Adenocarcinoma Ex Goblet Cell Carcinoid (GCC) of the Appendix: Report of Five Cases and Pitfalls in Diagnosis of GCC

Abstract

Neoplasm of the appendix is relatively rare. Only 0.9-1.4% of all appendectomy specimens is found to have it. One in particular is the adenocarcinoma ex goblet cell carcinoid. The exact histopathogenesis and pathologic classification of this neoplasm are yet to be elucidated. Herein we report five cases to emphasize the importance of meticulous sampling and the possibility of misdiagnosis due to the presence of diverticulitis and acute appendicitis in some of these patients. All of our patients initially were presented with symptoms of or mimicking appendicitis, with radiology imaging suggestive of acute appendicitis or an appendiceal abscess. The pathologic examination of the appendectomy specimen revealed the incidental finding of the adenocarcinoma ex goblet cell carcinoid with focal positivity of synaptophysin and chromogranin. Two of our patients had diverticulitis and perforated appendicitis, which may lead to a misdiagnosis of the goblet cell carcinoid due to the absence of a discrete mass formation and focal localization of these tumor cells. Therefore, meticulous sampling is imperative in the diagnosis of this entity.

Goblet Cell Adenocarcinoma of the Appendix: A Systematic Review and Incidence and Survival of 1,225 Cases From an English Cancer Registry

“…More rarely, authors used a small-cell lung cancer-based regimen such as carboplatin and etoposide, a NEN regimen such as streptozocin and 5-FU, or an ovarian cancer regimen such as carboplatin and docetaxel ( 58 ). Some authors combined systemic chemotherapy with targeted therapy such as bevacizumab ( 58 , 101 ). In the largest series of 2,552 patients, 70.2% of patients with stage IV disease received some form of chemotherapy; however, this was not associated with improved survival (HR 0.9, 95% CI: 0.49–1.82, p = 0.86) ( 18 ).…”

Diagnosis, Assessment, and Therapeutic Strategy for Colorectal Mixed Adenoneuroendocrine Carcinoma

“…Adenocarcinoma ex GCC, poorly differentiated adenocarcinoma (group C), was defined by focal evidence of goblet cells, not otherwise morphologically distinguishable from a poorly differentiated adenocarcinoma. A favorable 5-year survival rate of 100% was revealed in GCC group A patients; in contrast, survival rates of 36 and 0% were shown for group B and group C patients, respectively [9] . Although GCC does not meet the criteria for MANEC, an undifferentiated subtype shows poor outcomes similar to those of MANEC.…”

Adenocarcinoma Ex-Goblet Cell: a Retrospective Experience

“…In a similar vein, adenocarcinoma ex-goblet cell carcinoids (AGCCs) are rare chimeric neoplasms of appendiceal origin, comprising < 5% of appendiceal tumors, which have long been misunderstood [1]. These tumors have been called many names over the years from adenocarcinoid, mixed carcinoid/adenocarcinoma to adenocarcinoma with neuroendocrine differentiation.…”

“…AGCC patients are defined by unique clinicopathologic features including female predominance, young age at diagnosis (mean age 58.8), transmural (T3/T4) primary lesions, peritoneal predilection, and absence of hematogenous metastases [1–3]. In the local setting, these patients are typically treated with more aggressive surgeries such as right hemicolectomies rather than appendectomies.…”

“…The Chimera was a mythical beast first described by Homer in the Iliad as Ba thing of immortal make, not human, lion-fronted and snake behind, a goat in the middle, and shorting out the breath of the terrible flame of bright fire.” Its disparate parts made it difficult to categorize, formidable, and a challenge to defeat. In a similar vein, adenocarcinoma ex-goblet cell carcinoids (AGCCs) are rare chimeric neoplasms of appendiceal origin, comprising < 5% of appendiceal tumors, which have long been misunderstood [1]. These tumors have been called many names over the years from adenocarcinoid, mixed carcinoid/adenocarcinoma to adenocarcinoma with neuroendocrine differentiation.…”