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Adnexal Tumours of the Skin J Clin Pathol: First Published As 10.1136/Jcp.44.7.543 on 1 July 1991

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Adnexal Tumours of the Skin J Clin Pathol: First Published As 10.1136/Jcp.44.7.543 on 1 July 1991 J Clin Pathol 199 1;44:543-548 543 Troublesome tumours 1: Adnexal tumours of the skin J Clin Pathol: first published as 10.1136/jcp.44.7.543 on 1 July 1991. Downloaded from D Cotton Introduction these are very unusual,6 and the confusion due Most adnexal tumours are benign and, if com- to the term "cylindroma" being used for a pletely excised, cause no further concern. It different, malignant, tumour of other sites may therefore be thought that there is little causes considerable difficulty. Again, duct dif- need for further subclassification. The major ferentiation is CEA positive, but the bulk of arguments for considering them further can tumour cells in all these tumours (poromas, be summarised as follows: (1) if you are not spiradenomas, and cylindromas) are CEA sure what it is, it may be something else; (2) negative. All of the above mentioned tumours clinical associations with specific subtypes will have features reminiscent of the sweat gland not become apparent if the lesions are never on electron microscopical examination and subtyped; and (3) there is academic and obses- they stain variably positive with middle sional satisfaction to be derived from weight cytokeratin antibodies such as PKKI meticulously identifying lesions as accurately and are negative for CAM5 2, S100, epithelial as possible. membrane antigen (EMA) and human milk Given these justifications I will comment on fat globule 1 (HMFG 1). what I consider to be useful and interesting Poroma, spiradenoma, and cylindroma are aspects of certain adnexal tumours. The first all derived from the outer cells of the duct and division is into tumours showing affinity with behave as benign "epitheliomas" or eccrine glands and those showing affinity with "basalomas" as these terms are variously used the pilosebaceous system. The question of by Lever7 and Pinkus.8 They are the whether tumours are derived from a given equivalent of those basal cell carcinomas structure or from some primordial and, at which arise from the pilosebaceous unit. least, pluripotential stem cell is unresolved, Their largely benign nature is probably due to probably unresolvable, and is, anyway, of the fact that, unlike the hair follicle, the little practical concern; the important point is eccrine sweat gland does not actively cycle and that tumours do resemble normal structures Hair proliferate. follicle epitheliomas are http://jcp.bmj.com/ and that this enables us to classify them and, much more akin to epidermal epitheliomas as our experience of them accumulates, to because the epidermis also proliferates and predict their behaviour. regenerates actively, and such lesions con- stitute the common basal cell carcinomas. Malignant transformation of these eccrine Eccrine sweat gland tumours tumours is indicated by cellular pleomor- sweat The eccrine gland consists of several phism, invasion, and high mitotic rate; ab- on September 30, 2021 by guest. Protected copyright. morphological and functional regions and normal mitotic figures are generally highly there is at least one recognisable tumour for suggestive of malignancy. each of these (fig 1). The outer cells of the The cells lining the inner aspect of the coiled intraepidermal duct give rise to the eccrine duct and those of the secretory coil are eccrine poroma. This tumour may nest within concerned with active secretion/resorption the epidermis as hidroacanthoma simplex, or processes and their benign neoplasms are it may become detached from the epidermis adenomas-the so-called hidradenomas. These and occur as an intradermal nodule, termed a are more prone to malignant transformation dermal duct tumour; actual ducts are rare but and occur as simple adenocarcinomas termed are positive with carcinoembryonic antigen hidradenocarcinomas. Because the cells lining (CEA) antibody when they occur. Malignant the duct have different functions and express poromas have been clearly described and their different markers from those forming the behaviour is relatively unpredictable.' It has secretory coil, the hidradenomas are rather been claimed that the clear cell acanthoma of variable in form and in their marker expres- Degos has a similar histogenesis,2 but the sion. Those derived from the duct show gen- evidence for this is poor and the lesion is eral staining with CEA antibody in most of Department of probably reactive rather than neoplastic.3 4 their cells; those from the secretory coil show Pathology, University The intradermal straight duct gives rise to CAM 5-2 and S100 positivity but no CEA of Sheffield Medical eccrine School, Beech Hill spiradenomas, and again ductular dif- antibody positivity.' The intercellular can- Road, Sheffield ferentiation is rare but CEA positive when it aliculi of the secretory coil express HMFG1 S10 2RY occurs. Highly vascular and giant forms5 and EMA positivity, and small spidery areas D Cotton occur which can give some diagnostic dif- corresponding to this can be seen to stain with Correspondence to: ficulty but malignant transformation is again these markers in many Most Dr D Cotton hidradenomas. rare. The coiled duct seems to be the origin of hidradenomas show a mixture of differentia- Accepted for publication 21 November 1990 eccrine cylindromas; malignant variants of tion towards both duct lining cells and 544 Cotton The eccrine sweat gland '.