Difference between revisions of "Parathyroid carcinoma"

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{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Parathyroid carcinoma - 1 -- high mag.jpg
| Width      =
| Caption    = Parathyroid carcinoma. [[H&E stain]].
| Synonyms  =
| Micro      = vascular invasion, local invasion, metastases; nuclear atypia ''not'' diagnostic
| Subtypes  =
| LMDDx      = [[parathyroid adenoma]], [[parathyroid hyperplasia]], thyroid lesions
| Stains    =
| IHC        = [[PAX8]] +ve, Ki-67 >6% +ve, [[GATA3]] +ve, parafibromin -ve
| EM        =
| Molecular  = +/-HRPT2 gene mutations
| IF        =
| Gross      =
| Grossing  =
| Staging    =
| Site      = [[parathyroid gland]]
| Assdx      =
| Syndromes  = familial primary hyperparathyroidism
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = very rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = poor
| Other      =
| ClinDDx    = [[parathyroid adenoma]], [[parathyroid hyperplasia]], [[thyroid cancer]]
| Tx        = surgical excision
}}
'''Parathyroid carcinoma''' is a rare epithelial malignancy of the [[parathyroid gland]].
'''Parathyroid carcinoma''' is a rare epithelial malignancy of the [[parathyroid gland]].


==General==
==General==
*Extremely rare.
*Extremely rare.
*May be seen in the context of ''familial primary hyperparathyroidism'' (PHPT).<ref name=pmid28949121>{{Cite journal  | last1 = Cetani | first1 = F. | last2 = Pardi | first2 = E. | last3 = Marcocci | first3 = C. | title = Parathyroid carcinoma: a clinical and genetic perspective. | journal = Minerva Endocrinol | volume = 43 | issue = 2 | pages = 144-155 | month = Jun | year = 2018 | doi = 10.23736/S0391-1977.17.02737-7 | PMID = 28949121 }}</ref>


==Microscopic==
==Microscopic==
Line 12: Line 45:
*+/-[[Metastasis]] - '''diagnostic feature'''.
*+/-[[Metastasis]] - '''diagnostic feature'''.


Note:
DDx:
*Diagnosis of ''parathyroid carcinoma'' is like that of ''malignant [[pheochromocytoma]]'' - cytology useless, tissue invasion and metastases are the key features.
*[[Parathyroid adenoma]].
*[[Parathyroid hyperplasia]].
*[[Poorly differentiated thyroid carcinoma]] and other thyroid cancers.


===Images===
===Images===
<gallery>
Image: Parathyroid carcinoma - 1 -- low mag.jpg | PC - low mag. (WC)
Image: Parathyroid carcinoma - 1 -- intermed mag.jpg | PC - intermed. mag. (WC)
Image: Parathyroid carcinoma - 1 - alt -- intermed mag.jpg | PC - intermed. mag. (WC)
Image: Parathyroid carcinoma - 1 -- high mag.jpg | PC - high mag. (WC)
Image: Parathyroid carcinoma - 1 -- very high mag.jpg | PC - very high mag. (WC)
Image: Parathyroid carcinoma - 2 -- low mag.jpg | PC - low mag. (WC)
Image: Parathyroid carcinoma - 2 -- intermed mag.jpg | PC - intermed. mag. (WC)
Image: Parathyroid carcinoma - 3 -- very low mag.jpg | PC - very low mag. (WC)
Image: Parathyroid carcinoma - 3 -- low mag.jpg | PC - low mag. (WC)
Image: Parathyroid carcinoma - 3 -- intermed mag.jpg | PC - intermed. mag. (WC)
Image: Parathyroid carcinoma - 3 -- high mag.jpg | PC - high mag. (WC)
</gallery>
*[http://emedicine.medscape.com/article/280908-overview Parathyroid carcinoma (medscape.com)].
*[http://emedicine.medscape.com/article/280908-overview Parathyroid carcinoma (medscape.com)].


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*Ki-67 >6% of cells positive - supports diagnosis.<ref name=pmid7860042>{{Cite journal  | last1 = Abbona | first1 = GC. | last2 = Papotti | first2 = M. | last3 = Gasparri | first3 = G. | last4 = Bussolati | first4 = G. | title = Proliferative activity in parathyroid tumors as detected by Ki-67 immunostaining. | journal = Hum Pathol | volume = 26 | issue = 2 | pages = 135-8 | month = Feb | year = 1995 | doi =  | PMID = 7860042 }}</ref>
*Ki-67 >6% of cells positive - supports diagnosis.<ref name=pmid7860042>{{Cite journal  | last1 = Abbona | first1 = GC. | last2 = Papotti | first2 = M. | last3 = Gasparri | first3 = G. | last4 = Bussolati | first4 = G. | title = Proliferative activity in parathyroid tumors as detected by Ki-67 immunostaining. | journal = Hum Pathol | volume = 26 | issue = 2 | pages = 135-8 | month = Feb | year = 1995 | doi =  | PMID = 7860042 }}</ref>
**Parathyroid adenomas and hyperplasias ~ 3%.
**Parathyroid adenomas and hyperplasias ~ 3%.
*[[PAX8]] +ve.<ref name=pmid22498579>{{Cite journal  | last1 = Ordóñez | first1 = NG. | title = Value of PAX 8 immunostaining in tumor diagnosis: a review and update. | journal = Adv Anat Pathol | volume = 19 | issue = 3 | pages = 140-51 | month = May | year = 2012 | doi = 10.1097/PAP.0b013e318253465d | PMID = 22498579 }}</ref>
*GATA3 +ve.<ref>{{cite journal |authors=Ordóñez NG |title=Value of GATA3 immunostaining in the diagnosis of parathyroid tumors |journal=Appl Immunohistochem Mol Morphol |volume=22 |issue=10 |pages=756–61 |date=2014 |pmid=25046229 |doi=10.1097/PAI.0000000000000007 |url=}}</ref>
*Parafibromin -ve.<ref name=pmid15475453>{{cite journal |authors=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 |title=Loss of parafibromin immunoreactivity is a distinguishing feature of parathyroid carcinoma |journal=Clin Cancer Res |volume=10 |issue=19 |pages=6629–37 |date=October 2004 |pmid=15475453 |doi=10.1158/1078-0432.CCR-04-0493 |url=}}</ref>
==Molecular==
*HRPT2 gene mutations (10 of 15 sporadic cases of parathyroid carcinoma).<ref name=pmid14585940>{{cite journal |authors=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 |title=Somatic and germ-line mutations of the HRPT2 gene in sporadic parathyroid carcinoma |journal=N Engl J Med |volume=349 |issue=18 |pages=1722–9 |date=October 2003 |pmid=14585940 |doi=10.1056/NEJMoa031237 |url=}}</ref>
Note:
*The HRPT2 gene encodes the parafibromin protein.<ref name=pmid14585940/>


==See also==
==See also==

Latest revision as of 22:28, 9 October 2021

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.