Zelger B G, Steiner H, Kutzner H, Rütten A, Zelger B
Department of Pathology, Univeristy of Innsbruck, Austria.
Histopathology. 1997 Sep;31(3):258-62. doi: 10.1046/j.1365-2559.1997.2490854.x.
To describe a series of five granular cell dermatofibromas as an unusual and rare manifestation of fibrohistiocytic tissue response.
Five granular cell dermatofibromas were collected out of 136 tumours filed as granular cell tumours. Clinically, all lesions occurred on the shoulder or back of middle-aged adults (two women, three men), mostly with the clinical diagnosis of a fibrohistiocytic lesion. Histology revealed well-circumscribed, dermal to subcutaneous lesions dominated by periodic acid-Schiff (PAS) positive, granular cells. Acanthosis above, as well as storiform arrangement of spindle cells, sclerotic collagen and some interspersed lymphohistiocytic infiltrate at the periphery of the lesion, indicated the fibrohistiocytic origin. Lesions showed prominent reactivity with NK1C3 (CD57), as well as for macrophage markers KiM1p and KP1 (CD68). In contrast to classic Schwannian/neurogenic granular cell tumours, granular cell dermatofibromas were S100 protein negative, but showed variable reactivity for factor XIIIa (10-50%) in 4/5, for smooth muscle specific actin (10-50%) in 2/5 and with E9 (10-30%) in 3/5 lesions. Electron microscopy in one case revealed large pools of phago-lysosomes and variably sized glycogen granules in granular cells.
Our series delineates granular cell dermatofibroma as a distinct clinicopathological variant of fibrohistiocytic tissue response which needs to be distinguished from other tumours with granular cell features.
描述一系列五例颗粒细胞皮肤纤维瘤，作为纤维组织细胞性组织反应的一种不寻常且罕见的表现。
在136例记录为颗粒细胞瘤的肿瘤中收集到五例颗粒细胞皮肤纤维瘤。临床上，所有病变均发生在中年成年人的肩部或背部（两名女性，三名男性），大多数临床诊断为纤维组织细胞性病变。组织学显示界限清楚的真皮至皮下病变，以过碘酸雪夫（PAS）阳性的颗粒细胞为主。病变上方棘层肥厚，以及梭形细胞的束状排列、硬化性胶原和病变周边一些散在的淋巴组织细胞浸润，提示其纤维组织细胞起源。病变对NK1C3（CD57）以及巨噬细胞标志物KiM1p和KP1（CD68）显示出显著反应。与经典的施万/神经源性颗粒细胞瘤不同，颗粒细胞皮肤纤维瘤S100蛋白阴性，但4/5的病例对因子ⅩⅢa显示出不同程度的反应（10 - 50%），2/5的病例对平滑肌特异性肌动蛋白显示出不同程度的反应（10 - 50%），3/5的病变对E9显示出不同程度的反应（10 - 30%）。一例病例的电子显微镜检查显示颗粒细胞中有大量吞噬溶酶体池和大小不一的糖原颗粒。
我们的系列病例描述了颗粒细胞皮肤纤维瘤是纤维组织细胞性组织反应的一种独特的临床病理变体，需要与其他具有颗粒细胞特征的肿瘤相鉴别。
