Malignant ectomesenchymoma in children: The European pediatric Soft tissue sarcoma Study Group experience
Publication date
2023-02
Authors
Milano, Giuseppe Maria
Orbach, Daniel
Casanova, Michela
Berlanga, Pablo
Schoot, Reineke A
Corradini, Nadege
Brennan, Bernadette
Ramirez-Villar, Gema L.
Lyngsie Hjalgrim, Lisa
Van Noesel, Max M.
Editors
Advisors
Supervisors
Document Type
Article
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Abstract
Malignant ectomesenchymoma (MEM) is an extremely rare soft tissue tumor typical of young children, currently included in the category of skeletal muscle malignancies and characterized by a neuroblastic component. This study describes a series of 10 patients prospectively registered in the European paediatric Soft tissue sarcoma Study Group (EpSSG) database Of the 10 cases, seven had an initial local diagnosis of rhabdomyosarcoma. All patients received chemotherapy according to rhabdomyosarcoma strategy, four had radiotherapy. Overall, six patients were alive in first remission, two in second remission and one after second tumor. Only the patient with initially metastatic tumor died of disease.
Keywords
children, EpSSG, malignant ectomesenchymoma, outcome, treatment, Sarcoma/therapy, Humans, European People, Child, Preschool, Muscle Neoplasms, Rhabdomyosarcoma/therapy, Child, Soft Tissue Neoplasms/therapy, Hematology, Oncology, Pediatrics, Perinatology, and Child Health, Research Support, Non-U.S. Gov't, Journal Article
Citation
Milano, G M, Orbach, D, Casanova, M, Berlanga, P, Schoot, R A, Corradini, N, Brennan, B, Ramirez-Villar, G L, Lyngsie Hjalgrim, L, van Noesel, M M, Alaggio, R & Ferrari, A 2023, 'Malignant ectomesenchymoma in children : The European pediatric Soft tissue sarcoma Study Group experience', Pediatric Blood and Cancer, vol. 70, no. 2, e30116, pp. 1-5. https://doi.org/10.1002/pbc.30116