Anaplastic Hemangiopericytoma of the Jugular Foramen: Case Report and Systematic Review

Abstract

Hemangiopericytomas (HPCs) and solitary fibrous tumors (SFTs) are rare tumors of mesenchymal origin. Here, the authors present a rare case of anaplastic HPC in the jugular foramen (JF). The authors also conduct a systematic review of the literature to examine the current fund of knowledge on JF HPC/SFTs. A systematic MEDLINE search was conducted using key words "hemangiopericytoma" OR "solitary fibrous tumor" AND "jugular foramen" OR "extracranial" OR "skull base." Clinicopathologic characteristics and outcomes of the present case were reviewed and compared with those in the literature. A 41-year-old male, who had undergone stereotactic radiation therapy 6 years ago for a presumed glomus jugulare tumor, presented to our institution with worsening dysphagia, hoarseness, persistent tongue weakness, and radiographic evidence of tumor progression. The patient underwent uncomplicated gross total resection with sacrifice of the infiltrated hypoglossal nerve. Histopathologic evaluation revealed anaplastic HPC/SFT (World Health Organization grade III). Review of the literature yielded 9 additional cases of JF HPC/SFT in 5 males (56%) and 4 females (44%), with a mean age of 49.6 years old. Patients commonly presented with pain (37.5%) and lower cranial nerve deficits (100%). Preoperative diagnoses included glomus jugulare (n= 2) or JF schwannomas (n= 3). All patients underwent microsurgical resection of the lesion, except for 1 who refused all treatment after diagnostic biopsy. The authors present the only reported case of anaplastic HPC of the JF. The illustrative case and those found on systematic review of the literature highlight the importance of tissue diagnosis and appropriate management.