Malignant syringoma: causes, symptoms, diagnosis, treatment

Malignant syringoma (syn: sclerosing carcinoma of the duct of the sweat gland, syringomatous carcinoma, microcystic adrenal carcinoma, syringoid ecrcine carcinoma, ecrin epithelioma, basal cell epithelioma with ecrinic differentiation, ekrinnaya carcinoma with syringomatous structures, ekstrynaya basalioma, etc.).

Based on literature data and own observations, P. Abenoza, AB Ackerman (1990) concluded that all these numerous names actually describe the different degree of differentiation of a single tumor process - tubular ecrcine carcinoma, and suggested the term "syringomatous carcinoma of high, moderate and low degree of differentiation ".

This is a rare tumor, which often occurs in men and women. The average age of patients is 45 years, the growth is relatively slow - for years, occasionally - for decades. 85% of malignant syringes with high differentiation are located on the face skin, especially often on the upper lip (35%), cheeks (16%), periorbital (10%). In these places there is a condensation, and subjective patients indicate paresthesia. The tumor usually manifests as a solitary knot or plaque with a smooth surface, with a diameter of 1-3 cm or more, in long-term elements may occur ulceration. The sites of compaction at the localization on the lip usually indicate a transmural growth towards the mucosa.

Pathomorphology of malignant syringoma. The tumor is characterized by the presence in the central sections of tubular structures, as in the syringome, lined with a double-row epithelium; cysts with signs of keratinization, here and there with deposits of calcium salts, violations of the integrity of the cystic wall and granulomatous reaction in the surrounding stroma. On the periphery - strands of small dark cells, with infiltrating growth in the dermis and subcutaneous adipose tissue. Part of the cords with the projected lumens. In the stroma surrounding the strands, with the coloring of toluidine blue, metachromasia is revealed, and in the ultrastructural study the cells forming strands have large nuclei, which is characteristic of processes of increased synthesis of nucleic acids. Sometimes tumor cells penetrate the bundles of peripheral nerve fibers, the advent of medium-sized vessels and cause the destruction of skin appendages. In the literature, observations of syringomatous carcinoma with the predominance of bright cells rich in glycogen have been described.

Syringomatous carcinoma of moderate degree of differentiation, according to P. Abenoza, AB Ackerman (1990), is more common in women, the average age of patients is 61 years. The tumor exists for years, the primary localization is the skin of the scalp, palms, back, lower limbs. Clinically it looks like a dense plaque with fuzzy boundaries up to a diameter of 5 cm.

Pathomorphology. The tumor is characterized by the presence of nuclear atypia, the absence of cysts with keratinization, ductal structures of irregular configuration and various sizes, solid or adenocystic accumulations of cells of the basaloid type.

In syringomatous carcinoma of low degree of differentiation, typical "syringoid" structures are difficult to determine, nuclear atypia, many mitosis figures, strands of tumor cells between bundles of collagen fibers of the dermis with single microfragments of tubular differentiation are sharply expressed. The risk of metastasis is high. It is necessary to differentiate from carcinoma of any other localization, in particular of the mammary gland.

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