[A rare case of adrenal hemangioblastoma and literature review].
A retrospective analysis was conducted on the clinical data of a patient with adrenal hemangioblastoma (HB), pathologically confirmed at the First Medical Center of Chinese PLA General Hospital. A review of the literature relating to adrenal HB was also undertaken. The patient was a 36-year-old male in whom a right adrenal mass was incidentally detected during a physical examination. The mass measured approximately 4.7 cm×4.2 cm and had a CT attenuation value of approximately 55 HU. MRI showed an irregular mass with a slightly long T1, mixed long/short T2 signals, mildly high signal intensity on diffusion weighted imaging, and a slightly low signal on apparent diffusion coefficient. No signal drop was observed on opposed-phase imaging. Dynamic contrast-enhanced scanning revealed progressive, marked enhancement. 68Ga DOTATATE PET-CT demonstrated a slightly hypodense mass in the right adrenal gland (maximum standardized uptake value 17.3). Functional evaluation showed an elevated chromogranin A (CgA) level of 294.64 pmol/L (normal range: 64-204 pmol/L), with no other abnormalities detected. The preoperative diagnosis was pheochromocytoma, and the patient underwent surgery after adequate preoperative preparation with phenoxybenzamine hydrochloride. Immunohistochemical results-S-100 (focal weak+), melan-A (-), inhibin-α (partial+), FLI-1 (+), CK (-), Syn (partial+), CgA (-), Ki67 (2%+), CD34 (+), CD31 (+), and desmin (-)-confirmed the diagnosis of adrenal HB. Genetic testing detected no VHL gene mutation. No recurrence or metastasis was observed during more than two years of follow-up. A review of the literature revealed that this was the first reported case of adrenal HB in China. Globally, only six cases have been reported to date, four of which were clearly related to von Hippel-Lindau syndrome. Adrenal HB is exceptionally rare and lacks characteristic clinical manifestations. Significant enhancement of the solid component with flow-void vessels on MRI is considered a relatively distinctive imaging feature. Definitive diagnosis relies on pathology and immunohistochemistry. Surgical resection remains the primary treatment, and the prognosis is generally favorable.