Requena L, Kutzner H, Hügel H, Rütten A, Furio V
Department of Dermatology, Fundación Jiménez Diaz, Universidad Autónoma, Madrid, Spain.
J Cutan Pathol. 1996 Oct;23(5):445-57. doi: 10.1111/j.1600-0560.1996.tb01434.x.
Infantile myofibromatosis is a distinctive type of fibromatosis that usually develops during the immediate perinatal period. There are variants with solitary and multiple tumors. Lesions confined to the skin, soft tissue, and bone carry a good prognosis, showing spontaneous regression. The prognosis, however, is much less favorable when visceral lesions are present and the outcome may be fatal. Only recently it became obvious that there is an adult counterpart of infantile myofibromatosis, characterized by solitary lesions that have a predilection for involve the dermis and show no tendency to regression, although they have an entirely benign biological behavior. These lesions have been named cutaneous myofibroma or solitary myofibroma of adults. We have studied the clinical, histopathological and immunohistochemical characteristics of 53 examples of cutaneous adult myofibroma. In addition, 2 cases were examined ultrastructurally. The patients were mostly adults with ages ranging from 6-83 years. The lesions presented as solitary, usually painless nodules of variable duration on the skin, usually located on the extremities. Histopathologically, four patterns were identified: nodular or cellular type, multinodular or biphasic type, leiomyoma-like or fascicular type, and vascular type. A correlation between the histopathologic pattern and the lesional age was observed: vascular type of cutaneous adult myofibroma in early lesions, nodular and multinodular lesions in fully developed lesions, and leiomyoma-like or fascicular type in late lesions. Immunohistochemically, the spindle cells were desmin negative, but expressed immunoreactivity for vimentin, pan-smooth muscle actin, and alpha-smooth muscle actin. Ultrastructurally, neoplastic cells showed characteristics of undifferentiated mesenchymal cells with features of fibroblasts, myofibroblasts and pericytes. Primitive vascular formations were seen in the form of irregular clefts between adjoining cells. We conclude that cutaneous adult myofibroma is a little-known benign vascular neoplasm probably derived from myopericytes.
婴儿肌纤维瘤病是一种独特类型的纤维瘤病，通常在围生期即刻发病。有单发和多发肿瘤的变异型。局限于皮肤、软组织和骨骼的病变预后良好，可自发消退。然而，当存在内脏病变时，预后则差得多，结局可能是致命的。直到最近才明确，婴儿肌纤维瘤病有成人对应物，其特征为单发病变，好发于真皮，虽具有完全良性的生物学行为但无消退倾向。这些病变被命名为成人皮肤肌纤维瘤或单发成人肌纤维瘤。我们研究了53例成人皮肤肌纤维瘤的临床、组织病理学和免疫组化特征。此外，对2例进行了超微结构检查。患者多为成年人，年龄范围为6至83岁。病变表现为皮肤上单发的、通常无痛的结节，持续时间不等，通常位于四肢。组织病理学上，确定了四种模式：结节状或细胞型、多结节状或双相型、平滑肌瘤样或束状型以及血管型。观察到组织病理学模式与病变年龄之间存在相关性：早期病变中的成人皮肤肌纤维瘤为血管型，完全发展的病变为结节状和多结节状病变，晚期病变为平滑肌瘤样或束状型。免疫组化方面，梭形细胞结蛋白阴性，但对波形蛋白、泛平滑肌肌动蛋白和α平滑肌肌动蛋白呈免疫反应性。超微结构上，肿瘤细胞显示未分化间充质细胞的特征，具有成纤维细胞、肌成纤维细胞和周细胞的特点。原始血管形成表现为相邻细胞之间不规则的裂隙。我们得出结论，成人皮肤肌纤维瘤是一种鲜为人知的良性血管肿瘤，可能起源于肌周细胞。
