Brian C Gulack1, Kristy L Rialon2, Brian R Englum3, Jina Kim4, Lindsay J Talbot5, Obinna O Adibe6, Henry E Rice7, Elisabeth T Tracy8. 1. Department of Surgery, Duke University Medical Center, Durham, NC. Electronic address: brian.gulack@duke.edu. 2. Department of Surgery, Duke University Medical Center, Durham, NC. Electronic address: Kristy.rialon@duke.edu. 3. Department of Surgery, Duke University Medical Center, Durham, NC. 4. Department of Surgery, Duke University Medical Center, Durham, NC. Electronic address: jina.kim1@duke.edu. 5. Department of Surgery, Duke University Medical Center, Durham, NC. Electronic address: Lindsay.jones@duke.edu. 6. Department of Surgery, Duke University Medical Center, Durham, NC. Electronic address: Obinna.adibe@duke.edu. 7. Department of Surgery, Duke University Medical Center, Durham, NC. Electronic address: henry.rice@duke.edu. 8. Department of Surgery, Duke University Medical Center, Durham, NC. Electronic address: Elisabeth.tracy@duke.edu.
Abstract
PURPOSE: Adrenocortical carcinoma (ACC) is a rare tumor in children with important distinctions from the adult disease. We reviewed the National Cancer Data Base (NCDB) to determine factors associated with long-term survival. METHODS: The NCDB was queried for patients less than 18 years of age who were diagnosed with ACC between 1998 and 2011. Kaplan-Meier analysis was utilized to determine factors significantly associated with overall survival. RESULTS: A total of 111 patients were included (median age: 4 years, 69% female). ACC was more common in the youngest cohort, with 48% of cases occurring in children younger than the age of 3. Median tumor size was 9.5 cm (IQR: 6.5-13.0), and 87% of patients underwent some form of surgical resection. Among children with available data, 19 of 62 presented with metastases. Overall 1- and 3-year survival was 70% and 64%, respectively. Age, tumor size, extension of tumor into surrounding tissue, and metastatic disease were all found to be significantly associated with survival. Among patients who underwent a surgical procedure, margin status was also found to be significantly associated with survival. CONCLUSION: Age, tumor size, extension of tumor, metastatic disease, and margin status are significantly associated with long-term survival in children with adrenocortical carcinoma.
PURPOSE:Adrenocortical carcinoma (ACC) is a rare tumor in children with important distinctions from the adult disease. We reviewed the National Cancer Data Base (NCDB) to determine factors associated with long-term survival. METHODS: The NCDB was queried for patients less than 18 years of age who were diagnosed with ACC between 1998 and 2011. Kaplan-Meier analysis was utilized to determine factors significantly associated with overall survival. RESULTS: A total of 111 patients were included (median age: 4 years, 69% female). ACC was more common in the youngest cohort, with 48% of cases occurring in children younger than the age of 3. Median tumor size was 9.5 cm (IQR: 6.5-13.0), and 87% of patients underwent some form of surgical resection. Among children with available data, 19 of 62 presented with metastases. Overall 1- and 3-year survival was 70% and 64%, respectively. Age, tumor size, extension of tumor into surrounding tissue, and metastatic disease were all found to be significantly associated with survival. Among patients who underwent a surgical procedure, margin status was also found to be significantly associated with survival. CONCLUSION: Age, tumor size, extension of tumor, metastatic disease, and margin status are significantly associated with long-term survival in children with adrenocortical carcinoma.
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