CASE REPORT article

Front. Pediatr.

Sec. Pediatric Oncology

Volume 13 - 2025 | doi: 10.3389/fped.2025.1489754

First Case of Pediatric Laryngeal Inflammatory Rhabdomyoblastic Tumor-Diagnostic Dilemma and Insights Into Conservative Management of Residual Lesion

Provisionally accepted
Nadhirah Mohd ShakriNadhirah Mohd Shakri1,2Hamidah AliasHamidah Alias2,3Aisyah Harizah Abdullah AlwiAisyah Harizah Abdullah Alwi1,2Reena Rahayu Md ZinReena Rahayu Md Zin2,4Thean Yew KewThean Yew Kew2,5Marina Mat BakiMarina Mat Baki1,2*
  • 1Deparment of Otorhinolaryngology-Head and Neck Surgery, Faculty of Medicine, National University of Malaysia, Kuala Lumpur, Malaysia
  • 2Hospital Canselor Tuanku Muhriz, Cheras, Kuala Lumpur, Kuala Lumpur, Malaysia
  • 3Department of Paediatrics, Faculty of Medicine, National University of Malaysia, Kuala Lumpur, Malaysia
  • 4Department of Pathology, Faculty of Medicine, National University of Malaysia, Kuala Lumpur, Malaysia
  • 5Department of Radiology, Faculty of Medicine, National University of Malaysia, Cheras, Kuala Lumpur, Malaysia

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

Keywords: Histiocyte-rich rhabdomyoblastic tumour, Inflammatory leiomyosarcoma, inflammatory rhabdomyoblastic tumour, Rhabdomyosarcoma, Larynx

Received: 04 Feb 2025; Accepted: 19 May 2025.

Copyright: © 2025 Mohd Shakri, Alias, Abdullah Alwi, Md Zin, Kew and Mat Baki. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Marina Mat Baki, Deparment of Otorhinolaryngology-Head and Neck Surgery, Faculty of Medicine, National University of Malaysia, Kuala Lumpur, Malaysia

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Supplementary Material