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 w...
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Frontiers Media S.A.
2025-06-01
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| Series: | Frontiers in Pediatrics |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fped.2025.1489754/full |
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| author | M. S. Nadhirah M. S. Nadhirah A. Hamidah A. Hamidah A. A. Aishah Harizah A. A. Aishah Harizah R. M. Z. Reena R. M. Z. Reena T. Y. Kew T. Y. Kew M. B. Marina M. B. Marina |
| author_facet | M. S. Nadhirah M. S. Nadhirah A. Hamidah A. Hamidah A. A. Aishah Harizah A. A. Aishah Harizah R. M. Z. Reena R. M. Z. Reena T. Y. Kew T. Y. Kew M. B. Marina M. B. Marina |
| author_sort | M. S. Nadhirah |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-e6e45993e2394792ab4c704e26770420 |
| institution | DOAJ |
| issn | 2296-2360 |
| language | English |
| publishDate | 2025-06-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Pediatrics |
| spelling | doaj-art-e6e45993e2394792ab4c704e267704202025-08-20T03:10:04ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602025-06-011310.3389/fped.2025.14897541489754Case Report: First case of a pediatric laryngeal inflammatory rhabdomyoblastic tumor—diagnostic dilemma and insights into the conservative management of a residual lesionM. S. Nadhirah0M. S. Nadhirah1A. Hamidah2A. Hamidah3A. A. Aishah Harizah4A. A. Aishah Harizah5R. M. Z. Reena6R. M. Z. Reena7T. Y. Kew8T. Y. Kew9M. B. Marina10M. B. Marina11Department of Otorhinolaryngology—Head and Neck Surgery, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, MalaysiaDepartment of Otorhinolaryngology—Head and Neck Surgery, Hospital Canselor Tuanku Muhriz, Bandar Tun Razak, Cheras, Kuala Lumpur, MalaysiaDepartment of Otorhinolaryngology—Head and Neck Surgery, Hospital Canselor Tuanku Muhriz, Bandar Tun Razak, Cheras, Kuala Lumpur, MalaysiaDepartment of Paediatrics, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, MalaysiaDepartment of Otorhinolaryngology—Head and Neck Surgery, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, MalaysiaDepartment of Otorhinolaryngology—Head and Neck Surgery, Hospital Canselor Tuanku Muhriz, Bandar Tun Razak, Cheras, Kuala Lumpur, MalaysiaDepartment of Otorhinolaryngology—Head and Neck Surgery, Hospital Canselor Tuanku Muhriz, Bandar Tun Razak, Cheras, Kuala Lumpur, MalaysiaDepartment of Pathology, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, MalaysiaDepartment of Otorhinolaryngology—Head and Neck Surgery, Hospital Canselor Tuanku Muhriz, Bandar Tun Razak, Cheras, Kuala Lumpur, MalaysiaDepartment of Radiology, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, MalaysiaDepartment of Otorhinolaryngology—Head and Neck Surgery, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, MalaysiaDepartment of Otorhinolaryngology—Head and Neck Surgery, Hospital Canselor Tuanku Muhriz, Bandar Tun Razak, Cheras, Kuala Lumpur, MalaysiaAn 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.https://www.frontiersin.org/articles/10.3389/fped.2025.1489754/fullhistiocyte-rich rhabdomyoblastic tumorinflammatory leiomyosarcomainflammatory rhabdomyoblastic tumorrhabdomyosarcomalarynx |
| spellingShingle | M. S. Nadhirah M. S. Nadhirah A. Hamidah A. Hamidah A. A. Aishah Harizah A. A. Aishah Harizah R. M. Z. Reena R. M. Z. Reena T. Y. Kew T. Y. Kew M. B. Marina M. B. Marina Case Report: First case of a pediatric laryngeal inflammatory rhabdomyoblastic tumor—diagnostic dilemma and insights into the conservative management of a residual lesion Frontiers in Pediatrics histiocyte-rich rhabdomyoblastic tumor inflammatory leiomyosarcoma inflammatory rhabdomyoblastic tumor rhabdomyosarcoma larynx |
| title | Case Report: First case of a pediatric laryngeal inflammatory rhabdomyoblastic tumor—diagnostic dilemma and insights into the conservative management of a residual lesion |
| title_full | Case Report: First case of a pediatric laryngeal inflammatory rhabdomyoblastic tumor—diagnostic dilemma and insights into the conservative management of a residual lesion |
| title_fullStr | Case Report: First case of a pediatric laryngeal inflammatory rhabdomyoblastic tumor—diagnostic dilemma and insights into the conservative management of a residual lesion |
| title_full_unstemmed | Case Report: First case of a pediatric laryngeal inflammatory rhabdomyoblastic tumor—diagnostic dilemma and insights into the conservative management of a residual lesion |
| title_short | Case Report: First case of a pediatric laryngeal inflammatory rhabdomyoblastic tumor—diagnostic dilemma and insights into the conservative management of a residual lesion |
| title_sort | case report first case of a pediatric laryngeal inflammatory rhabdomyoblastic tumor diagnostic dilemma and insights into the conservative management of a residual lesion |
| topic | histiocyte-rich rhabdomyoblastic tumor inflammatory leiomyosarcoma inflammatory rhabdomyoblastic tumor rhabdomyosarcoma larynx |
| url | https://www.frontiersin.org/articles/10.3389/fped.2025.1489754/full |
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