Adolescent- and adult-onset neuroblastic tumor: A retrospective multicenter observational study of patients diagnosed in France between 2000 and 2020.

BACKGROUND: Adult- and adolescent-onset neuroblastomas are rare, with no established therapy. In addition, rare pheochromocytomas may harbor neuroblastic components. This study was designed to collect epidemiological, diagnostic and therapeutic data in order to better define the characteristics of malignant peripheral neuroblastic tumors (MPNT) and composite pheochromocytomas (CP) with MPNT. PROCEDURE: Fifty-nine adults and adolescents (aged over 15 years) diagnosed with a peripheral or composite neuroblastic tumor, who were treated in one of 17 institutions between 2000 and 2020, were retrospectively studied. RESULTS: Eighteen patients with neuroblastoma (NB) or ganglioneuroblastoma (GNB) had locoregional disease, and 28 patients had metastatic stage 4 NB. Among the 13 patients with CP, 12 had locoregional disease. Fifty-eight percent of the population were adolescents and young adults under 24 years of age. The probability of 5-year event-free survival (EFS) was 40% (confidence interval: 27%-53%). CONCLUSIONS: Outcomes were better for patients with localized tumor than for patients with metastases. For patients with localized tumor, in terms of survival, surgical treatment was the best therapeutic option. Multimodal treatment with chemotherapy, surgery, radiotherapy, and immunotherapy-based maintenance allowed long-term survival for some patients. Adolescent- and adult-onset neuroblastoma appeared to have specific characteristics associated with poorer outcomes compared to pediatric neuroblastoma. Nevertheless, complete disease control improved survival. The presence of a neuroblastic component in pheochromocytoma should be considered when making therapeutic management decisions. The development of specific tools/resources (Tumor Referral Board, Registry, biology, and trials with new agents or strategies) may help to improve outcomes for patients.

Duration of palliative care involvement and immunotherapy treatment near the end of life among patients with cancer who died in-hospital.

BACKGROUND: Immune checkpoint inhibitors (ICIs) have revolutionised cancer treatment, but their use near the end of life in patients with advanced cancer is poorly documented. This study investigated the association between administration of ICI therapy in the last month of life and the duration of involvement of the palliative care (PC) team, among patients with advanced cancer who died in-hospital. METHODS: In a retrospective, multicentre study, we included all patients who died in 2018 of melanoma, head and neck carcinoma, non-small cell lung cancer or urothelial or renal cancer, in 2 teaching hospitals and one community hospital in France. The primary outcome was the association between ICI therapy in the last month of life and duration of involvement of the PC team in patient management. RESULTS: Among 350 patients included, 133 (38%) received anti-cancer treatment in the last month of life, including 71/133 (53%) who received ICIs. A total of 207 patients (59%) received palliative care, only 127 (36%) 30 days before death. There was a significant association between ongoing ICI therapy in the last month of life and shorter duration of PC management (p = 0.04). Receiving ICI therapy in the last month of life was associated with an increased risk of late PC initiation by multivariate regression analysis (hazard ratio 1.668; 95% CI 1.022-2.722). CONCLUSION: ICI therapy is frequently used close to the end of life in patients with advanced cancer. Innovative new anti-cancer treatments should not delay PC referral. Improved collaboration between PC and oncological teams is needed to address this issue.