Case Report: First case of a pediatric laryngeal inflammatory rhabdomyoblastic tumor—diagnostic dilemma and insights into the conservative management of a residual lesion

An inflammatory rhabdomyoblastic tumor (IRMT), formerly known as a histiocyte-rich rhabdomyoblastic tumor (HR-RMT), has been identified as a skeletal muscle neoplasm exhibiting a mild clinical course. However, recent studies suggested that an HR-RMT has significant pathological and genetic overlap with inflammatory leiomyosarcoma, leading to the proposal of the newly defined entity, IRMT. We report the first case of an IRMT affecting the larynx in an adolescent girl who presented with a 3-month history of hoarseness. Endoscopic examination revealed a left paraglottic mass, which was surgically removed. No disease progression was observed over a 4-year follow-up. We discuss the diagnostic and management challenges, especially the role of adjuvant therapies in pediatric patients. This case highlights the importance of a precise diagnosis and careful management and emphasizes the need to increase awareness of IRMT.

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Journal
Frontiers in Pediatrics
Published
2025-06-11
DOI
https://doi.org/10.3389/fped.2025.1489754
Primary Topic
Tumors and Oncological Cases
Type
article
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article

Case Report: First case of a pediatric laryngeal inflammatory rhabdomyoblastic tumor—diagnostic dilemma and insights into the conservative management of a residual lesion

Rai Reena, Thean Yean Kew, Hamidah Alias, Aishah Harizah Abdullah Alwi et al.
Frontiers in Pediatrics
Tumors and Oncological Cases
article

Case Report: First case of a pediatric laryngeal inflammatory rhabdomyoblastic tumor—diagnostic dilemma and insights into the conservative management of a residual lesion

Rai Reena, Thean Yean Kew, Hamidah Alias, Aishah Harizah Abdullah Alwi, Marina Mat Baki, M. S. Nadhirah
article en

Abstract

An inflammatory rhabdomyoblastic tumor (IRMT), formerly known as a histiocyte-rich rhabdomyoblastic tumor (HR-RMT), has been identified as a skeletal muscle neoplasm exhibiting a mild clinical course. However, recent studies suggested that an HR-RMT has significant pathological and genetic overlap with inflammatory leiomyosarcoma, leading to the proposal of the newly defined entity, IRMT. We report the first case of an IRMT affecting the larynx in an adolescent girl who presented with a 3-month history of hoarseness. Endoscopic examination revealed a left paraglottic mass, which was surgically removed. No disease progression was observed over a 4-year follow-up. We discuss the diagnostic and management challenges, especially the role of adjuvant therapies in pediatric patients. This case highlights the importance of a precise diagnosis and careful management and emphasizes the need to increase awareness of IRMT.

Frontiers in PediatricsVol. 13
Hospital Kuala Lumpur (MY), University Kebangsaan Malaysia Medical Centre (MY), National University of Malaysia (MY)
Good health and well-being
Openalex Percentile: Top 81%
Tumors and Oncological Cases
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Case Report: First case of a pediatric laryngeal inflammatory rhabdomyoblastic tumor—diagnostic dilemma and insights into the conservative management of a residual lesion — Rai Reena, Thean Yean Kew, et al. · Frontiers in Pediatrics (2025) | TGRS Research Map | TGRS