Difference between revisions of "Granular cell tumour"

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Granular cell tumour (view source)

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| Width      =
| Width      =
| Caption    = Granular cell tumour. [[H&E stain]].
| Caption    = Granular cell tumour. [[H&E stain]].
| Micro      = cells with abundant eosinophilic granular cytoplasm (granules ~ 1-3 micrometers, poorly demarcated on LM), +/-[[pseudoepitheliomatous hyperplasia]]  
| Micro      = cells (usually epithelioid) with (usually abundant) eosinophilic granular cytoplasm (granules ~ 1-3 micrometers, poorly demarcated on LM), +/-[[pseudoepitheliomatous hyperplasia]]  
| Subtypes  =
| Subtypes  = benign (common), malignant (uncommon)
| LMDDx      = [[squamous cell carcinoma]], [[oncocytoma]], adjacent ulcer  
| LMDDx      = [[squamous cell carcinoma]], [[oncocytoma]], adjacent ulcer, [[xanthoma]], [[melanocytic nevus]] with neurotization
| Stains    = PAS +ve
| Stains    = PAS +ve
| IHC        = S-100 +ve, CD68 +ve (cytoplasmic), vimentin +ve (membranous), calretinin +ve (usually)
| IHC        = S-100 +ve, CD68 +ve (cytoplasmic), vimentin +ve (membranous), calretinin +ve (usually)
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| Rads      =
| Rads      =
| Endoscopy  =
| Endoscopy  =
| Prognosis  = usu. benign, may be malignant
| Prognosis  = usually good (benign)
| Other      =
| Other      =
| ClinDDx    =
| ClinDDx    = xanthoma, other [[skin]] lesions
| Tx        = 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.
The '''granular cell tumour''' is a rare histomorphologically distinctive neoplasm found at many sites. The classic location is the [[head and neck pathology|head and neck]].


==General==
==General==
*A nerve shealth tumour.<ref name=pmid22668577>{{cite journal |authors=Izquierdo F, Suárez-Vilela D, Honrado E |title=Perineurial cells in granular cell tumors and neoplasms with perineural invasion: an immunohistochemical study |journal=Am J Dermatopathol |volume=34 |issue=8 |pages=800–9 |date=December 2012 |pmid=22668577 |doi=10.1097/DAD.0b013e31824ba93b |url=}}</ref>
*Rare.
*Rare.
*Usually benign.
*Usually benign.
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***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>


Aside:
Treatment:
*[[Pseudoepitheliomatous hyperplasia]] is seen in:
*Complete excision.<ref>{{cite journal |author=Toelen C, Goovaerts G, Huyghe M |title=Granular cell tumor of the abdominal wall: case report and review of the literature |journal=Acta Chir. Belg. |volume=113 |issue=5 |pages=364–6 |year=2013 |pmid=24294803 |doi= |url=}}</ref><ref>{{cite journal |author=Emesz M, Arlt EM, Krall EM, ''et al.'' |title=[Granular cell tumors of the orbit : Diagnostics and therapeutic aspects exemplified by a case report.] |journal=Ophthalmologe |volume= |issue= |pages= |year=2013 |month=November |pmid=24173669 |doi=10.1007/s00347-013-2972-1 |url=}}</ref>
*#[[Fungi|Fungal infections]].
*Followup.<ref name=pmid24652564>{{cite journal |author=Paksoy M, Eken M, Ayduran E, Altin G |title=Two cases of granular cell tumors of the head and neck at different sites |journal=Ear Nose Throat J |volume=93 |issue=3 |pages=E15–7 |year=2014 |month=March |pmid=24652564 |doi= |url=}}</ref>
*#[[Inflammatory papillary hyperplasia]].
*#Granular cell tumour.
*#Adjacent to an [[ulcer]].


===Sites===
===Sites===
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*Granular cell tumour.
*Granular cell tumour.
*[[Lipoma]].
*[[Lipoma]].
*Xanthoma.
*[[Xanthoma]].


