Goblet cell carcinoid tumors

Goblet Cell Carcinoid Tumors

Goblet cell carcinoid tumors represent a unique entity. Its pathologic features and clinical behaviour are distinct from the classical carcinoid tumor as well as from adenocarcinoma of the appendix. The mean age at diagnosis is about 50 year. Morphologically, goblet cell carcinoids (GCC) arise in the mucosa without conventional dysplasia of the appendiceal epithelium. The fundamental morphological features include presence of mucin containing goblet-shaped epithelial cell arranged in round or oval clusters, neoplastic cells demonstrating focal positive immunoreactivity for neuroendocrine markers (CgA or synaptophysin).

Clinical Presentation of Globlet cell carcinoid tumors
Metastatic disease is quite common in this group of tumors, with lymph node metastases in about 10% of the cases at diagnosis. It has also spread to the peritoneum and omentum and some of them present rather aggressive growth pattern.

Diagnosis of Globlet cell carcinoid tumors
The diagnosis is usually made incidentally due to abdominal symptoms and CT or abdominal US is demonstrating a lesion in the distal ileum or appendix.

Treatment of Globlet cell carcinoid tumors
Surgery is the primary treatment. Metastatic disease should be treated like colorectal cancer with cytotoxic treatment.

Prognosis of Globlet cell carcinoid tumors
There are different types of GCCs based on histopathology. The most benign group presenting a 5-year survival of 100%, whereas the worst group with high proliferation index present a 3-year survival of only 17%.