Open AccessPrimitive myxoid mesenchymal tumor of infancy. Review of literature
Author(s) -
В. А. Фоченкова,
Г. Г. Радулеску,
Н. А. Рысев,
И. С. Клецкая,
Д. М. Коновалов,
М. Б. Белогурова
Publication year - 2021
Publication title -
rossijskij žurnal detskoj gematologii i onkologii
Language(s) - English
Resource type - Journals
eISSN - 2413-5496
pISSN - 2311-1267
DOI - 10.21682/2311-1267-2020-7-4-48-55
Subject(s) - mesenchymal stem cell , soft tissue , medicine , metastasis , mesenchymal tumor , pathology , sarcoma , cancer , immunohistochemistry
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.