Anand Rohit, Gupta Sonal
Department of Pedodontics, Sardar Patel Post Graduate Institute of Dental Sciences, Lucknow-UP, India.
J Dent Child (Chic). 2010 Sep-Dec;77(3):180-2.
The hemangiopericytoma is a malignant vascular tumor arising from mesenchymal cells with pericytic differentiation. Hemangiopericytoma is most commonly seen in adults, and only 5% to 10% of cases occur in children. The tumor is extremely rare in the head and neck region (16%). Cytogenic abnormalities have been present in some hemangiopericytoma cases. Surgical resection remains the mainstay treatment. Adjuvant chemotherapy and radiotherapy is appropriate for cases of incomplete resections and life-threatening tumors particularly in children. Late relapses may occur and require long-term follow-up. A 4-year-old child patient with hemangiopericytoma of the maxilla presented with firm, recurrent, but painless jaw mass. Radiographic investigations revealed a poorly circumscribed radiolucency. The lesion biopsy showed wellcircumscribed multiple lobules of tumor mass consisting of tightly packed, spindleshaped cells. Chemotherapy and radiotherapy of the lesion was conducted. The role of the pediatric dental team is extensive in children with hemangiopericytoma, who require a regular dental review. The patient's oncologist should be immediately contacted if there is any suspicion of recurrence.
血管外皮细胞瘤是一种起源于具有周细胞分化的间充质细胞的恶性血管肿瘤。血管外皮细胞瘤最常见于成年人，仅5%至10%的病例发生在儿童。该肿瘤在头颈部区域极为罕见（16%）。一些血管外皮细胞瘤病例存在细胞遗传学异常。手术切除仍然是主要的治疗方法。辅助化疗和放疗适用于不完全切除和危及生命的肿瘤病例，尤其是儿童患者。可能会发生晚期复发，需要长期随访。一名4岁上颌骨血管外皮细胞瘤患儿表现为颌部有质地坚硬、反复出现但无痛的肿块。影像学检查显示边界不清的透射区。病变活检显示肿瘤肿块有边界清晰的多个小叶，由紧密排列的梭形细胞组成。对该病变进行了化疗和放疗。儿科牙科团队在患有血管外皮细胞瘤的儿童中作用广泛，这些儿童需要定期进行牙科检查。如果怀疑有复发，应立即联系患者的肿瘤医生。
