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Türkiye Klinikleri Journal of Case Reports, vol.30, no.1, pp.62-66, 2022 (Peer-Reviewed Journal)
Malignant ectomesenchymoma is a rare pediatric soft tissue tumor that contains both neuroectodermal and mesenchymal components. It is frequently found in the pelvis, paratesticular soft tissue, external genitalia and the head-neck region. We present a 12-month-old boy
with a recurrent pelvic tumor excised 5 months ago and diagnosed as neuroblastoma. Histological consultation of the initial tumor tissue revealed
a biphasic pattern with rhabdomyosarcomatous and neural components. The mesenchymal component was embryonal rhabdomyosarcoma showing desmin, myogenin, and Myo-D1 positivity. The neuroectodermal component was mainly Schwannian stroma stained with S100 antibody and
scattered synaptophysin and chromogranin positive ganglion cells were also present. The patient was diagnosed with malignant ectomesenchymoma. A chemotherapy regime was started and surgical approach to the recurrent tumor was planned. We present here a pediatric case of malignant ectomesenchymoma because of its rarity and it is important that this entity should be considered in the differential diagnosis for appropriate
clinical management.