Malignant ectomesenchymoma in children: The European pediatric Soft tissue sarcoma Study Group experience
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.; Alaggio, Rita; Ferrari, Andrea
(2023) Pediatric Blood and Cancer, volume 70, issue 2, pp. 1 - 5
(Article)
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
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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.
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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
ISSN: 1545-5009
Publisher: Wiley-Liss Inc.
Note: Funding Information: Data management and statistical processing have been funded by Alice's Arc, children's cancer charity focusing on rhabdomyosarcoma, United Kingdom (alicesarc.org). Open access funding provided by BIBLIOSAN. Publisher Copyright: © 2022 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC. © 2022 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.
(Peer reviewed)
