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
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
Molecular abnormality
  • Activating mutation of RET
Treatment
  • Mostly palliative
  • Total thyroidectomy
  • Radiation therapy
  • Chemotherapy



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