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Fibrous hamartoma of infancy with sarcomatous transformation: an unusual morphology

BACKGROUND: Fibrous hamartoma of infancy (FHI) is a rare soft tissue lesion arising as a subcutaneous mass involving the axilla, trunk, and upper arm in infants and children <2yrs. Sarcomatous transformation in FHI is described in anecdotal cases in the literature. CASE REPORT: We describe one su...

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Autores principales: Kapatia, Gargi, Chatterjee, Debajyoti, Gupta, Kirti, Trehan, Amita
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hospital Universitário da Universidade de São Paulo 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9083879/
https://www.ncbi.nlm.nih.gov/pubmed/35574046
http://dx.doi.org/10.4322/acr.2021.380
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author Kapatia, Gargi
Chatterjee, Debajyoti
Gupta, Kirti
Trehan, Amita
author_facet Kapatia, Gargi
Chatterjee, Debajyoti
Gupta, Kirti
Trehan, Amita
author_sort Kapatia, Gargi
collection PubMed
description BACKGROUND: Fibrous hamartoma of infancy (FHI) is a rare soft tissue lesion arising as a subcutaneous mass involving the axilla, trunk, and upper arm in infants and children <2yrs. Sarcomatous transformation in FHI is described in anecdotal cases in the literature. CASE REPORT: We describe one such example arising as a mass in the lower back in a 3-month-old infant. On histology, the tumor contained classic triphasic morphology; however, brisk mitotic activity noted at multiple foci was diagnostically challenging to categorize. The tumor was evaluated for ETV6-NTRK3 fusion to exclude other common differentials. CONCLUSION: While FHI may be frequently encountered in infants, rare sarcomatous transformation are known to occur and merits special attention as it can be misdiagnosed. Also, a close follow-up is warranted as the lesion is known to recur locally.
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spelling pubmed-90838792022-05-14 Fibrous hamartoma of infancy with sarcomatous transformation: an unusual morphology Kapatia, Gargi Chatterjee, Debajyoti Gupta, Kirti Trehan, Amita Autops Case Rep Clinical Case Report BACKGROUND: Fibrous hamartoma of infancy (FHI) is a rare soft tissue lesion arising as a subcutaneous mass involving the axilla, trunk, and upper arm in infants and children <2yrs. Sarcomatous transformation in FHI is described in anecdotal cases in the literature. CASE REPORT: We describe one such example arising as a mass in the lower back in a 3-month-old infant. On histology, the tumor contained classic triphasic morphology; however, brisk mitotic activity noted at multiple foci was diagnostically challenging to categorize. The tumor was evaluated for ETV6-NTRK3 fusion to exclude other common differentials. CONCLUSION: While FHI may be frequently encountered in infants, rare sarcomatous transformation are known to occur and merits special attention as it can be misdiagnosed. Also, a close follow-up is warranted as the lesion is known to recur locally. Hospital Universitário da Universidade de São Paulo 2022-04-28 /pmc/articles/PMC9083879/ /pubmed/35574046 http://dx.doi.org/10.4322/acr.2021.380 Text en Copyright © 2022 The Authors. https://creativecommons.org/licenses/by/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Clinical Case Report
Kapatia, Gargi
Chatterjee, Debajyoti
Gupta, Kirti
Trehan, Amita
Fibrous hamartoma of infancy with sarcomatous transformation: an unusual morphology
title Fibrous hamartoma of infancy with sarcomatous transformation: an unusual morphology
title_full Fibrous hamartoma of infancy with sarcomatous transformation: an unusual morphology
title_fullStr Fibrous hamartoma of infancy with sarcomatous transformation: an unusual morphology
title_full_unstemmed Fibrous hamartoma of infancy with sarcomatous transformation: an unusual morphology
title_short Fibrous hamartoma of infancy with sarcomatous transformation: an unusual morphology
title_sort fibrous hamartoma of infancy with sarcomatous transformation: an unusual morphology
topic Clinical Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9083879/
https://www.ncbi.nlm.nih.gov/pubmed/35574046
http://dx.doi.org/10.4322/acr.2021.380
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