Can we cure patients with abdominal Desmoplastic Small Round Cell Tumor? Results of a retrospective multicentric study on 100 patients.
Surg Oncol
; 29: 107-112, 2019 Jun.
Article
em En
| MEDLINE
| ID: mdl-31196472
ABSTRACT
BACKGROUND:
Despite being associated with a very poor prognosis, long-term survivors across all series of Desmoplastic Small Round Cell Tumor (DSRCT) have been reported. AIM OF THE STUDY To analyze patients 'characteristics associated with a prolonged survival after DSRCT diagnosis.METHODS:
All consecutive patients treated for DSRCT in nine French expert centers between 1991 and 2018 were retrospectively analyzed. Patients with a follow-up of less than 2 years were excluded and cure defined as being disease-free at least 5 years.RESULTS:
100â¯pts were identified (median age 25 years, 89% male). 27 had distant metastases at diagnosis and 80â¯pts underwent upfront chemotherapy (CT). 71â¯pts were operated, 20â¯pts without prior CT). Surgery was macroscopically complete (CC0/1) in 50â¯pts. Hyperthermic intraperitoneal Chemotherapy (HIPEC) was administered during surgery in 15â¯pts 54â¯pts had postoperative CT and 26â¯pts had postoperative whole abdomino-pelvic RT (WAP-RT). After a median follow-up of 103 months (range 23-311), the median overall survival (OS) was 25 months. The 1- year, 3-year and 5-year OS rates were 90%, 35% and 4% respectively. 5 patients were considered cured after a median disease-free interval of 100 months (range 22-139). Predictive factors of cure were female sex (HRâ¯=â¯0.49, pâ¯=â¯0.014), median PCI<12 (HRâ¯=â¯0.32, pâ¯=â¯0.0004), MD Anderson stage I (HRâ¯=â¯0.25, pâ¯<â¯0.0001), CC0/1 (HRâ¯=â¯0.34, pâ¯<â¯0.0001), and WAP-RT (HRâ¯=â¯0.36, pâ¯=â¯0.00013). HIPEC did not statistically improve survival.CONCLUSION:
Cure in DSRCT is possible in 5% of patients and is best achieved combining systemic chemotherapy, complete cytoreductive surgery and WAP-RT. Despite aggressive treatment, recurrence is common and targeted therapies are urgently needed.Palavras-chave
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MEDLINE
Assunto principal:
Neoplasias Peritoneais
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Quimioterapia do Câncer por Perfusão Regional
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Tumor Desmoplásico de Pequenas Células Redondas
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Procedimentos Cirúrgicos de Citorredução
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Hipertermia Induzida
Idioma:
En
Ano de publicação:
2019
Tipo de documento:
Article