Fluctuating hypertension possibly leading to severe cardiomyopathy in SDHB-associated hereditary paraganglioma: from heart transplant candidacy to long-term lanreotide management: a case report.

INTRODUCTION

Paragangliomas are rare catecholamine-secreting neuroendocrine tumors that can cause significant cardiovascular complications, including catecholamine-induced cardiomyopathy. While surgical resection is the standard of care, some patients are not candidates for surgery or heart transplantation due to tumor biology, genetic risk, or patient preference.

CASE SUMMARY: A 68-year-old man with longstanding hypertension, hereditary paraganglioma-pheochromocytoma syndrome (SDHB mutation), and advanced nonischemic cardiomyopathy was evaluated for heart transplantation. Imaging revealed a highly vascular retroperitoneal paraganglioma, and biochemical testing demonstrated markedly elevated chromogranin A with mildly increased plasma free normetanephrine. Biopsy confirmed paraganglioma. Due to transplant ineligibility related to malignancy risk and the patient's decision to decline surgical resection, medical therapy with lanreotide was initiated. Following treatment, the patient demonstrated biochemical improvement with declining chromogranin A levels, stabilization of blood pressure, improvement in B-type natriuretic peptide, modest recovery of left ventricular ejection fraction, and reduced metabolic activity on serial somatostatin receptor PET/CT imaging, with stable tumor size. Functional status remained excellent without further heart failure hospitalizations.

CONCLUSION

This case highlights lanreotide as a potential non-surgical therapeutic option for paraganglioma-associated cardiomyopathy in select high-risk patients. Systemic tumor control may contribute to cardiovascular stabilization when surgical or transplant options are not feasible.