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The '''granular cell tumour''' is a rare | {{ Infobox diagnosis | ||
| Name = {{PAGENAME}} | |||
| Image = Granular_cell_tumor_(3)_skin.jpg | |||
| Width = | |||
| Caption = Granular cell tumour. [[H&E stain]]. | |||
| Micro = cells (usually epithelioid) with (usually abundant) eosinophilic granular cytoplasm (granules ~ 1-3 micrometers, poorly demarcated on LM), +/-[[pseudoepitheliomatous hyperplasia]] | |||
| Subtypes = benign (common), malignant (uncommon) | |||
| LMDDx = [[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 | |||
| Molecular = | |||
| IF = | |||
| Gross = yellow nodule | |||
| Grossing = | |||
| Site = typically [[head and neck pathology|head and neck]] - other sites: [[breast]], [[skin]], tongue, [[esophagus]] + more | |||
| Assdx = | |||
| Syndromes = [[LEOPARD syndrome]] | |||
| Clinicalhx = | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = rare | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = usually good (benign) | |||
| Other = | |||
| 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 pathology|head and neck]]. | |||
==General== | ==General== | ||
*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> | |||
**PTPN11 = protein-tyrosine phosphatase non-receptor type 11.<ref>{{OMIM|176876}}</ref> | |||
***Gene implicated in ''[[Noonan syndrome]] 1''. | |||
*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> | |||
Treatment: | |||
*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> | |||
*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> | |||
===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== | ==Gross== | ||
*Yellow nodule. | *Yellow nodule. | ||
DDx of yellow nodule: | |||
*Granular cell tumour. | |||
*[[Lipoma]]. | |||
*[[Xanthoma]]. | |||
==Microscopic== | ==Microscopic== | ||
Features: | Features: | ||
* | *Cells eosinophilic or grey granular cytoplasm - '''key feature'''. | ||
**Granules 1-3 micrometers | **Cytoplasm usually abundant. | ||
**Granules (represent abundant lysosomes<ref name=pmid10410172/>): | |||
***Size: 1-3 micrometers. | |||
***Poorly demarcated (on light microscopy). | |||
**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. | |||
**May mimic [[SCC]]. | |||
DDx: | |||
*[[Squamous cell carcinoma]]. | |||
*[[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== | ||
| Line 24: | Line 99: | ||
*CD68 +ve (cytoplasmic). | *CD68 +ve (cytoplasmic). | ||
*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> | |||
===Images=== | |||
<gallery> | |||
Image:Granular_cell_tumor_(3)_skin.jpg | GCT. (WC) | |||
Image:Granular_cell_tumor_(4)_S-100.JPG | GCT - S-100. (WC) | |||
</gallery> | |||
==EM== | |||
*Abundant lysosomes.<ref name=pmid10410172>{{Cite journal | last1 = Ordóñez | first1 = NG. | title = Granular cell tumor: a review and update. | journal = Adv Anat Pathol | volume = 6 | issue = 4 | pages = 186-203 | month = Jul | year = 1999 | doi = | PMID = 10410172 }}</ref> | |||
**Round structures with variable (electron) density. | |||
Images: | Images: | ||
*[http:// | *[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== | ||
*[[Esophagus]]. | *[[Esophagus]]. | ||
*[[Head and neck pathology]]. | |||
==References== | ==References== | ||
{{Reflist| | {{Reflist|2}} | ||
[[Category:Weird stuff]] | [[Category:Weird stuff]] | ||
[[Category:Diagnosis]] | |||