Cecum Large Cell Neuroendocrine Carcinoma: A Case Report and Literature Review

盲腸大細胞神經內分泌惡性腫瘤：病例報告及文獻回顧

楊博翔(Po-Hsiang Yang) ； 黃敬文(Ching-Wen Huang) ； 蔡祥麟(Hsiang-Lin Tsai) ； 蘇偉智(Wei-Chih Su) ； 張琮琨(Tsung-Kun Chang) ； 陳伯榕(Po-Jung Chen) ； 陳彥成(Yen-Cheng Chen) ； 吳建廷(Chien-Ting Wu) ； 連培因(Peir-In Liang) ； 王照元(JawYuan Wang)

《南臺灣醫學雜誌》 18卷2期 (2022/12) Pp. 65-72

https://doi.org/10.6726/MJST.202212_18(2).0003

摘要

Colorectal large-cell neuroendocrine carcinomas (LCNECs) are extremely rare and the prognosis of patients with LCNEC have very poor prognosis with reported median overall survival time between 4 and 16 months. Herein, we report a 57-year-old man who presented with abdominal pain in the right lower quadrant with bloody stool for 1 month. Abdominal computed tomography revealed suspected cecum cancer with adjacent infiltration with metastatic lymphadenopathy in the pericolic region; clinical stage was cT3N1M0, stage IIIB. The patient underwent right hemicolectomy with lymph node dissection, and histological results confirmed poorly differentiated large cell neuroendocrine carcinoma with regional lymph node metastases (pT3N2aM0, stage IIIB). Four cycles of adjuvant chemotherapy with cisplatin and etoposide were administered; however, due to disease progression at the precaval region, a further 12 cycles of chemotherapy with the FOLFIRI regimen (5-fluorouracil, leucovorin, and irinotecan) were conducted. The precaval region tumor did not enlarge further and serum level of chromogranin A (CgA) decreased significantly. Follow-up at the outpatient department continued until August, 2021 with progression-free survival of 21 months. In this case, although poor prognosis was initially reported, better prognosis could be achieved through aggressive treatment. Moreover, the literature concerning LCNEC was reviewed.

關鍵字

cecum ； large cell neuroendocrine carcinoma ； poorly differentiated ； FOLFIRI

並列摘要

大腸直腸癌中的大細胞神經內分泌惡性腫瘤是一種極為罕見的腫瘤且通常有很差的預後，文獻報告中位存活期為4到16個月。於此分享一位57歲男性，其表現的症狀為右下腹痛伴隨解血便且時間長達一個月。腹部電腦斷層顯示懷疑有盲腸癌並伴隨有周邊組織浸潤及在大腸周圍的淋巴結癌細胞轉移（cT3N1M0，臨床分期IIIB期）。他接受了右半結腸切除及淋巴結廓清。病理報告確認是盲腸大細胞神經內分泌惡性腫瘤及區域淋巴結癌細胞轉移（pT3N2aM0，病理分期IIIB期），後續接受四次的輔助化學治療（Cisplatin+Etoposide），但之後因為疾病復發在下腔靜脈區域而接受12次FOLFIRI化學治療。下腔靜脈區域的腫瘤沒有持續擴大但chromogranin A（CgA）濃度顯著下降，他持續接受門診追蹤到2021年8月，疾病無惡化存活期為21個月。雖然大細胞神經內分泌惡性腫瘤被文獻報告為預後很差，但藉由積極的治療還是有相當好的預後，同時我們也回顧了大腸直腸的大細胞神經內分泌惡性腫瘤相關的文獻。

並列關鍵字

盲腸 ； 大細胞神經內分泌惡性腫瘤 ； 分化不良 ； FOLFIRI