BCC伴附件分化（basal cell carcinoma with adnexal differentiation）这种变异型在临床上和预后方面，与其它类型BCC没有区别。它主要是BCC的一个组织学分类和鉴别诊断问题。

组织学上，表现为两个方面。一方面是BCC的成分，例如连接表皮的基底细胞蓓蕾样的结构，具有BCC特征的片巢以及巢周边裂隙等，可以表现出前述任何一种BCC的形态结构（经典的或其它诸如腺样或管状的、色素性、硬化性的等变异型成分）；另一方面，表现出各种皮肤附件成分分化，多见的是毛源性分化（如毛鞘上皮、毛乳头等），也可见皮脂腺甚至汗腺分化。

具有汗腺分化的时候要特别注意与汗腺癌鉴别，因为其预后不一样。

BCC伴附件分化中的有的病例，可能会与皮肤附件肿瘤重叠。这时，要注意重叠的良性成分的含量。如果说是以良性成分占绝对优势，有学者建议，这种情况最好还是诊断为良性附件肿瘤，如：毛发上皮瘤、毛母细胞瘤和毛鞘瘤，甚至基底细胞样毛源性错构瘤等。

本例的组织病理形态可以看出，肿瘤与表皮关系密切，巢周裂隙易见，基底样细胞的区域占优势，细胞巢的形状甚不规则，有的区域呈浸润状，间有毛源性分化。IHC标记BerBP4阳性较强，而且Ki-67标记增殖指数较高。患者年龄偏大，结合生长部位，诊断为BCC伴毛源性分化可能更为妥当。

仅供参考！

 本例的分类在BCC伴附件分化的范畴，它在组织学上是以皮肤附件分化为特征的变异型的BCC。

从组织发生学来看，它与毛母细胞瘤相同起源，表达的CK谱也可有相同之处，但其表型类同于不成熟的毛母细胞瘤，而且表现出胎儿毛囊早期发育的一些特征，并向任何一种前述类型的BCC形态过渡，抑或表现出毛鞘-皮脂腺单位的分化。所以，有的学者建议，将这样的BCC再命名为：毛母细胞癌。但这个命名目前还未被正式分类所接受，仍然称为BCC伴附件分化。

 基底细胞癌的形态复杂多变。是所有单一病种的上皮性肿瘤中分型最为复杂的恶性肿瘤。参阅WHO分类，对基底细胞癌（BCC）的分型如下：

1）浅表型BCC

2）结节型BCC

3）微小结节型BCC

4）浸润性BCC

5）纤维上皮型BCC

6）BCC伴附件分化

7）BCC鳞状分化（或译为基底细胞鳞癌，不同其它器官的基底样鳞癌）（basaosguamous carcinoma）

8）角化型BCC

9）其它变异型：

   （1） 囊性BCC

    （2）腺样BCC

    （3）硬化性BCC

    （4）漏斗形囊状BCC

    （5）色素性BCC

    （6）其它罕见的杂类：

         a. 透明细胞型BCC

         b. “印戒细胞”型BCC

         c. 颗粒细胞型BCC

         d. 巨细胞型（怪异细胞型）BCC

         e. 釉质样BCC

         f. 内分泌型BCC

         g. 神经鞘样BCC

 谢谢金主任！

我将查阅些文献，再做小结。

 这是金主任帮我回答了关于诊断的问题

XLJin8  文章：1290 积分：7265 经验：1608 注册：2009-3-4 3:39

发表于 2010-6-12 8:50 {资料} {好友} {短信} {引用} {快回} {编辑} {删除}  第 17 楼       ......毛母细胞瘤主要发生在头皮, 肿瘤1厘米左右大小。毛胚基样基底样细胞巢和纤维间质二种成分构成.....肿瘤主要位于真皮内，与表皮不相连......基底样细胞巢的周边细胞排列成栅栏状, 与间质紧密相连, 无收缩裂隙......纤维性间质围绕细胞巢,可形成透明胶原带...... ......少数肿瘤的形态与基底细胞癌可有部分重叠, 如鉴别诊断困难, 诊断为”恶性附件肿瘤伴毛囊分化” (Malignant adnexal neoplasm with desc ription of  apparent differentiation)。 参考文献: Lever’s Histopathology of the Skin, 9th edition, 2009;861-862. 参考文献: Lever’s Histopathology of the Skin, 9th edition, 2009;861-862.

