Melanotic Xp11 translocation renal cancer

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Melanotic Xp11 translocation renal cancer
Diagnosis in short

Melanotic Xp11.2 translocation renal cancer. H&E stain.

LM melanin-like pigment, clear cells - epithelioid morphology - similar to Xp11.2 translocation carcinoma
LM DDx PEComa, malignant melanoma, Xp11.2 translocation carcinoma
IHC S-100 -ve, TFE3 +ve, keratin -ve
Molecular Xp11 translocation
Grossing notes partial nephrectomy grossing, Total nephrectomy for tumour grossing
Site kidney - see kidney tumours

Prevalence case reports
Prognosis unknown
Clin. DDx other renal tumours
Treatment excision

Melanotic Xp11 translocation renal cancer is a very rare kidney tumour that is evolving as an entity.[1]

General

  • Evolving entity.[2][1]
  • Very rare.

Microscopic

Features:

DDx:

Images

IHC

  • TFE3 +ve.
  • Keratin -ve.

Molecular

  • TFE3 rearrangement.

See also

References

  1. 1.0 1.1 Rao, Q.; Shen, Q.; Xia, QY.; Wang, ZY.; Liu, B.; Shi, SS.; Shi, QL.; Yin, HL. et al. (Sep 2015). "PSF/SFPQ is a very common gene fusion partner in TFE3 rearrangement-associated perivascular epithelioid cell tumors (PEComas) and melanotic Xp11 translocation renal cancers: clinicopathologic, immunohistochemical, and molecular characteristics suggesting classification as a distinct entity.". Am J Surg Pathol 39 (9): 1181-96. doi:10.1097/PAS.0000000000000502. PMID 26274027.
  2. Argani, P.; Aulmann, S.; Karanjawala, Z.; Fraser, RB.; Ladanyi, M.; Rodriguez, MM. (Apr 2009). "Melanotic Xp11 translocation renal cancers: a distinctive neoplasm with overlapping features of PEComa, carcinoma, and melanoma.". Am J Surg Pathol 33 (4): 609-19. doi:10.1097/PAS.0b013e31818fbdff. PMID 19065101.