Boisseau-Garsaud A M, Vergier B, Beylot-Barry M, Nastasel-Menini F, Dubus P, de Mascarel A, Eghbali H, Beylot C
Service de Dermatologie, Bergonié, Bordeaux, France.
J Cutan Pathol. 1996 Jun;23(3):275-82. doi: 10.1111/j.1600-0560.1996.tb01479.x.
A 28-year-old man presented with a histiocytic sarcoma of a very uncommon origin, as it had developed for several years like a benign cutaneous histiocytosis resembling generalized eruptive histiocytoma before becoming acute, with nodal and massive pulmonary involvement. Despite various chemotherapies, the patient died within 8 months. Skin biopsies showed histiocytic proliferation in the dermis and node biopsies showed histiocytic proliferation with a sinusoidal pattern. Immunohistochemical analysis, performed on paraffin-embedded sections, demonstrated strong labeling of tumoral cells for CD68 and moderate labeling for CD3 and CD4. CD30 labeling was negative. S-100 protein was positive on a Langerhans' cell reactive subpopulation. Electron microscopy confirmed the histiocytic nature of malignant cells and showed cytoplasmic inclusions such as regularly laminated bodies, dense bodies and pleomorphic inclusions. No Birbeck granules were seen. A gene rearrangement study of T-cell receptor gamma and immunoglobulin heavy chain genes showed a germline configuration. Histiocytic sarcoma is an extremely rare true histiocytic malignancy, the existence of which has been recently debated since it has often been mistaken in the past for large cell lymphomas. Such a deceptive onset as benign cutaneous histiocytosis has not been described in the literature to our knowledge.
一名28岁男性患者，患有起源极为罕见的组织细胞肉瘤。该病起初表现为良性皮肤组织细胞增生，类似泛发性发疹性组织细胞瘤，持续数年，随后病情急转，出现淋巴结及肺部广泛受累。尽管接受了多种化疗，患者仍在8个月内死亡。皮肤活检显示真皮层有组织细胞增生，淋巴结活检显示组织细胞呈窦状增生。对石蜡包埋切片进行免疫组化分析，结果显示肿瘤细胞对CD68呈强阳性标记，对CD3和CD4呈中度阳性标记，CD30标记为阴性。S-100蛋白在朗格汉斯细胞反应性亚群中呈阳性。电子显微镜检查证实了恶性细胞的组织细胞性质，并显示出细胞质内含物，如规则层状小体、致密小体和多形性内含物，未见伯贝克颗粒。T细胞受体γ基因和免疫球蛋白重链基因的基因重排研究显示为种系构型。组织细胞肉瘤是一种极其罕见的真正的组织细胞恶性肿瘤，其存在近来备受争议，因为过去它常被误诊为大细胞淋巴瘤。据我们所知，文献中尚未描述过如此以良性皮肤组织细胞增生为起始表现的病例。