==Microscopic==
==Microscopic==
Features:
Features:
*Cells with abundant eosinophilic granular cytoplasm - '''key feature'''.
*Cells eosinophilic or grey granular cytoplasm - '''key feature'''.
**Cytoplasm usually abundant.
**Granules (represent abundant lysosomes<ref name=pmid10410172/>):  
**Granules (represent abundant lysosomes<ref name=pmid10410172/>):  
***Size: 1-3 micrometers.
***Size: 1-3 micrometers.
***Poorly demarcated (on light microscopy).
***Poorly demarcated (on light microscopy).
*Nested architecture.
**Poorly defined cellular borders.
*Nested architecture or syncytial growth pattern.
*+/-Pustulo-ovoid bodies (focal eosinophilic granules surrounded by a clear halo).<ref name=pmid22760808>{{Cite journal  | last1 = Fernandes | first1 = BF. | last2 = Belfort Neto | first2 = R. | last3 = Odashiro | first3 = AN. | last4 = Pereira | first4 = PR. | last5 = Burnier | first5 = MN. | title = Clinical and histopathological features of orbital granular cell tumor: case report. | journal = Arq Bras Oftalmol | volume = 75 | issue = 2 | pages = 137-9 | month =  | year =  | doi =  | PMID = 22760808 }}</ref>
*+/-Pseudoepitheliomatous hyperplasia.
*+/-Pseudoepitheliomatous hyperplasia.
**May mimic [[SCC]].
**May mimic [[SCC]].
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*[[Squamous cell carcinoma]].
*[[Squamous cell carcinoma]].
*[[Oncocytoma]].
*[[Oncocytoma]].
*[[Melanocytic nevus]] with neurotization.
*[[Xanthoma]].
===Malignant features===
Features of malignancy (3 of 6 required):<ref name=pmid9669341>{{cite journal |author=Fanburg-Smith JC, Meis-Kindblom JM, Fante R, Kindblom LG |title=Malignant granular cell tumor of soft tissue: diagnostic criteria and clinicopathologic correlation |journal=Am. J. Surg. Pathol. |volume=22 |issue=7 |pages=779–94 |year=1998 |month=July |pmid=9669341 |doi= |url=}}</ref>
*[[Necrosis]].
*Spindled tumour cells.
*Vesciular nuclei with large [[nucleoli]].
*Mitoses (>2/10 [[HPF]] at 200x).
*High [[NC ratio]].
*Pleomorphism.
Predictive of poor outcome:<ref name=pmid9669341/>
*Ki-67 >10%.


==Special stains==
==Special stains==
*PAS +ve.
*PAS +ve.
*PASD +ve (pustulo-ovoid bodies).


==IHC==
==IHC==
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*Vimentin +ve (membranous).
*Vimentin +ve (membranous).
*Calretinin +ve (90-95%).<ref name=pmid12579997>{{Cite journal  | last1 = Fine | first1 = SW. | last2 = Li | first2 = M. | title = Expression of calretinin and the alpha-subunit of inhibin in granular cell tumors. | journal = Am J Clin Pathol | volume = 119 | issue = 2 | pages = 259-64 | month = Feb | year = 2003 | doi = 10.1309/GRH4-JWX6-J9J7-QQTA | PMID = 12579997 }}</ref>
*Calretinin +ve (90-95%).<ref name=pmid12579997>{{Cite journal  | last1 = Fine | first1 = SW. | last2 = Li | first2 = M. | title = Expression of calretinin and the alpha-subunit of inhibin in granular cell tumors. | journal = Am J Clin Pathol | volume = 119 | issue = 2 | pages = 259-64 | month = Feb | year = 2003 | doi = 10.1309/GRH4-JWX6-J9J7-QQTA | PMID = 12579997 }}</ref>
*SOX10 +ve (11 of 11 cases).<ref name=pmid37026754>{{cite journal |authors=Malik F, Bernieh A, Saad AG |title=Esophageal Granular Cell Tumor in Children: A Clinicopathologic Study of 11 Cases and Review of the Literature |journal=Am J Clin Pathol |volume=160 |issue=1 |pages=106–112 |date=July 2023 |pmid=37026754 |doi=10.1093/ajcp/aqad025 |url=}}</ref>


===Images===
===Images===
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Images:
Images:
*[http://pathhsw5m54.ucsf.edu/cts/grancell.html Granular cell tumour - several LM and EM images (ucsf.edu)].
*[http://pathhsw5m54.ucsf.edu/cts/grancell.html Granular cell tumour - several LM and EM images (ucsf.edu)].
==Sign out==
<pre>
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.
</pre>
===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==
==See also==
Michael
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