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Pheochromocytoma is a rare pathology, even more so its malignant presentation. It is an aggressive entity but with an acceptable survival, liable to palliative treatment with good results. Currently available therapeutic options are surgical debulking, radiopharmaceutical therapy (IMIBG Y and Lu-DOTATATE), chemotherapy and targeted therapy. The timing at which metastatic patients benefit most from systemic therapy has not yet been established. However, at some point they will require resective surgical treatment. Expectant management is accepted whenever is possible. When the symptomatology is flowery and difficult to manage medically, the therapeutic range is imposed being the treatment option a combination between surgical reduction and 131I-MIBG.
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guarneri et al. (2023) studied this question.
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