Poorly Differentiated Adenocarcinoma with Signet-ring Cells of the Vater's Ampulla, without Jaundice but with Disseminated Carcinomatosis
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A 49-year-old man was hospitalized because of a 2-month history of purpura in his extremities and for back pain. Laboratory findings showed alkaline phosphatase to be greatly elevated, and platelet counts and coagulation factor showed that the patient had disseminated intravascular coagulation (DIC). Compression fractures of the thoracic vertebrae were found on radiological examination. The histological findings from bone marrow showed metastasis of adenocarcinoma with signet-ring cells, although the primary site was unknown. To reduce tumor cells in number and improve DIC, 11 cycles of 5-Fluorouracil and leucovorin therapy were done, and the patient survived for 12 months. Autopsy showed a 0.8 cm diameter, poorly differentiated adenocarcinoma with the signetring cell type in the lamina propria of the Vater's ampulla. Many metastatic foci and micro tumor emboli were found in the lung and in bone marrow. The sections of thestomach, the gallbladder, urinary bladder, prostate, and thyroid gland showed no malignant cells. These findings suggest that the origin of the cancer may have been located in the Vater's ampulla. This is a rare case of an ampullary tumor of poorly differentiated adenocarcinoma with the signet-ring cell type, without jaundice but with multiple metastasis. 5-Fluorouracil and leucovorin were effective for increasing survival time and improving quality of life.症例は49歳男性で2ケ月にわたる四肢の紫斑と背部痛を主訴に来院した. 生化学検査におけるALPの著明な上昇と血小板数及び凝固能の低下より播種性血管内凝固症候群(DIC)と診断された. レントゲン写真にて胸椎の圧迫骨折が認められた. 骨髄穿刺にて転移性印環細胞癌が証明されたが原発巣は不明であった. 腫瘍量の縮小とDIC改善の目的にて計11回の5-Fluorouraci1及びleucovorinの投与を行い, DICは改善, 患者はその後12ケ月間生存した. 剖検では0.8cm径の未分化腺癌(印環細胞癌)がファーター乳頭固有粘膜層に認められた. 多数の転移細胞及び腫瘍塞栓が肺及び骨髄に認められた. 他の臓器に原発巣と考えられえる腫瘍は認められなかった. これらの所見より原発巣はファーター乳頭部と判断した. この症例は, 黄疸を伴わないが全身播種が認められた乳頭部の印環細胞癌であり希少な症例と考えられた. 5-Fluorouracil及びleucovorinが生存期間とADLの改善に有効であった.
- 2003-07-25
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