-.lf i Hidroacanthoma simplex Intraepidermal duct C.inPoroma :: 4 ~~~~~Dermal duct tumour J Clin Pathol: first published as 10.1136/jcp.44.7.543 on 1 July 1991. Downloaded from Syringoma Spiradenoma Intradermal duct straight Hdaeoa Secretory coil (Hidradenoma) Figure 1 Variousfunctional regions of the eccrine sweat gland and the tumours derivedfrom them. secretory coil cells sometimes recapitulating the normal gland with "ductal" areas nearer the epidermis and "secretory coil" areas deeper in the skin. In some hidradenomas the outer cells differentiate to myoepithelial cells and either produce or induce large volumes of stromal mucins. This compresses the aden- Hair papilla omatous part of the lesion and produces the Figure 2 Vertical section ofhairfollicle showing the characteristic mixed tumour of skin or chon- various morphological elements. droid syringoma. The stroma differs from nor- mal dermis in being mucopolysaccharide-rich epithelial interactions peculiar to the hair folli- and is the counterpart of the "private stroma" cle and which can be recognised in distorted seen around the normal sweat gland. All form within some tumours. As with most varieties of hidradenomas can be malignant adnexal tumours, the main area of concern is and the criteria are those for adenocarcinomas distinguishing between benign and malignant of any site. Whatever their nature, no eccrine lesions; unfortunately there are no reliable tumour, unless it is very large and very old, morphological correlates to help in the mar- ever shows calcification; this is the hallmark of ginal cases. pilosebaceous tumours. Tumours ofthe hair follicle show differentia- tion towards many of the normal follicular elements and are generally named accordingly. Pilosebaceous tumours It is much more difficult to confirm these http://jcp.bmj.com/ While all skin adnexal structures develop in a morphological impressions with techniques similar manner from embryonic epidermal such as immunocytochemistry and electron anlagen, the convention has been to separate microscopy than is the case with sweat gland eccrine sweat glands from pilosebaceous com- tumours. The reason why immunocyto- plexes and to view the latter as a somewhat chemistry is less useful in classifying hair integrated entity. This seems logical as the follicle tumours than eccrine sweat gland biological functions of apocrine and sebaceous tumours is that the different layers of the hair on September 30, 2021 by guest. Protected copyright. glands seem to be related to maintaining the follicle represent metabolic variations on the "condition" of hair and the whole complex is theme of keratinisation and there is consider- morphologically closely related. When it able overlap in specificity to the available cyto- comes to adnexal tumours, however, there are keratin markers. In eccrine sweat glands dif- clearly some which are apocrine, some which ferent areas of the gland express markers that are sebaceous, and a large number which are show almost no overlapping specificities (CEA, pilar, notwithstanding the fact that there are HMFG1, S100, CAM5-2, PKK1) (table 1). also many The themselves The various benign hair follicle tumours hybrids. hybrids have been reviewed and classified most satisfac- support this classification as I see many sweat in and little has gland tumours with apparently mixed sweat torily by Headington 1976, gland histogenesis but relatively few that also been added since that time.'0 Malignant hair show any pilosebaceous differentiation. follicle tumours have been recently reviewed tumours show by Wick and Coffin, although they comment Similarly, pilar may apocrine ... been and sebaceous areas, but very rarely do they that, "... only a handful have show eccrine With these documented."." differentiation. Table 2 is modified from Headington's paper reservations in mind I will review some exam- of hair ples of pilosebaceous tumours under the and shows the histogenic classification various subheadings of pilar, apocrine, and follicle tumours based on morphology. This classification is based not only on the perceived sebaceous neoplasms. morphological similarities between the tumours and the normal components ofthe hair HAIR FOLLICLE
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