Goblet cell carcinoid tumors of the appendix: An overview

Abstract

Goblet cell carcinoid is an enigmatic and rare tumor involving the appendix almost exclusively. Since its identification in 1969, understanding of this disease has evolved greatly, but issues regarding its histogenesis, nomenclature and management are still conjectural. The published English language literature from 1966 to 2009 was retrieved via PubMed and reviewed. Various other names have been used for this entity such as adenocarcinoid, mucinous carcinoid, crypt cell carcinoma, and mucin-producing neuroendocrine tumor, although none have been found to be completely satisfactory or universally accepted. The tumor is thought to arise from pluripotent intestinal epithelial crypt-base stem cells by dual neuroendocrine and mucinous differentiation. GCCs present in the fifth to sixth decade and show no definite sex predominance. The most common clinical presentation is acute appendicitis, followed by abdominal pain and a mass. Fifty percent of the female patients present with ovarian metastases. The histologic hallmark of this entity is the presence of clusters of goblet cells in the lamina propria or submucosa stain for various neuroendocrine markers, though the intensity is often patchy. Atypia is usually minimal, but carcinomatous growth patterns may be seen. These may be of signet ring cell type or poorly differentiated adenocarcinoma. Recently molecular studies have shown these tumors to lack the signatures of adenocarcinoma but they have some changes similar to that of ileal carcinoids (allelic loss of chromosome 11q, 16q and 18q). The natural history of GCC is intermediate between carcinoids and adenocarcinomas of the appendix. The 5-year overall survival is 76%. The most important prognostic factor is the stage of disease. Appendectomy and right hemicolectomy are the main modalities of treatment, followed by adjuvant chemotherapy in select cases. There is some debate about the surgical approach for these tumors, and a summary of published series and recommendations are provided.

Goblet cell carcinoid of the appendix: a case report

“…tarafından tanımlanmış olmakla birlikte (1,3) terim olarak kullanımı ilk kez 1974 yılında, Subbuswamy ve ark. tarafından, tümörü oluşturan temel hücrelerin Goblet hücrelerine olan benzerliğinden ve tümörün adenokarsinomdan daha çok karsinoid tümör histomorfolojisi göstermesinden dolayı önerilmiştir (3,4). GHKT'lerin, konvansiyonel endokrin tümörlerden daha agresif seyrettikleri, ancak adenokarsinomlar gibi malign davranış göstermediği bilindiği için histolojik ayırımları büyük önem taşır (4,6,7,11-13).…”

“…Sıklıkla 5-6. dekatlarda görülen bu tümörler, olgumuzda olduğu gibi sıklıkla akut karın şikayeti ile hastaneye başvuran ve akut apendisit tanısıyla operasyona alınan hastaların apendektomi materyalinin histopatolojik incelenmesiyle tanınırlar (3,4,12,(14)(15)(16). Karakteristik histolojik görünümlerine ek olarak bizim olgumuzda da görüldüğü gibi mezoapendikse yaygın invazyon ve perinöral invazyon gösterirler (5,6).…”

“…Goblet hücreli karsinoid hem nöroendokrin hem de müsinöz farklılaşma gösteren pluripotent intestinal kök hücrelerinden köken alır (3,4,5). Goblet hücreli karsinoid, adenokarsinoid, müsinöz karsinoid, kript hücreli karsinoid, müsin üreten nöroendokrin tümör gibi çeşitli isimlerle bilinir (3,6,7).…”

“…Goblet hücreli karsinoid, adenokarsinoid, müsinöz karsinoid, kript hücreli karsinoid, müsin üreten nöroendokrin tümör gibi çeşitli isimlerle bilinir (3,6,7). Dünya Sağlık Örgütünün (WHO) 2010 yılında yayınladığı nöroendokrin neoplazm sınıflamasına göre "GHKTler, nöroendokrin tümör grubunda yer almaktadır (8)(9)(10).…”

Goblet cell carcinoid of the appendix accompanied by adenomatous polyp with high-grade dysplasia at the cecum

“…Because its behavior is more aggressive than that of the other standard carcinoid tumors, more extensive surgical approaches may be required (6). A five-year survey revealed a survival rate of 60%-84% (7). Metastatic disease develops in approximately 20% of patients.…”