clinicaloncologyКлинический случай в онкологииClinical Case in Oncology3034-14773034-4018ОНКОПРАКТИК10.62546/3034-1477-2024-2-1-45-56clinicaloncology-24Research ArticleКЛИНИЧЕСКИЙ СЛУЧАЙCLINICAL CASEДесмопластическая мелкокруглоклеточная опухоль: клинический случай и обзор литературыDesmoplastic small round cell tumor: clinical case and literature reviewhttps://orcid.org/0009-0008-4582-4878НаталенкоК. Е.NatalenkoK. E.

Наталенко Кирилл Евгеньевич  — 3.1.6, врач-онколог отделения противоопухолевой лекарственной терапии химиотерапевтическое) №11

198255, Санкт-Петербург, пр. Ветеранов, д. 56

Natalenko Kirill Evgenyevich  — oncologist

St. Petersburg

natalenko.kirill@gmail.comКоркинаА. А.KorkinaA. A.

Коркина Алиса Андреевна — клинический ординатор кафедры онкологии

199106, Санкт-Петербург, 21-линия В.О., д. 8а

Korkina Alisa Аndreevna — resident

St. Petersburg

korkinaalisa@gmail.comСПб ГБУЗ «Городской клинический онкологический диспансер»РоссияCity Clinical Oncology DispensaryRussian FederationФГБОУ ВО «Санкт-Петербургский государственный университет»РоссияSt. Petersburg State UniversityRussian Federation202421062024214556Copyright © Наталенко К.Е., Коркина А.А., 20242024Наталенко К.Е., Коркина А.А.Natalenko K.E., Korkina A.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.oncocase.ru/jour/article/view/24

Десмопластическая мелкокруглоклеточная опухоль (ДМКО) — очень редкая и агрессивная злокачественная опухоль брюшной полости, впервые описанная в  1989  г. [1]. Заболеваемость ДМКО составляет примерно 0,2 случая на 1 000 000 населения [2]. ДМКО обычно встречается у подростков и молодых мужчин. ДМКО характеризуется высокой агрессивностью и, несмотря на  комплексное лечение, прогноз при данном заболевании остается крайне неблагоприятным. Медиана выживаемости без прогрессирования (ВБП) составляет от 4 до 21 месяца, общая выживаемость (ОВ) находится в пределах 17 до 60 месяцев, а порог 5-летней выживаемости преодолевают лишь 10–20% больных ДМКО [3]. В настоящий момент не существует протоколов лечения ДМКО в связи с редкостью нозологии. В данной статье мы представляем клинический случай лечения пациента с ДМКО с множественными метастазами по брюшине, получавшего лечение по схеме VAC-IE, с максимальным эффектом лечения в виде частичного регресса.

Desmoplastic small round cell tumour (DSRCT) is a very rare and aggressive malignant tumour first described in 1989 [1]. The incidence of DSRCT is approximately 0.2 cases per 1,000,000 population [2]. DSRCT usually occurs in adolescents and young males. DSRCT is characterised by high aggressiveness and despite comprehensive treatment, the prognosis in this disease remains extremely poor. Median progression-free survival (PFS) ranges from 4 to 21 months, overall survival (OS) ranges from 17 to 60 months, and only 10–20% of DSRCT patients cross the 5-year survival threshold [3]. Currently, there are no treatment protocols for DSRCT due to the rarity of the nosology. In this article, we present a clinical case of a patient with DSRCT with multiple peritoneal metastases treated with the VAC-IE regimen with a maximum treatment effect of partial regression.

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The authors declare that there are no conflicts of interest present.