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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Molecular-defined melanoma: Implications for clinical practice

BJMO - volume 13, issue 7, november 2019

Y. Van Herck MD, O. Bechter MD, PhD

SUMMARY

Due to the development of genome-directed therapy and standardised methods of molecular analysis, molecular diagnostics has become an important part of daily practice in clinical oncology, especially in diseases like malignant melanoma where molecular testing has therapeutic implications. Melanoma has one of the highest mutation frequency of all cancers analysed in the TGCA (The Cancer Genome Atlas) and is characterised by an enormous genetic heterogeneity.1 The discovery of ‘driver mutations’ directly involved in melanomagenesis has led to the development of small-molecule kinase inhibitors. The emergence of BRAF and MEK inhibitors has completely changed treatment paradigm for BRAF-mutant metastatic melanoma with dramatically improvement of therapeutic outcomes. Despite high response rates, the duration of response remains limited, mainly due to the development of acquired treatment resistance. In this review the authors try to outline the importance of molecular oncology for malignant melanoma by giving an overview of the most frequent and potentially clinically relevant molecular alterations, the targeted therapies already used in clinical routine and by discussing the problem of acquired resistance and treatment strategies being developed to circumvent these obstacles.

(BELG J MED ONCOL 2019;13(7):277–85)

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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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