# Silent Metastatic Pheochromocytoma: Clinical, Diagnostic, and Management Challenges in a Resource-Limited Setting

## TL;DR

A rare case of metastatic pheochromocytoma with normal hormone levels is presented, highlighting diagnostic and management challenges in low-resource settings.

## Contribution

Highlights the challenges of diagnosing metastatic pheochromocytoma in resource-limited settings with atypical symptoms and normal catecholamine levels.

## Key findings

- Metastatic pheochromocytoma can present with normal metanephrine levels and atypical symptoms.
- Socioeconomic barriers delay treatment and worsen outcomes in metastatic pheochromocytoma.
- Comprehensive evaluation including imaging and histopathology is crucial for diagnosis.

## Abstract

Pheochromocytomas are rare neuroendocrine tumors arising from adrenal chromaffin cells, typically characterized by excess catecholamine secretion, which causes symptoms such as hypertension and palpitations. However, some pheochromocytomas and paragangliomas are non-functional and biochemically silent, complicating their diagnosis. Metastatic pheochromocytoma is uncommon and challenging to manage, especially in resource-limited settings.

We present the case of a 50-year-old man with progressive back pain, weight loss, weakness, and intermittent sweating. Imaging revealed bilateral adrenal masses with extensive lymphadenopathy and probable hypopharyngeal metastasis. Surprisingly, plasma and urinary metanephrines were normal. A biopsy of a cervical lymph node confirmed metastatic pheochromocytoma, demonstrating chromogranin and GATA3 positivity and a Ki-67 index of 20%. Despite multidisciplinary input, the patient’s lack of health insurance delayed treatment and resulted in death within one month.

This case highlights the diagnostic challenges of metastatic pheochromocytoma presenting with atypical symptoms and normal catecholamine levels. It emphasizes the need for comprehensive evaluation beyond biochemical tests, including imaging and histopathology. Additionally, it illustrates how socioeconomic barriers can limit access to timely care, adversely affecting outcomes. Awareness and early multidisciplinary management are essential to improving prognosis in metastatic pheochromocytoma.