The rhabdoid variant of adrenocortical carcinoma-Report of three cases and literature review

Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy. Extensive rhabdoid morphology in ACC has been described recently in very few cases. The proportion of rhabdoid morphology and the role of SMARCB1/ INI1 expression in these tumor cells to diagnose the specific variant is not describe...

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Main Authors: Pavithra Ayyanar, Mukund N. Sable, Amit K. Adhya, Manoj K. Das, Madhabananda Kar, Pritinanda Mishra
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2023-07-01
Series:Indian Journal of Pathology and Microbiology
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Online Access:https://journals.lww.com/10.4103/ijpm.ijpm_515_21
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author Pavithra Ayyanar
Mukund N. Sable
Amit K. Adhya
Manoj K. Das
Madhabananda Kar
Pritinanda Mishra
author_facet Pavithra Ayyanar
Mukund N. Sable
Amit K. Adhya
Manoj K. Das
Madhabananda Kar
Pritinanda Mishra
author_sort Pavithra Ayyanar
collection DOAJ
description Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy. Extensive rhabdoid morphology in ACC has been described recently in very few cases. The proportion of rhabdoid morphology and the role of SMARCB1/ INI1 expression in these tumor cells to diagnose the specific variant is not described in the literature. We reviewed the clinicopathological features of nine cases of adrenocortical neoplasm. Out of which, three cases of ACC showed predominant rhabdoid morphology. Large discohesive cells with abundant cytoplasm containing eosinophilic inclusions, eccentric vesicular nucleus, and prominent nucleoli. INI1 immunostain was retained in all cases. We reported the rhabdoid variant of ACC, a novel entity, and its diagnostic approach from their histological mimickers. Identifying more cases of this entity will help to clearly understand the pathogenesis, biologic behaviour, and any specific molecular alterations in the future.
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issn 0377-4929
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publishDate 2023-07-01
publisher Wolters Kluwer Medknow Publications
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series Indian Journal of Pathology and Microbiology
spelling doaj-art-e9f67fb00f5a4bfe84cd093fc5b4f7d42025-02-07T13:35:16ZengWolters Kluwer Medknow PublicationsIndian Journal of Pathology and Microbiology0377-49292023-07-0166362763110.4103/ijpm.ijpm_515_21The rhabdoid variant of adrenocortical carcinoma-Report of three cases and literature reviewPavithra AyyanarMukund N. SableAmit K. AdhyaManoj K. DasMadhabananda KarPritinanda MishraAdrenocortical carcinoma (ACC) is a rare and aggressive malignancy. Extensive rhabdoid morphology in ACC has been described recently in very few cases. The proportion of rhabdoid morphology and the role of SMARCB1/ INI1 expression in these tumor cells to diagnose the specific variant is not described in the literature. We reviewed the clinicopathological features of nine cases of adrenocortical neoplasm. Out of which, three cases of ACC showed predominant rhabdoid morphology. Large discohesive cells with abundant cytoplasm containing eosinophilic inclusions, eccentric vesicular nucleus, and prominent nucleoli. INI1 immunostain was retained in all cases. We reported the rhabdoid variant of ACC, a novel entity, and its diagnostic approach from their histological mimickers. Identifying more cases of this entity will help to clearly understand the pathogenesis, biologic behaviour, and any specific molecular alterations in the future.https://journals.lww.com/10.4103/ijpm.ijpm_515_21adrenocortical carcinomainii1rhabdoid variantsmarcb1
spellingShingle Pavithra Ayyanar
Mukund N. Sable
Amit K. Adhya
Manoj K. Das
Madhabananda Kar
Pritinanda Mishra
The rhabdoid variant of adrenocortical carcinoma-Report of three cases and literature review
Indian Journal of Pathology and Microbiology
adrenocortical carcinoma
inii1
rhabdoid variant
smarcb1
title The rhabdoid variant of adrenocortical carcinoma-Report of three cases and literature review
title_full The rhabdoid variant of adrenocortical carcinoma-Report of three cases and literature review
title_fullStr The rhabdoid variant of adrenocortical carcinoma-Report of three cases and literature review
title_full_unstemmed The rhabdoid variant of adrenocortical carcinoma-Report of three cases and literature review
title_short The rhabdoid variant of adrenocortical carcinoma-Report of three cases and literature review
title_sort rhabdoid variant of adrenocortical carcinoma report of three cases and literature review
topic adrenocortical carcinoma
inii1
rhabdoid variant
smarcb1
url https://journals.lww.com/10.4103/ijpm.ijpm_515_21
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