Adrenocortical carcinoma.
Adrenocortical carcinoma is a rare disease with a poor prognosis. Patients can present with a hormonal syndrome or with general symptoms from an abdominal mass. The pathogenesis is unknown. Sometimes the adrenocortical carcinoma is associated with tumour syndromes such as the Beckwith-Wiedemann and Li-Fraumeni syndrome; however, most tumours are sporadic. Using one of the international classification methods, histopathological research can in almost all cases distinguish between adrenocortical adenoma and carcinoma. complete surgical resection is the treatment of choice for adrenocortical carcinoma. Mitotane is given when surgery is not possible, after incomplete resection or for metastatic disease. Frequently used chemotherapeutic combinations are etoposide, doxorubicin, cisplatin and mitotane (EDP/M) and streptozotocin and mitotane (SZ/M). International and national cooperation has resulted in a randomised trial aimed at determining a standard therapy in advanced adrenocortical carcinoma. The Dutch Adrenal Network is a national cooperation of endocrinologists, pathologists and oncologists from all eight academic centres and Máxima Medical centre. The network combines knowledge and expertise and gives patients the opportunity to receive optimal treatment in their own district.
van Ditzhuijsen
C I M
CI
Department of Internal Medicine, Máxima Medical Centre Eindhoven, Ds. Th. Fliednerstraat 1, 5600 PD Eindhoven, the Netherlands.
van de Weijer
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R
Haak
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HR
D000046:Academic Medical Centers / Q000458:organization & administration*; D000306:Adrenal Cortex Neoplasms / Q000175:diagnosis / Q000188:drug therapy* / Q000601:surgery; D018268:Adrenocortical Carcinoma / Q000175:diagnosis / Q000188:drug therapy* / Q000601:surgery; D000971:Antineoplastic Combined Chemotherapy Protocols; D002945:Cisplatin / Q000008:administration & dosage; D002986:Clinical Trials as Topic; D004317:Doxorubicin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D006801:Humans; D038622:Internationality; D008939:Mitotane / Q000008:administration & dosage; D009426:Netherlands; D013311:Streptozocin / Q000008:administration & dosage
D013311:Streptozocin; D005047:Etoposide; D008939:Mitotane; D004317:Doxorubicin; D002945:Cisplatin