Pheochromocytoma Multisystem Crisis Complicated by Severe Acute Pancreatitis

Abstract

A 43-year-old man developed headache, dizziness, abdominal pain, and vomiting. His blood pressure was 203/121 mmHg, heart rate 122 beats/min, body temperature 39.1°C, and respiratory rate 24/min. He had elevated levels of creatinine at 2.95 mg/dL and lipase at 1,364 U/L as well as an extremely low calcium level at 5.2 mg/dL. Hypertriglyceridemia and hyperglycemia were seen. Chest and abdominal computed tomography showed interstitial pneumonia, severe pancreatitis, and a right adrenal tumor. The patient also developed vertebral artery dissection and medullary infarction. After right adrenalectomy, the patient was diagnosed with pheochromocytoma multisystem crisis (PMC). Acute pancreatitis might augment numerous life-threatening manifestations of PMC.

Keywords: acute pancreatitis; pheochromocytoma multisystem crisis.

Figure 1.

A serum lipoprotein analysis by agarose gel electrophoresis of the patient. Approximate proportions of each fraction; α: 5.2% (reference ranges for men: 26.9-50.5%), preβ: 31.3% (7.9-23.8%), and β: 18.6% (35.3-55.5%). Ex1+ex2=44.9% (extra fractions). Generally, fraction α represents HDL predominance, fraction preβ includes mostly VLDL and LDL, and fraction β mostly contains LDL. Ex1 (tailing) was considered to be CM, and ex2 (residue) was also thought to be CM. HDL: high-density lipoprotein, VLDL: very-low-density lipoprotein, LDL: low-density lipoprotein, CM: chylomicron

Figure 2.

Chest computed tomography findings showing ground-glass opacity and interstitial pneumonia in the right lobe. No pleural or pericardial effusion was observed.

Figure 3.

Abdominal computed tomography findings showing severe pancreatitis with diffuse edematous inflammation (arrowheads) and an enlarged (45-mm diameter, arrow) right adrenal tumor. Ascites was seen, but no gallstones were observed.

Figure 4.

The clinical course of the patient. The patient was admitted, and on Day 13, pheochromocytoma attack was seen. Hypertension was well controlled with the α-blocker doxazosin (gradually increased to 16 mg/day), and pheochromocytoma attack ceased. After adrenalectomy, all symptoms and laboratory abnormalities, including hyperamylasemia, hyperglycemia, and hypertriglyceridemia, were improved. AMY: amylase, PG: plasma glucose, LDL-C: low-density lipoprotein cholesterol, HDL-C: high-density lipoprotein cholesterol, TG: triglyceride

Figure 5.

Brain MRI showed right vertebral artery dissection on T1 FLAIR imaging (A, arrow) and right medullary infarction on diffusion-weighted imaging (B, arrow).

Figure 6.

The right adrenal tumor showed uptake of the trace on an ¹²³I-MIBG scintigram.

Figure 7.

Macroscopically, the right adrenal mass (4×3.5 mm) had a brown-colored appearance (A, arrows). The tumor showed a zellballen pattern, low cellularity, the absence of comedo-necrosis, the presence of vascular invasion, and the absence of capsular invasion, indicating pheochromocytoma (Hematoxylin and Eosin staining, ×100) (B). Ki67 labelling index 6/223 (2.7%) (C, ×100). Immunohistochemical staining was positive for synaptophysin (D, ×100), chromogranin (E, ×100), and neuron-specific enolase (NSE) (F, ×100).

Figure 8.

Schematic image of hypertriglyceridemia in this patient. TG: triglyceride, PMC: pheochromocytoma multisystem crisis, CM: chylomicron, VLDL: very low-density lipoprotein, LDL: low-density lipoprotein, HDL: high-density lipoprotein, LPL: lipoprotein lipase