OBJECTIVE: Treatment of metastatic adrenocortical carcinoma (ACC) is challenging and long-term survival rates are exceedingly low. Long-term outcome data for pediatric patients who received mitotane is very limited. METHODS: We describe the case of a 2-year-old boy with ACC with a lung metastasis. He was treated with surgery, chemotherapy, and mitotane, and remains disease-free 13 years after diagnosis. RESULTS: The key endocrine issues learned from this case include: adrenal-derived sex-steroid and insulin-like growth factor-2 levels are correlated with disease status; very high doses of glucocorticoid and mineralocorticoid are required while on treatment of mitotane; and central precocious puberty needs to be detected and treated in a timely manner to preserve final adult height. CONCLUSION: We report a case of pediatric ACC with metastasis that was successfully treated with surgery, chemotherapy, and adjuvant therapy with mitotane. Appropriate endocrine testing and management are important for long-term survival and quality of life.
OBJECTIVE: Treatment of metastatic adrenocortical carcinoma (ACC) is challenging and long-term survival rates are exceedingly low. Long-term outcome data for pediatric patients who received mitotane is very limited. METHODS: We describe the case of a 2-year-old boy with ACC with a lung metastasis. He was treated with surgery, chemotherapy, and mitotane, and remains disease-free 13 years after diagnosis. RESULTS: The key endocrine issues learned from this case include: adrenal-derived sex-steroid and insulin-like growth factor-2 levels are correlated with disease status; very high doses of glucocorticoid and mineralocorticoid are required while on treatment of mitotane; and central precocious puberty needs to be detected and treated in a timely manner to preserve final adult height. CONCLUSION: We report a case of pediatric ACC with metastasis that was successfully treated with surgery, chemotherapy, and adjuvant therapy with mitotane. Appropriate endocrine testing and management are important for long-term survival and quality of life.
Authors: Patrícia Zancanella; Mara A D Pianovski; Brás H Oliveira; Sima Ferman; Gislaine C Piovezan; Leniza L Lichtvan; Suely Z Voss; Sérvio Túlio Stinghen; Luiz G Callefe; Guilherme A Parise; Maria Helena A Santana; Bonald C Figueiredo Journal: J Pediatr Hematol Oncol Date: 2006-08 Impact factor: 1.289
Authors: Vasileios Chortis; Angela E Taylor; Petra Schneider; Jeremy W Tomlinson; Beverly A Hughes; Donna M O'Neil; Rossella Libé; Bruno Allolio; Xavier Bertagna; Jérôme Bertherat; Felix Beuschlein; Martin Fassnacht; Niki Karavitaki; Massimo Mannelli; Franco Mantero; Giuseppe Opocher; Emilio Porfiri; Marcus Quinkler; Mark Sherlock; Massimo Terzolo; Peter Nightingale; Cedric H L Shackleton; Paul M Stewart; Stefanie Hahner; Wiebke Arlt Journal: J Clin Endocrinol Metab Date: 2012-11-16 Impact factor: 5.958
Authors: N Boulle; E Baudin; C Gicquel; A Logié; J Bertherat; A Penfornis; X Bertagna; J P Luton; M Schlumberger; Y Le Bouc Journal: Eur J Endocrinol Date: 2001-01 Impact factor: 6.664
Authors: B G Robinson; I B Hales; A J Henniker; K Ho; B M Luttrell; I R Smee; J N Stiel Journal: Clin Endocrinol (Oxf) Date: 1987-10 Impact factor: 3.478
Authors: Philip D Oddie; Benjamin B Albert; Paul L Hofman; Craig Jefferies; Stephen Laughton; Philippa J Carter Journal: Endocrinol Diabetes Metab Case Rep Date: 2018-08-23