Abstract
Primitive myxoid mesenchymal tumor of infancy (PMMTI) is an aggressive tumor with a high rate of local recurrence, rare metastasis and poor response to chemotherapy.PMMTI is a rare soft-tissue sarcoma that preferentially affects infants. It is isolated from the group of infantile fibrosarcomas or myofibroblastic tumors. PMMTI differs from other tumors in the clinical course, morphological features and primarily molecular genetic changes. ETV6-NTRK3 gene fusion is characteristic of infantile fibrosarcomas. However, it is not detected in patients with PMMTI. BCOR ITD had been described in patients with PMMTI and it allowed to define this tumor from the group of infantile fibrosarcomas.Unified treatment approaches have not been identified for this tumor yet. Radical surgical removal is preferred treatment. BCOR ITD is a target. Nowadays search for targeted drugs is ongoing and it could help to solve the problem of PMMTI treatment.
Highlights
Primitive myxoid mesenchymal tumor of infancy (PMMTI) is an aggressive tumor with a high rate of local recurrence, rare metastasis and poor response to chemotherapy
PMMTI is a rare soft-tissue sarcoma that preferentially affects infants. It is isolated from the group of infantile fibrosarcomas or myofibroblastic tumors
PMMTI differs from other tumors in the clinical course, morphological features and primarily molecular genetic changes
Summary
Ключевые слова: дети, примитивная миксоидная мезенхимальная опухоль младенцев, мягкотканные саркомы, BCOR, детская онкология. Для цитирования: Фоченкова В.А., Радулеску Г.Г., Рысев Н.А., Клецкая И.С., Коновалов Д.М., Белогурова М.Б. PMMTI is a rare soft-tissue sarcoma that preferentially affects infants It is isolated from the group of infantile fibrosarcomas or myofibroblastic tumors. BCOR ITD had been described in patients with PMMTI and it allowed to define this tumor from the group of infantile fibrosarcomas. Фоченкова: анализ научного материала, обзор публикаций по теме статьи, разработка дизайна статьи, подготовка списка литературы, написание текста рукописи, составление резюме Г.Г. Белогурова: анализ научного материала, разработка дизайна статьи, написание текста рукописи, научное редактирование статьи. В журнале “The American Journal of Surgical Pathology” была опубликована работа группы ученых, в которой при молекулярно-генетическом исследовании ПММОМ обнаружена мутация BCOR-ITD
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