Incidentally diagnosed bilateral pheochromocytoma accompanied by urothelial carcinoma of the bladder

Damla Tufekci; Yasemin Emur Gunay; Ahmet Suat Demir; Muhammet Cuneyt Bilginer; Hulya Coskun; Irfan Nuhoglu; Ozge Ucuncu; Mustafa Kocak

doi:10.1530/endoabs.73.AEP50

Introduction

Adrenal abnormalities can be detected incidentally during the imaging performed for malignancies. These abnormalities may include adrenal pathological conditions independent of primary malignancies. Pheochromocytomas detected as part of some familial syndromes are often bilateral.In this report, we present a case of isolated sporadic bilateral pheochromocytoma that was incidentally detected during malignancy workup and staging, which is a rare occurrence.

Case presentation

The 51-year-old female patient was referred to our clinic due to the detection of bilateral adrenal mass on positron emission computed tomography (PET-CT) that was performed following the surgery for urothelial carcinoma of the bladder. The patients 24-hour urine and plasma catecholamines indicated approximately 30 times higher values of metanephrine and normetanephrine (Table 1). Fundus examination indicated grade 1–2 hypertensive retinopathy. On 24-h Holter monitoring, mean blood pressure was 152/98 mmHg.Abdominal magnetic resonance imaging (MRI) showed a lesion that was isointense in T1-weighted images and slightly hyperintense in T2-weighted images without signal loss in the external phase sequence, measuring 43 × 23 mm in the right adrenal gland and 26 × 29 mm in the left. Based on these findings, the patient was diagnosed as bilateral pheochromocytoma and the patient was negative for Von Hippel-Lindau (VHL) and RET gene mutations. Bilateral cortex-sparing surgery was performed. The catecholamine levels of the patient decreased postoperatively. The pathological result was reported as pheochromocytoma, with a diameter of 4.5 cm on the right side and 5 cm on the left, which was positive for s-100, synaptophysin, chromogranin, and neuron-specific enolase (NSE) and had a low ki-67 index. The PASS (pheochromocytoma of the adrenal gland scaled score) score was 1 and 3 for the right and left glands, respectively. No pathological lesion was detected in the adrenal glands in follow-up imaging.

Conclusion

Almost 5–10% of pheochromocytomas occur bilaterally. Although bilateral masses are rare, they do not have any syndromic features. Nevertheless, the number of pheochromocytoma cases detected in the presymptomatic stage is increasing due to the growing use of advanced imaging techniques and genetic tests.

Keywords: Bilateral pheochromocytoma, adrenal incidentaloma, urothelial carcinoma

**Table 1.** Clinical characteristics
TEST	Preoperative value	Reference value	Postoperative value
Plasma Metanephrine	608.71 pg/ml	≤ 90	15.5
Plasma Normetanephrine	5877.92 pg/ml	≤ 180	177.68
Plasma Adrenaline	168.81 ng/l	≤ 90	66.86
Plasma Noradrenaline	895.32 ng/l	≤ 500	189.72
Urine Metanephrine	1496.51 μg/24 h	50–250	88.95
Urine Normetanephrine	8370.15 μg/24 h	100–500	369.96
Urine Adrenaline	51.6 μg/24 h	0–20	7.32
Urine Noradrenaline	145.76 μg/24 h	15–80	45.83

Endocrine Abstracts

AEP50