Difference between revisions of "Clear cell sugar tumour of the lung"
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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== | ||
Latest revision as of 17:21, 25 March 2021
Clear cell sugar tumour of the lung is a very rare benign lung tumour.
General
- A PEComa.
- Benign.[1]
- The sugar part in the name clear cell sugar tumour comes from the presence of glycogen; the tumour is PAS positive.[2]
Microscopic
Features:[1]
- Sheets or trabeculae.
- Irregular epithelioid cells with:
- Focally clear cytoplasm.
DDx:
Images
stains
IHC
- HMB-45 +ve (nuclear & cytoplasmic).
Molecular
- TFE3 gene rearrangements - present in subset of cases.
See also
References
- ↑ 1.0 1.1 1.2 Kim, WJ.; Kim, SR.; Choe, YH.; Lee, KY.; Park, SJ.; Lee, HB.; Chung, MJ.; Jin, GY. et al. (Dec 2008). "Clear cell "sugar" tumor of the lung: a well-enhanced mass with an early washout pattern on dynamic contrast-enhanced computed tomography.". J Korean Med Sci 23 (6): 1121-4. doi:10.3346/jkms.2008.23.6.1121. PMC 2610653. PMID 19119463. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2610653/.
- ↑ 2.0 2.1 Tsilimigras DI, Bakopoulos A, Ntanasis-Stathopoulos I, Gavriatopoulou M, Moris D, Karaolanis G, Spartalis E, Vagios S, Kalfa M, Salla C, Avgerinos DV (2018). "Clear cell "sugar tumor" of the lung: Diagnostic features of a rare pulmonary tumor". Respir Med Case Rep 23: 52–54. doi:10.1016/j.rmcr.2017.12.001. PMC 5734796. PMID 29276673. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5734796/.
- ↑ 3.0 3.1 3.2 Argani P, Aulmann S, Illei PB, Netto GJ, Ro J, Cho HY, Dogan S, Ladanyi M, Martignoni G, Goldblum JR, Weiss SW (October 2010). "A distinctive subset of PEComas harbors TFE3 gene fusions". Am J Surg Pathol 34 (10): 1395–406. doi:10.1097/PAS.0b013e3181f17ac0. PMID 20871214.