Difference between revisions of "Extrarenal malignant rhabdoid tumour"

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#redirect [[Soft_tissue_lesions#Extrarenal_malignant_rhabdoid_tumour]]
'''Extrarenal malignant rhabdoid tumour''' is a rare malignant tumour typically seen in children.
 
It is essentially identical to the ''[[renal malignant rhabdoid tumour]]''.<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
 
==General==
*Usually children < 2 years old.
*Very poor prognosis ~ mean survival in one series 9 months.<ref name=pmid25085603>{{Cite journal  | last1 = Uwineza | first1 = A. | last2 = Gill | first2 = H. | last3 = Buckley | first3 = P. | last4 = Owens | first4 = C. | last5 = Capra | first5 = M. | last6 = O'Sullivan | first6 = C. | last7 = McDermott | first7 = M. | last8 = Brett | first8 = F. | last9 = Farrell | first9 = M. | title = Rhabdoid tumor: the Irish experience 1986-2013. | journal = Cancer Genet | volume = 207 | issue = 9 | pages = 398-402 | month = Sep | year = 2014 | doi = 10.1016/j.cancergen.2014.05.015 | PMID = 25085603 }}</ref>
*In the CNS it is known as ''[[atypical teratoid-rhabdoid tumour]]''.<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
 
==Microscopic==
Features:<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
*Variable architecture.
*Round cells.
*Eccentric vesicular nucleus.
*Prominent [[nucleolus]] -- '''key feature'''.
 
DDx:
*[[Desmoplastic small round cell tumour]].<ref name=pmid25133149>{{Cite journal  | last1 = Liang | first1 = L. | last2 = Tatevian | first2 = N. | last3 = Bhattacharjee | first3 = M. | last4 = Tsao | first4 = K. | last5 = Hicks | first5 = J. | title = Desmoplastic small round cell tumor with atypical immunohistochemical profile and rhabdoid-like differentiation. | journal = World J Clin Cases | volume = 2 | issue = 8 | pages = 367-72 | month = Aug | year = 2014 | doi = 10.12998/wjcc.v2.i8.367 | PMID = 25133149 }}</ref>
*Rhabdoid [[melanoma]].<ref name=pmid24551314>{{Cite journal  | last1 = Ishida | first1 = M. | last2 = Iwai | first2 = M. | last3 = Yoshida | first3 = K. | last4 = Kagotani | first4 = A. | last5 = Okabe | first5 = H. | title = Rhabdoid melanoma: a case report with review of the literature. | journal = Int J Clin Exp Pathol | volume = 7 | issue = 2 | pages = 840-3 | month =  | year = 2014 | doi =  | PMID = 24551314 }}</ref>
 
==IHC==
*INI1 ([[SMARCB1]]) -ve.
**[[AKA]] BAF47.
 
Others:<ref name=pmid25623976>{{Cite journal  | last1 = Wang | first1 = H. | last2 = Ma | first2 = Y. | last3 = Li | first3 = J. | last4 = Zhang | first4 = D. | last5 = Wu | first5 = B. | last6 = Fang | first6 = C. | last7 = Chen | first7 = L. | title = [Extrarenal malignant rhabdoid tumor of childhood: a clinicopathologic analysis of 8 cases]. | journal = Zhonghua Bing Li Xue Za Zhi | volume = 43 | issue = 12 | pages = 805-8 | month = Dec | year = 2014 | doi =  | PMID = 25623976 }}</ref>
*Vimentin +ve.
*EMA +ve.
*AE1/AE3 +ve.
*CAM5.2 +ve.
 
==See also==
*[[Soft tissue lesions]].
*[[Atypical teratoid/rhabdoid tumour]] (AT/RT).
*[[Renal malignant rhabdoid tumour]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Soft tissue lesions]]

Revision as of 02:56, 4 April 2015

Extrarenal malignant rhabdoid tumour is a rare malignant tumour typically seen in children.

It is essentially identical to the renal malignant rhabdoid tumour.[1]

General

Microscopic

Features:[1]

  • Variable architecture.
  • Round cells.
  • Eccentric vesicular nucleus.
  • Prominent nucleolus -- key feature.

DDx:

IHC

Others:[5]

  • Vimentin +ve.
  • EMA +ve.
  • AE1/AE3 +ve.
  • CAM5.2 +ve.

See also

References

  1. ↑ 1.0 1.1 1.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 627. ISBN 978-0781765275.
  2. ↑ Uwineza, A.; Gill, H.; Buckley, P.; Owens, C.; Capra, M.; O'Sullivan, C.; McDermott, M.; Brett, F. et al. (Sep 2014). "Rhabdoid tumor: the Irish experience 1986-2013.". Cancer Genet 207 (9): 398-402. doi:10.1016/j.cancergen.2014.05.015. PMID 25085603.
  3. ↑ Liang, L.; Tatevian, N.; Bhattacharjee, M.; Tsao, K.; Hicks, J. (Aug 2014). "Desmoplastic small round cell tumor with atypical immunohistochemical profile and rhabdoid-like differentiation.". World J Clin Cases 2 (8): 367-72. doi:10.12998/wjcc.v2.i8.367. PMID 25133149.
  4. ↑ Ishida, M.; Iwai, M.; Yoshida, K.; Kagotani, A.; Okabe, H. (2014). "Rhabdoid melanoma: a case report with review of the literature.". Int J Clin Exp Pathol 7 (2): 840-3. PMID 24551314.
  5. ↑ Wang, H.; Ma, Y.; Li, J.; Zhang, D.; Wu, B.; Fang, C.; Chen, L. (Dec 2014). "[Extrarenal malignant rhabdoid tumor of childhood: a clinicopathologic analysis of 8 cases].". Zhonghua Bing Li Xue Za Zhi 43 (12): 805-8. PMID 25623976.
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