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上肢先天性纤维肉瘤——病例报告及文献复习

Congenital fibrosarcoma of the upper extremity--a case report and review of literature.

作者信息

Sachdev Ritesh, Singhal Niti, Mandal Ashish K

机构信息

Department of Pathology, Maulana Azad Medical College, Bahadur Shah Zafar Marg, Delhi.

出版信息

Indian J Pathol Microbiol. 2005 Oct;48(4):474-6.

Abstract

Fibrosarcoma is rare in pediatric age group. Its diagnosis in infants may be difficult clinically and histologically. A twenty-day infant presented with a rapidly growing mass in the right forearm. Histologically a gray white fleshy mass was seen occupying the entire forearm and distal arm. The tumor showed sheets of closely packed spindle cells with minimal pleomorphism. The cells were positive for vimentin, negative jor smooth muscle actin, desmin and S-100. The diagnosis of congenital fibrosarcoma was made. Most cases of infantile fibrosarcomas present in the first year of life and although they have an alarming presentation, in children they have a much better prognosis as compared to adults. Diagnosis can be made on histology with immunohistochemistry.

摘要

纤维肉瘤在儿童年龄组中较为罕见。在婴儿中,其临床和组织学诊断可能具有挑战性。一名20天大的婴儿右前臂出现迅速生长的肿块。组织学检查可见灰白色肉质肿块占据整个前臂和手臂远端。肿瘤表现为紧密排列的梭形细胞片,异型性极小。细胞波形蛋白阳性,平滑肌肌动蛋白、结蛋白和S-100阴性。诊断为先天性纤维肉瘤。大多数婴儿纤维肉瘤病例在出生后第一年内出现,尽管其表现令人担忧,但与成人相比,儿童患者的预后要好得多。可通过组织学检查及免疫组织化学进行诊断。

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