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下颌骨孤立性婴儿肌纤维瘤病。三例报告。

Solitary infantile myofibromatosis of the mandible. Report of three cases.

作者信息

Vigneswaran N, Boyd D L, Waldron C A

机构信息

Department of Oral Pathology, Emory University School of Postgraduate Dentistry, Atlanta, Ga.

出版信息

Oral Surg Oral Med Oral Pathol. 1992 Jan;73(1):84-8. doi: 10.1016/0030-4220(92)90160-r.

DOI:10.1016/0030-4220(92)90160-r
PMID:1603571
Abstract

Infantile myofibromatosis (IMF) is a benign localized (solitary) or generalized (multicentric) proliferation of fibroblastic tissue occurring exclusively in infants and children. Three cases of solitary IMF involving the posterior region of the mandible of young children are reported. These lesions manifested clinically as asymptomatic bony expansion and roentgenographically as circumscribed lytic areas. Microscopically these tumors showed a distinct zoning phenomenon of curving bundles or intertwining fascicles of plump, spindle-shaped cells at the periphery and solid sheets of less differentiated round cells in the center. Positive immunostaining for vimentin and actin, with the lack of desmin and S-100 protein reactivity, confirmed their myofibroblastic nature of these cells and supported the diagnosis of IMF. All three lesions were treated by curettage and the follow-up showed no incidence of recurrence or any other complications. As we demonstrate in these case reports, IMF should be included in the differential diagnosis of spindle cell neoplastic processes in children.

摘要

婴儿肌纤维瘤病(IMF)是一种仅发生于婴儿和儿童的成纤维组织的良性局限性(孤立性)或广泛性(多中心性)增生。本文报告了3例累及幼儿下颌骨后部区域的孤立性IMF病例。这些病变临床上表现为无症状性骨质膨胀,X线表现为局限性溶骨性区域。显微镜下,这些肿瘤显示出明显的分区现象,周边为弯曲束状或交织成束的丰满梭形细胞,中心为分化程度较低的圆形细胞实性片块。波形蛋白和肌动蛋白免疫染色阳性,而结蛋白和S-100蛋白反应阴性,证实了这些细胞的肌成纤维细胞性质,支持IMF的诊断。所有3例病变均行刮除术治疗,随访显示无复发及其他并发症发生。正如我们在这些病例报告中所展示的,IMF应纳入儿童梭形细胞肿瘤性病变的鉴别诊断中。

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