背景:纤维瘤病样神经肿瘤(LPF-NT)是一种罕见的软组织,通常发生在皮下组织,以CD34和S100蛋白阳性纺锤形肿瘤细胞的细胞增殖为特征,具有浸润性生长模式。
目的:描述5例主要出现在真皮中,以扩大其形态范围。
方法:审查了H&E幻灯片,所有病例均进行CD34、SOX10、S100、ALK、和NTRK1,其中一些带有额外的染色。
结果:患者为3名男性和2名女性,平均年龄为44.8岁(14-68岁)。组织病理学,所有病例的特征是单一形态的致密真皮浸润,轻度非典型,丰满到纺锤形的肿瘤细胞,CD34、S100和NTRK1染色呈弥漫性阳性,但S100、EMA阴性,NKIC3,MNF116,SMA,ALK,和Desmin。
结论:临床信息有限。
结论:LPL-NT主要位于真皮。我们的病例中有60%显示LPL-NT的典型区域与更丰满的细胞混合,例如婴儿期纤维错构瘤中的细胞。我们建议不仅在皮下梭形细胞肿瘤中,而且在主要涉及真皮的肿瘤中,都使用一组CD34,S100和NTRK1抗体,以便做出准确的诊断。
BACKGROUND: Lipofibromatosis-like neural tumor (LPF-NT) is a rare soft tissue typically occurring in the subcutis, characterized by a cellular proliferation of CD34- and S100-protein positive spindle-shaped tumor cells with an infiltrative growth pattern.
OBJECTIVE: To describe five cases arising mainly in the dermis in order to expand their morphologic spectrum.
METHODS: H&E slides were reviewed, and all cases were stained for CD34, SOX10, S100, ALK, and NTRK1 and some of them with additional staining.
RESULTS: Patients were three males and two females with a mean age of 44.8 years (14-68 years). Histopathologically, all cases were characterized by a dense dermal infiltration by monomorphous, mildly atypical, plump to spindle-shaped tumor cells, staining diffusely positive for CD34, S100, and NTRK1 but were negative for S100, EMA, NKIC3, MNF116, SMA, ALK, and desmin.
CONCLUSIONS: Limited clinical information.
CONCLUSIONS: LPL-NT can be located mainly in the dermis. Sixty percent of our cases showed typical areas of LPL-NT intermingled with more plump cells like the ones in fibrous hamartoma of infancy. We recommend a panel of CD34, S100, and NTRK1 antibodies not only in subcutaneous spindle cell neoplasms but also in the ones predominantly involving the dermis in order to make an accurate diagnosis.