谢谢金主任！

 这是一位网友对诊断的回答

wang4160  文章：1151 积分：5660 经验：1247 注册：2007-1-31 18:12

发表于 2010-6-11 9:10 {资料} {好友} {短信} {引用} {快回} {编辑} {删除}  第 5 楼        倾向基底细胞癌！ 阿克曼上说：基底细胞癌与毛母细胞瘤的鉴别本身就是个难点，来源与组成差不多，分化方向也差不多，同时也说了一句似乎只有癌会发生浸润现象！也就是说对周围组织的浸润成为诊断的关键！另外从表浅型基底细胞癌来看，似乎基底细胞大多起源于表皮基底层（应该也能起源于附属器基底细胞层），因此与表皮的关系，能否成为一个辅助指标呢？？ 有人提到收缩裂隙，这张片子可以看到，不知道能否作为诊断依据！！！ 个人理解，请各位老师指教！

 有幸请上海著名皮肤肿瘤病理专家—皮肤病理老前辈—孔金诚教授会诊该病例。

孔教授会诊意见：基底细胞癌，伴毛源性分化。

谢谢孔教授！

 BerEP4在汗腺和外毛鞘发生的肿瘤也可阳性，但毛鞘表达BerEP4不如在BCC表达的强。

本例主要是区分BCC和毛母。

谢谢Dr,djdnx.

 突击学习，本例形态学集中在基底细胞癌和毛母细胞瘤的鉴别。这两种瘤形态学相似，老师就其鉴别诊断的免疫组化分析提出问题，就BerEP4可以做为基底细胞肿瘤和鳞状细胞癌鉴别的重要marker，但是在基底细胞癌和毛母细胞瘤（Modern Pathology (2008) 21, 178–185; doi:10.1038/modpathol.3801000; published online 7 December 2007，57% of desmoplastic trichoepithelioma）中都有表达，

明天在学习继续写

续上篇

Basal cell carcinoma vs. trichoblastoma (trichoepithelioma)

Immunopanel—BerEP4, CD10, Bcl-2, CD34, CK20, Cam5.2

In trichoblastomas, CD10 typically highlights the peritumoral stroma, including papillary mesenchymal bodies, with minimal patchy staining of basaloid cells. In contrast, in BCC, the stroma is negative and basaloid cells variably strongly positive with CD10. Diffuse Bcl-2 positivity is reported in BCC, whereas the basal layer alone is highlighted in TE. The authors have found this to be variable and unreliable in practice. CD34 may highlight the peritumoural stroma in the desmoplastic variant of TE and not in infiltrative BCC, but this is also an unreliable finding in the authors’ experience. Merkel cells can be highlighted with Cam5.2 or CK20 and are absent from BCC but increased in number in trichoblastoma.

MINI-SYMPOSIUM: CUTANEOUS EPITHELIAL TUMOURS

The use of immunohistochemistry in the differential diagnosis of common epithelial tumours of the skin

D.S.A. Sanders ^{, a,} and R.A. Carr^a

^aDepartment of Pathology, Lakin Road, Warwick CV34 5BJ, UK

Available online 25 August 2007.

 

BerEP4

BerEP4 is a monoclonal antibody to a 34/49-kDa glycoprotein on the surface of most epithelial cells, with the exception of superficial layers of squamous epithelia.¹ BerEP4 stains the vast majority of non-cutaneous epithelia and is highly conserved in the tumours derived from them, including non-cutaneous squamous cell carcinomas (SCC).¹ In normal skin, there is reported positivity of the lower part of telogen hairs (secondary hair germ), matrix and outer root sheath (ORS) of vellus hairs, early anagen of terminal follicles (but not any part of mature anagen follicles), and the lower part of the epithelial strand of late catagen follicles.^{[2] and [3]} Similar strong positive staining is seen in lining cells of eccrine and apocrine coils, with more variable staining of sweat duct lining cells and the acrosyryngium.3 F.J. Jimenez, J.L. Burchette Jr, J.M. Grichnik and M.G. Hitchcock, Ber-EP4 immunoreactivity in normal skin and cutaneous neoplasms, Mod Pathol 8 (1995), pp. 854–858. View Record in Scopus | Cited By in Scopus (32)³ The epidermis, follicular mantle, sebaceous glands and all non-epithelial tissues are negative^{[1] and [3]} (Fig. 1A–C).

