Mishra Sudhansu Sekhar, Panigrahi Souvagya, Das Srikant, Senapati Satyabhusan
Department of Neurosurgery, SCB Medical College and Hospital, Cuttack, Odisha, India.
Surg Neurol Int. 2013 Apr 18;4:55. doi: 10.4103/2152-7806.110653. Print 2013.
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.
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.
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.
血管外皮细胞瘤（HPC）是一种罕见的肿瘤，起源于具有周细胞分化的间充质细胞，其恶性潜能尚不确定。它占软组织肉瘤的3% - 5%，占血管肿瘤的1%。首选的治疗方法是进行广泛的手术切除，对于切除不完全的病例则采用辅助放疗（RT）。
我们报告一例24岁女性患者，其颈部后方有一个生长迅速、血管丰富的肿块，深入颅颈（CV）交界处，伴有硬膜外/脊髓内及颅内受累。切开活检显示为一种具有HPC样特征的细胞性、血管丰富的肿瘤。患者接受了术前放疗，这减小了病变的大小并降低了其血管化程度，便于随后近乎完全切除。术后进一步放疗取得了良好的临床效果，术后2年未观察到肿瘤复发。
颈部软组织HPC伴深及CV交界处的情况并不常见。诊断这些病例需要高度的怀疑指数，可采用术前放疗（以减小病变大小/血管化程度）、积极的手术切除，随后还需进行术后辅助放疗。
