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

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COD: 27_09_2021_3567 Categorie: , , ,

Lavinia Amato, Manuel Valeri, Adolfo Petrina, Michela Boncompagni,
Nadia Pietropaoli, Roberto Ciaccarini

Department of General Surgery, University of Perugia, Medical School, Perugia, Italy
General Surgery, Santa Maria della Misericordia Hospital, Perugia, Italy
Pathological Anatomy and Histology, Santa Maria della Misericordia Hospital, Perugia, Italy

Ann Ital Chir, 2021; 10 – Sept. 27

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

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.