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

Cylinder (syn: Spiegler tumor, turbaned tumor, syringoma of the scalp, benign multiple basal cell epithelioma, etc.). Until the present udder, there is uncertainty about the histogenesis of the tumor.

Causes and pathogenesis of skin cylinders. The origin of the cylinders is unclear. They consider it to be an ecrcrinic tumor, but some believe that it develops from the apocrine glands, as well as the hair structures. The presence of family cases indicates an autosomal dominant type of inheritance.

Symptoms of skin cylinders. Neoplasm is manifested in the form of multiple solitary nodes on the skin of the head, neck, mainly in women in the 60-70 years. Approximately 10% of the cases of the cylinder are inherited but of an autosomal dominant type with varying degrees of penetrance. One of the significant features of the cylinder is its participation as one of the components of complex skin hamart, which can have a wide variety of combinations of the cylinder type - trichoepithelioma - parotid gland adenoma, cylinder - ecrina spiradenoma - parotid adenoma, cylinder - trichoepithelioma - milium in men of the same family in 3 generations, the hereditary multiple cylinder - gyerlipidemia type II, family cylinder - trichoepithelioma - milium - spiradenoma.

Clinically, the cylinder is a multiple knotty tumor formation with a smooth surface, a different shade of pink, a dense elastic consistency. Sometimes the tumor occupies almost the entire surface of the head, resembling a turban. Occasionally, the tumor can contain a cystic component that gives it a bluish tinge.

The disease is characterized by a slow development of solitary tumorous formations, mainly in young women, in some cases - in childhood. Tumors of rounded shape, dense to the touch, abruptly rise above the level of the skin, of various sizes, reach the size of large chestnuts, tomatoes. Fusing together, form conglomerates of tumors, covering in a number of cases the entire scalp (turbaned tumor). The skin above the tumor is devoid of hair, has a faint or bright pink color. Large and old elements are permeated with telangiectasia. 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 from cells similar to basal layer cells containing hyaline surrounded by a hyaline membrane are noted. Within the tumor islets there are two types of cells: in the center of the cell have a light-colored nucleus of the oval form, but the periphery of the cell, located in the form of a gulisade, of smaller dimensions, has dark-colored nuclei.

Pathomorphology. The tumor is localized in the dermis and subcutaneous adipose tissue. The epidermis over the tumor is thinned, with smoothed intergrowth outgrowths. The tumor consists of lobules of various sizes, usually round in shape, containing 2 types of cells: in the central parts of the cell with large yatras and abundant cytoplasm, and on the periphery of the cell with small nuclei and scant cytoplasm, sometimes forming palisade structures. The lobes are fringed with thick eosinophilic hyaline-like deposits, which are the substance of the multiplicated basal membrane and give the lobules a resemblance to the "cylinders". Partly the accumulations of this substance are visible inside the lobules between the tumor cells. The flow structures in some lobules have a lumen bounded by prismatic cells with an eosinophilic cytoplasm and contain a cuticle on the luminal surface. Occasionally, tubules, expanding, form cystic structures. There are foci of keratinization and follicular differentiation. A 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 basal membrane - collagen IV and V types. Laminin, fibronectin, proteoglycans. The substance gives a positive PAS-response and is resistant to diastase. In studies with cell culture, it has been shown that a membrane-like substance is produced by tumor epithelial cells. In connection with the predominance of these or those structures, four morphological types of cylinders are distinguished: undifferentiated, hydratenomatous, trichoepitheliomatous and mixed.

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

The hydratenomatous type is characterized by the presence of cavities in the cells described above, reminiscent of the structure of the excretory ducts of the sweat glands.

In the trichoepithelioma type, in addition to the cavities and cysts, structures that indicate differentiation in the direction of the hair follicles are determined. There are flat-epithelial cysts, similar to those with trichoepitheliomas. Differential diagnosis of cylinders is not difficult, it is based on the detection of thick eosinophilic membranes surrounding its lobules.

Immunomorphological studies reveal a rather variegated picture, in particular, the expression of a-antihemhemotrypsin, lysozyme, globulin factor 1 of female milk, a-smooth muscle actin and cytokeratins 8 and 18, which is interpreted as a sign of histogenetic association with the secretory department of the apocrine gland. At the same time, positive expression of nerve growth factor, protein S-100, CD44, CD34 is regarded as evidence of histogenetic connection with the secretory department of 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 departments, also revealed cytokeratin 14, characteristic for duct differentiation.

Histogenesis. In the WHO histological classification, the cylinder is placed both in the section of 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 cells look immature. The secretory cells contain granules similar to those in the cells of the eccrine glands, but the connection between the cells of the cylinders and the hair follicles attests to the apocryphal differentiation of the cylinders. From these positions one can once again confirm the validity of the conclusions of A.K. Apatenko (1973), who, noting the presence in the cylinder of signs of eccrine, apocrine and piloidal differentiation, regarded it as a histogenetically heterogeneous tumor, the source of which are various combinations of elements of the epidermis and embryonic rudiments of the pilosebate and apocrine complex.

Differential diagnosis. The disease must be differentiated with basaloma, dermatofibrosarcoma, lipomatosis.

Treatment of skin cylinders. Large tumors are removed surgically, sometimes using plastic, cryodestruction.

[1], [2], [3], [4], [5], [6], [7]