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Malignant Paraganglioma: Long-term Considerations and Interventions
Background: Catecholamine-secreting tumors are neuroendocrine tumors classified as intra- or extra-adrenal. Extra-adrenal paragangliomas are rarer and more often multifocal, recurrent, and metastatic, which is consistent with our patient’s presentation. Paragangliomas also have a 60% malignancy rate compared to the 25% rate of adrenal pheochromocytomas. The Journal of Clinical Endocrinology and Metabolism (JCEM) determined that infra-diaphragmatic paraaortic paragangliomas have higher rates of metastasis compared to those in other locations. Surgical resection of metastatic disease depends on extent of metastasis and long-term goals of treatment. Adjuvant therapies such as chemotherapy, radiation, or targeted therapies should be considered to decrease disease recurrence and/or manage residual disease. Case Presentation: An elderly 68-year-old man presented with progressive lethargy, uncontrollable sleepiness, forceful vomiting, and wheezing. Upon admission, he was found to have hypertensive urgency, with a blood pressure of 187/110 and tachycardia. EKG revealed prolonged QTc. His labs showed elevated TSH, free T4, creatinine kinase, blood glucose, and leukocytosis. His urinalysis showed glucose above a thousand and an elevated specific gravity. His blood pressure and blood glucose remained elevated, requiring an increase in home metoprolol dose and the addition of amlodipine and hydralazine. Throughout admission, he was pale, and complained of overnight heat intolerance, diaphoresis, and anxiety regarding his hospitalization. A CT scan of the abdomen and pelvis with contrast revealed a 5.9 cm x 5.6 cm x 7.9 cm left para-aortic/retroperitoneal mass with central necrosis, most likely representing a paraganglioma. Bilateral pulmonary nodules and an 8 mm enhancing focus in the liver concerning for metastasis were also noted. Plasma and 24-hour urine normetanephrines were elevated at 16.25 and 10,904, respectively. Urine normetanephrine to creatine ratio was elevated at 4,948. The patient declined IR-guided biopsy. Discussion: For patients like this with extensive metastatic disease, excision is mostly a debulking procedure used to resolve the immediate symptoms and does not prevent recurrence or metastasis to other sites in the future. A retrospective analysis was conducted between 1995 and 2020 on patients who underwent resection of their paragangliomas, went into remission, and then had recurrence, defined as local or distant metastasis. The recurrence rate for paragangliomas was approximately 6.5-17.4% after initial surgery. Long-term surveillance is necessary to monitor for disease recurrence or progression.