Primary mucinous carcinoma of the thyroid (PMCT) is an extremely rare, aggressive, and distinct malignant thyroid tumor, with fewer than 10 cases reported in literature. Characterized by cells producing excessive mucin, it carries a poor prognosis (survival ranging from 1 month to 2 years). It often presents as a rapid-growing, hard neck mass causing dysphonia.
ScienceDirect.com +2
Key Characteristics and Diagnosis
- Definition: A rare, mucin-producing primary cancer of the thyroid gland, first described in 1976.
- Pathology: Characterized by clusters of neoplastic epithelial cells surrounded by extracellular mucin.
- Immunohistochemistry: Typically positive for TTF1 and thyroglobulin, and negative for calcitonin.
- Differential Diagnosis: Must be distinguished from metastatic mucinous carcinoma (lung, breast, GI tract) or primary thyroid tumors with mucinous components (e.g., mucoepidermoid carcinoma).
Symptoms: Rapid neck mass, hoarseness, and dysphagia.
HCA Healthcare Scholarly Commons +6
Prognosis and Management
- Prognosis: Generally poor due to rapid progression and aggressive behavior.
Treatment: Early and complete surgical resection (often with neck dissection) is critical to improving survival.
National Institutes of Health (NIH) | (.gov) +4
Because this condition is so rare, diagnosis requires comprehensive examination to rule out cancers originating elsewhere, particularly the esophagus or trachea