Milano GM, Orbach D, Casanova M, Berlanga P, Schoot R, Corradini N, Brennan B, Ramirez-Villar G, Lyngsie Hjalgrim L, van Noesel M, Alaggio R, Ferrari A
Pediatr Blood Cancer 2023 Feb;70(2):e30116. doi: 10.1002/pbc.30116. Epub 2022 Nov 28
This is a short report on 10 cases of malignant ectomesenchymoma (MEM) enrolled in the EpSSG NRSTS 2005 protocol (0.8% of the cases). MEM is an extremely rare soft tissue tumor typical of young children (often arising at genito-urinary sites), currently included in the category of skeletal muscle malignancies and characterized by a neuroblastic component. Of the 10 cases, 7 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.
More in-depth molecular investigations are needed to better study MEM tumorogenesis and its biphenotypic differentiation. Meanwhile, EpSSG proposes to treat these patients according to the RMS therapeutic strategy.
Keywords: EpSSG; children; malignant ectomesenchymoma; outcome; treatment.
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.
More in-depth molecular investigations are needed to better study MEM tumorogenesis and its biphenotypic differentiation. Meanwhile, EpSSG proposes to treat these patients according to the RMS therapeutic strategy.
Keywords: EpSSG; children; malignant ectomesenchymoma; outcome; treatment.
