Adrenocortical carcinoma (ACC) is a highly malignant tumor with an incidence of 1 to 1.6 cases per million per year. It presents with metastatic disease in up to 40% of cases. In advanced or recurrent disease treatment options are limited, and therapies using agents such as mitotane, cisplatin and adriamycin effect a tumor response rate of less than 30%. Pheochromocytomas have emerged as an endocrine malignancy with few options but with promising targets and very interesting genetics and these are being pursued. In adrenocortical cancer we are pursuing genetic and expression analyses to better understand these unique cancers and their diverse biology. Pheochromocytomas present a very rare disease with very unique biological and clinical properties and with increasingly complex and puzzling genetics. We are pursuing clinical strategies that will hopefully lead to better therapies and better understanding of how our therapies work and preclinical and laboratory studies to better understand the biology of cancers driven by mutations in the SDHB gene.

National Institute of Health (NIH)
National Cancer Institute (NCI)
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National Cancer Institute Division of Basic Sciences
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Jain, Meenu; Zhang, Lisa; He, Mei et al. (2012) Interleukin-13 receptor alpha2 is a novel therapeutic target for human adrenocortical carcinoma. Cancer 118:5698-708
Datrice, Nicole M; Langan, Russell C; Ripley, R Taylor et al. (2012) Operative management for recurrent and metastatic adrenocortical carcinoma. J Surg Oncol 105:709-13
Fassnacht, Martin; Terzolo, Massimo; Allolio, Bruno et al. (2012) Combination chemotherapy in advanced adrenocortical carcinoma. N Engl J Med 366:2189-97
Fonte, Jay S; Robles, Jeremyjones F; Chen, Clara C et al. (2012) False-negative ýýýýýýI-MIBG SPECT is most commonly found in SDHB-related pheochromocytoma or paraganglioma with high frequency to develop metastatic disease. Endocr Relat Cancer 19:83-93
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Martiniova, Lucia; Perera, Shiromi M; Brouwers, Frederieke M et al. (2011) Increased uptake of [¹²³I]meta-iodobenzylguanidine, [¹⁸F]fluorodopamine, and [³H]norepinephrine in mouse pheochromocytoma cells and tumors after treatment with the histone deacetylase inhibitors. Endocr Relat Cancer 18:143-57
Patterson, Erin E; Holloway, Alisha K; Weng, Julie et al. (2011) MicroRNA profiling of adrenocortical tumors reveals miR-483 as a marker of malignancy. Cancer 117:1630-9
King, Kathryn S; Prodanov, Tamara; Kantorovich, Vitaly et al. (2011) Metastatic pheochromocytoma/paraganglioma related to primary tumor development in childhood or adolescence: significant link to SDHB mutations. J Clin Oncol 29:4137-42
Veytsman, Irina; Nieman, Lynnette; Fojo, Tito (2009) Management of endocrine manifestations and the use of mitotane as a chemotherapeutic agent for adrenocortical carcinoma. J Clin Oncol 27:4619-29
Ghayee, Hans K; Havekes, Bas; Corssmit, Eleonora P M et al. (2009) Mediastinal paragangliomas: association with mutations in the succinate dehydrogenase genes and aggressive behavior. Endocr Relat Cancer 16:291-9

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