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Extrarenal malignant rhabdoid tumour (view source)
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'''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]] | |||