CLINICAL PRACTICE

Rare Gems: Navigating Goblet Cell Appendiceal Cancer


 

Background

Goblet cell adenocarcinoma (GCA), also known as goblet cell carcinoid, is a rare and distinct type of cancer originating from the appendix. It is characterized by cells that exhibit both mucinous and neuroendocrine differentiation, presenting a more aggressive nature compared to conventional carcinoids and a higher propensity for metastasis.

Case Presentation

A 60-year-old male presented with complaints of abdominal pain, nausea, vomiting, constipation, and weight loss worsening in the last month. He had a history of heavy alcohol intake, smoking, and family history of colon cancer in his grandfather. Initial workup with abdominal CT revealed findings suggestive of early bowel obstruction and possible malignancy. Subsequent EGD showed esophagitis, and colonoscopy identified a cecal mass. Biopsies confirmed malignant cells of enteric type with goblet cell features. Staging CT during hospitalization did not reveal distant metastasis initially. However, diagnostic laparoscopy later identified widespread peritoneal carcinomatosis, precluding surgical intervention. The case was discussed in tumor boards, leading to the initiation of palliative FOLFOX + Bevacizumab chemotherapy. After completing 7 cycles, restaging imaging showed stable disease. Subsequently, the patient experienced worsening obstructive symptoms with CT abdomen and pelvis demonstrating disease progression. Given his condition, decompressive gastrostomy was not feasible. The patient decided to transition to comfort measures only.

Discussion

Goblet cell adenocarcinoma is a rare appendiceal tumor with amphicrine differentiation, occurring at a rate of 0.01–0.05 per 100,000 individuals annually and comprising approximately 15% of all appendiceal neoplasms. These tumors often disseminate within the peritoneum, contributing to their aggressive behavior and challenging management.

Conclusions

Metastatic goblet cell adenocarcinoma presents significant treatment challenges and is associated with a poor prognosis. Tailored treatment strategies, vigilant monitoring, and ongoing research efforts are essential for optimizing patient outcomes and enhancing quality of life in this aggressive cancer

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