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ABSTRACT: We report a case of intra-abdominal plexiform neurofibromatosis, including periportal, mesenteric, and gastrointestinal tract
involvement, in a patient with von Recklinghausen’s disease/neurofibromatosis type 1 (NF-1). A 26-year-old man with familial
NF-1 was admitted to hospital for further examination of an abnormal hepatic mass along the portal vein. Esophagogastroduodenoscopy
revealed antral wall thickening and swelling of the papilla of Vater. Mucosal biopsies taken from the duodenum revealed possible
ganglioneuromatosis. Abdominal ultrasonography, contrast-enhanced computed tomography, and magnetic resonance imaging revealed
an abnormal periportal mass with serpiginous extension into the liver along the portal vein and the mesentery, which is the
typical spread pattern of plexiform neurofibromatosis. A laparotomy and cholecystectomy for gallstones were performed, and
this patient was diagnosed as having intra-abdominal plexiform neurofibromatosis. This is the 15th case of intrahepatic periportal
plexiform neurofibromatosis and the 16th case of diffuse ganglioneuromatosis associated with NF-1 in the English literature.
The imaging findings of the lesion have been followed for 10years; there has been slight growth of the mass, but no malignant
transformation has been found. The previously reported cases are reviewed.
KeywordsNeurofibromatosis type 1 (von Recklinghausen’s disease)–Intestinal diffuse ganglioneuromatosis–Intrahepatic periportal plexiform neurofibromatosis
Clinical Journal of Gastroenterology 04/2012; 4(5):292-297.
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ABSTRACT: We examined the expressions of adhesion molecules (E-cadherin, beta-catenin, CD44s, and CD44v6) and Ki-67 labeling index (Ki-67 LI) in low- and moderate-grade dysplasia and invasive carcinoma components in ten noninvasive intraductal papillary mucinous neoplasms (IPMNs) of the pancreas and eight invasive carcinomas associated with IPMNs of the pancreas using immunohistochemical methods. There was no significant difference in regard to the proportion of components expressing either E-cadherin or beta-catenin in more than 70% of the tumor cells between the low- and moderate-grade dysplasia components. In contrast, the proportion of those in invasive carcinoma components was significantly lower than in low- or moderate-grade dysplasia components. Also, there was no significant difference in the proportion of components expressing CD44s or CD44v6 in more than 5% of tumor cells among low-grade dysplasia, moderate-grade dysplasia, and invasive carcinoma components. In contrast, the Ki-67 LI values increased in the order of low-grade dysplasia, moderate-grade dysplasia, and invasive carcinoma components, with significant differences among them. The present results indicate that carcinoma components are associated with a decrease in tumor cells expressing E-cadherin and beta-catenin and have the highest proliferative activity.
Medical Molecular Morphology 12/2009; 42(4):222-9. · 1.39 Impact Factor
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ABSTRACT: A 26-year-old woman presented with pain in the left hypochondrium, for which pancreatectomy and splenectomy was performed, with total gross excision of a mass. A tumor measuring 11 x 9 cm was found in the pancreas. On cut surface there were two cysts filled with a necrotic substance and hemorrhagic content. Spindle or ovoid-shaped cells, in the sarcomatous component, had diffusely infiltrated along the inner side of the walls of one cyst. Osteoclast-like giant cells (OGC) were also contained in the sarcomatous component. Adenoma components of mucinous epithelium with foci of borderline and adenocarcinomatous components were seen on the inner side of the other cyst. An ovarian-type stroma beneath the epithelial component was seen in the cyst wall. A diagnosis of undifferentiated carcinoma with OGC arising in a mucinous cystic neoplasm (MCN) of the pancreas, was made. Seven months after the initial operation the patient had a local recurrence, and the tumor was removed. One month after the second operation, the patient was free of symptoms. Only four cases of undifferentiated carcinoma with OGC arising in MCN, involving an ovarian-type stroma of the pancreas, have been reported.
Pathology International 07/2008; 58(6):383-9. · 1.62 Impact Factor
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Takashi Fujisawa,
Wataru Sano,
Sachiko Ouchi, Shigemitsu Ueyama,
Toshiyuki Mori,
Daisuke Tsuchihashi,
Osamu Uchikoga,
Maki Koyanagi,
Hisashi Yoshimura,
Shiro Tachibana,
Hirotsugu Hirano
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ABSTRACT: A 60-year-old man complaining of black stool, body weight loss, and anemia, was examined and diagnosed with advanced gastric cancer (M, type 3, por 2, cT3, cN3, cH0, cP0, cM0, cStage IV). A poor prognosis was predicted, yet we tried neoadjuvant chemotherapy (NAC) expecting downstaging of the tumor. Considering the efficacy and safety, we chose S-1+CDDP as the NAC regimen. S-1 (120 mg/day) was administered orally for 21 days, followed by CDDP (75 mg/body) div on day 8. Distal partial gastrectomy and lymph node dissection (D2) were performed, with Billroth I reconstruction. Histological examination of the resected stomach and lymph nodes revealed no residual cancer cells, suggesting complete histological remission (grade 3) according to the Japanese classification of gastric carcinoma.
Gan to kagaku ryoho. Cancer & chemotherapy 01/2008; 34(13):2297-300.
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ABSTRACT: Clostridium perfringens (C.P) gas gangrene is one of the most fulminant infectious diseases. We encountered fulminant massive gas gangrene in a 56- year-old man with alcoholic liver cirrhosis. The patient died 14 hours after diagnosis of gas gangrene (54 hours after admission). Dramatic changes in abdominal CT imaging revealed development of a massive volume of gas in the intra-portal vein, retroperitoneum and abdominal subcutaneous tissue within 24 hours. We also proved C.P infection by immunohistological staining, leading to a diagnosis of C.P gas gangrene.
Internal Medicine 06/2005; 44(5):499-502. · 0.94 Impact Factor
