Goblet cell carcinoid
Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
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| Goblet cell carcinoid | |
|---|---|
| Synonyms | GCC, goblet cell adenocarcinoid |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Abdominal pain, appendicitis, bowel obstruction |
| Complications | Metastasis, peritoneal carcinomatosis |
| Onset | Middle-aged adults |
| Duration | Chronic |
| Types | Classic, mixed, signet ring cell type |
| Causes | Unknown |
| Risks | Smoking, family history |
| Diagnosis | Histopathology, immunohistochemistry |
| Differential diagnosis | Adenocarcinoma, carcinoid tumor |
| Prevention | None known |
| Treatment | Surgery, chemotherapy |
| Medication | 5-fluorouracil, oxaliplatin |
| Prognosis | Variable, depends on stage |
| Frequency | Rare |
| Deaths | Dependent on stage and treatment |
A type of tumor found in the appendix
Goblet Cell Carcinoid
Goblet cell carcinoid (GCC) is a rare type of neuroendocrine tumor that primarily arises in the appendix. It is characterized by the presence of both goblet cells and neuroendocrine cells, which gives it a unique histological appearance. Unlike typical carcinoid tumors, goblet cell carcinoids exhibit both endocrine and exocrine features.
Pathophysiology
Goblet cell carcinoids are thought to originate from pluripotent stem cells in the appendiceal epithelium. These tumors display a mixed phenotype, with both mucin-producing goblet cells and neuroendocrine cells. The presence of mucin is a distinguishing feature, as it is not typically found in other types of neuroendocrine tumors.
Clinical Presentation
Patients with goblet cell carcinoid often present with symptoms similar to acute appendicitis, such as abdominal pain, nausea, and vomiting. In some cases, the tumor may be discovered incidentally during an appendectomy or other abdominal surgery. Advanced cases may present with signs of metastasis, particularly to the peritoneum and ovaries.
Diagnosis
The diagnosis of goblet cell carcinoid is typically made through histopathological examination of the appendiceal tissue. The presence of both goblet cells and neuroendocrine cells is confirmed using special staining techniques, such as immunohistochemistry. Common markers used in the diagnosis include chromogranin A, synaptophysin, and cytokeratin.
Treatment
The primary treatment for goblet cell carcinoid is surgical resection. An appendectomy is often performed, and in cases where the tumor has spread, more extensive surgery such as a right hemicolectomy may be necessary. The role of chemotherapy and radiation therapy is not well established, but they may be considered in cases of advanced disease.
Prognosis
The prognosis for patients with goblet cell carcinoid varies depending on the stage of the disease at diagnosis. Early-stage tumors confined to the appendix have a relatively good prognosis following surgical resection. However, the presence of metastasis significantly worsens the outlook. Regular follow-up and monitoring are essential for managing potential recurrence or progression.
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Contributors: Prab R. Tumpati, MD