Hemangiopericytoma of neck extending to craniovertebral junction treated by surgery, pre- and postoperative radiotherapy

Abstract

Background:Hemangiopericytoma (HPC) is a rare tumor of uncertain malignant potential arising from mesenchymal cells with pericytic differentiation. It accounts for 3-5% of soft tissue sarcomas, and 1% of vascular tumors. The treatment of choice is a primary wide surgical resection with adjuvant radiotherapy (RT) reserved for cases of incomplete removal.Case Description:We report a case of a 24-year-old female with a rapidly growing, highly vascular swelling in nape of the neck extending deep into the craniovertebral (CV) junction accompanied by extradural/intraspinal, and intracranial involvement. An incisional biopsy revealed a cellular, highly vascular tumor with HPC-like features. The patient received preoperative RT, which reduced both the size and vascularity of the lesion, facilitating subsequent near complete resection. Further postoperative RT resulted in a good clinical outcome, with no tumor recurrence observed at 2 postoperative years.Conclusion:HPC of the soft tissues of neck accompanied by deep extension to the CV junction is uncommon. A high index of suspicion is required to diagnose these cases. which may be treated with preoperative RT (to reduce the lesion size/vascularity), aggressive surgical resection, followed by postoperative adjunctive radiation treatment as well.