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.
Highlights
Hemangiopericytoma (HPC) is a rare tumor of uncertain malignant potential arising from mesenchymal cells that exhibits pericytic differentiation
Just above 300 cases of HPC have been reported since Stout and Murray described HPCs as “vascular tumors arising from Zimmerman’s pericytes” in 1942.[5]. Primary wide surgical resection is the treatment of choice and is typically accompanied by adjuvant postoperative radiotherapy (RT) if tumor removal has been incomplete
We present a case in which HPC was treated with subtotal resection accompanied by both preoperative and postoperative RT
Summary
Hemangiopericytoma of neck extending to craniovertebral junction treated by surgery, pre‐ and postoperative radiotherapy. Available FREE in open access from: http://www.surgicalneurologyint.com/text.asp?2013/4/1/55/110653
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