Papillary hidradenoma of the skin: causes, symptoms, diagnosis, treatment

Hidradenoma papillaris is a rare benign multilobulated tumor of the apocrine sweat glands, localized in the dermis.

P. Abenoza, AB Ackerman (1990) from 1979 to 1987 among 750 thousand biopsies found only 219 hidradenomas - in 5% of cases these were eccrine (poroid) hidradenomas, in 95% - apocrine (clear cell) hidradenomas. 99% of tumors were solitary. The ratio of men and women was 1:1. 80% of patients were over 40 years old. In 50% of cases, hidradenoma was localized on the skin of the face and scalp, in 21% - on the skin of the extremities, in 20% - on the trunk. Contrary to popular belief, only in 6 cases the tumor was localized in the skin of the mammary gland and not a single case of tumor localization on the vulva and perianal area was registered.

The causes and pathogenesis of hidradenoma papillae cutanea are unknown.

Symptoms of papillary hidradenoma of the skin. Clinically, hidradenoma is a cyst-like, clearly demarcated cutaneous and/or intradermal nodule, grayish-bluish in color, sometimes with the release of light or bloody fluid, with an average diameter of 1-3 cm.

The disease is easy to diagnose. It is characterized by the formation of tumor-like formations in women in the area of the labia majora, perineum, and less often in other areas of the skin where there are apocrine sweat glands.

The tumor is spherical in shape, soft in consistency, mobile, painless, the diameter varies from 1-2 to 4-5 cm. Large tumors protrude above the surface of the skin.

Histopathology. In the deep layers of the dermis, a tumor surrounded by a capsule, not connected to the epidermis, is noted. The tumor is glandular in structure, consists of tubules and cystic formations lined with prismatic cells.

Pathomorphology. The authors distinguish two main forms of hidradenoma - poroid (with eccrine differentiation) and clear cell (apocrine). The first variant at low magnification is multilobular with a different ratio of solid and cystic components. The tumor is more often cystic, localized in the deep parts of the dermis, surrounded by a fibrous capsule, and is not connected with the epidermis covering it. The cyst walls are lined with keratinized epithelium, tubular structures and papillary outgrowths are found in the lumen. Poroid (eccrine poroma type) and cuticular cells, foci of necrosis, nuclear polymorphism, and sometimes multinuclear macrophages are determined cytologically. Poroid cells are small, dark, with scanty cytoplasm, cuticular cells are larger, with light cytoplasm and intercellular bridges, resembling cuticular cells of a normal duct of an eccrine sweat gland. The poroid variant of hidradenoma must be differentiated from simple hydracanthoma, eccrine poroma - in both cases, hidradenoma is not associated with the epidermis.

Clear cell hidradenoma is divided into solid, cystic and solid-cystic variants. According to the cellular composition, they may contain clear, squamoid (eosinophilic, granular, polygonal), mucinous cells and apocrine cells lining tubular structures. The latter are present in almost any variant of hidradenoma. The tumor is usually located in the dermis, but sometimes a continuous connection with the hyperplastic epidermis and the follicle infundibulum is noted. The cytoplasm of clear cells has a high content of glycogen, which is removed during treatment with diastase. Squamoid cells have a round or oval nucleus, barely noticeable nucleoli and finely dispersed chromatin. There are many tonofilaments in the cytoplasm. Mucinous cells are difficult to detect in hidradenoma; they usually line cystic cavities and are characterized by balloon-shaped cytoplasm rich in mucin. The cells lining the tubules can be of two types - prismatic and cylindrical. The former line duct-like structures (a sign of ductal differentiation), the latter line gland-like structures and to some extent reflect differentiation toward the secretory part of the apocrine gland.

Histogenesis. Histochemical and electron microscopic methods revealed signs of secretion by the type of apocrine glands: a positive reaction to lysosomal enzymes and a negative phosphorylase reaction. The histogenetic connection of this tumor with apocrine glands was confirmed by ultrastructural studies. The glandular cellular elements had a pronounced Golgi complex and electron-dense formations with "pinching" of the apical part of the cells (secretory granules) into the lumen of the duct.

Clear cell hidradenoma is differentiated from metastasis of clear cell renal cell carcinoma. The latter is characterized by the absence of a lobular structure, abundant vascularization with massive erythrocyte extravasates, and the presence of abundant fatty inclusions in the cytoplasm of cells.

With a superficially taken biopsy, there may be difficulties in differential diagnosis with the clear cell variant of squamous cell carcinoma, neoplasms of the sebaceous gland and trichilemmal differentiation in reactive hyperplasia of the follicular epithelium of viral genesis.

Treatment of papillary hidradenoma of the skin. Surgical excision of the tumor is performed.

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