Composite Large Cell Neuroendocrine Carcinoma and Adenocarcinoma of the Ampulla of Vater

Large Cell Neuroendocrine Tumors (LCNEC) in the ampulla of Vater are extremely rare. This report addresses a case of concurrent LCNEC and adenocarcinoma in the ampulla of Vater. A 60-year-old male patient experienced fever, body chills and jaundice. He had a periampullary ulcerative lesion and underwent radical pancreaticoduodenectomy. Histopathologically, the tumor consisted of an LCNEC component and an adenocarcinoma component. Simultaneous LCNEC and adenocarcinoma has been reported in a few cases. Our patient had a coexisting LCNEC and an adenocarcinoma of the ampulla of Vater. We also present a review of the literature

Pancreatic Cyst in von Hippel-Lindau Disease

The von Hippel-Lindau (VHL) disease is an autosomal dominant disorder by germline mutation of VHL tumor suppressor gene. The affected patients mainly develop cancers of the central nervous system, renal cell carcinoma, pheochromocytoma, and pancreatic cysts. Pancreatic involvement was observed 12~77% in VHL patients and included true cysts, serous cystadenomas, and neuroendocrine tumors. We experienced two cases of pancreatic cyst of the von Hippel-Lindau disease and reviewed associated literatures.

Ruptured Solitary Splenic Metastasis of Gastric Cancer after Gastric Cancer Operation

Solitary malignant splenic metastasis is uncommon and usually occurs in association with widely disseminated metastatic disease. Splenic metastasis usually occurs late in the disease course, with widespread involvement of other organs and rarely shows any presenting symptoms. Virtually all primary tumors have been known to metastasize to the spleen. The common ones include melanoma, lung, breast and ovary cancer, but metastasis from gastric cancer is very rare. When solitary spleen metastasis is suspected in a clinical setting, aggressive treatment is indicated such as splenectomy followed by combined modality treatment to prevent dissemination of the disease. We experienced a case of solitary spleen metastasis and rupture after gastric cancer operation and reviewed the associated literatures.

Value of Elevated Ki67 Index (>10%) and p53 Protein Expression as Prognostic Factors in GISTs

PURPOSE: Although gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors and express CD117, the prediction of their malignant potential remains difficult. The aim of this study is to evaluate the prognostic accuracy of elevated Ki67 index and p53 overexpression in combination with classical prognostic factors (tumor size and mitotic index). METHODS: A retrospective study was conducted in 84 patients who had been re-evaluated for confirmation of diagnosis based on immunohistochemical analysis with CD117 expression, between Jan 1991 and Dec 2001. Cases were classified as very low, low, intermediate and high-risk groups according to 2001 NIH consensus symposium. Elevated Ki67 index was assigned to the lesion that displayed 10% or more of immunoreactive cells. And p53 expression was assigned to the area with 5% or more of eosinophilic nucleus. RESULTS: Elevated Ki67 was noted in 37 (44.0%) out of 84 cases. High-risk patients showed elevated Ki67 index more frequently (P<0.0001) and there was significant relation between elevated Ki67 and survival rate (P=0.0417). The p53 expression was noted in 32 (38.1%) out of 84 cases. The p53 expression was significantly higher in high-risk patients (P=0.0081) than low-risk patients. But, there was no significant relation between p53 expression and survival rate. As a result of multivariate analysis, tumor size (P=0.0059), mitotic index (P=0.0016) and elevated Ki67 index (P=0.0384) were proved as significant independent prognostic factors. CONCLUSION: According to the results of our retrospective study, p53 expression is related to disease progression but its value as a prognostic factor in GISTs is uncertain. It is suggested that tumor size, mitotic rate and elevated Ki67 index are the helpful prognostic factors in GISTs.

A Case of Primary Retroperitoneal Synovial Sarcoma

A retroperitoneal Synovial Sarcoma is defined as a tumor arising in the retroperitoneal space, with an origin of mesodermal structures. The majority of synovial sarcomas (85 to 95%) occur in the extremities, located near the large joints. They are uncommon, and usually appear as a non- specific soft tissue mass, with no specific imaging feature. However, in some cases, radiological findings and anatomic location of the tumor may help the diagnosis. On CT examination, these tumors may be shown as hypo-dense masses and have an irregular enhancement in the periphery, with a poor enhancement in the central area, reflecting the necrotic, cystic and hemorrhagic changes. Histologically, there are two types of synovial sarcoma: biphasic and monophasic. Both types have about a 40% mortality rate. The known prognostic factors are; frequent mitotic figures, extensive tumor necrosis, and poor differentiation. Surgical ablation remains the mainstay of their management. Regardless of adequate surgical and adjunctive therapies, the recurrence rate ranges from 28 to 36%. Here, we present a case of a retroperitoneal synovial sarcoma, with a review of the literature.

Colonic Complication of Acute Necrotizing Pancreatitis: a Case Report

We present a case of a colonic involvement associated with necrotizing pancreatitis, with a review of the literature. A 10 year old boy had an appendectomy at the local clinic ten days ago. On admission, he complained nausea, vomiting and severe constipation. His abdomen was distended and he had tenderness on the left abdomen. Laboratory and radiologic studies revealed findings consistent with acute pancreatitis with colonic complication. He was treated conservatively for 30 days but did not improve. On hospital 30th day, abdominal pain developed and his vital sign changed. Abdominal CT suggested ischemic change of the transverse colon. At laparotomy, the left colon showed stenosis. The greatly distended transverse colon was resected and a transverse end colostomy was done. He was discharged at postoperative 45th day with improvement and colostomy closure was performed 8 months later.

A Case of Infantile Digital Fibromatosis Treated with Skin Graft / 대한피부과학회지

Infantile digital fibromatosis is a rare benign tumor on the fingers and toes of infants and childhood which is characterized by fibroblastic proliferation. Usually the lesions occur singly or severally on the dorsal or lateral aspects of the distal phalanges of the toes and fingers. The thumb and great toe are usually spared. These asymptomatic, firm, red, smooth nodules, some lcm diameter, occur during the first year of life. Forty-seven percent occur in the first month of life. This disease can occur after trauma. The lesions do not metastasize. Occasionally, spontaneous regression has been reported. After excision, recurrence can be possible. A 2 years old female infant had three large bean sized erythematous masses on the left 2,3,4th fingers. The erythematous aacule was developed at 6 months old and grew slowly. We could find inclusion body stained bright red with Massons trichrome and purple with PTAH in cytoplasm of fibroblast. The patient was treated with a simple excision followed by a skin graft. During 5 rnonths after operation, recurrence was not occurred.