Clear cell sugar tumour of the lung

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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. //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. //www.ncbi.nlm.nih.gov/pmc/articles/PMC5734796/.
  3. 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.