Abstract Introduction Malignant pheochromocytomas are histologically and biochemically identical to benign pheochromocytomas, with malignancy diagnosed only upon documented metastasis. Risk factors for malignancy include pathogenic variants in succinate dehydrogenase subunit B (SDHB), larger tumor size, and certain von Hippel-Lindau variants. This case demonstrates the diagnostic challenges and therapeutic evolution in malignant pheochromocytoma management, emphasizing personalized medicine approaches in the era of novel radionuclide therapies. Clinical Case A 27-year-old female presented with hypertensive crisis during pregnancy (BP: 200/100 mmHg), with persistent hypertension postpartum. Secondary hypertension workup revealed pheochromocytoma. Right adrenalectomy was performed, removing a 13 cm tumor with focal adipose tissue invasion. Postoperatively, persistent hypertension and abdominal MRI revealed a 24x27 mm solid lesion in the surgical bed with suspected metastatic lymph nodes, indicating recurrence. Three years after initial surgery, Ga-68 PET imaging showed pathological uptake in the right adrenal region and multiple lymph nodes (paraaortic, paracaval, mediastinal, paraesophageal, supraclavicular). Planned debulking surgery was aborted due to vena cava infiltration and extensive metastatic disease. The patient tested negative for SDH mutations. Medical oncology initiated cisplatin-etoposide chemotherapy, followed by 100 mCi I-131 MIBG therapy after multidisciplinary evaluation. Follow-up Ga-68 PET studies revealed widespread nodular lesions with variable pathological activity in multiple locations including bilateral adrenal regions, mediastinum, and supraclavicular areas. Lutetium-177 therapy was administered in September 2024. Lesions were evaluated as stable on PET imaging performed after lutetium therapy. The patient's blood pressure is currently being monitored and regulated with doxazosin therapy. Conclusion This case emphasizes the importance of careful evaluation for pheochromocytoma in pregnancy-related hypertensive crises and demonstrates that novel radionuclide therapies like Lutetium-177 offer promising options for conventional therapy-resistant malignant pheochromocytomas. Genetic mutation analyses and targeted radionuclide therapies highlight the significance of personalized medicine approaches in malignant pheochromocytoma management.Figure 1:Pathological increased activity is observed in upper abdominal, mediastinal, bilateral supraclavicular and axillary lymph nodes, right adrenalectomy bed, and multiple foci in both lungs.
Aydemir et al. (Thu,) studied this question.