# A Case of Pheochromocytoma With Coagulation Necrosis Due to Hypertensive Crisis Aggravated by Contrast-Enhanced CT Scan and Negative 123I-Metaiodobenzylguanidine (MIBG) Scintigraphy

## TL;DR

A rare case of pheochromocytoma with coagulation necrosis caused by a hypertensive crisis worsened by a CT scan and showed negative results on a standard imaging test.

## Contribution

First reported case of massive tumor necrosis due to a hypertensive crisis exacerbated by contrast-enhanced CT.

## Key findings

- 123I-MIBG scintigraphy was negative despite histological confirmation of pheochromocytoma.
- Contrast-enhanced CT scan worsened the hypertensive crisis, leading to tumor necrosis.
- Clinical diagnosis should consider pheochromocytoma even with negative imaging results.

## Abstract

123I-metaiodobenzylguanidine (123I-MIBG) scintigraphy is a highly sensitive and specific imaging test for the diagnosis of pheochromocytoma. Typical pheochromocytomas are positive on 123I-MIBG scintigraphy; however, cases of paragangliomas eliciting negative results have been reported. We encountered a case of hypertensive crisis resulting in extensive coagulative necrosis of a pheochromocytoma and negative findings on 123I-MIBG scintigraphy. A 50-year-old Japanese female presented with an acute onset of vomiting, epigastralgia, and abdominal pain. Immediately after contrast-enhanced CT, the patient developed respiratory failure and was intubated. The CT scan revealed a 5-cm left adrenal mass, and a pheochromocytoma crisis was suspected. The patient’s condition stabilized following phentolamine administration. Regarding the assessment for pheochromocytoma, plasma metanephrine levels were not markedly increased, and 123I-MIBG scintigraphy was negative. However, a histological examination of the left adrenal mass revealed extensive coagulative necrosis of the entire adrenal mass, comprising trabecular and alveolar growth of large polygonal cells that were immunopositive for chromogranin A/synaptophysin, thereby suggesting a diagnosis of pheochromocytoma. There have been three reported cases of 123I-MIBG scintigraphy-negative pheochromocytomas because of pure avascular necrosis without hemorrhage or rupture. To the best of our knowledge, this is the first reported case of massive tumor necrosis due to hypertensive crisis exacerbated after contrast-enhanced CT imaging. In conclusion, pheochromocytoma cannot be ruled out even with negative findings on 123I-MIBG scintigraphy. Accordingly, clinical judgment must be made based on a comprehensive assessment of the clinical course and pathological diagnosis, especially for cases involving a hypertensive crisis.