinical condition improved slowly over the subsequent 2 weeks and she was discharged on oral iron supplements. No evidence of mono-clonality was seen on flow cytometry for immuno-phenotyping of lymph node tissue.A 6 year old girl presented at a local hospital with an acute onset of abdominal pain, nausea and bilious vomiting. A history of constipation, recurrent vomiting associated with high fever in the preceding 12 months was elicited. Clinical examination was reported to be normal at the time. Complete blood counts revealed haemoglobin (Hb) 94 g/l, WBC 18.4 × 09/l with thrombocytosis. She had lost 7 kgs in weight (from the first presentation), had recurrent abdominal pain and required a second admission for a partial obstruction which resolved with conservative management.Three months later, she moved interstate and presented to the gastroenterology clinic at our hospital with continued weight loss and an exacerbation of abdominal pain and vomiting of fecal matter. Upper and lower endoscopies were normal. MRI of the abdomen revealed apparent thickening of a loop of proximal jejunum in the left mid abdomen with enlarged mesenteric lymph nodes. CT scan revealed evidence of a subtle attenuation in the mesentery with diffuse lymphadenopathy Figure . The symA rheumatology consultation was made. Review of the case revealed multiple episodes of subacute small bowel obstruction with chronic anaemia, weight loss, and a chronic inflammatory process with fibrosis on mesenteric biopsy and elevated acute phase reactants. Further investigations revealed a borderline positive ANA of 1:40 (speckled pattern), a negative dSDNA and rheumatoid factor (RF). Total serum proteins were 82 g/l, albumin 40 g/l (32-48 g/l), globulins 42 g/l (23-35 g/l), and serum IgG 15.7 g/l (6-12.3 g/L) indicating mild hypergammaglobulinemia. IgG subclasses IgG1 11.1(4.0-10.8 g/L), IgG2 4.3 (0.8-4.1 g/L), IgG 30.4 (0.1-1.4 g/L), IgG4 1.47 (<1.9 g/L) were unremarkable. An abdominal angiogram was done to screen for major vessel vasculitis such as polyarteritis nodosa, and was negative.9/l platelets 490 × 109/l and ESR 3 mm/hr. Subcutaneous methotrexate (10 mg/m2 ), total dose 10 mg once a week was started for long term immunomodulation and steroid sparing effects. At 6 months follow - up from the onset of treatment, she had gained 3 kgs, was completely asymptomatic and her laboratory investigations remained normal. Her repeat CT scan this time had revealed resolution of the lymphadenopathy with normal mesentery  now 12.5 mg. Figure A diagnosis of idiopathic sclerosing mesenteritis was made and the child was treated with 3 daily pulses of methyl prednisolone therapy repeated monthly for 3 months. She made a remarkable and rapid clinical recovery with marked reduction in fatigue, abdominal pain and resolution of fever within 2 weeks. Four weeks into treatment, laboratory investigations revealed significant improvement; Hb 118 g/l, WBC 10.1 × 10y Figure . CurrentFirst reported in 1924 as 'retractile sclerosing mesenteritis', ISM was reprised in a case series which reported it to be a benign disease in general. ISM is considered a rare disorder of late adult life with relatively few cases reported in world literature . Only 3 Various patho-physiological mechanisms suggested include previous surgery, trauma, hypoxia, allergy, infection and autoimmunity ,9,10. CoThe onset of the disease may be acute, insidious or have recurring episodes before the actual diagnosis is made . Varied In the pediatric cases, of the 17 children, 4 were described as mesenteric panniculitis while the remaining 13 termed sclerosing mesenteritis. Eleven (64.7%) children presented with abdominal pain, 7 (41%) had vomiting, 4 (23.5%) with bowel symptoms in the form of constipation or diarrhoea, 8 (47%) with associated fever and 2 (11.7%) with weight loss. Only 5 (29%) children had a clinically palpable mass corroborated on exploration. Laboratory investigations were usually non specific ranging from normal to neutrophilic leucocytosis and variable elevation of acute phase reactants. Five of the 13 (38%) children tested demonstrated anemia and 9 (62%) leucocytosis. 4 (30%) children had normal white cell counts. Elevated acute phase reactants ESR/CRP were only reported in 5 children -26.Most cases are diagnosed on surgical intervention, but advances in imaging techniques have helped identify features that may suggest a diagnosis of ISM. The various imaging patterns reported in children has been summarised in Table Most histopathological analyses show varied degree of fibrosis, chronic inflammation, and fat necrosis with the infiltrate being predominantly lymphocytes and eosinophils with no evidence of vasculitis or vascular thickening. Foci of dystrophic calcification have been occasionally reported . The finNo specific guidelines regarding the management of ISM exist and various medical and surgical modalities that have been tried. Use of anti-inflammatory, anti fibrotic and immunomodulatory agents such as cortico-steroids, methotrexate, azathioprine, colchicine, thalidomide and Tamoxifen have all been reported either alone or in combination with varied degrees of success ,7. ThaliSurgical exploration for diagnostic and therapeutic  purposes has been reported with all the children having some form of surgical intervention with only two cases not having actual tissue resection. Resection or debulking probably has a role in alleviating symptoms but may not help in prevention of disease progression.Widely believed to have a good outcome in adults, the paucity of reported pediatric cases and long term follow up makes it difficult to plan management and prognosticate in children.Decisions regarding the most appropriate form and duration of therapy in children should probably be guided by the severity and chronicity of symptomatology, growth of the child, and inflammatory markers. It seems rational that unless there is severe obstruction, medical/non-surgical management might be considered. Serial imaging is probably not indicated unless warranted by a clinical deterioration. Studies in adults have reported median duration of medical treatment between 2-30 months with recurrences up to 2 years after discontinuation though long term outcomes are not well reported ). Acute Mortality secondary to ISM has been reported in 2 children and 4 adults ,18,33). Written informed consent was obtained from the parents of the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.VV was involved in the review of literature, analysis and drafting the manuscript. KJM was involved in management of the case and conceptualising the design and drafting of manuscript and will act as a guarantor. Both authors read and approved the final manuscript.


