Melanotic Xp11 translocation renal cancer
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Melanotic Xp11 translocation renal cancer is a very rare kidney tumour that is evolving as an entity.[1]
| Melanotic Xp11 translocation renal cancer | |
|---|---|
| Diagnosis in short | |
| 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 |
General
Microscopic
Features:
- Melanin-like pigment.
- Clear cells, epithelioid morphology - similar to Xp11.2 translocation carcinoma.
DDx:
- PEComa - may have TFE3 rearrangement.
- Malignant melanoma.
- Xp11.2 translocation carcinoma.
Images
MXTRC - low mag.
MXTRC - intermed. mag.
MXTRC - high mag.
MXTRC - very high mag.
MXTRC - low mag.
MXTRC - intermed. mag.
MXTRC - high mag.
MXTRC - high mag.
IHC
- TFE3 +ve.
- Keratin -ve.
Molecular
- TFE3 rearrangement.
See also
References
- ↑ 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.
- ↑ 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.