Medullary thyroid carcinoma

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Medullary thyroid carcinoma
Diagnosis in short

Medullary thyroid carcinoma. H&E stain.

LM nuclei with neuroendocrine features (round nuclei with salt-and-pepper chromatin), +/-amyloid deposits (fluffy appearing acellular eosinophilic material), +/-C-cell hyperplasia
Stains congo red +ve (amyloid deposits)
IHC calcitonin +ve, CEA +ve, chromogranin A +ve, synaptophysin +ve, thyroglobulin -ve (usually)
Gross usu. well-circumscribed, white, gray or yellow, gritty, firm
Site thyroid gland

Associated Dx C-cell hyperplasia
Syndromes multiple endocrine neoplasia IIa, multiple endocrine neoplasia IIb

Prevalence uncommon
Blood work serum calcitonin elevated
Prognosis poor

Medullary thyroid carcinoma, abbreviated MTC, is an uncommon epithelial malignancy of the thyroid gland that may be syndromic.

General

Medical school memory device - 3 M's:

Epidemiology:

  • Very rare.
  • Poor prognosis.
  • May be genetic (MEN IIa/b syndrome).
  • Arises from C cells (which produce calcitonin).

Syndromic tumours - typically:[1]

Serology:

  • Serum calcitonin classically elevated.[2]

Gross

Features:[1]

  • Usu. well-circumscribed.
  • White, gray or yellow.
  • Gritty.
  • Firm.

Image:

Microscopic

Features:

  • Nuclei with "neuroendocrine features".
    • Small, round nuclei.
    • Coarse chromatin (salt and pepper nuclei).
  • +/-Amyloid deposits - fluffy appearing acellular eosinophilic material in the cytoplasm.
  • +/-C-cell hyperplasia - seen with familial forms of MTC.
    • C cells (AKA parafollicular cell): abundant cytoplasm - clear/pale.

Note:

  • The amyloid is formed from calcitonin.[3]

DDx:

Images

www:

Stains

  • Congo-red +ve (amyloid present) - mnemonic: CRAP -- congo red amyloid protein.

IHC

Features:[4]

EM

  • Neurosecretory granules.
    • Feature seen in neuroendocrine tumours.

Images: Neurosecretory granules (ucsf.edu).

See also

References

  1. 1.0 1.1 Nosé, V. (Apr 2011). "Familial thyroid cancer: a review.". Mod Pathol 24 Suppl 2: S19-33. doi:10.1038/modpathol.2010.147. PMID 21455198.
  2. Vainas, I.; Marthopoulos, A.; Chrisoulidou, A.; Raptou, K.; Tziomalos, K.; Pazaitou-Panayiotou, K. (Jul 2013). "Calcitonin stimulation tests for the early diagnosis and follow-up of patients with C cell disease: a descriptive analysis.". Hippokratia 17 (3): 246-51. PMID 24470736.
  3. Khurana, R.; Agarwal, A.; Bajpai, VK.; Verma, N.; Sharma, AK.; Gupta, RP.; Madhusudan, KP. (Dec 2004). "Unraveling the amyloid associated with human medullary thyroid carcinoma.". Endocrinology 145 (12): 5465-70. doi:10.1210/en.2004-0780. PMID 15459123.
  4. URL: http://pathologyoutlines.com/thyroid.html#medullary. Accessed on: 17 January 2011.
  5. SB. 7 January 2010.
  6. de Micco, C.; Chapel, F.; Dor, AM.; Garcia, S.; Ruf, J.; Carayon, P.; Henry, JF.; Lebreuil, G. (Mar 1993). "Thyroglobulin in medullary thyroid carcinoma: immunohistochemical study with polyclonal and monoclonal antibodies.". Hum Pathol 24 (3): 256-62. PMID 8454270.