Papillotubular adenoma of the ampullary region in a rare tumor which has the potential for malignanttransformation. Although reported more frequently nowadays, ampullary tumors are difficult to diagnosebefore operation unless they cause obstructive jaundice. Occasionally they are detected byultrasonography, computed tomography or ERCP in patients who complain of nonspecific upperabdominal discomfort.        We report a case of a periampullary papilotubular adenoma causing generalized pruritus and weightloss but no jaundice. The tumor was extirpated by pancreaticoduodenectomy, and the patient hasremained healthy for over five years.


Transitional cell carcinoma (TCC) of the ovary is a rare, recently recognized, subtype of ovarian surface epithelial cancer.A 69-year-old postmenopausal woman presented with a 2-year history of progressive enlargement of an abdominal mass. Abdominal computed tomography showed a pelvic mass. CA-125 was normal. A staging operation with total abdominal hysterectomy, bilateral salpingo-oophorectomy, infracolic omentectomy and pelvic lymph node dissection was performed. After surgery, the pathologic report of the right ovarian tumour was TCC, grade 3, stage IC. The patient underwent 3 cycles of chemotherapy: carboplatin and paclitaxel. She is regularly followed up and has been disease free for 10 monthsTransitional cell carcinoma (TCC) of the ovary is a rare subtype of epithelial ovarian cancer. Surgical resection is the primary therapeutic approach, and patient outcomes after chemotherapy are better than for other types of ovarian cancers. Transitional cell carcinoma (TCC) of the ovary is a rare, recently recognized, subtype of ovarian surface epithelial cancer. In a study by Silva et al, focal or diffuse TCC pattern was seen in 88 of 934 ovarian cancers . Here, wA 69-year-old postmenopausal woman presented with a 2-year history of progressive enlargement of an abdominal mass. She had experienced weight loss of about 4 kg during the 6 months prior to admission. Physical examination showed a pelvic mass. Abdominal ultrasound showed a pelvic mass measuring 31 × 35 mm with homogeneous echogenicity. Abdominal computed tomography (CT) showed a homogeneous cyst on the right side of the pelvis, which was larger than 35 mm in maximal diameter with a solid component. There was no evidence of lymphadenopathy. The liver and kidneys were unremarkable (Figure TCC of the ovary is a recently recognized subtype of ovarian surface epithelial cancer. It has been described as a primary ovarian carcinoma in which definite urothelial features are present but no benign, metaplastic and/or proliferating Brenner tumor can be identified. TCC of the ovary was first defined by Austin and Norris . They reAs described in detail by Eichhorn and Young, ovarian TCC typically showed undulating, diffuse, insular and trabecular growth patterns . The tumThe immunophenotype of TCC of the ovary is similar to that of other surface carcinomas of the ovary, but differs from that of TCC of the bladder. In addiThe relative influences of tumor biology and treatment strategies remain undetermined. Gershenson et al. concluded that advanced-stage ovarian TCC was significantly more chemosensitive and associated with better prognosis than poorly differentiated serous carcinoma. KommossOptimal surgical resectability followed by cisplatin-based chemotherapy might contribute to the survival benefit . In theiWritten informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.ET, IL and HM analyzed and interpreted the patient data regarding its oncological features. MFT and YA have been involved in drafting the manuscript and HE has given final approval of the version to be published. All authors read and approved the final manuscript.