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Open Access Green as soon as Postprint is submitted to ZB.
Malignant ectomesenchymoma in children and adolescents: Report from the Cooperative Weichteilsarkom Studiengruppe (CWS).
Pediatr. Blood Cancer 60, 224-229 (2013)
BACKGROUND: Malignant ectomesenchymoma (MEM) is a soft tissue tumor with heterologous rhabdomyoblastic components believed to arise from pluripotent migratory neural crest cells. To date merely 50 cases have been published and the knowledge about the course of disease and optimal treatment is limited. METHODS: Six patients with MEM were registered 1996-2009. The diagnosis was confirmed according to current criteria. Their treatment and outcome was analyzed. RESULTS: The median age of the three females and three males was 0.6 years (range, 0.2-13.5). The mesenchymal component in all tumors was rhabdomyosarcoma (RMS), the neural component ganglioneuroblastoma/neuroblastoma (n = 5) and peripheral primitive neuroectodermal tumor in one case. Five patients presented with localized, one with metastatic disease. All but one patient received multiagent chemotherapy during their initial treatment. The tumors of 4/5 patients with localized MEM were at least grossly resected at best surgery; the patient without gross resection was additionally irradiated. Three of four evaluable tumors responded well to induction chemotherapy. All patients achieved a first complete remission (CR), but three recurrences (two local, one systemic) occurred. The individual with metastatic MEM did not survive, but all five patients with localized MEM are currently alive in CR with a median follow-up of 5 years (range: 2.1-13.7). CONCLUSIONS: Risk-factors and outcome of MEM appear to be comparable with other highly malignant pediatric soft tissue sarcoma when a multimodal treatment strategy including chemotherapy and adequate local treatment is pursued. We propose that treatment of patients with MEM be done according to pediatric protocols similar to other rhabdomyosarcoma-like soft tissue sarcoma.
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Publication type
Article: Journal article
Document type
Scientific Article
Keywords
ectomesenchymoma; rhabdomyosarcoma; soft tissue sarcoma
Language
english
Publication Year
2013
Prepublished in Year
2012
HGF-reported in Year
2012
ISSN (print) / ISBN
1545-5009
e-ISSN
1096-911X
Journal
Pediatric Blood and Cancer
Quellenangaben
Volume: 60,
Issue: 2,
Pages: 224-229
Publisher
Wiley
Reviewing status
Peer reviewed
Institute(s)
CCG Osteosarcoma (PATH-KOS)
POF-Topic(s)
30504 - Mechanisms of Genetic and Environmental Influences on Health and Disease
Research field(s)
Enabling and Novel Technologies
PSP Element(s)
G-520800-001
PubMed ID
22535600
Scopus ID
84871130340
Erfassungsdatum
2012-11-23