Intracranial hemangioblastomas with special types of imaging findings: A report of 8 cases

Abstract

Objective To investigate the diagnosis and treatment of intracranial hemangioblastomas (HBs) with special imaging findings in order to improve the clinical diagnostic level of this disease. Methods The clinical data of 8 patients with HBs who had atypical imaging findings admitted to Department of Neurosurgery, Huashan Hospital, Fudan University from May 2014 to April 2018 were retrospectively analyzed. Eight patients underwent CT and MRI, 7 of which underwent magnetic resonance spectroscopy (MRS) and 3 underwent positron emission tomography (PET)-CT. One case was diagnosed as Von Hippel-Lindau (VHL)-HB before surgery due to multiple lesions and the other 7 were misdiagnosed as other intracranial tumors. Eight patients with intracranial HB underwent microsurgery. Results All 8 cases showed atypical imaging findings. Four cases of cystic HBs presented the partial enhanced cystic wall or enhanced ring lesion without the nodules. The other 4 cases of substantial HBs showed severe peritumoral edema surrounding the lesions. Eight tumors were completely resected by microsurgery and HBs were confirmed by the postoperative pathology. The severe edema in 4 patients completely disappeared after dehydration therapy. Seven patients underwent VHL gene test, and 3 were found to be VHL-HB (including the patient with multiple lesions), which were recruited in the management of VHL disease. Postoperative follow-up duration was 32±17.6 months (3-50 months). Conclusion Those intracranial HBs with atypical imaging findings are very rare and misdiagnosis is common based on preoperative MRI results. MRS, PET-CT and other examinations might have supporting value for the diagnosis of HB and its definite diagnosis relies on pathological examination. Microsurgical resection is the main treatment for HB. Key words: Hemangioblastoma; Magnetic resonance imaging; Diagnosis; Treatment outcome