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Goblet cell carcinoid of the appendix.
Payam S Pahlavan1, Rani Kanthan
1Department of Physiology and Pathophysiology, University of Heidelberg, Heidelberg, Germany. payam.pahlavan@physiologie.uni-heidelberg.de
World Journal of Surgical Oncology
|June 22, 2005
Summary
Goblet cell carcinoid (GCC) of the appendix is a rare, aggressive tumor. Diagnosis requires careful pathology, and management involves surgery, with chemotherapy for advanced cases, offering a 5-year survival of 60-84%.
Area of Science:
- Gastroenterology
- Surgical Oncology
- Pathology
Background:
- Goblet cell carcinoid (GCC) of the appendix is a rare neoplasm with mixed adenocarcinoma and carcinoid features.
- GCC is considered more aggressive than conventional carcinoid tumors, with unclear malignant potential.
- Clinical presentations are varied, necessitating a broad differential diagnosis.
Purpose of the Study:
- To review the published literature on GCC of the appendix.
- To focus on diagnosis, histopathological aspects, clinical manifestations, and management strategies.
- To provide insights into the behavior and treatment of this rare appendiceal neoplasm.
Main Methods:
- A systematic review of English-language studies published between 1966 and 2004.
- Medline database search using keywords: "goblet cell carcinoid," "adenocarcinoid," "mucinous carcinoid," and "crypt cell carcinoma" of the appendix.
- Analysis of data from 57 papers encompassing nearly 600 diagnosed patients.
Main Results:
- Mean age of presentation was 58.89 years, with equal male and female representation.
- Common presentations include acute appendicitis (22.5%) and asymptomatic findings (5.4%); submucosal growth and lack of a defined mass complicate diagnosis.
- Appendectomy with right hemicolectomy was the most common surgical treatment; 5-year survival ranges from 60% to 84%.
Conclusions:
- GCC of the appendix is a rare tumor that should be considered in various abdominal surgical scenarios.
- Histopathological features like increased Paneth cells and mucin secretion may indicate aggressive behavior.
- Management includes appendectomy with right hemicolectomy for localized disease, with chemotherapy and cytoreductive surgery for advanced or metastatic cases.