Exploring heterogeneity of adrenal cortical tumors in children: The French pediatric rare tumor group (Fracture) experience.
Adolescent
Adrenal Cortex Neoplasms
/ classification
Antineoplastic Combined Chemotherapy Protocols
/ therapeutic use
Child
Child, Preschool
Combined Modality Therapy
Drug Resistance, Neoplasm
Female
Follow-Up Studies
Humans
Infant
Male
Neoplasm Recurrence, Local
/ pathology
Neoplasm, Residual
/ pathology
Prognosis
Retrospective Studies
Salvage Therapy
Survival Rate
adrenal cortical tumors
children
risk stratification
second-line therapy
tumor rupture
Journal
Pediatric blood & cancer
ISSN: 1545-5017
Titre abrégé: Pediatr Blood Cancer
Pays: United States
ID NLM: 101186624
Informations de publication
Date de publication:
02 2020
02 2020
Historique:
received:
24
07
2019
revised:
25
09
2019
accepted:
29
10
2019
pubmed:
19
11
2019
medline:
23
4
2020
entrez:
19
11
2019
Statut:
ppublish
Résumé
Pediatric adrenal cortical tumors are characterized by a wide spectrum of behavior. Questions remain regarding intermediate disease stages with isolated tumor rupture or relapse. To describe clinical characteristics, treatment strategy, and outcome of patients depending on disease stage, tumor rupture, or in case of a refractory tumor, to discuss optimal management. Pediatric patients with histological material reviewed and treated between 2000 and 2018 in 23 French oncology centers were included. Among 95 cases, 59% of patients had stage I tumors (n = 55), 16% had stage II tumors (n = 16), 19% had stage III tumors (n = 17), and 5% had stage IV tumors (n = 5) (missing data: 2). Overall, 27% of patients (n = 25) had an unfavorable histology. Initial tumor resection was performed for 90% of patients (n = 86). Systemic therapies included mitotane in 20 cases and chemotherapy in 13 cases. Among 17 stage III patients, 12 had microscopic residual tumor due to an initial biopsy (n = 5), intraoperative rupture (n = 8), or surgical resection with microscopic residue or tumor spillage surgery (n = 1) (two patients with two modalities). After a median follow-up of 96 months (25-119), four early progressions and two relapses occurred. A total of seven patients died, including five of disease. Stage III diseases due to microscopic residual disease correlated with a worse prognosis: 5-year progression-free survival 44% (95% CI, 22-87%) versus 82% (95% CI, 73-91%) for the whole cohort (P < .0001). Among the 14 patients with refractory disease, only 3 were alive and free of disease after multimodal second-line therapy. Stage III diseases due to a microscopic residual tumor have a dismal prognosis, arguing for the systematic use of adjuvant therapy. Patients with a relapsed disease should be included in experimental studies.
Types de publication
Journal Article
Multicenter Study
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
e28086Informations de copyright
© 2019 Wiley Periodicals, Inc.
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