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阑尾杯状细胞类癌的极罕见发现。病例报告。

An extremely rare finding of goblet cell carcinoid of the appendix. A case report.

出版信息

Ann Ital Chir. 2021 Sep 27;10:S2239253X21035672.

Abstract

Goblet cell carcinoid or carcinoma (GCC) refers to an extremely rare appendiceal tumor usually diagnosed on post-operative histology as an incidental finding. Primary cancers of the vermiform appendix are quite rare, representing less than 1% of all gastrointestinal malignancies. GCCs are considered as a distinct entity of appendiceal tumors, consisting of both epithelial (glandular) and neuroendocrine elements containing goblet cells. This entity tends to be more aggressive than typical carcinoid tumors as it often presents with metastatic disease. Therefore, an early recognition and an appropriate grading is essential. The 5-year overall survival is 14-22% in stage III-IV disease. As a matter of fact, GCC warrants more aggressive surgical and medical (chemotherapy) treatments than typical carcinoid tumors. We, hereby, report a case of a 67-year old male presenting with an acute abdominal obstruction and a severe tenderness predominant in his right lower quadrant, together with an endoscopic and radiological suspect of left colonic malignancy and acute appendicitis. Left hemicolectomy and appendicectomy were performed and pathological specimens revealed a lowgrade adenocarcinoma of the descending colon and a high-grade appendiceal goblet cell carcinoid. Subsequent right hemicolectomy was performed according to the current guidelines. GCCs are more aggressive compared with conventional appendiceal tumors but less aggressive compared with adenocarcinomas and they often present with serosal and mesoappendiceal involvement. The lack of a standardized classification system for GCC and the discrepancies in specific reliable markers are responsible for an insufficient prognostic and predictive value at diagnosis. KEY WORDS: Appendiceal neoplasms, Carcinoid tumor, Colectomy, Goblet cells, Immunochemistry.

摘要

杯状细胞类癌或癌(GCC)是一种极其罕见的阑尾肿瘤,通常在术后组织学检查中被诊断为偶然发现。阑尾原发性癌症非常罕见,占所有胃肠道恶性肿瘤的比例不到 1%。GCC 被认为是阑尾肿瘤的一种独特实体,由上皮(腺体)和神经内分泌成分组成,包含杯状细胞。这种实体往往比典型类癌肿瘤更具侵袭性,因为它经常表现为转移性疾病。因此,早期识别和适当分级至关重要。在 III-IV 期疾病中,5 年总生存率为 14-22%。事实上,GCC 需要比典型类癌肿瘤更积极的手术和医疗(化疗)治疗。我们在此报告一例 67 岁男性,因急性肠梗阻和右下象限剧烈压痛就诊,内镜和影像学检查怀疑为左结肠恶性肿瘤和急性阑尾炎。进行了左半结肠切除术和阑尾切除术,病理标本显示降结肠癌为低级别腺癌,阑尾为高级别杯状细胞类癌。根据目前的指南,随后进行了右半结肠切除术。与传统阑尾肿瘤相比,GCC 更具侵袭性,但与腺癌相比侵袭性较小,且常伴有浆膜和阑尾系膜受累。缺乏 GCC 的标准化分类系统以及特定可靠标志物的差异是导致诊断时预后和预测价值不足的原因。关键词:阑尾肿瘤、类癌肿瘤、结肠切除术、杯状细胞、免疫组织化学。

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