Difference between revisions of "Melanotic Xp11 translocation renal cancer"
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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Melanotic_Xp11.2_translocation_renal_cancer_-_b1_--_high_mag.jpg | |||
| Width = | |||
| Caption = Melanotic Xp11.2 translocation renal cancer. [[H&E stain]]. | |||
| Synonyms = | |||
| Micro = melanin-like pigment, clear cells - epithelioid morphology - similar to [[Xp11.2 translocation carcinoma]] | |||
| Subtypes = | |||
| LMDDx = [[PEComa]], [[malignant melanoma]], [[Xp11.2 translocation carcinoma]] | |||
| Stains = | |||
| IHC = S-100 -ve, TFE3 +ve, keratin -ve | |||
| EM = | |||
| Molecular = Xp11 translocation | |||
| IF = | |||
| Gross = | |||
| Grossing = [[partial nephrectomy grossing]], [[Total nephrectomy for tumour grossing]] | |||
| Staging = | |||
| Site = [[kidney]] - see ''[[kidney tumours]]'' | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = case reports | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = unknown | |||
| Other = | |||
| ClinDDx = other renal tumours | |||
| Tx = excision | |||
}} | |||
'''Melanotic Xp11 translocation renal cancer''' is a very rare [[kidney tumour]] that is evolving as an entity.<ref name=pmid26274027>{{Cite journal | last1 = Rao | first1 = Q. | last2 = Shen | first2 = Q. | last3 = Xia | first3 = QY. | last4 = Wang | first4 = ZY. | last5 = Liu | first5 = B. | last6 = Shi | first6 = SS. | last7 = Shi | first7 = QL. | last8 = Yin | first8 = HL. | last9 = Wu | first9 = B. | title = 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. | journal = Am J Surg Pathol | volume = 39 | issue = 9 | pages = 1181-96 | month = Sep | year = 2015 | doi = 10.1097/PAS.0000000000000502 | PMID = 26274027 }}</ref> | '''Melanotic Xp11 translocation renal cancer''' is a very rare [[kidney tumour]] that is evolving as an entity.<ref name=pmid26274027>{{Cite journal | last1 = Rao | first1 = Q. | last2 = Shen | first2 = Q. | last3 = Xia | first3 = QY. | last4 = Wang | first4 = ZY. | last5 = Liu | first5 = B. | last6 = Shi | first6 = SS. | last7 = Shi | first7 = QL. | last8 = Yin | first8 = HL. | last9 = Wu | first9 = B. | title = 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. | journal = Am J Surg Pathol | volume = 39 | issue = 9 | pages = 1181-96 | month = Sep | year = 2015 | doi = 10.1097/PAS.0000000000000502 | PMID = 26274027 }}</ref> | ||
Latest revision as of 05:16, 2 January 2018
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
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
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.