Difference between revisions of "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.<ref>{{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>
{{ 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>
 
==General==
*Evolving entity.<ref name=pmid19065101>{{Cite journal  | last1 = Argani | first1 = P. | last2 = Aulmann | first2 = S. | last3 = Karanjawala | first3 = Z. | last4 = Fraser | first4 = RB. | last5 = Ladanyi | first5 = M. | last6 = Rodriguez | first6 = MM. | title = Melanotic Xp11 translocation renal cancers: a distinctive neoplasm with overlapping features of PEComa, carcinoma, and melanoma. | journal = Am J Surg Pathol | volume = 33 | issue = 4 | pages = 609-19 | month = Apr | year = 2009 | doi = 10.1097/PAS.0b013e31818fbdff | PMID = 19065101 }}</ref><ref name=pmid26274027/>
*Very rare.
 
==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===
<gallery>
Image: Melanotic Xp11.2 translocation renal cancer -- low mag.jpg | MXTRC - low mag.
Image: Melanotic Xp11.2 translocation renal cancer -- intermed mag.jpg | MXTRC - intermed. mag.
Image: Melanotic Xp11.2 translocation renal cancer -- high mag.jpg | MXTRC - high mag.
Image: Melanotic Xp11.2 translocation renal cancer -- very high mag.jpg | MXTRC - very high mag.
 
Image: Melanotic Xp11.2 translocation renal cancer - b -- low mag.jpg | MXTRC - low mag.
Image: Melanotic Xp11.2 translocation renal cancer - b -- intermed mag.jpg | MXTRC - intermed. mag.
Image: Melanotic Xp11.2 translocation renal cancer - b1 -- high mag.jpg | MXTRC - high mag.
Image: Melanotic Xp11.2 translocation renal cancer - b2 -- high mag.jpg | MXTRC - high mag.
</gallery>
 
==IHC==
*TFE3 +ve.
*Keratin -ve.
 
==Molecular==
*TFE3 rearrangement.


==See also==
==See also==

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

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

Microscopic

Features:

DDx:

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. ↑ 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.
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