神経内分泌腫瘍。胆嚢の稀で進行性の腫瘍

Neuroendocrine Tumor: A Rare, Aggressive Tumor of the Gallbladder.

• Ishtiaq Hussain
• Deepika Sarvepalli
• Hammad Zafar
• Sundas Jehanzeb
• Waqas Ullah

抄録

内視鏡的超音波診断（EUS）の助けを借りて診断された稀で侵攻性のある胆嚢神経内分泌癌（GB-NEC）のWereporta症例。65歳の無症状の男性で、高血圧の既往歴があり、腹部大動脈瘤のスクリーニングのために腹部超音波検査を受けた。腹部超音波検査では，腹部超音波検査の付随所見である胃付近に混合性のエコー発生部を認めた．この患者は上部消化管（GI）内視鏡検査を受け、生検で肛門部腫瘤を発見した。組織標本には未分類型の上皮性間葉系腫瘍が認められ、最終的には胃の部分切除術を行った。外科病理検査では、切除した組織標本の低悪性度の漿膜下胃腸間質腫瘍（GIST）が報告された。この患者はその後退院し、毎年腹部コンピュータ断層撮影（CT）でフォローアップするように勧められた。2年後、腹部CTで胆嚢後面眼底に3.7cm×2.0cmの腫瘤が新たに認められた。その後，腹腔鏡下胆嚢摘出術を受け，切除生検でT3NXM1神経内分泌小細胞癌と診断された．その後、カルボプラチンとエトポシドによる全身化学療法を6サイクル受け、当初は良好な奏効を示した。しかし，8ヵ月目の経過観察時に造影剤を用いた繰り返しCT腹・骨盤検査を行ったところ，膵頭部に浸潤性の腫瘤が間隔をおいて発生していた。その後、消化器内科を受診し、一時的にステントを留置した括約筋切開術と腹腔神経叢神経分解術を施行した。また、膵腫瘤の経十二指腸細針吸引（FNA）を行ったところ、転移性小細胞癌が認められた。これらの所見をもとに、カルボプラチン／エトポシド化学療法を3サイクル、免疫療法を1サイクル追加した。しかし、化学療法に対する反応が悪く、最終的にはホスピスケアを選択した。

We report a case of rare and aggressive gallbladder neuroendocrine carcinoma (GB-NEC), diagnosed with the help of endoscopic ultrasound (EUS). A 65-year-old asymptomatic male, with a past medical history of hypertension, underwent abdominal ultrasound for the screening of an abdominal aortic aneurysm. He was found to have a mixed echogenicity area near the stomach, an incidental finding on abdominal ultrasound. The patient had an upper gastrointestinal (GI) endoscopy exam, which revealed an antral mass that was biopsied. The tissue specimen showed an epithelioid mesenchymal tumor of unclassified type and, eventually, the patient underwent partial gastrectomy. Surgical pathology reported a low-grade sub-serosal gastrointestinal stromal tumor (GIST) of the resected tissue specimen. He was later discharged and advised to follow up with abdominal computed tomography (CT) every year. Two years later, his abdominal CT revealed a new 3.7 cm x 2.0 cm mass in the posterior gallbladder fundus. Subsequently, the patient underwent laparoscopic cholecystectomy and the excisional biopsy reported a T3NXM1 neuroendocrine small cell carcinoma. Then, he received six cycles of systemic chemotherapy with carboplatin and etoposide, showing excellent response initially. However, a repeat CT abdomen/pelvis with contrast, on his eighth-month follow-up, demonstrated the interval development of an infiltrative mass in the pancreatic head. The gastroenterology team was then consulted, who performed sphincterotomy with temporary stent placement and celiac plexus neurolysis. Also, a transduodenal fine-needle aspiration (FNA) of the pancreatic mass was performed, which revealed metastatic small cell carcinoma. Based on these findings, the patient received an additional three cycles of carboplatin/etoposide chemotherapy, along with one cycle of immunotherapy. However, the patient had a poor response to chemotherapy, and he eventually chose hospice care.

Copyright © 2019, Hussain et al.