Endocrine Abstracts

Background

Pheochromocytomas are rare tumors originating from chromaffin cells and characterized by excessive catecholamine synthesis. They are usually benign lesions. Hypertension(HT), tachycardia, sweating and headache are frequently observed. We aimed to present a high malignancy suspected pheochromocytoma case diagnosed in a young age female.

Case presentation

A 20-year-old female patient was admitted to the emergency department due to high blood pressure, tinnitus and headache. The patient was hypertensive (220/110 mmHg) and had tachycardia (130/bpm). For the etiology of HT, renal doppler USG was performed. An extra-renal solid mass lesion (pheochromocytoma?) was reported. She was referred to our clinic. Surrenal MRI revealed a lesion in the left paraaortic area with 40 × 37 mm enhancement in the area corresponding to the adrenal region. 24-hour urinary normetanephrine and metanephrine levels were found 10 times higher than upper limit of the reference range. Laparoscopic partial adenomectomy was performed by the urologist. After surgery, blood pressure was return to normal range without any medication. In histopathology, Ki 67 proliferation index was reported as 20%. Periadrenal adipose tissue invasion, atypical mitosis, lymphovascular invasion and capsule invasion were found positive. Calculated PASS score was reported 6/21 and it was evaluated as high suspicion of malignancy. Genetic analysis (VHL, MEN, SDH) result was negative. The patient had an identical twin. Pheochromocytoma symptoms of the identical twin were not present. In the literature some cases of identical twins which have concominant pheochromocytoma were reported. We performed pheochromositoma screening tests for identical twin and no pathology was found.

Conclusion

Although pheochromocytoma is most common in the 4th and 5th decades, it can be seen in all age ranges. The majority of patients are sporadic, there are also hereditary forms. Genetic background should be investigated in patients diagnosed with pheochromocytoma at the young age. Although our case was diagnosed with pheochromocytoma at a young age, her genetic tests was not showed any mutation.