会阴部包块 基底毛囊错构瘤讨论病例【中美加宫颈病理交流群病例139】

小王

陈晓微
我上个会阴部位的病变。78岁，发现会阴部小包块，略黑，稍隆起，0.5CM。诊断、鉴别诊断和治疗？

陈健
考虑毛囊或毛母，鉴别基底

张恒明
毛母细胞瘤

石丽华
毛母鉴别基底

陈晓微
基底毛囊错构瘤（basal follicular harmatoma )主要的鉴别诊断是基底细胞癌。由于病人的年龄，建议按基底细胞癌处理。这是我从我们这里的皮肤病理医生那里学来的，分享一下

景晓峰
@陈晓微，纽约 同意你的诊断，诊断毛囊瘤也可以。最近我有一个累似的肿瘤，发生在背部，分化成熟的毛囊更明显。由于这是宫颈学的群，不便发在这里与大家分享。

陈晓微
@景晓峰-中车永济电机医院病理科 我也犹豫的一会儿，还是送上来分享了像我这样对皮肤病理了解不多的，应该是很好的学习。经过与皮肤病理医生探讨，我翻了书，其实还是很典型的形态特征，只是我们不常看。

汪俊
@陈晓微，纽约 陈老师，为什么处理方式跟基底细胞癌一样的呢？

陈晓微
@汪俊（香港大学深圳医院）群管 这是皮肤病理医生的建议。这也是为什么我送上来的原因。我们诊断，但很少建议怎么处理。皮肤病理医生说因为病人的年龄大，要稍微激进点

李国霞
basaloid follicular harmatoma，基底细胞样毛囊错构瘤。
Basaloid follicular hamartoma (BFH) is a benign adnexal tumour characterized by superficial dermal proliferation of thin basaloid cords in a fibrous stroma.
These lesions are regarded as malformation of hair follicle.
Basaloid follicular hamartoma may be congenital or acquired.
The congenital form can be further divided into the generalized and unilateral type.
The acquired form may present as localized and solitary lesions.
Congenital, generalized Basaloid follicular hamartoma (BFH) is associated with systemic diseases such as alopecia, cystic fibrosis, hypohidrosis, and myasthenia gravis.
Sporadic cases present as unilateral or localized lesions.
These lesions are usually located on the scalp, face and rarely on the chest, back and extremities.
Clinically, basaloid follicular hamartoma presents as a brown or skin coloured papule or plaque.
Microscopic features:The tumour shows thick cords and thin strands of anastomosing basaloid proliferations that arise from hair follicles and are enclosed by loose fibrous stroma.
Immunohistochemistry: The lesion shows CD34-positive, mildly cellular myxoid stroma with many CK20-positive Merkel cells scattered within the basaloid cellular strands, which exhibited BCL-2 positivity only within the outermost cells bordering the stroma.
These immunohistochemical features are the opposite of those displayed by basal cell carcinomas, which require more aggressive surgical management.

陈晓微
@李国霞，上海闵行 谢谢啦我没做IHC

xiaofeng

基底毛囊错构瘤，第一次见到，谢谢分享！