Zedek Daniel C, White Wain L, McCalmont Timothy H
Depts. of Pathology and Dermatology, University of California, San Francisco, 94115, USA.
J Cutan Pathol. 2009 Nov;36(11):1185-90. doi: 10.1111/j.1600-0560.2009.01263.x.
Cellular neurothekeoma is a benign lesion most commonly found on the face and upper extremities in the first two decades of life.
Retrospective clinicopathologic review of 12 examples of cellular neurothekeoma typified by prominent stromal sclerosis, a distinctive variant that we refer to as desmoplastic cellular neurothekeoma.
The mean age was 30 years (range, 3-55 years, 3 males, 9 females). The site was the head and neck in 3 cases, upper extremity in 4, lower extremity in 2, and trunk/abdomen in 3. All cases showed fascicles of slightly spindled and polygonal cells arrayed haphazardly in a prominent sclerotic background in the dermis and superficial subcutis. The cells displayed pale cytoplasm with indistinct membranes and vesicular nuclei with a single nucleolus. Lesional cells expressed NKI/C3, laminin, CD68, and CD10 and lacked expression of S-100 protein, EMA, and CD34. Clinical follow up was available on 10 cases with a mean duration of 24 months (range, 11-42 months) with no local recurrences or metastases.
The immunohistochemical staining pattern, clinical findings, and benign nature are similar to "conventional" cellular neurothekeomas. The differential diagnosis includes desmoplastic melanocytic lesions, desmoplastic spindle cell carcinoma, dermatofibroma, "immature" scar, plexiform fibrohistiocytic tumor, perineurioma, and piloleiomyoma.
细胞性神经鞘黏液瘤是一种良性病变，最常见于生命的前二十年，好发于面部和上肢。
对12例以显著间质硬化为特征的细胞性神经鞘黏液瘤进行回顾性临床病理分析，这是一种独特的变异型，我们称之为促纤维增生性细胞性神经鞘黏液瘤。
患者平均年龄30岁（范围3 - 55岁，男性3例，女性9例）。病变部位：头颈部3例，上肢4例，下肢2例，躯干/腹部3例。所有病例均显示在真皮和皮下浅层有显著硬化背景下，排列紊乱的轻度梭形和多边形细胞束。细胞胞质淡染，细胞膜不清，核呈泡状，有单个核仁。病变细胞表达NKI/C3、层粘连蛋白、CD68和CD10，不表达S - 100蛋白、EMA和CD34。10例患者有临床随访资料，平均随访时间24个月（范围11 - 42个月），无局部复发或转移。
免疫组化染色模式、临床特征及良性本质与“传统”细胞性神经鞘黏液瘤相似。鉴别诊断包括促纤维增生性黑素细胞病变、促纤维增生性梭形细胞癌、皮肤纤维瘤、“不成熟”瘢痕、丛状纤维组织细胞瘤、神经束膜瘤和毛发平滑肌瘤。
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