Difference between revisions of "Granular cell tumour"
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*Rare. | *Rare. | ||
*Usually benign. | *Usually benign. | ||
*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''. | ||
Revision as of 02:27, 15 January 2013
The granular cell tumour is a rare histomorphologically distinctive neoplasm found at many sites. The classic location is the head and neck.
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]
- Gene implicated in Noonan syndrome 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:
- Pseudoepitheliomatous hyperplasia is seen in:
- Fungal infections.
- Inflammatory papillary hyperplasia.
- Granular cell tumour.
- Adjacent to an ulcer.
Sites
May be seen in any number of sites:
- Granular cell tumour of the breast.
- Granular cell tumour of the skin.
- Granular cell tumour of the tongue.
- Granular cell tumour of the esophagus.
Gross
- Yellow nodule.
DDx of yellow nodule:
- Granular cell tumour.
- Lipoma.
- Xanthoma.
Microscopic
Features:
- Cells with abundant eosinophilic granular cytoplasm - key feature.
- Granules (represent abundant lysosomes[4]):
- Size: 1-3 micrometers.
- Poorly demarcated (on light microscopy).
- Granules (represent abundant lysosomes[4]):
- Nested architecture.
- +/-Pseudoepitheliomatous hyperplasia.
- May mimic SCC.
DDx:
Special stains
- PAS +ve.
IHC
Features:[5]
- S100 +ve.
- CD68 +ve (cytoplasmic).
- Vimentin +ve (membranous).
- Calretinin +ve (90-95%).[6]
Images:
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EM
- Abundant lysosomes.[4]
- Round structures with variable (electron) density.
Images:
See also
References
- ↑ 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.
- ↑ Online 'Mendelian Inheritance in Man' (OMIM) 176876
- ↑ 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.
- ↑ 4.0 4.1 Ordóñez, NG. (Jul 1999). "Granular cell tumor: a review and update.". Adv Anat Pathol 6 (4): 186-203. PMID 10410172.
- ↑ 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.
- ↑ 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.