Peritoneal Metastatic Goblet-Cell Carcinoid Tumor Treated With Cytoreductive Surgery and Intraperitoneal Chemotherapy

Abstract

We report a case of a goblet-cell carcinoid tumor of the appendix which metastasized to the peritoneum and was treated by using cytoreductive surgery (CRS) with intraperitoneal chemotherapy. A 47-year-old male presented with chronic constipation and was diagnosed as having a rectal adenocarcinoma with a signet-ring-cell component under colonoscopy. Computed tomography suggested peritoneal metastases with diffuse nodular parietal peritoneal thickening of the entire abdomen and focal invasion of the upper rectum by a seeding mass. CRS with intraperitoneal chemotherapy was done under the diagnosis of a rectal adenocarcinoma with peritoneal metastases. The pathologic diagnosis was a goblet-cell carcinoid tumor of the appendix with peritoneal metastasis. The histological discrepancy between a peritoneal metastatic mass and a rectal mass was due to the mixed histological pattern of a goblet-cell carcinoid tumor. A metastatic mass may not share identical immunohistochemical characteristics from its origin. This histologic discrepancy necessitates caution in diagnosing a distant metastasis of a goblet-cell carcinoid tumor.

Goblet cell carcinoids of the appendix: Tumor biology, mutations and management strategies

“…Yan et al[17] in their series of 26 patients reported that nine patients (35%) with metastatic GCC failed to stain for neuroendocrine marker. The explanation for this finding remains elusive[17-21]. …”

Goblet cell adenocarcinoma of the ascending colon: An underrecognized diagnostic pitfall

“… 13 Cytoreductive surgery and intraperitoneal chemotherapy have been employed for the peritoneal disease. 17 Extra‐appendiceal GCA usually exhibit aggressive behavior and present in advanced stages. 1 , 10 Prognosis depends on stage and tumor grade.…”

Colonic Goblet Cell Carcinoid: Rarity of a Rarity! A Case Report and Review of Literature

“…The colorectal neuro-endocrine tumour (NET) including GCCC does not present with any specific symptoms, however maypresent with abdominal pain, large mass, constipation, intestinal obstruction, tenesmus, haematochezia or anal pain, however as other goblet cell carcinomas, GCCCs tumour does not illicit carcinoid syndrome. These tumours can be detected incidentally during routine investigations such as colonoscopy (17,30,31,32).…”

“…Synaptophysin (Trans-membrane Channel Protein) is a common specific but not sensitive endocrine marker, it is used in carcinoid tumour, Merkel cell carcinoma (MCC) and small cell carcinoma (3,32,40,44,45).…”

“…Whereas surgical resection of GCC remains the mainstay of resectable disease, chemotherapy options offered are 5-fluorouracil (5-FU) and leucovorin (LV) (28). Cytoreductive surgery (CRS) and intraperitoneal chemotherapy have been employed for the peritoneal disease (29,32).…”