BJMO - volume 15, issue 1, january 2021
E. Roussel MD, PhD, D. Hompes MD, PhD, M. Bex MD, O. Bechter MD, PhD, S. Jentjens MD, PhD, I. Fourneau MD, PhD, R. Sciot MD, PhD, M. Baldewijns MD, PhD, M. Albersen MD, PhD
Succinate dehydrogenase deficient renal cell carcinoma (SDH-RCC) is a very rare but distinct renal neo-plasm, most often presenting at a young age and commonly associated with paragangliomas, pheochromocytomas and gastro-intestinal stromal tumours as a hereditary cancer syndrome. Although SDH-RCCs often have a relatively indolent disease course, higher nuclear grade, coagulative necrosis and sarcomatoid dedifferentiation may indicate aggressive disease. Radical surgery and (targeted) radiation therapy are valuable options in the treatment of these rare tumours. Genetic testing for germline SDH mutations is crucial. First-line relatives with germline SDH mutations should undergo periodical screening since early detection is paramount. The strong presence of the Warburg effect in SDH-related tumours make these the hallmark tumour for 18Fluorodeoxyglucose Positron Emission Tomography based screening and follow-up.
(BELG J MED ONCOL 2021;15(1):44-7)
Read moreBJMO - volume 7, issue 3, july 2013
P. Schöffski MD, MPH , D. Hompes MD, PhD, A. Wozniak PhD, H. Dumez MD, PhD, I. Samson MD, M. Stas PhD, F. Sinnaeve MD, O. Bechter MD, PhD, M. Debiec-Rychter MD, PhD, E. Van Limbergen MD, PhD, S. Pans MD, PhD, R. Sciot MD, PhD
Sarcomas are a group of rare solid tumours arising from mesenchymal or connective tissue. This review focuses on soft tissue sarcoma and covers general topics such as the epidemiology, age distribution, site of disease, histogenesis, histological subtypes, prognosis and outcome of treatment. In more detail the article reviews current systemic treatment standards and selected adverse events of agents such as doxorubicin, ifosfamide, trabectedin and pazopanib, and briefly highlights some drugs that are used off-label in rare subtypes of sarcoma.
(BELG J MED ONCOL 2013;7(3):80–88)
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