Figure 1. BerEP4 staining in normal skin and basal cell carcinoma (BCC): (A) weak staining in upper eccrine duct and acrosyryngium; (B) weak staining in lower sweat duct and strong staining in sweat gland coils; (C) outer root sheath telogen and anagen vellus buds (inset showing perifollicular Merkel cells); (D) nodular BCC; (E) metatypical and infiltrative BCC with weaker staining; (F) sebeorrhoeic keratosis (left side negative) in collision with superficial BCC; (G) fibroepithelioma of Pinkus; and (H) perineural invasion from a micronodular BCC.

 

 

Carcinoembryonic antigen

Carcinoembryonic antigen (CEA) is a glycoprotein initially described as a colonic oncofetal antigen,³² but subsequently shown to be expressed in a variety of normal human tissues³³ including squames.³⁴ Anti-CEA antibodies are now designated ‘CD66’ with a subclassification a–e depending on which epitope of the antigen is recognized. In normal skin, membranous CEA positivity can occasionally be seen in suprabasal squamous cells of the epidermis, and on the inner lining of sweat gland coils (including canaliculi), dermal ducts and acrosyringium.³⁵ Monoclonal CEA can be used as a marker of ductal/glandular differentiation in skin tumours, and may also highlight mature squamous differentiation. However, in the authors’ experience, immunostaining is sometimes inconsistent and less sensitive for lumina than EMA.

 

 

Bcl-2, p53 and Ki-67

Bcl-2 is an anti-apoptotic protein residing on the outer mitochondrial membrane. It is implicated in the pathogenesis of several common cancers by inhibiting programmed cell death. In normal skin, Bcl-2 stains the majority of keratinocytes in the basal epidermis, cells of the ORS, mesenchymal cells of the follicular papillae, and clear cells of eccrine glands.⁵⁷ Diffuse cytoplasmic Bcl-2 expression is reported in BCC^{[57] and [58]} and is reported to be useful in the distinction of BCC (diffuse staining) from TE (staining of basal layer only)⁵⁹ and BCC and solar keratosis (latter negative).⁶⁰

p53 is a tumour-suppressor gene and many p53 mutations result in a protein product that is unusually stable and becomes detectable by immunohistochemistry. Ultra-violet light is known to induce both overexpression of wild-type p53 and cause specific mutations in the p53 gene, and a discrepancy between cutaneous tumours with positive immunostaining and those with mutation has been reported.⁶¹ Nuclear accumulation of p53 protein is a feature of the majority of malignant adnexal and epidermal tumours including BCC,⁶² SCC and Bowen's disease,⁶³ and is confined to the areas of basal atypia in solar keratosis.⁶³ Hence, p53 immunostaining may have some utility in the differential diagnosis of intra-epidermal tumours (Fig. 7) and aids the distinction between basaloid proliferations (follicular induction) over dermatofibromas (rarely p53 positive) and the superficial subtype of BCC that they resemble.⁶⁴ p53 immunostaining is of lesser value in differentiating benign from malignant skin tumours, as evidenced by similar staining patterns reported between BCC and TE, and eccrine poroma and porocarcinoma.^{[65] and [66]}

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Full-size image (175K)

Figure 7. Clonal Bowen's: (A) low power of a clonal tumour and (B) high power showing uniform but atypical, acantholytic cells. Negative Cam5.2 is against extramammary Paget's and negative S100 against melanoma. p53 diffusely strongly positive.

View Within Article

 

 

Ki-67 (MIB 1) is a proliferation marker with a pattern of nuclear positivity. Demonstration of the proliferation index in skin tumours, in conjunction with the mitotic index, is used by many pathologists to help differentiate between benign and potentially malignant tumours. p53 positivity in conjunction with Ki-67 positivity is reported to be a feature of malignancy in the differential diagnosis of hidradenoma from hidradenocarcinoma, although histological parameters remain paramount.⁶⁷

 

原文太长，截取部分，希望对大家分析有帮助，继续学习

 BerEP4和CK 20是鉴别基底细胞癌和其它皮肤附件肿瘤的比较好的标志物，基底细胞癌表达前者阳性，其它类型一般为阴性。后者表达的意义则相反。本例标记Ki-67的阳性率在不同区域约45-70%.

请参阅现有的IHC标记结果继续发表意见。谢谢！

 等待学习

IHC:

图1 BerEP4； 图2 Ki-67； 图3 CK20; 图4 CEA