Busam K J, Mentzel T, Colpaert C, Barnhill R L, Fletcher C D
Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts 02115, USA.
Am J Surg Pathol. 1998 Sep;22(9):1067-72. doi: 10.1097/00000478-199809000-00004.
Cellular neurothekeoma is a recently characterized benign cutaneous neoplasm arising usually on the upper trunk or head and neck of children or young adults. Typical histology is that of a lobulated dermal tumor composed of spindle and epithelioid cells, arranged in fascicles and nests, lacking immunoreactivity for S-100 protein, but usually being NK1/C3 positive. We present 10 new cases of cellular neurothekeoma with atypical histologic features that have not been described previously and that suggested the possibility of malignancy. The age range of affected patients was 1 to 44 years (median, 20.5 years); sites included the head and neck (three cases), the upper limbs (two cases), the lower limbs (two cases), and the trunk (two cases). Atypical findings in individual cases included large size (up to 6 cm), deep penetration (extending into skeletal muscle or subcutaneous fat, or both), diffusely infiltrative borders, vascular invasion, high mitotic rate, and marked cytologic pleomorphism. Clinical follow-up was available in 7 of 10 cases. Although the atypical features raised concern about the biologic potential of these lesions, preliminary follow-up (1-5 years) has shown no recurrence and suggests that complete surgical excision of these lesions is curative. These new data expand the morphologic spectrum of cellular neurothekeoma.
细胞性神经鞘黏液瘤是一种最近才被明确特征的良性皮肤肿瘤，通常发生于儿童或年轻成人的上躯干、头部或颈部。典型的组织学表现为分叶状真皮肿瘤，由梭形细胞和上皮样细胞组成，呈束状和巢状排列，对S-100蛋白缺乏免疫反应性，但通常NK1/C3呈阳性。我们报告了10例具有非典型组织学特征的细胞性神经鞘黏液瘤新病例，这些特征此前未曾描述过，提示有恶性的可能性。受影响患者的年龄范围为1至44岁（中位数为20.5岁）；部位包括头颈部（3例）、上肢（2例）、下肢（2例）和躯干（2例）。个别病例的非典型表现包括体积大（达6厘米）、深部浸润（延伸至骨骼肌或皮下脂肪，或两者皆有）、边界弥漫性浸润、血管侵犯、高有丝分裂率和明显的细胞多形性。10例中有7例有临床随访资料。尽管这些非典型特征引发了对这些病变生物学潜能的担忧，但初步随访（1至5年）未显示复发，提示这些病变完整手术切除可治愈。这些新数据扩展了细胞性神经鞘黏液瘤的形态学谱。
