Granular cell tumour

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Granular cell tumour
Diagnosis in short

Granular cell tumour. H&E stain.

LM cells (usually epithelioid) with (usually abundant) eosinophilic granular cytoplasm (granules ~ 1-3 micrometers, poorly demarcated on LM), +/-pseudoepitheliomatous hyperplasia
Subtypes benign (common), malignant (uncommon)
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 usually good (benign)
Clin. DDx xanthoma, other skin lesions
Treatment complete excision (benign) or wide excision (malignant)

The granular cell tumour is a rare histomorphologically distinctive neoplasm found at many sites. The classic location is the head and neck.

General

Treatment:

Sites

May be seen in any number of sites:

Gross

  • Yellow nodule.

DDx of yellow nodule:

Microscopic

Features:

  • Cells eosinophilic or grey granular cytoplasm - key feature.
    • Cytoplasm usually abundant.
    • Granules (represent abundant lysosomes[7]):
      • Size: 1-3 micrometers.
      • Poorly demarcated (on light microscopy).
  • Nested architecture.
  • +/-Pseudoepitheliomatous hyperplasia.

DDx:

Malignant features

Features of malignancy (3 of 6 required):[8]

Predictive of poor outcome:[8]

  • Ki-67 >10%.

Special stains

  • PAS +ve.

IHC

Features:[9]

  • S100 +ve.
  • CD68 +ve (cytoplasmic).
  • Vimentin +ve (membranous).
  • Calretinin +ve (90-95%).[10]

Images

EM

  • Abundant lysosomes.[7]
    • Round structures with variable (electron) density.

Images:

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SKIN LESION, NECK, EXCISION:
- GRANULAR CELL TUMOUR WITH BENIGN FEATURES, INCOMPLETELY EXCISED.

COMMENT:
The lesional cells stain as follows:
POSITIVE: S-100, CD68, vimentin, calretinin.
NEGATIVE: HMB-45.

A conservative re-excision is recommended.

Micro

The sections show skin with nests of cells in the mid dermis, extending to the subcutis. The cells that make up the nests are epithelioid and have an abundant granular grey cytoplasm, and round regular nuclei without obvious nucleoli. No mitotic activity is readily apparent. No necrosis is identified. No nuclear pleomorphism is apparent. The lesional cells focally wrap around nerve. The lesion is completely excised in the plane of section.

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. Toelen C, Goovaerts G, Huyghe M (2013). "Granular cell tumor of the abdominal wall: case report and review of the literature". Acta Chir. Belg. 113 (5): 364–6. PMID 24294803.
  5. Emesz M, Arlt EM, Krall EM, et al. (November 2013). "[Granular cell tumors of the orbit : Diagnostics and therapeutic aspects exemplified by a case report.]". Ophthalmologe. doi:10.1007/s00347-013-2972-1. PMID 24173669.
  6. Paksoy M, Eken M, Ayduran E, Altin G (March 2014). "Two cases of granular cell tumors of the head and neck at different sites". Ear Nose Throat J 93 (3): E15–7. PMID 24652564.
  7. 7.0 7.1 Ordóñez, NG. (Jul 1999). "Granular cell tumor: a review and update.". Adv Anat Pathol 6 (4): 186-203. PMID 10410172.
  8. 8.0 8.1 Fanburg-Smith JC, Meis-Kindblom JM, Fante R, Kindblom LG (July 1998). "Malignant granular cell tumor of soft tissue: diagnostic criteria and clinicopathologic correlation". Am. J. Surg. Pathol. 22 (7): 779–94. PMID 9669341.
  9. 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.
  10. 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.