Neuroendocrine Carcinomas of the Colon and Rectum: Result of a 15-year Experience

目的 結直腸神經內分泌癌為少見之惡性腫瘤。本研究回顧林口長庚紀念醫院過去治療結直腸神經內分泌癌之經驗，並就其臨床與病理特徵進行綜述。 方法 本研究回顧並收集林口長庚紀念醫院之結直腸癌及病理學資料庫於1992 至2007 年間，經診斷罹患結直腸癌之11,000 餘名病患中，病理診斷結果為結直腸神經內分泌癌者共11 例。11 例病患之病理檢查結果皆由同一位病理科醫師加以重新檢閱。另回顧分析上述病患之個別醫療紀錄，並就其臨床病理及人口統計學特徵，如神經內分泌之病理分類、腫瘤位置、腫瘤期別、治療 (手術方式、化學治療或放射線治療) 成效、轉移情形，以及存活率等進行分析。 結果 5 例病患於初次診斷時即發現有遠處轉移的情形，緩和性放射線治療或化學治療對於上述病患之存活率似無法提供適當之改善，整體存活率自診斷日起算不到11 個月。 11 名病患之初次診斷結果顯示其中75% 有淋巴結轉移，45% 有遠處轉移，整體之六個月、一年與三年之存活率依序約為73%、45% 以及20%。上述結果與前人文獻相符，即相較於常見之同期腺癌，結直腸神經內分泌癌之侵襲性更強，預後亦較差。然而，兩名IIA 與IIIB 期直腸小細胞神經內分泌癌病患在接受cisplatin 合併etoposide 之輔助性化學治療後，其存活率有正面且具積極意義的初步結果，治療後存活期達10 年以上，且期間無疾病再發或轉移的情形。 結論 結直腸神經內分泌癌為一罕見但具侵襲性之疾病，占本院收治所有結直腸癌病例的0.1% 以下。積極給予II 期與III 期結直腸神經內分泌癌患者進行cisplatin 合併etoposide 之輔助性化學治療，可能有助提高病患之存活率，此值得未來進一步研究。
Purpose. The experience of the uncommon malignancy, neuroendocrine carcinomas of the colon and rectum with emphasis on the pathology and clinical characteristics at a single hospital was reviewed. Methods. Of more than 11,000 colon or rectal cancers removed from July 1992 to June 2007 at Chang Gung Medical Center in Taipei, 11 cases diagnosed as colon or rectal neuroendocrine carcinoma were evaluated. Pathology was reviewed by a single pathologist. Medical records were retrospectively reviewed and patients were analyzed in terms of clinicopathologic and demographic characteristics including neuroendocrine type, tumor location, tumor stage, responses to treatment (operative procedure, chemotherapy or radiotherapy), metastases, and survival. Results. Five patients had distantmetastasis at the time of diagnosis. Palliative chemotherapy or radiotherapy did not seem to offer a modest improvement in survival for these patients, with an overall survival of less than 11 months after diagnosis. Lymph node metastasis was found in 75%, and the distant metastasis in 45% of the 11 patients at the time of diagnosis. Overall survival rates for six-month, one-year, and three-year survival were 73 percent, 45 percent, 20 percent, respectively. These findings are in accordance with other publications, which demonstrate that the neuroendocrine carcinomas behave aggressively and are associated with worse prognosis than that of conventional adenocarcinomas of the same stage. Unexpectedly, improved results were found for two stage IIA and IIIB rectal small cell neuroendocrine carcinoma patients administered with adjuvant chemotherapy treatment with cisplatin and etoposide at our hospital. They were alive without evidence of disease at more than 10 years after treatment. Conclusions. Neuroendocrine malignancies are rare but behave aggressively in the colon or rectum, accounting for less than 0.1 percent of all colorectal cancers at our institution. Aggressive adjuvant chemotherapy with cisplatin and etoposide might offer a better chance of long-term survival for patients with stage II and III neuroendocrine carcinomas in the colon and rectum, which deserves further investigation.