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Difference between revisions of "Clear cell sugar tumour of the lung"
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Clear cell sugar tumour of the lung (view source)
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'''Clear cell sugar tumour of the lung''' is a very rare [[lung tumour]]. | '''Clear cell sugar tumour of the lung''' is a very rare benign [[lung tumour]]. | ||
==General== | ==General== | ||
*A [[PEComa]]. | *A [[PEComa]]. | ||
*Benign.<ref name=pmid19119463>{{Cite journal | last1 = Kim | first1 = WJ. | last2 = Kim | first2 = SR. | last3 = Choe | first3 = YH. | last4 = Lee | first4 = KY. | last5 = Park | first5 = SJ. | last6 = Lee | first6 = HB. | last7 = Chung | first7 = MJ. | last8 = Jin | first8 = GY. | last9 = Lee | first9 = YC. | title = Clear cell "sugar" tumor of the lung: a well-enhanced mass with an early washout pattern on dynamic contrast-enhanced computed tomography. | journal = J Korean Med Sci | volume = 23 | issue = 6 | pages = 1121-4 | month = Dec | year = 2008 | doi = 10.3346/jkms.2008.23.6.1121 | PMID = 19119463 | PMC = 2610653 | URL = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2610653/?tool=pubmed }}</ref> | *Benign.<ref name=pmid19119463>{{Cite journal | last1 = Kim | first1 = WJ. | last2 = Kim | first2 = SR. | last3 = Choe | first3 = YH. | last4 = Lee | first4 = KY. | last5 = Park | first5 = SJ. | last6 = Lee | first6 = HB. | last7 = Chung | first7 = MJ. | last8 = Jin | first8 = GY. | last9 = Lee | first9 = YC. | title = Clear cell "sugar" tumor of the lung: a well-enhanced mass with an early washout pattern on dynamic contrast-enhanced computed tomography. | journal = J Korean Med Sci | volume = 23 | issue = 6 | pages = 1121-4 | month = Dec | year = 2008 | doi = 10.3346/jkms.2008.23.6.1121 | PMID = 19119463 | PMC = 2610653 | URL = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2610653/?tool=pubmed }}</ref> | ||
*The ''sugar'' part in the name ''clear cell sugar tumour'' comes from the presence of glycogen; the tumour is [[PAS]] positive.<ref name=pmid29276673>{{cite journal |authors=Tsilimigras DI, Bakopoulos A, Ntanasis-Stathopoulos I, Gavriatopoulou M, Moris D, Karaolanis G, Spartalis E, Vagios S, Kalfa M, Salla C, Avgerinos DV |title=Clear cell "sugar tumor" of the lung: Diagnostic features of a rare pulmonary tumor |journal=Respir Med Case Rep |volume=23 |issue= |pages=52–54 |date=2018 |pmid=29276673 |pmc=5734796 |doi=10.1016/j.rmcr.2017.12.001 |url=}}</ref> | |||
==Microscopic== | ==Microscopic== | ||
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*Irregular epithelioid cells with: | *Irregular epithelioid cells with: | ||
**Focally clear cytoplasm. | **Focally clear cytoplasm. | ||
DDx: | |||
*[[Clear cell tumours]]. | |||
===Images=== | ===Images=== | ||
*[http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20080802170404452 Clear cell sugar tumour of the lung (surgicalpathologyatlas.com)]. | *[http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20080802170404452 Clear cell sugar tumour of the lung (surgicalpathologyatlas.com)]. | ||
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2610653/figure/F3/ CCST (nih.gov)].<ref name=pmid19119463/> | *[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2610653/figure/F3/ CCST (nih.gov)].<ref name=pmid19119463/> | ||
==stains== | |||
*[[PAS]] +ve.<ref name=pmid29276673>{{cite journal |authors=Tsilimigras DI, Bakopoulos A, Ntanasis-Stathopoulos I, Gavriatopoulou M, Moris D, Karaolanis G, Spartalis E, Vagios S, Kalfa M, Salla C, Avgerinos DV |title=Clear cell "sugar tumor" of the lung: Diagnostic features of a rare pulmonary tumor |journal=Respir Med Case Rep |volume=23 |issue= |pages=52–54 |date=2018 |pmid=29276673 |pmc=5734796 |doi=10.1016/j.rmcr.2017.12.001 |url=}}</ref> | |||
==IHC== | ==IHC== | ||
*HMB-45 +ve (nuclear & cytoplasmic). | *HMB-45 +ve (nuclear & cytoplasmic). | ||
==Molecular== | |||
*TFE3 gene rearrangements - present in subset of cases. | |||
**Typically younger patients.<ref name=pmid20871214>{{cite journal |authors=Argani P, Aulmann S, Illei PB, Netto GJ, Ro J, Cho HY, Dogan S, Ladanyi M, Martignoni G, Goldblum JR, Weiss SW |title=A distinctive subset of PEComas harbors TFE3 gene fusions |journal=Am J Surg Pathol |volume=34 |issue=10 |pages=1395–406 |date=October 2010 |pmid=20871214 |doi=10.1097/PAS.0b013e3181f17ac0 |url=}}</ref> | |||
**Epithelioid morphology.<ref name=pmid20871214/> | |||
**Minimal staining with muscle markers.<ref name=pmid20871214/> | |||
==See also== | ==See also== | ||