Pheochromocytoma in the organ of Zuckerkandl with distant skeletal metastases – A case report

Abstract

Pheochromocytomas are rare catecholamine-secreting neuroendocrine tumors that originate from chromaffin cells in the adrenal medulla. They cause severe hypertension and various clinical manifestations. While most arise in the adrenal medulla, some occur in extra-adrenal locations.We present a case report with clinical, laboratory and radiographic data, along with a brief literature review. We report a 33-year-old woman who presented with flushing and sharp lower abdominal pain. Abdominal ultrasound and urinary metanephrines suggested a pheochromocytoma. Subsequent Computed tomography (CT) and Ga-DOTATATE Positron emission tomography/Computed tomography (PET/CT) scans confirmed a pheochromocytoma in the organ of Zuckerkandl with distant bony metastasis. Extra-adrenal pheochromocytomas, or paragangliomas, are rare tumors found in specific anatomical locations. Their diagnostic challenges stem from variable clinical presentations and imaging findings. CT scans and Ga-DOTATATE PET/CT scans are crucial in diagnosis and prognosis. Surgical resection can cure localized cases, while metastatic disease requires palliative options, such as chemotherapy and I131-MIBG, due to a poorer prognosis.

Keywords

• Pheochromocytoma
• Paraganglioma
• Zuckerkandl
• Bone metastasis