Cécile Picard1, Cécile Faure-Conter2, Pierre Leblond2, Laurence Brugières3, Cécile Thomas-Teinturier4, Frédéric Hameury5, Anne-Sophie Defachelles6, Arnauld Verschuur7, Hervé J Brisse8, Sabine Sarnacki9, Frédérique Dijoud1, Yves Reguerre10, Pascal Chastagner11, Matthieu Carton12, Daniel Orbach13. 1. Institut de Pathologie Multisite, Groupement hospitalier Est, Hospices Civils de Lyon, UCBL Lyon 1 University, Lyon, France. 2. Département de Cancérologie pédiatrique, Institut d'Hématologie et d'Oncologie Pédiatrique, Lyon, France. 3. Département de Cancérologie de l'Enfant et l'Adolescent, Gustave Roussy Cancer Campus, Villejuif, France. 4. Department of Pediatric Endocrinology, AP-HP, Hôpitaux universitaires Paris-Sud, site Bicêtre, Le Kremlin-Bicêtre, France. 5. Département de chirurgie viscérale pédiatrique, Groupement hospitalier Est, Hospices Civils de Lyon, Le Kremlin-Bicêtre, France. 6. Département d'hématologie et de Cancérologie pédiatrique, Centre Oscar Lambret, Lille, France. 7. Service d'hématologie-oncologie pédiatrique, Hôpital de La Timone, AP-HM, Marseille, France. 8. Département d'imagerie médicale, Institut Curie, PSL University, Paris, France. 9. Département de chirurgie viscérale pédiatrique, Hôpital Necker, AP-HP, Paris, France. 10. Département d'hématologie et de Cancérologie pédiatrique, Centre Hospitalier Universitaire, Saint Denis de La Réunion, France. 11. Département d'Hématologie et de Cancérologie pédiatrique, Centre Hospitalier Universitaire, Nancy, France. 12. Institut Curie, PSL Research University, DRCI, Biométrie, Saint-Cloud, France. 13. SIREDO Oncology Center, Institut Curie, PSL University, Paris, France.
Abstract
INTRODUCTION: Pediatric adrenal cortical tumors are characterized by a wide spectrum of behavior. Questions remain regarding intermediate disease stages with isolated tumor rupture or relapse. OBJECTIVES: 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. MATERIAL AND METHODS: Pediatric patients with histological material reviewed and treated between 2000 and 2018 in 23 French oncology centers were included. RESULTS: 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. CONCLUSIONS: 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.
INTRODUCTION:Pediatric adrenal cortical tumors are characterized by a wide spectrum of behavior. Questions remain regarding intermediate disease stages with isolated tumor rupture or relapse. OBJECTIVES: 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. MATERIAL AND METHODS: Pediatric patients with histological material reviewed and treated between 2000 and 2018 in 23 French oncology centers were included. RESULTS: 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 patientsdied, 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. CONCLUSIONS: 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.
Authors: Michaela Kuhlen; Christina Pamporaki; Marina Kunstreich; Stefan A Wudy; Michaela F Hartmann; Mirko Peitzsch; Christian Vokuhl; Guido Seitz; Michael C Kreissl; Thorsten Simon; Barbara Hero; Michael C Frühwald; Peter Vorwerk; Antje Redlich Journal: Front Endocrinol (Lausanne) Date: 2022-06-17 Impact factor: 6.055