Difference between revisions of "TFE3-rearranged renal cell carcinoma"

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#redirect [[Kidney_tumours#Renal_tumour_with_Xp11.2_translocation]]
'''Renal tumour with Xp11.2 translocation''' is an uncommon [[malignant]] [[kidney tumour]].
 
==General==
*Defined by the presence of a fusion gene formed with TFE3 @ Xp11.2.
*TFE3 is the gene involved in the translocation seen in [[alveolar soft part sarcoma]] (ASPS).
*Poor prognosis ~ 50% present at stage IV, majority of lymph node metastases.
*~1/3 of childhood RCC.<ref name=pmid17667536>{{Cite journal  | last1 = Argani | first1 = P. | last2 = Olgac | first2 = S. | last3 = Tickoo | first3 = SK. | last4 = Goldfischer | first4 = M. | last5 = Moch | first5 = H. | last6 = Chan | first6 = DY. | last7 = Eble | first7 = JN. | last8 = Bonsib | first8 = SM. | last9 = Jimeno | first9 = M. | title = Xp11 translocation renal cell carcinoma in adults: expanded clinical, pathologic, and genetic spectrum. | journal = Am J Surg Pathol | volume = 31 | issue = 8 | pages = 1149-60 | month = Aug | year = 2007 | doi = 10.1097/PAS.0b013e318031ffff | PMID = 17667536 }}</ref>
 
==Microscopic==
Features:<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref>
*Large cells.
*Clear or eosinophilic cytoplasm.
*Papillae or nests.
*[[Psammoma bodies]] - common.<ref name=pmid17102051>{{Cite journal  | last1 = Prasad | first1 = SR. | last2 = Humphrey | first2 = PA. | last3 = Catena | first3 = JR. | last4 = Narra | first4 = VR. | last5 = Srigley | first5 = JR. | last6 = Cortez | first6 = AD. | last7 = Dalrymple | first7 = NC. | last8 = Chintapalli | first8 = KN. | title = Common and uncommon histologic subtypes of renal cell carcinoma: imaging spectrum with pathologic correlation. | journal = Radiographics | volume = 26 | issue = 6 | pages = 1795-806; discussion 1806-10 | month =  | year =  | doi = 10.1148/rg.266065010 | PMID = 17102051 }}</ref>
**Calcification is considered the '''classic histomorphologic feature'''.
*[[Hyaline bodies]] - common.
 
Notes:
*Looks clear cell RCC ''or'' papillary RCC ''or'' a hybrid between the two.
*May resemble [[alveolar soft part sarcoma]].
 
DDx:
*[[Clear cell RCC]].
*[[Papillary RCC]].
*[[Epithelioid angiomyolipoma]].
*[[Clear cell papillary renal cell carcinoma]].
 
===Images===
<gallery>
Image:Xp11.2_translocation_renal_cell_carcinoma_-_intermed_mag.jpg | Xp11.2 translocation RCC - intermed. mag. (WC/Nephron)
Image:Xp11.2_translocation_renal_cell_carcinoma_-_high_mag.jpg | Xp11.2 translocation RCC - high mag. (WC/Nephron)
</gallery>
www:
*[http://www.flickr.com/photos/40764007@N08/7403237732/in/photostream/ Translocation carcinoma (flickr.com)].
 
==IHC==
*TFE3 +ve (nucleus) - '''key feature'''.<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref>
*CD10 +ve.
*Vimentin +ve.
*CK7 -ve.
**Positive in ~20% of cases.<ref name=pmid21804394>{{Cite journal  | last1 = He | first1 = H. | last2 = Zhou | first2 = GX. | last3 = Zhou | first3 = M. | last4 = Chen | first4 = L. | title = The distinction of clear cell carcinoma of the female genital tract, clear cell renal cell carcinoma, and translocation-associated renal cell carcinoma: an immunohistochemical study using tissue microarray. | journal = Int J Gynecol Pathol | volume = 30 | issue = 5 | pages = 425-30 | month = Sep | year = 2011 | doi = 10.1097/PGP.0b013e318214dd4f | PMID = 21804394 }}</ref>
 
Others:
*HMB-45 & Melan A -ve.
**Positive in ''epithelioid [[angiomyolipoma]]''.
 
==Molecular==
*[[Translocation]] involving TFE3, e.g. t(X;1)(p11.2;q21).<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref>
 
==See also==
*[[Kidney tumours]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]

Revision as of 16:16, 23 November 2013

Renal tumour with Xp11.2 translocation is an uncommon malignant kidney tumour.

General

  • Defined by the presence of a fusion gene formed with TFE3 @ Xp11.2.
  • TFE3 is the gene involved in the translocation seen in alveolar soft part sarcoma (ASPS).
  • Poor prognosis ~ 50% present at stage IV, majority of lymph node metastases.
  • ~1/3 of childhood RCC.[1]

Microscopic

Features:[2]

  • Large cells.
  • Clear or eosinophilic cytoplasm.
  • Papillae or nests.
  • Psammoma bodies - common.[3]
    • Calcification is considered the classic histomorphologic feature.
  • Hyaline bodies - common.

Notes:

DDx:

Images

  • Xp11.2 translocation RCC - intermed. mag. (WC/Nephron)

  • Xp11.2 translocation RCC - high mag. (WC/Nephron)

www:

IHC

  • TFE3 +ve (nucleus) - key feature.[2]
  • CD10 +ve.
  • Vimentin +ve.
  • CK7 -ve.
    • Positive in ~20% of cases.[4]

Others:

Molecular

See also

References

  1. ↑ Argani, P.; Olgac, S.; Tickoo, SK.; Goldfischer, M.; Moch, H.; Chan, DY.; Eble, JN.; Bonsib, SM. et al. (Aug 2007). "Xp11 translocation renal cell carcinoma in adults: expanded clinical, pathologic, and genetic spectrum.". Am J Surg Pathol 31 (8): 1149-60. doi:10.1097/PAS.0b013e318031ffff. PMID 17667536.
  2. ↑ 2.0 2.1 2.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 285. ISBN 978-0781765275.
  3. ↑ Prasad, SR.; Humphrey, PA.; Catena, JR.; Narra, VR.; Srigley, JR.; Cortez, AD.; Dalrymple, NC.; Chintapalli, KN.. "Common and uncommon histologic subtypes of renal cell carcinoma: imaging spectrum with pathologic correlation.". Radiographics 26 (6): 1795-806; discussion 1806-10. doi:10.1148/rg.266065010. PMID 17102051.
  4. ↑ He, H.; Zhou, GX.; Zhou, M.; Chen, L. (Sep 2011). "The distinction of clear cell carcinoma of the female genital tract, clear cell renal cell carcinoma, and translocation-associated renal cell carcinoma: an immunohistochemical study using tissue microarray.". Int J Gynecol Pathol 30 (5): 425-30. doi:10.1097/PGP.0b013e318214dd4f. PMID 21804394.
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