Articles

Succinate dehydrogenase B deficient renal cell carcinoma in a young adult with paraganglioma: Management of a rare, hereditary malignancy

BJMO - volume 15, issue 1, january 2021

E. Roussel MD, 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

SUMMARY

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)

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Algorithms for molecular testing in solid tumours

BJMO - volume 13, issue 7, november 2019

Ir A. Hébrant PhD, M. Lammens MD, PhD, C. Van den Broecke MD, N. D’Haene MD, PhD, J. Van den Oord MD, PhD, A. Vanderstichele MD, PhD, A. Dendooven MD, PhD, P. Neven MD, PhD, K. Punie MD, G. Floris MD, PhD, J. Van der Meulen MD, HA. Poirel MD, PhD, C. Dooms MD, PhD, S. Rottey MD, PhD, T. Boterberg MD, PhD, L. Brochez MD, PhD, M.C. Burlacu MD, G. Costante MD, D. Creytens MD, PhD, P. De Paepe MD, PhD, R. De Pauwn MD, B. Decallonne MD, PhD, F. Dedeurwaerdere MD, H. Denys MD, PhD, L. Ferdinande MD, PhD, R. Forsyth MD, PhD, M. Garmyn MD, PhD, T. Gevaert MD, PhD, J. De Grève MD, PhD, E. Govaerts MD, E. Hauben MD, PhD, J. Kerger MD, O. Kholmanskikh Van Criekingen MD, PhD, V. Kruse MD, PhD, Y. Lalami MD, L. Lapeire MD, PhD, P. Lefesvre MD, PhD, J.P. Machiels MD, PhD, B. Maes MD, PhD, G. Martens MD, PhD, M. Remmelink MD, PhD, I. Salmon MD, PhD, R. Sciot MD, PhD, S. Tejpar MD, PhD, K. Van de Vijver MD, PhD, L. Van de Voorde MD, I. Van den Berghe MD, A. Van den Bruel MD, K. Vandecasteele MD, PhD, L. Vanwalleghem MD, K. Vermaelen MD, PhD, R. Salgado MD, PhD, E. Wauters MD, PhD, B. Weynand MD, PhD, E. Van Valckenborgh PhD, G. Raicevic PhD, M. Van den Bulcke PhD, P. Pauwels MD, PhD

SUMMARY

In order to advise the Federal Government on the reimbursement of molecular tests related to Personalised Medicine in Oncology, the Commission of Personalised Medicine (ComPerMed), represented by Belgian experts, has developed a methodology to classify molecular testing in oncology. The different molecular tests per cancer type are represented in algorithms and are annotated with a test level reflecting their relevance based on current guidelines, drug approvals and clinical data. The molecular tests are documented with recent literature, guidelines and a brief technical description. This methodology was applied on different solid tumours for which molecular testing is a clear clinical need.

(BELG J MED ONCOL 2019;13(7):286–95)

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Soft tissue sarcoma: the clinically relevant basics and an update on systemic therapy options for patients with advanced disease

BJMO - 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 , O. Bechter MD, PhD, M. Debiec-Rychter MD, PhD, E. Van Limbergen MD, PhD, S. Pans MD, PhD, R. Sciot MD, PhD

Summary

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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