Unilateral simultaneous renal oncocytoma and angiomyolipoma: case report

A rare case of synchronous angiomyolipoma and oncocytoma in the same kidney of a 70 year old man is presented. A left renal mass was found incidentally by ultrasound. Computerized tomography and magnetic resonance imaging revealed a 1,3 cm mass in the mid-portion of the left kidney, whereas on the lower pole of the same kidney, a 3,3 cm mass was also revealed, consistent with angiomyolipoma. A working diagnosis of renal cell carcinoma was made. A radical nephrectomy was performed. Microscopically, the tumor of the lower pole was found to be an angiomyolipoma, whereas the mid-portion tumor was an oncocytoma. Until now, only 16 cases of unilateral simultaneous presence of renal angiomyolipoma and oncocytoma have been reported. Of these cases, all except one were female and three were associated with the tuberous sclerosis complex. It is well worth remarking, that renal oncocytoma overlap with other renal neoplasms, therefore nephrectomy remains the treatment of choice

Mixed acinar-endocrine carcinoma of the pancreas: a case report and review of the literature

and review of the literatur

Concurrent insulinoma and pancreatic adenocarcinoma: report of a rare case and review of the literature

Pancreatic adenocarcinoma is the 5th leading cause of cancer-related death in Western countries and insulinomas are rare endocrine neoplasms of the pancreas. The concurrent appearance of pancreatic adenocarcinoma and insulinoma is very rare and to the best of our knowledge has never been reported again. Herein, we present such an occurrence in a 74-year-old man. Resection of a mass in the uncinate process of the pancreas revealed pancreatic adenocarcinoma with severe desmoplastic reaction. Two years later, due to symptomatology persistence the patient was re-examined and a new 2cm mass in the uncinate process was found leading to surgery, which demonstrated a 2cm endocrine islet-cell tumor. Establishing a diagnosis in patients with insulinoma is difficult and the imaging studies still have low sensitivity and specificity except for intra-operative ultrasonography, which is the most accurate method detecting 90% of these lesions

A rare combination of an endocrine tumour of the common bile duct and a follicular lymphoma of the ampulla of Vater: a case report and review of the literature

Carcinoid tumours of the common bile duct represent an extremely rare entity. Similarly, primary follicular lymphomas of the ampulla of Vater constitute an infrequent neoplasia. Herein, we report the first case of a synchronous development of a carcinoid tumour of the common bile duct and an ampullary follicular lymphoma that was treated surgically with a Whipple's procedure, due to inability to establish definitive preoperative diagnosis despite the extensive diagnostic investigation

Malignant potential of intrahepatic biliary papillomatosis: a case report and review of the literature

BACKGROUND: Biliary papillomatosis (BP) is a rare disease entity with a strong malignant potential. It is characterized by multiple papillary adenomas involving both the intrahepatic and extrahepatic biliary tree. BP was considered in the past to be a disease with low malignant potential. However, a current review of the English literature revealed a high rate of malignant occurrence of approximately 41% and histological analysis along with the expression pattern of mucin core proteins (MUC) and mucin carbohydrate antigens suggests that BP is a borderline or low grade malignant neoplasm with a high malignant potential. CASE PRESENTATION: A 68 year-old male patient was referred to our hospital due to the presence of sudden right upper quadrant abdominal pain, nausea and dark urine. Imaging workup demonstrated dilatation of the left hepatic duct without the presence of a space-occupying lesion. A left hepatectomy and cholecystectomy were carried out and histological analysis revealed a moderately to poorly differentiated carcinoma of the left hepatic duct in the background of biliary papillomatosis. Postoperative course was uneventful. Unfortunately, two years after initial diagnosis the patient rapidly deteriorated and died from multiple pulmonary secondary deposits. CONCLUSION: BP should not be considered to be a benign disease. The clinical behavior, the high recurrence rate and the even higher malignant transformation occurrence, as well as the presence of carcinogenetic indicators (K-ras mutation, overexpression of p53, MUC and Tn antigens) strongly support that BP is a low-grade neoplasm with high malignant potential

KIT exon 11 codon 557/558 deletion/insertion mutations define a subset of gastrointestinal stromal tumors with malignant potential

AIM: To study the association of the frequency and pattern of KIT and PDGFRA mutations and clinicopathological factors in a group of patients with gastrointestinal stromal tumors (GIST). METHODS: Thirty patients with GIST were examined. Exons 9, 11, 13, and 17 of the KIT and exons 12 and 18 of the PDGFRA gene were analyzed for the presence of mutations by PCR amplification and direct sequencing. RESULTS: KIT or PDGFRA mutations were detected in 21 of the 30 patients (70%). Sixteen patients had mutations within KIT exon 11, three within KIT exon 9, and two within PDGFRA exon 18. GISTs with KIT exon 9 mutations were predominantly located in the small intestine, showed a spindle cell phenotype, and were assessed as potentially malignant. GISTs with KIT exon 11 mutations were located in the stomach and intestine, showed mainly a spindle cell phenotype, and were scored as potentially malignant (P < 0.05). Tumors with KIT exon 11 codon 557/558 deletion/insertion mutations were found to be associated with a potentially malignant clinical behaviour (P < 0.003). GISTs with PDGFRA mutations located in stomach showed a mixed cell phenotype and were classified as of very low or low moderate malignant potential. CONCLUSION: Determination of KIT and PDGFRA mutations should be additional parameters for the better prediction of GISTs clinical behaviour. Tumors with deletion/insertion mutations affecting codons 557/558 of the KIT gene seem to represent a distinct subset of malignant GISTs. (c) 2008 WJG. All rights reserved

Gastric glomus tumor: A case report

Gastric glomus tumors are rare mesenchymal tumors of the gastrointestinal tract. We describe a 72-year-old patient who presented with episodes of melena and was subsequently investigated for a tumor of the antrum of the stomach. Surgical resection revealed a 2 x 2 x 1.7 cm well circumscribed submucosal tumor, extending into the muscularis propria. The histopathologic examination of the specimen demonstrated a glomus tumor of the stomach. We discuss the preoperative investigation, the diagnostic problems and the surgical treatment of the patient with this rare submucosal lesion