Disease relevance of Carcinoma, Adenoid Cystic

• An unusual protocol based on a preliminary clinical study on cylindromas metastasized to the lung was proposed to brain glioma patients: Day 2 100 mg/m2 i.v. Cis platinum (Cis PII) followed at days 3 and 5 by 6 Gy irradiation (RT) in two fractions and three days [1].
• Invasive breast carcinomas were consistently devoid of nuclear p63 staining, with the exception of the two adenoid-cystic carcinomas, of the two ductal carcinomas with squamous metaplasia, and of 11 (4.6%) ductal carcinomas not otherwise specified, showing p63 immunoreactivity in a minor fraction (5-15%) of the neoplastic cells [2].
• Five of the 7 patients had squamous cell carcinoma (SCC), one had adenoid-cystic carcinoma and 1 had a small-cell neuroendocrine cancer of the Bartholin gland [3].

High impact information on Carcinoma, Adenoid Cystic

• Here we have identified CYLD by detecting germline mutations in 21 cylindromatosis families and somatic mutations in 1 sporadic and 5 familial cylindromas [4].
• In the present study, we show that mice ectopically expressing the human Cubitus interruptus homolog GLI-1 in the skin develop tumors closely resembling human BCCs as well as other hair follicle-derived neoplasias, such as trichoepitheliomas, cylindromas, and trichoblastomas [5].
• To determine the molecular defects leading to extracellular matrix accumulation, the ultrastructural, immunological, and biochemical properties of cylindroma tissue and isolated cells were analyzed [6].
• Brooke-Spiegler syndrome (BSS, familial cylindromatosis or turban tumor syndrome) is an inherited disease characterized by neoplasms of the skin appendages such as cylindroma, trichoepithelioma, and spiradenoma [7].
• Cytokeratin 7 labeled predominantly the central basaloid cells, and smooth muscle actin stained peripheral myoepithelial cells in breast and dermal cylindromas [8].

Chemical compound and disease context of Carcinoma, Adenoid Cystic

• Cylindromas of the breast and skin did not express cytokeratin 20, gross cystic disease fluid protein 15, or estrogen or progesterone receptor [8].
• In both cylindromas and spiradenomas, zonal staining patterns were apparent: modified myoepithelial cells were positive for smooth-muscle-type actin, while the luminal cells mainly expressed ductal markers (CK 6 and CK 19) and, less prominently, secretory-coil markers including CK 7 [9].

Biological context of Carcinoma, Adenoid Cystic

• This observation suggests that the cyld1 gene may be the only tumour suppressor gene implicated in the development of cylindromas [10].

Anatomical context of Carcinoma, Adenoid Cystic

• Basement membrane components and keratin in the dominantly inherited form of cylindroma [11].
• All spiradenomas and cylindromas we studied demonstrated actin and/or S-100 protein positivity in basal epithelial cells, consistent with myoepithelial differentiation [12].
• Human milk fat globulin (HMFG) and lysozyme, two markers associated with apocrine differentiation, are expressed by tubular cells in spiradenomas and cylindromas [13].
• Two of three eccrine hidradenocarcinomas, and two of three porocarcinomas presented p53 overexpression, whereas in one case of malignant cylindroma and adenoid cystic carcinoma we did not find p53-positivity [14].

Gene context of Carcinoma, Adenoid Cystic

• Cylindromas and spiradenomas presented focal CD44 positivity, virtually limited to clear cells [15].
• The cells of the cylindroma lacked vimentin but expressed additionally CK 7, CK 8 and CK 18 [16].
• Some sera were negative on cylindroma [17].
• In all cylindromas, a thin continuous and sharply limited immunolabelling for the alpha1 (IV) collagen chain was abutted to the tumoral cell aggregates [18].
• Both spiradenomas and cylindromas expressed identical cytokeratin patterns [13].

References