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:
- Parathyroid adenoma.
- Parathyroid hyperplasia.
- Poorly differentiated thyroid carcinoma and other thyroid cancers.
Images
PC - low mag. (WC)
PC - intermed. mag. (WC)
PC - intermed. mag. (WC)
PC - high mag. (WC)
PC - very high mag. (WC)
PC - low mag. (WC)
PC - intermed. mag. (WC)
PC - very low mag. (WC)
PC - low mag. (WC)
PC - intermed. mag. (WC)
PC - high mag. (WC)
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
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 7.0 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.