Parathyroid carcinoma

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Parathyroid carcinoma
Diagnosis in short

Parathyroid carcinoma. H&E stain.

LM vascular invasion, local invasion, metastases; nuclear atypia not diagnostic
LM DDx parathyroid adenoma, parathyroid hyperplasia, thyroid lesions
IHC PAX8 +ve, Ki-67 >6% +ve, GATA3 +ve, parafibromin -ve
Molecular +/-HRPT2 gene mutations
Site parathyroid gland

Syndromes familial primary hyperparathyroidism

Prevalence very rare
Prognosis poor
Clin. DDx parathyroid adenoma, parathyroid hyperplasia, thyroid cancer
Treatment surgical excision

Parathyroid carcinoma is a rare epithelial malignancy of the parathyroid gland.

General

  • Extremely rare.
  • May be seen in the context of familial primary hyperparathyroidism (PHPT).[1]

Microscopic

Features:[2]

  • Histologically normal parathyroid cells.
    • Cytologic features not reliable for diagnosis.
  • Fibrous capsule.
  • Invasion of surrounding tissue - key feature.
  • +/-Metastasis - diagnostic feature.

DDx:

Images

IHC

  • Ki-67 >6% of cells positive - supports diagnosis.[3]
    • Parathyroid adenomas and hyperplasias ~ 3%.
  • PAX8 +ve.[4]
  • GATA3 +ve.[5]
  • Parafibromin -ve.[6]

Molecular

  • HRPT2 gene mutations (10 of 15 sporadic cases of parathyroid carcinoma).[7]

Note:

  • The HRPT2 gene encodes the parafibromin protein.[7]

See also

References

  1. Cetani, F.; Pardi, E.; Marcocci, C. (Jun 2018). "Parathyroid carcinoma: a clinical and genetic perspective.". Minerva Endocrinol 43 (2): 144-155. doi:10.23736/S0391-1977.17.02737-7. PMID 28949121.
  2. Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1128. ISBN 978-1416031215.
  3. Abbona, GC.; Papotti, M.; Gasparri, G.; Bussolati, G. (Feb 1995). "Proliferative activity in parathyroid tumors as detected by Ki-67 immunostaining.". Hum Pathol 26 (2): 135-8. PMID 7860042.
  4. Ordóñez, NG. (May 2012). "Value of PAX 8 immunostaining in tumor diagnosis: a review and update.". Adv Anat Pathol 19 (3): 140-51. doi:10.1097/PAP.0b013e318253465d. PMID 22498579.
  5. Ordóñez NG (2014). "Value of GATA3 immunostaining in the diagnosis of parathyroid tumors". Appl Immunohistochem Mol Morphol 22 (10): 756–61. doi:10.1097/PAI.0000000000000007. PMID 25046229.
  6. Tan MH, Morrison C, Wang P, Yang X, Haven CJ, Zhang C, Zhao P, Tretiakova MS, Korpi-Hyovalti E, Burgess JR, Soo KC, Cheah WK, Cao B, Resau J, Morreau H, Teh BT (October 2004). "Loss of parafibromin immunoreactivity is a distinguishing feature of parathyroid carcinoma". Clin Cancer Res 10 (19): 6629–37. doi:10.1158/1078-0432.CCR-04-0493. PMID 15475453.
  7. 7.0 7.1 Shattuck TM, Välimäki S, Obara T, Gaz RD, Clark OH, Shoback D, Wierman ME, Tojo K, Robbins CM, Carpten JD, Farnebo LO, Larsson C, Arnold A (October 2003). "Somatic and germ-line mutations of the HRPT2 gene in sporadic parathyroid carcinoma". N Engl J Med 349 (18): 1722–9. doi:10.1056/NEJMoa031237. PMID 14585940.