Hayashi T, Tsuda N, Chowdhury P R, Anami M, Kishikawa M, Iseki M, Kobayashi K
Department of Laboratry Medicine, Scientific Data Center for the Atomic Bomb Disaster, Nagasaki, Japan.
Mod Pathol. 1995 Jun;8(5):548-52.
An 8-yr-old Japanese boy developed infantile digital fibromatosis in the right ring finger with recurrence and another lesion in the right middle finger. Histologic investigation of the tumor disclosed that the size and frequency of inclusion bodies were inversely proportional to the degree of fibrosis. Electron microscopic study revealed a variety of stages in the development of inclusion bodies, ranging from small, dense aggregates of filaments into bundles with dense bodies traversing the cytoplasm to typical inclusion bodies that also contained cytoplasmic organelles. In areas of dense fibrosis, the cytoplasm of the tumor cells showed areas of constriction and compression by adjacent bundles of collagen. The tendency for a decrease in the number of inclusion bodies in these areas necessitated a differential diagnosis from other fibrous or fibro-histiocytic lesions. Our findings suggest that the tumor may undergo a decrease in the numbers of inclusion bodies and spontaneously may become fibrotic with time. Thus, even as a form of fibromatosis featuring both recurrence and multiple lesions, it may not have a consistently aggressive nature. These findings support the concept that infantile digital fibromatosis should be managed by limited excision rather than by immediate aggressive surgical treatment.
一名8岁日本男孩右环指发生婴儿指纤维瘤病并复发,右中指出现另一处病变。对肿瘤进行组织学检查发现,包涵体的大小和数量与纤维化程度呈反比。电子显微镜研究揭示了包涵体形成的各个阶段,从小的、密集的细丝聚集体到有致密小体穿过细胞质的束状结构,再到也含有细胞质细胞器的典型包涵体。在致密纤维化区域,肿瘤细胞的细胞质显示出被相邻胶原束挤压和收缩的区域。这些区域包涵体数量减少的趋势使得需要与其他纤维性或纤维组织细胞性病变进行鉴别诊断。我们的研究结果表明,肿瘤可能会出现包涵体数量减少,并可能随时间自发纤维化。因此,即使作为一种具有复发和多发病变特点的纤维瘤病形式,它可能也并非始终具有侵袭性。这些发现支持了婴儿指纤维瘤病应采用有限切除而非立即积极手术治疗的观点。