[Dermatofibrosarcoma protuberans developing for 25 years with 27 recurrences. Histological and ultrastructural study].

Annales d'anatomie pathologique Pub Date : 1980-01-01
N Mandalenakis, L Venne, J G Paquin, Y McKay, S Allard, J Bourgeois
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Abstract

The authors report the results of a histological and ultrastructure study of a case of a protuberant dermatofibrosarcoma evolving for the last 25 years with 27 relapses. Histologically the tumour is dermic, non-encapsulated, invading the hypodermis right from the beginning and mainly made up of fusiform cells which often have a storiform appearance and proliferate in a varying degree of collagenous stroma which includes an oedematous or myxoid component. During the course of the evolution of this tumour, there were zones of cystic degeneration, but in the last 10 relapses in 1978 and 1979, we noted the predominance of cellular and compact zones with an increase in the number of mitoses. The ultrastructure study showed tumour cells containing a fairly well developed endoplasmic reticulum in the cytoplasm, a prominent Golgi apparatus and some pinocytic vesicles. Some of the cells also contained lysosomes and phagolysosomes, whereas others contained cytoplasmic filaments and contractile elements. The nuclei were in general elongated and irregular. Sometimes the cytoplasmic membrane was covered by an amorphous substance with a membranoid appearance. Between the cells sometimes there were junctions of the tight kind. In conclusion, the authors consider that this tumour has the histological characteristics of a protuberant dermatofibrosarcoma and at the ultrastructure level, it is made up of cells which put it in the group of myofibroblastic and histiocytic proliferating lesions.

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结节性皮肤纤维肉瘤病程25年,复发27次。组织学和超微结构研究]。
作者报告了一个组织和超微结构研究的结果,一个突出的皮肤纤维肉瘤发展为过去25年27次复发。组织学上,肿瘤是真皮的,非包膜的,从一开始就侵入皮下,主要由梭状细胞组成,梭状细胞通常具有故事状外观,并在不同程度的胶原基质中增殖,胶原基质包括水肿或粘液成分。在肿瘤的演变过程中,有囊性变性区,但在1978年和1979年的最后10次复发中,我们注意到细胞和致密区占主导地位,有丝分裂数量增加。超微结构研究显示,肿瘤细胞的细胞质中含有相当发达的内质网,突出的高尔基体和一些胞泡。有些细胞还含有溶酶体和吞噬溶酶体,而另一些细胞则含有细胞质丝和收缩元件。细胞核一般呈细长和不规则。有时细胞质膜被膜样无定形物质覆盖。细胞之间有时有紧密的连接。总之,作者认为该肿瘤具有突出性皮肤纤维肉瘤的组织学特征,在超微结构水平上,它由细胞组成,属于肌成纤维细胞和组织细胞增生性病变。
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