Multicentric dermal-type cylindromas of the parotid glands in a patient with florid turban tumor

Abstract

Dermal‐type cylindromas of parotid glands in a patient with turban tumor, and various adnexal tumors, represent a rare diathesis, apparently not reported previously. A Caucasian man, born in 1916, had a scalpectomy for turban tumor in 1957. In 1957, 1960, and 1974, dermal type cylindromas were excised from portions of both parotid glands, developing from intercalated and striated ducts; they were considered benign, based on long term followup. Adnexal tumors included multiple dermal cylindromas, trichoepitheliomas, eccrine spiradenomas, and Bowen's disease, and occurred on almost all skin surfaces. In November, 1976, a portion of a right parotidectomy included a 3.5 × 3‐cm malignant tumor having the characteristics of an invasive poorly differentiated spindle cell epidermoid carcinoma, which showed no evidence of originating from a malignant basal cell tumor. Several encapsulated and developing benign basal cell tumors were included in the specimen. Dermal‐type cylindromas of the parotid gland are considered to be a variant of basal cell adenoma, or monomorphic adenoma, basal cell type, since they consist of similar cell components, although of somewhat different arrangement and with prominent focal deposition of hyaline. Dermal‐type cylindromas should be excised conservatively as they appear. They must be differentiated from adenoid cystic carcinoma, which is an infiltrating neoplasm and requires a radical surgical approach.

Basal Cell Adenocarcinoma and Basal Cell Adenoma of the Salivary Glands: A Clinicopathological Review of Seventy Tumors with Comparison of Morphologic Features and Growth Control Indices

“…Any membranous area seen in the other tumors was \5 %. None of our patients had a history of skin adnexal tumors such as cylindroma, although this finding has been reported by others [8,18,19]. One of our patients presented with a basal cell adenoma in the parotid gland and subsequently presented with a basal cell adenocarcinoma in the contralateral parotid gland.…”

Cylindroma of the Breast of Skin Adnexal Type

“…They can also present in an autosomal dominant familial form as multiple tumors, which may involve not only the scalp, head, and neck, but also unusual sites such as trunk and extremities, or occur in an association with other cutaneous tumors (including spiradenoma, trichoepithelioma, and milia) and salivary gland tumors (the membranous variant of basal cell adenoma). 15,23,31 In this familial form, known as turban tumor syndrome or Brooke-Spiegler syndrome, the lesion is generally first noted in adolescence or childhood. In affected families, mutations have been demonstrated in the CYLD gene, located on chromosome 16q12-13, which probably represents a tumor suppressor gene.…”

Salivary Gland Neoplasia: A Review for the Practicing Pathologist

“…This is evident in the rare familial autosomal dominant Turban Tumor (Brooke-Spiegler) syndrome, localized to a tumor suppressor gene on chromosome 16q12-q13. In this condition individuals develop numerous cutaneous dermal cylindromas, trichoepitheliomas and eccrine spiradenomas with occasional similar appearing (dermal analogue) salivary membranous type basal cell adenomas (11)(12)(13). Other salivary tumors known to have a familial occurrence include pleomorphic adenoma, acinic cell carcinoma (14), Warthin's tumor and lymphoepithelial carcinoma (15).…”

“…The membranous type of adenoma, also know as the dermal analogue tumor, was first described in 1977 by Headington and colleagues (11,12). It occurs mainly in the parotid gland or may arise in a parotid lymph node thereby simulating a metastasis.…”