Clinicopathological and treatment response characteristics of updated rhabdomyosarcoma histomolecular subtypes: An Asian population-based study.

Autor: How GY; Department of Paediatric Surgery, KK Women's and Children's Hospital, Singapore, Singapore., Kuick CH; Department of Pathology and Laboratory Medicine, KK Women's and Children's Hospital, Singapore, Singapore., Yong MH; Department of Pathology and Laboratory Medicine, KK Women's and Children's Hospital, Singapore, Singapore., Soh SY; KK Women's and Children's Hospital, VIVA-KKH Paediatric Brain and Solid Tumour Programme, Children's Blood and Cancer Centre, Singapore, Singapore.; Department of Paediatric Subspecialties Haematology/Oncology Service, KK Women's and Children's Hospital, Singapore, Singapore, Singapore.; Duke NUS Medical School, Duke NUS Medical School, Singapore, Singapore., Hee EX; KK Women's and Children's Hospital, VIVA-KKH Paediatric Brain and Solid Tumour Programme, Children's Blood and Cancer Centre, Singapore, Singapore., Wong MK; KK Women's and Children's Hospital, VIVA-KKH Paediatric Brain and Solid Tumour Programme, Children's Blood and Cancer Centre, Singapore, Singapore., Quek R; Duke NUS Medical School, Duke NUS Medical School, Singapore, Singapore.; National Cancer Centre Singapore, Division of Medical Oncology, Singapore, Singapore., Harunal MF; Duke NUS Medical School, Duke NUS Medical School, Singapore, Singapore.; National Cancer Centre Singapore, Division of Medical Oncology, Singapore, Singapore., Selvarajan S; Duke NUS Medical School, Duke NUS Medical School, Singapore, Singapore.; Department of Anatomical Pathology, Singapore General Hospital, Singapore, Singapore., Sittampalam K; Duke NUS Medical School, Duke NUS Medical School, Singapore, Singapore.; Department of Anatomical Pathology, Singapore General Hospital, Singapore, Singapore., Dhamne CA; National University of Singapore, Division of Paediatric Haematology and Oncology, Singapore, Singapore., Lee V; Department of Pathology, National University of Singapore, Singapore, Singapore., Chang KT; Department of Pathology and Laboratory Medicine, KK Women's and Children's Hospital, Singapore, Singapore.; KK Women's and Children's Hospital, VIVA-KKH Paediatric Brain and Solid Tumour Programme, Children's Blood and Cancer Centre, Singapore, Singapore.; Duke NUS Medical School, Duke NUS Medical School, Singapore, Singapore., Loh AH; Department of Paediatric Surgery, KK Women's and Children's Hospital, Singapore, Singapore.; KK Women's and Children's Hospital, VIVA-KKH Paediatric Brain and Solid Tumour Programme, Children's Blood and Cancer Centre, Singapore, Singapore.; Duke NUS Medical School, Duke NUS Medical School, Singapore, Singapore.
Jazyk: angličtina
Zdroj: Asia-Pacific journal of clinical oncology [Asia Pac J Clin Oncol] 2023 Jul 11. Date of Electronic Publication: 2023 Jul 11.
DOI: 10.1111/ajco.13975
Abstrakt: Aim: New histomolecular subtypes of rhabdomyosarcoma have recently been defined but their corresponding clinical characteristics are not well described. Also, these clinical phenotypes vary greatly by age and ethnicity but have not been profiled in Asian populations. Thus, we sought to determine the landscape of rhabdomyosarcoma subtypes in a national Asian cohort and compare clinical characteristics among age groups and molecular subtypes.
Methods: We performed a retrospective population-based study of all rhabdomyosarcoma patients in Singapore public hospitals from 2004 to 2014 (n = 67), and assigned histomolecular subtypes according to the updated 2020 WHO classification of soft tissue tumors following central pathology review and molecular profiling.
Results: Age-specific prevalence followed a tri-modal peak. There were significantly more embryonal and alveolar (p = 0.032) and genitourinary (non-bladder/prostate) tumors (p = 0.033) among children. Older age was associated with complete resection among spindle cell/sclerosing tumors (p = 0.027), with the omission of chemotherapy among embryonal tumors (p = 0.001), and with poorer survival among embryonal and alveolar tumors (p = 0.026, p = 0.022, respectively). Overall survival differed with stage, group, and surgical resection, adjusted for age group (p = 0.004, p = 0.001, p = 0.004, respectively). Spindle-cell/sclerosing tumors showed an indolent phenotype with a significantly lower incidence of nodal metastasis (p = 0.002), but two of 15 patients with MYOD1 mutations had a contrastingly aggressive disease.
Conclusion: Disease and treatment response profiles of rhabdomyosarcoma subtypes vary significantly between adults and children, especially surgical resectability. In our Asian population, poorer outcomes were observed in adults with embryonal and alveolar tumors, while activating mutations influence the behavior of otherwise favorable spindle cell/sclerosing tumors.
(© 2023 The Authors. Asia-Pacific Journal of Clinical Oncology published by John Wiley & Sons Australia, Ltd.)
Databáze: MEDLINE
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