Difference between revisions of "Clear cell sugar tumour of the lung"

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==Molecular==
==Molecular==
*TFE3 gene fusions - subset of cases.<ref name=pmid20871214>{{cite journal |vauthors=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>
*TFE3 gene rearrangements - present in subset of cases.
**Typically younger patients.<ref name=pmid20871214>{{cite journal |vauthors=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==

Revision as of 15:22, 31 December 2020

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.
    • Typically younger patients.[3]
    • Epithelioid morphology.[3]
    • Minimal staining with muscle markers.[3]

See also

References

  1. ↑ 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. ↑ 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. ↑ 3.0 3.1 3.2 "A distinctive subset of PEComas harbors TFE3 gene fusions". Am J Surg Pathol 34 (10): 1395–406. October 2010. doi:10.1097/PAS.0b013e3181f17ac0. PMID 20871214.
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