Lyss A P
Washington University School of Medicine, St Louis, MO.
Semin Oncol. 1988 Apr;15(2):129-37.
Malignancies of the appendix are extremely rare. Of the reported cases, carcinoid tumors are most common, with mucinous cystadenocarcinomas and adenocarcinomas next in frequency. Adenocarcinoids, which share morphologic and clinical features that are a composite of adenocarcinomas and carcinoids, have been described. Concomitant second malignancies, most often in the GI tract, occur with greater than expected frequency in patients with appendiceal tumors. Treatment and prognosis are markedly different for each of the histologic variants of appendiceal malignancy. In general, carcinoid tumors (smaller than 2 cm in diameter) may be treated by appendectomy alone and are associated with long term survival. Adenocarcinomas, unless well differentiated and extremely superficial and localized, required hemicolectomy and confer a prognosis that is, stage for stage, similar to that of adenocarcinoma of the colon. Mucinous cystadenocarcinomas may spread widely through the abdomen, resulting in P peritonei, but can be associated with long survival duration if surgical treatment is aggressive. There is evidence that adenocarcinoids develop from simultaneous neoplastic change in two stem cell populations. Metastases may show histologic features of adenocarcinomas alone, of carcinoid tumors alone, or may resemble a composite neoplasm. The prognosis is variable and treatment recommendations are not well defined.
阑尾恶性肿瘤极为罕见。在已报道的病例中，类癌肿瘤最为常见，黏液性囊腺癌和腺癌次之。腺类癌具有腺癌和类癌的综合形态学及临床特征，已有相关描述。阑尾肿瘤患者并发第二恶性肿瘤的情况极为常见，其中大多数发生在胃肠道。阑尾恶性肿瘤的每种组织学类型在治疗和预后方面均有显著差异。一般来说，直径小于2厘米的类癌肿瘤可仅行阑尾切除术，且患者长期存活。腺癌除非分化良好、极为表浅且局限，否则需要行半结肠切除术，其分期与结肠癌腺癌相似。黏液性囊腺癌可能广泛扩散至腹部，导致腹膜假黏液瘤，但如果积极进行手术治疗，患者可能长期存活。有证据表明腺类癌由两个干细胞群同时发生肿瘤性改变发展而来。转移灶可能仅表现为腺癌的组织学特征，或仅表现为类癌肿瘤的组织学特征，也可能类似混合性肿瘤。其预后不一，治疗建议尚不明确。
