Difference between revisions of "Granular cell tumour"

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*May seen in the context of ''LEOPARD syndrome'' and a mutation in the ''PTPN11 gene''.<ref name=pmid19054014>{{Cite journal  | last1 = Schrader | first1 = KA. | last2 = Nelson | first2 = TN. | last3 = De Luca | first3 = A. | last4 = Huntsman | first4 = DG. | last5 = McGillivray | first5 = BC. | title = Multiple granular cell tumors are an associated feature of LEOPARD syndrome caused by mutation in PTPN11. | journal = Clin Genet | volume = 75 | issue = 2 | pages = 185-9 | month = Feb | year = 2009 | doi = 10.1111/j.1399-0004.2008.01100.x | PMID = 19054014 }}</ref>
*May seen in the context of ''LEOPARD syndrome'' and a mutation in the ''PTPN11 gene''.<ref name=pmid19054014>{{Cite journal  | last1 = Schrader | first1 = KA. | last2 = Nelson | first2 = TN. | last3 = De Luca | first3 = A. | last4 = Huntsman | first4 = DG. | last5 = McGillivray | first5 = BC. | title = Multiple granular cell tumors are an associated feature of LEOPARD syndrome caused by mutation in PTPN11. | journal = Clin Genet | volume = 75 | issue = 2 | pages = 185-9 | month = Feb | year = 2009 | doi = 10.1111/j.1399-0004.2008.01100.x | PMID = 19054014 }}</ref>
**PTPN11 = protein-tyrosine phosphatase non-receptor type 11.<ref>{{OMIM|176876}}</ref>
**PTPN11 = protein-tyrosine phosphatase non-receptor type 11.<ref>{{OMIM|176876}}</ref>
***Gene implicated in ''Noonan syndrome 1''.
***Gene implicated in ''[[Noonan syndrome]] 1''.
*May mimic (well-differentiated) [[squamous cell carcinoma]] - histopathologically.
*May mimic (well-differentiated) [[squamous cell carcinoma]] - histopathologically.
**There is a well-described phenomenon called ''pseudoepitheliomatous hyperplasia''.<ref name=pmid16487362>{{cite journal |author=Abu-Eid R, Landini G |title=Morphometrical differences between pseudoepitheliomatous hyperplasia in granular cell tumours and squamous cell carcinomas |journal=Histopathology |volume=48 |issue=4 |pages=407–16 |year=2006 |month=March |pmid=16487362 |doi=10.1111/j.1365-2559.2006.02350.x |url=}}</ref>
**There is a well-described phenomenon called ''pseudoepitheliomatous hyperplasia''.<ref name=pmid16487362>{{cite journal |author=Abu-Eid R, Landini G |title=Morphometrical differences between pseudoepitheliomatous hyperplasia in granular cell tumours and squamous cell carcinomas |journal=Histopathology |volume=48 |issue=4 |pages=407–16 |year=2006 |month=March |pmid=16487362 |doi=10.1111/j.1365-2559.2006.02350.x |url=}}</ref>

Revision as of 06:24, 27 February 2012

The granular cell tumour is a rare beast.

General

  • Rare.
  • Usually benign.
  • May seen in the context of LEOPARD syndrome and a mutation in the PTPN11 gene.[1]
    • PTPN11 = protein-tyrosine phosphatase non-receptor type 11.[2]
  • May mimic (well-differentiated) squamous cell carcinoma - histopathologically.
    • There is a well-described phenomenon called pseudoepitheliomatous hyperplasia.[3]

Aside:

Gross

  • Yellow nodule.

Note:

  • DDx of yellow nodule: granular cell tumour, lipoma, xanthoma.

Microscopic

Features:

  • Cells with abundant eosinophilic granular cytoplasm - key feature.
    • Granules:
      • Size: 1-3 micrometers.
      • Poorly demarcated.
  • Nested architecture.
  • +/-Pseudoepitheliomatous hyperplasia.

DDx:

Special stains

  • PAS +ve.

IHC

Features:[4]

  • S100 +ve.
  • CD68 +ve (cytoplasmic).
  • Vimentin +ve (membranous).
  • Calretinin +ve (90-95%).[5]

Images:

See also

References

  1. Schrader, KA.; Nelson, TN.; De Luca, A.; Huntsman, DG.; McGillivray, BC. (Feb 2009). "Multiple granular cell tumors are an associated feature of LEOPARD syndrome caused by mutation in PTPN11.". Clin Genet 75 (2): 185-9. doi:10.1111/j.1399-0004.2008.01100.x. PMID 19054014.
  2. Online 'Mendelian Inheritance in Man' (OMIM) 176876
  3. Abu-Eid R, Landini G (March 2006). "Morphometrical differences between pseudoepitheliomatous hyperplasia in granular cell tumours and squamous cell carcinomas". Histopathology 48 (4): 407–16. doi:10.1111/j.1365-2559.2006.02350.x. PMID 16487362.
  4. Rekhi, B.; Jambhekar, NA. (Jun 2010). "Morphologic spectrum, immunohistochemical analysis, and clinical features of a series of granular cell tumors of soft tissues: a study from a tertiary referral cancer center.". Ann Diagn Pathol 14 (3): 162-7. doi:10.1016/j.anndiagpath.2010.01.005. PMID 20471560.
  5. Fine, SW.; Li, M. (Feb 2003). "Expression of calretinin and the alpha-subunit of inhibin in granular cell tumors.". Am J Clin Pathol 119 (2): 259-64. doi:10.1309/GRH4-JWX6-J9J7-QQTA. PMID 12579997.