Granular cell tumour
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The granular cell tumour is a rare histomorphologically distinctive neoplasm found at many sites. The classic location is the head and neck.
| Granular cell tumour | |
|---|---|
| Diagnosis in short | |
_skin.jpg) Granular cell tumour. H&E stain. | |
|
| |
| LM | cells with abundant eosinophilic granular cytoplasm (granules ~ 1-3 micrometers, poorly demarcated on LM), +/-pseudoepitheliomatous hyperplasia |
| LM DDx | squamous cell carcinoma, oncocytoma, adjacent ulcer, xanthoma, melanocytic nevus with neurotization |
| Stains | PAS +ve |
| IHC | S-100 +ve, CD68 +ve (cytoplasmic), vimentin +ve (membranous), calretinin +ve (usually) |
| EM | abundant lysosomes |
| Gross | yellow nodule |
| Site | typically head and neck - other sites: breast, skin, tongue, esophagus + more |
|
| |
| Syndromes | LEOPARD syndrome |
|
| |
| Prevalence | rare |
| Prognosis | usu. benign, may be malignant |
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:
- Squamous cell carcinoma.
- Oncocytoma.
- Melanocytic nevus with neurotization.
- Xanthoma.
Special stains
- PAS +ve.
IHC
Features:[5]
- S100 +ve.
- CD68 +ve (cytoplasmic).
- Vimentin +ve (membranous).
- Calretinin +ve (90-95%).[6]
Images
GCT. (WC)
GCT - S-100. (WC)
_S-100.JPG)
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.