Fetsch J F, Laskin W B, Miettinen M
Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
Hum Pathol. 2001 Jul;32(7):704-14. doi: 10.1053/hupa.2001.25903.
This report describes the clinicopathologic features and immunohistochemical findings identified in 37 cases of a distinctive soft tissue tumor that has a predilection for the hands and feet. The study group included 25 male and 12 female subjects ranging in age from 14 to 72 (mean, 43; median, 46) years. The patients presented with solitary masses 0.6 to 5.0 cm (mean, 1.75 cm) that were present from 3 months to 30 years (median duration, approximately 3 years) before surgical intervention and involved the toes (n = 20), fingers (n = 13), and palm (n = 4). Twenty of the cases were documented to involve the nail region. Histologically, the tumors were typically located in the dermis or subcutis and composed of spindled and stellate-shaped cells with random, loose storiform, and fascicular growth patterns. The lesional cells were embedded in myxoid or collagenous matrix, often with mildly to moderately accentuated vasculature and increased numbers of mast cells. There was generally slight to mild nuclear atypia; only 3 cases had more substantial atypia. Mitotic figures were infrequent. Occasional multinucleated stromal cells were noted in 19 cases. The process showed immunoreactivity for CD34 (21 of 23 cases), epithelial membrane antigen (18 of 25 cases), and CD99 (11 of 13 cases). No immunoreactivity was detected for actins, desmin, keratins, or HMB-45, and only 1 of 23 tumors had weak reactivity for S100 protein. The surgical specimens consisted of biopsy or partial resection specimens (n = 4), local excisions (n = 29), and amputated or partially amputated digits (n = 4). Detailed follow-up, available for 18 patients (mean follow-up interval, 10.1 years), revealed 1 recurrence after local excision and 2 instances of persistent or progressive disease after partial excision. A differential diagnosis of fibrous histiocytoma, dermatofibrosarcoma protuberans, acquired (digital) fibrokeratoma, sclerosing perineurioma, cutaneous myxoma (superficial angiomyxoma), and acral myxoinflammatory fibroblastic sarcoma is discussed.
本报告描述了37例一种特殊软组织肿瘤的临床病理特征及免疫组化结果，该肿瘤好发于手足部位。研究组包括25名男性和12名女性，年龄范围为14至72岁（平均43岁；中位数46岁）。患者表现为单个肿块，大小为0.6至5.0厘米（平均1.75厘米），在手术干预前已存在3个月至30年（中位病程约3年），累及脚趾（20例）、手指（13例）和手掌（4例）。其中20例记录显示累及甲床区域。组织学上，肿瘤通常位于真皮或皮下组织，由梭形和星状细胞组成，呈随机、疏松的席纹状和束状生长模式。病变细胞包埋于黏液样或胶原性基质中，常伴有轻度至中度的血管增生和肥大细胞数量增多。一般有轻微至轻度的核异型性；只有3例有更明显的异型性。核分裂象少见。19例可见散在的多核间质细胞。该病变对CD34（23例中的21例）、上皮膜抗原（25例中的18例）和CD99（13例中的11例）呈免疫反应性。对肌动蛋白、结蛋白、角蛋白或HMB - 45未检测到免疫反应性，23例肿瘤中只有1例对S100蛋白有弱反应性。手术标本包括活检或部分切除标本（4例）、局部切除标本（29例）以及截肢或部分截肢的手指（4例）。对18例患者进行了详细随访（平均随访间隔10.1年），结果显示局部切除后有1例复发，部分切除后有2例出现持续性或进展性疾病。文中讨论了与纤维组织细胞瘤、隆突性皮肤纤维肉瘤、获得性（指部）纤维角化瘤、硬化性神经鞘瘤、皮肤黏液瘤（浅表血管黏液瘤）和肢端黏液炎性纤维母细胞肉瘤的鉴别诊断。
