Cylindroma of the skin: causes, symptoms, diagnosis, treatment

Cylindroma (syn.: Spiegler's tumor, turban tumor, syringoma of the scalp, benign multiple basal cell epithelioma, etc.). Until now, there is uncertainty regarding the histogenesis of the tumor.

Causes and pathogenesis of skin cylindroma. The origin of cylindroma is unclear. It is considered an eccrine tumor, but some believe that it develops from apocrine glands, as well as hair structures. The presence of familial cases indicates an autosomal dominant type of inheritance.

Symptoms of skin cylindroma. The neoplasm appears as multiple solitary nodes on the skin of the head, neck, mainly in women aged 60-70. In about 10% of cases, cylindroma is inherited in an autosomal dominant manner with varying degrees of penetrance. One of the significant features of cylindroma is its participation as one of the components of complex skin hamartomas, which can have a variety of combinations such as cylindroma - trichoepithelioma - adenoma of the parotid gland, cylindroma - eccrine spiradenoma - adenoma of the parotid gland, cylindroma - trichoepithelioma - milium in men of one family in 3 generations, hereditary multiple cylindroma - hyperlipidemia type II, familial cylindroma - trichoepithelioma - milium - spiradenoma.

Clinically, cylindroma is multiple nodular tumor formations with a smooth surface, various shades of pink, and a dense elastic consistency. Sometimes the tumor occupies almost the entire surface of the head, resembling a turban. Rarely, the tumor may contain a cystic component, giving it a bluish tint.

The disease is characterized by the slow development of solitary tumor-like formations, mainly in young women, in some cases - in childhood. Tumor-like formations are round in shape, dense to the touch, sharply rise above the skin level, of varying size, reaching the size of large chestnuts, tomatoes. Merging with each other, they form conglomerates of tumors, in some cases covering the entire scalp (turban tumor). The skin above the tumor is devoid of hair, has a weak or bright pink color. Large and old elements are penetrated by telangiectasias. Tumors are localized mainly on the scalp and face, less often - on other areas of the skin.

Histopathology. In the dermis, multiple papillomas and nests of cells similar to those of the basal layer, containing hyaline, surrounded by a hyaline membrane are noted. Within the tumor islands, there are two types of cells: in the center, the cells have a light-colored oval-shaped nucleus, but the periphery of the cells, located in the form of a gyrus, are smaller in size, and have dark-colored nuclei.

Pathomorphology. The tumor is localized in the dermis and subcutaneous adipose tissue. The epidermis above the tumor is thinned, with smoothed interpapillary outgrowths. The tumor consists of lobules of various sizes, usually rounded, containing 2 types of cells: in the central sections, cells with large nuclei and abundant cytoplasm, and along the periphery, cells with small nuclei and scanty cytoplasm, sometimes forming palisade structures. The lobules are bordered by thick eosinophilic hyaline-like deposits, which are the substance of the multiplexed basement membrane and make the lobules look like "cylinders". Partial accumulations of this substance are visible inside the lobules between the tumor cells. Ductal structures in some lobules have a lumen limited by prismatic cells with eosinophilic cytoplasm and contain cuticle on the luminal surface. Occasionally, tubules expand to form cystic structures. There are foci of keratinization and follicular differentiation. The tumor stroma sometimes contains a significant amount of mucin, in which hyaluronic acid is determined. The eosinophilic substance located around and inside the tumor lobules contains all the components of the basement membrane - collagen types IV and V. laminin, fibronectin, proteoglycans. The substance gives a positive PAS reaction and is resistant to diastase. In works with cell culture, it was shown that the membrane-like substance is produced by the epithelial cells of the tumor. Depending on the predominance of certain structures, four morphological types of cylindroma are distinguished: undifferentiated, hidradenomatous, trichoepitheliomatous and mixed.

In the undifferentiated type, the tumor cells are small with intensely stained nuclei, arranged in the form of cells surrounded by hyaline-like membranes.

The hidradenomatous type is characterized by the presence among the cells described above of cavities that resemble in structure the excretory ducts of sweat glands.

In the trichoepitheliomatous type, in addition to cavities and cysts, structures are determined that indicate differentiation in the direction of hair follicles. Flat epithelial cysts are encountered, similar to those in trichoepitheliomas. Differential diagnostics of cylindroma is not difficult, based on the detection of thick eosinophilic membranes surrounding its lobules.

Immunomorphological studies reveal a rather varied picture, in particular the expression of a-antichemotrypsin, lysozyme, human milk globulin factor 1, a-smooth muscle actin and cytokeratins 8 and 18, which is interpreted as a sign of a histogenetic connection with the secretory section of the apocrine gland. At the same time, positive expression of nerve growth factor, S-100 protein, CD44, CD34 is regarded as evidence of a histogenetic connection with the secretory section of the eccrine glands. A similar picture is revealed by M. Meubehm, HP Ficher (1997), who, in addition to the cytokeratin profile (7, 8, 18), characteristic of the secretory sections, also identified cytokeratin 14, characteristic of ductal differentiation.

Histogenesis. In the histological classification of the WHO, cylindroma is included in the section of both benign eccrine tumors and apocrine tumors. Electron microscopy reveals two types of cells: undifferentiated basal cells with small dark nuclei and cells with large light nuclei. Most of the cells appear immature. Secretory cells contain granules similar to those in the cells of the eccrine glands, but the connection of cylindroma cells with hair follicles indicates apocrine differentiation of cylindroma. From this position, we can once again confirm the validity of the conclusions of A.K. Apatenko (1973), who, noting the presence of signs of eccrine, apocrine and piloid differentiation in cylindroma, considered it a histogenetically heterogeneous tumor, the source of which are various combinations of epidermal elements and embryonic rudiments of the pilosebaceous and apocrine complex.

Differential diagnosis. The disease must be differentiated from basalioma, dermatofibrosarcoma, lipomatosis.

Treatment of skin cylindroma. Large tumors are removed surgically, sometimes plastic surgery and cryodestruction are used.

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