Medullary carcinoma
Medullary carcinoma
Updated:4/23/2019
© Jun Wang, MD, PhD
General features
- Neuroendocrine tumor derived from C cells
- Express calcitonin
- Either sporadic (nonhereditary) or hereditary (familial)
- If familial, associated with MEN2A or 2B
Clinical features
- Neck mass
- Presentations due to local advance: hoarseness, dysphagia, and respiratory difficulty
- Paraneoplastic syndromes, including Cushing or carcinoid syndrome
- Other presentations associated with metastases, including bone pain, neurological defects
Key pathological features
- Usually nonencapsulated solid, gray-tan-yellow, firm nodule
- May be infiltrative
- Round, polygonal or spindle cells in nests, cords or follicles
- Granular cytoplasm and uniform round/oval nuclei with punctate chromatin
- Amyloid deposits from calcitonin, prominent vascularity with glomeruloid configuration or long cords of vessels, coarse calcifications
Key laboratory finding
- Elevated serum calcitonin and chromogranin A
Marker
- Positive for calcitonin, CEA
- Negative for thyroglobulin
- Congo Red: stain amyloid
Serum marker
- CEA, calcitonin
- Activating mutation of RET
Treatment
- Mostly palliative
- Total thyroidectomy
- Radiation therapy
- Chemotherapy
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