Metastatic Pheochromocytoma

A 35-year-old man without previous history of systemic illness presents to our clinic with generalized weakness, more prominent on the right side, ptosis, and difficulty swallowing for six months.

Figure 1: Axial CT image and 3D CT reconstruction show a lytic lesion at the right mandibular angle (yellow arrows).

Figure 2: Axial CT images show multiple lytic lesions at the right iliac bone (yellow arrows). 3D CT reconstruction shows a large lytic lesion at the right iliac crest (red arrow).

Figure 3: DSA shows a highly vascularized lesion at C1 vertebra.

Figure 4: Sagittal CT image and 3D reconstruction shows lytic lesions at the right mandibular angle, odontoid process, and C4 vertebra (yellow and black arrows).

Figure 5: Axial CT image shows destruction of the C1 arch (yellow arrow).

Figure 6: Coronal and sagittal CT images show an aggressive, necrotic, and vascularized lesion occupying the right hepatic lobe and hepatorenal fossa.

Figure 7: Histological tissue section shows positive staining for chromogranin.

Figure 8: Histological tissue section shows positive staining for neuro-specific enolase (NSE).

Imaging studies disclosed multiple skeletal lytic lesions, a large hepatic lesion, and a highly vascularized cervical vertebral mass (Figures 1-6). Histological immunostaining for chromogranin and neurospecific enolase (NSE) was performed and were positive, thus confirming the diagnosis of metastatic pheochromocytoma (Figures 7 and 8).

Diagnosis: Pheochromocytoma with hepatic, mandibular, and vertebral metastasis

Diagnosis was also confirmed clinically with increased catecholamine levels as follows: urine total metanephrine (2,680 mcg/g creat; normal 94-445), urine normetanephrine (2,583 mcg/g creat; normal 67-390); plasma total metanephrine (1,559 pg/ml; normal < 205) and plasma normetanephrine (1,502 pg/ml; normal < 148).

Guido Santacana-Laffitte, MD, and Jomarie Cortés, MD, Department of Diagnostic Radiology, University of Puerto Rico School of Medicine