Clear cell sugar tumour of the lung

From Libre Pathology
Jump to navigation Jump to search

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 "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.