Overview
Metastatic and unresectable pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors that can arise from chromaffin cells of the adrenal medulla or sympathetic/parasympathetic ganglia 1. These tumors can secrete catecholamines, leading to significant morbidity and mortality 1.Diagnosis
Diagnosis is typically confirmed by elevated plasma or 24-hour urinary levels of metanephrines and normetanephrines 1.
Functional imaging with [131I]metaiodobenzylguanidine (MIBG) scintigraphy or somatostatin receptor positron emission tomography (PET) is recommended for staging and identifying metastatic sites 1.
Genetic testing is recommended for all patients with PPGL due to the high prevalence of germline mutations 1.Management
Medical Management:
* For symptomatic or progressive metastatic/unresectable PPGL, treatment options include somatostatin analogs, tyrosine kinase inhibitors (e.g., sunitinib, axitinib), and peptide receptor radionuclide therapy (PRRT) with [177Lu]DOTATATE 1.
* PRRT with [177Lu]DOTATATE is a recommended option for patients with somatostatin receptor-positive metastatic PPGL 1.
* Chemotherapy regimens, such as cyclophosphamide, vincristine, and dacarbazine (CVD), can be considered for aggressive or rapidly progressive disease 1.
Surgical Management:
* Surgical resection of primary or metastatic lesions may be considered in select cases to improve symptoms or control disease burden 1.Key Recommendations
For symptomatic or progressive metastatic/unresectable PPGL, consider treatment with somatostatin analogs, tyrosine kinase inhibitors, or PRRT with [177Lu]DOTATATE 1. (Evidence: Expert opinion)
PRRT with [177Lu]DOTATATE is a recommended option for patients with somatostatin receptor-positive metastatic PPGL 1. (Evidence: Expert opinion)
Genetic testing is recommended for all patients with PPGL due to the high prevalence of germline mutations 1. (Evidence: Expert opinion)References
1 Fishbein L, Del Rivero J, Else T, Howe JR, Asa SL, Cohen DL et al.. The North American Neuroendocrine Tumor Society Consensus Guidelines for Surveillance and Management of Metastatic and/or Unresectable Pheochromocytoma and Paraganglioma. Pancreas 2021. link