[A rare case of adrenal hemangioblastoma and literature review].
Abstract
Ga 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.
Fields of Study & Subjects
Cancer
Hypoxia
and Metabolism
Neuroblastoma Research and Treatments
Adrenal and Paraganglionic Tumors
Publication details
- Journal / source
- PubMed
- Volume
- 65
- Issue
- 1
- Pages
- 91–97
- Publication date
- 1 January 2026
- Language
- ZH