Sarcomatoid variant of Xp11 translocation renal cell carcinoma: A rare case report in an adult female

Xp11 translocation renal cell carcinoma (XPTRCC) is a very rare kidney neoplasm, which has been predominantly reported in young patients. Sarcomatoid transformation in renal cell carcinomas is known. However, its occurrence in XPTRCC is unreported so far in the literature. We report a unique case of...

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Main Authors: Rakhi V. Jagdale, Supriya Jain, Jaydeep N. Pol, Makarand V. Khochikar
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2023-01-01
Series:Indian Journal of Pathology and Microbiology
Subjects:
Online Access:https://journals.lww.com/10.4103/ijpm.ijpm_1159_21
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author Rakhi V. Jagdale
Supriya Jain
Jaydeep N. Pol
Makarand V. Khochikar
author_facet Rakhi V. Jagdale
Supriya Jain
Jaydeep N. Pol
Makarand V. Khochikar
author_sort Rakhi V. Jagdale
collection DOAJ
description Xp11 translocation renal cell carcinoma (XPTRCC) is a very rare kidney neoplasm, which has been predominantly reported in young patients. Sarcomatoid transformation in renal cell carcinomas is known. However, its occurrence in XPTRCC is unreported so far in the literature. We report a unique case of sarcomatoid transformation in a XPTRCC in a 23-year-old female, who presented with a huge right-sided renal mass and had metastatic deposits in lungs. Morphologically, clear cell morphology with papillary architecture along with foci of sarcomatoid transformation and rhabdoid differentiation were noted. Immunohistochemistry showed Pax-8 and TFE-3 expression in all components including the sarcomatous areas, whereas CK and EMA were expressed in conventional clear cell component. We present an extremely rare case of sarcomatous transformation in XPTRCC and discuss the case as determined by histopathology and immunocytochemistry. To our knowledge, this is the first case of sarcomatoid transformation XPTRCC being reported in the world literature.
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spelling doaj.art-19510bdb28fd45a1aeea593a3756d1282025-01-22T15:13:55ZengWolters Kluwer Medknow PublicationsIndian Journal of Pathology and Microbiology0377-49290974-51302023-01-0167117818110.4103/ijpm.ijpm_1159_21Sarcomatoid variant of Xp11 translocation renal cell carcinoma: A rare case report in an adult femaleRakhi V. JagdaleSupriya JainJaydeep N. PolMakarand V. KhochikarXp11 translocation renal cell carcinoma (XPTRCC) is a very rare kidney neoplasm, which has been predominantly reported in young patients. Sarcomatoid transformation in renal cell carcinomas is known. However, its occurrence in XPTRCC is unreported so far in the literature. We report a unique case of sarcomatoid transformation in a XPTRCC in a 23-year-old female, who presented with a huge right-sided renal mass and had metastatic deposits in lungs. Morphologically, clear cell morphology with papillary architecture along with foci of sarcomatoid transformation and rhabdoid differentiation were noted. Immunohistochemistry showed Pax-8 and TFE-3 expression in all components including the sarcomatous areas, whereas CK and EMA were expressed in conventional clear cell component. We present an extremely rare case of sarcomatous transformation in XPTRCC and discuss the case as determined by histopathology and immunocytochemistry. To our knowledge, this is the first case of sarcomatoid transformation XPTRCC being reported in the world literature.https://journals.lww.com/10.4103/ijpm.ijpm_1159_21sarcomatoid transformationtfe-3xp11 translocation carcinoma
spellingShingle Rakhi V. Jagdale
Supriya Jain
Jaydeep N. Pol
Makarand V. Khochikar
Sarcomatoid variant of Xp11 translocation renal cell carcinoma: A rare case report in an adult female
Indian Journal of Pathology and Microbiology
sarcomatoid transformation
tfe-3
xp11 translocation carcinoma
title Sarcomatoid variant of Xp11 translocation renal cell carcinoma: A rare case report in an adult female
title_full Sarcomatoid variant of Xp11 translocation renal cell carcinoma: A rare case report in an adult female
title_fullStr Sarcomatoid variant of Xp11 translocation renal cell carcinoma: A rare case report in an adult female
title_full_unstemmed Sarcomatoid variant of Xp11 translocation renal cell carcinoma: A rare case report in an adult female
title_short Sarcomatoid variant of Xp11 translocation renal cell carcinoma: A rare case report in an adult female
title_sort sarcomatoid variant of xp11 translocation renal cell carcinoma a rare case report in an adult female
topic sarcomatoid transformation
tfe-3
xp11 translocation carcinoma
url https://journals.lww.com/10.4103/ijpm.ijpm_1159_21
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AT supriyajain sarcomatoidvariantofxp11translocationrenalcellcarcinomaararecasereportinanadultfemale
AT jaydeepnpol sarcomatoidvariantofxp11translocationrenalcellcarcinomaararecasereportinanadultfemale
AT makarandvkhochikar sarcomatoidvariantofxp11translocationrenalcellcarcinomaararecasereportinanadultfemale