Malignant Phaeochromocytoma — SCE Medical Oncology MCQ

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HardOncological EmergenciesMalignant PhaeochromocytomaSCE Medical Oncology

A 55-year-old man with malignant pheochromocytoma (metastatic, SDHB-mutant) has progressive disease. What systemic therapy options exist?

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Correct answer: B — CVD chemotherapy (cyclophosphamide, vincristine, dacarbazine) or 177Lu-DOTATATE (if SSTR-positive) or temozolomide

Malignant phaeochromocytoma/paraganglioma has limited systemic therapy options. CVD chemotherapy (Averbuch protocol) is the most established regimen (~30-40% ORR). PRRT with 177Lu-DOTATATE is effective for somatostatin receptor-positive tumours. Temozolomide has activity, particularly in SDHB-mutant tumours (~30% ORR). Sunitinib has shown modest activity. Belzutifan (HIF-2α inhibitor) is approved for VHL-associated tumours. Comprehensive alpha-blockade (phenoxybenzamine/doxazosin) must precede any intervention.

Reference: ESMO 2024 NET/Phaeochromocytoma Guidelines; Averbuch et al Ann Intern Med 1988