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消化管カルチノイドの中でも胆管カルチノイドは極めてまれであり,Davies,Pilzらの報告を含めて7例にすぎない.われわれはカルチノイド症候群を来した本邦2例目の胆管カルチノイドを経験したので報告する.
Bile duct carcinoid is extremely rare disease. This case is an extrahepatic bile duct carcinoid which seems to be the eighth case reported in literature.
The patient, a 64 year-old man, was admitted to our hospital with chief complaint of right hypochondralgia. ERCP revealed an apple core-like defect contour about 2 cm in diameter and marked stenosis of the common bile duct was noted. Pancreatography showed almost normal pancreatic duct appearance. PTC demonstrated complete obstruction of the common bile duct and marked dilatation of the common hepatic and intrahepatic bile duct. Hence PTC drainage was performed. Celiac angiography and CT scan did not revealed any findings of the tumor and liver metastasis. Percutaneous transhepatic portography (PTP) disclosed no tumor infiltration to the portal vein around the common bile duct, and the tumor was diagnosed as resectable. Carcinoid syndrome, such as flushing, diarrhea, fever and hypotention, developed after PTP. Pancreatoduodenectomy was performed and reconstruction was made with Child's method. Operative finding showed H0, P0, S0, N0, Hinf0, G0, Panc1, D0, V0, that is Stage I, and an absolute curative resection was performed.
Resected specimen showed the tumor was 2.5×2.5×2.0 cm in size, nodular and grayish white in colour. Histologically the tumor was composed of clear cells with uniform round nuclei and arranged mainly in nodular nests manner. Argentaffin reaction is negative but showed positive argyrophilic reaction. An electron microscopic studies demonstrated round specific secretory granules, measuring 130~300 nm in diameter, in the cytoplasms of the tumor cells.
He was free from any recurrence or metastasis for five months after operation, then multiple liver metastasis were noted and died. Increased CEA levels (19.8 ng/ml) was noted with urinary 5-HIAA(11.5 mg/day), however serotonin levels (9.3 mg/dl)remained in normal range.
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