Uncommon spreading of primary malignant paraganglioma in a patient with SDHB mutation

Abstract

Pheochromocytomas (PHEOs) or paragangliomas (PGLs) are rare neuroendocrine tumors arising from adrenal medulla or from chromaffin cells outside the adrenal gland including parasympathetic ganglia, respectively.
Here we describe a case of a 56-year-old man evaluated for left hip pain. The anatomical and functional imaging showed a pelvic tumor spreading through the foramen obturatum to the proximal part of the left hip, histologicaly confirmed as PGL. On biochemical evaluation elevated plasma catecholamine and metanephrine levels were found. The diagnosis of metastatic PGL was done based on additional lesions found in bones, liver, and retroperitoneal lymphatic nodes. Treatment with systemic cyclophosphamide, vincristine, and dacarbazine (CVD) chemotherapy was initiated and with a substantial decrease in tumor burden. Spreading of PGL (PHEO) through the foramen obturatum infiltrating the left hip is very uncommon and has not been described previously.