Long-term survival outcomes of pediatric adrenal malignancies: An analysis with the upstaged SEER registry during 2000-2019

Abstract

ObjectiveTo investigate the clinicopathological characteristics and long-term survival outcomes of pediatric adrenal malignancies.MethodThis study retrospectively analyzed children with pathologically confirmed pediatric adrenal malignancies from Surveillance, Epidemiology, and End Results Database from 2000 to 2019. Kaplan-Meier curve was used to assess the overall survival (OS) and cancer-special survival (CSS), and the Log-Rank method was used to calculate statistical differences. Cox proportional hazards model and Fine-and-Grey model were used to calculate the hazard ratio (HR) of all-cause mortality risk and the sub-distribution HR (sHR) of disease-specific mortality risk, respectively, and their corresponding 95% confidence intervals (CI).Results1601 children were included in the study in which 1335 (83.4%) neuroblastoma, 151 (9.4%) ganglioneuroblastoma, 89 (5.6%) adrenocortical carcinoma, and 26 (1.6%) were diagnosed with other types malignancies. Metastatic disease accounted for the largest proportion (69.3%), and the proportion of metastases diagnosed by neuroblastoma was higher than that of adrenocortical carcinoma and ganglioneuroblastoma (73.9% vs. 45.7% vs. 47.2%). The 5-year OS and CSS of all cohort were 69.5% and 70.5%, respectively. Adrenal cortical carcinoma had the worst prognosis, with 5-year OS and CSS of 52.5% and 53.1%, respectively. Patients in recent years had no better OS and CSS than in previous years at diagnosis. The tumor stage remained the main prognostic predictor. Compared to metastatic adrenal tumors, the risk of all-cause mortality (adjusted HR: 0.12, 95% CI: 0.06-0.25, P < 0.001) and the risk of disease-specific mortality (adjusted sHR: 0.11, 95% CI: 0.05-0.25, P<0.001) was significantly lower for patients with localized diseases. Additionally, higher age, adrenal cortical carcinoma, and lack of complete tumor resection are independent risk factors for poor prognosis. Furthermore, it was found that the prognosis of patients who received chemotherapy was worse than those who did not, mainly because the former mostly had metastasis at the presentation and complete resection of the tumor cannot be achieved.ConclusionThe clinicopathological characteristics of pediatric adrenal malignancies have not changed significantly in the past two decades, while the prognosis of patients has improved. Early diagnosis of disease and complete resection of local tumors are the keys to improving prognosis.

Development and validation of a prognostic nomogram for patients with ganglioneuroblastoma: A SEER-based study

“…We found that patients who received chemotherapy had an adverse prognosis compared with those who did not receive or had an unknown chemotherapy status. Similarly, a SEER-based study of adrenal tumors in children reported a worse prognosis for patients in the chemotherapy group [ 28 ]. Notably, in the SEER database, patients who did not receive chemotherapy and those with unknown chemotherapy were grouped together, which might lead to bias in the results regarding value of chemotherapy.…”

Construction of a novel clinical nomogram to predict cancer-specific survival in patients with primary malignant adrenal tumors: a large population-based retrospective study

“…Many studies have been conducted on the prognosis, diagnosis, and treatment of malignant adrenal tumors. Researchers have found that patient age, the initial stage of diagnosis, and surgical resection might affect the prognosis of patients with adrenal malignancies ( 6 , 21 , 22 ). However, most studies limited the tumor to a single histological type or only studied one prognostic factor and only the OS of patients.…”

“…Tumor size and stage were probable variables that affected the OS in adrenal tumor patients ( 21 , 29–31 ). Wang et al stated that primary tumor size is a vital prognostic indicator for NB patients, and tumor size >4 cm may anticipate a poor prognosis ( 29 ).…”

“…Hue et al reported that ACC patients with tumors ≥5 cm were correlated to a high conversion rate and a subsequent elevation in margin positivity, thus having a poor prognosis ( 30 ). Furthermore, many reports revealed that patients with distant metastasis have significantly lower OS than those with localized or regional tumors ( 21 , 31 , 32 ). The results of our study supported the previous reports that distant metastasis and larger tumor size (63–111 mm, > 111 mm) exhibited worse CSS in patients with adrenal malignancies.…”

A real‐world study of immune checkpoint inhibitors in advanced triple‐negative breast cancer

“…LDH is a catalytic enzyme in the pathways of glycolysis and gluconeogenesis, and exists in most tissues of the body [ 24 , 25 , 26 ]. Cancer cells produce large amount of lactic acid to form an acidic tumor microenvironment, and local high concentration of lactic acid inhibits the function of cytotoxic T lymphocytes and reduces the release of IL‐2, interferon, perforin, and granzyme [ 27 ].…”