Primitive myxoid mesenchymal tumor of infancy. Review of literature-Reference-Cited by-同舟云学术

Primitive myxoid mesenchymal tumor of infancy. Review of literature

Published:2021-01-07 Issue:4 Volume:7 Page:48-55
ISSN:2413-5496
Container-title:Russian Journal of Pediatric Hematology and Oncology
language:
Short-container-title:Ross. ž. det. gematol. onkol.

Author:

Fochenkova V. A.¹^ORCID,Radulesku G. G.¹^ORCID,Rysev N. A.¹^ORCID,Kletskaya I. S.²^ORCID,Konovalov D. M.³^ORCID,Belogurova M. B.⁴^ORCID

Affiliation:

1. Saint Petersburg Clinical Scientific and Practical Center of Specialized Medical Assistance (Oncological)

2. Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia

3. Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia; Russian Medical Academy of Continuous Professional Education, Ministry of Health of Russia

4. Saint Petersburg Clinical Scientific and Practical Center of Specialized Medical Assistance (Oncological); Almazov National Medical Research Centre, Ministry of Health of Russia

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.

Publisher

OOO Grafika

Subject

Oncology,Hematology,Pediatrics, Perinatology and Child Health

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