Babin E, Goullet De Rugy M, Moreau S, Comoz F, Bourdon N, Roquier V, Valdazo A
Service ORL et de Chirurgie Cervico-Faciale, CHU Côte de Nacre, 14033 Caen, France.
Ann Otolaryngol Chir Cervicofac. 2000 Mar;117(2):118-25.
We report a case of desmoid tumor in the submandibular region in an 18-month-old girl. Head and neck desmoid tumors are uncommon in children and diagnosis is a difficult task because the tumors are often classified as different types of fibromatosis. This histologically benign affection is characterized by local expansion or destruction and tends to recur. Pathology gives the positive diagnosis, showing fibroblastic monoclonal proliferation between the cellular center and the collagen periphery. Electron microscopy evidences an abundant collagen network enclosing a polymorphous cellular proliferation. Immunohistochemistry defines vimentin and actin positive desmoid tumors. Surgery is usually the therapeutic choice. Chemotherapy and radiotherapy may be used in conjunction with surgery in situations of recurrence or unsatisfactory surgical margin. There is a risk of recurrence which can be detected with regular follow-up examinations.
我们报告一例18个月大女童下颌下区硬纤维瘤病例。头颈部硬纤维瘤在儿童中并不常见，诊断颇具难度，因为这些肿瘤常被归类为不同类型的纤维瘤病。这种组织学上为良性的病变以局部扩张或破坏为特征，且易于复发。病理检查给出阳性诊断，显示在细胞中心和胶原外周之间有成纤维细胞单克隆增殖。电子显微镜检查证明有丰富的胶原网络包裹着多形性细胞增殖。免疫组织化学确定波形蛋白和肌动蛋白阳性的硬纤维瘤。手术通常是治疗的选择。在复发或手术切缘不理想的情况下，化疗和放疗可与手术联合使用。存在复发风险，可通过定期随访检查检测到。
