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      Brooke-Spiegler Syndrome with Multiple Scalp Cylindromas and Bilateral Parotid Gland Adenomas

      1 , * , 2

      Case Reports in Radiology

      Hindawi Publishing Corporation

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          Abstract

          A 62-year-old female presented with numerous soft tissue lesions of her scalp and bilateral preauricular region. Several of these have been biopsied or removed with a diagnosis of cylindromas. Cylindromas are benign tumors with a differentiation towards apocrine sweat glands that increase in number and size throughout life. Multiple scalp cylindromas may coalesce and cover the entire scalp, resulting in the “turban tumor.” These are often associated with the autosomal dominant Brooke-Spiegler syndrome with coexistent facial trichoepitheliomas and spiradenomas. There is a very rare association between cylindromas and basal cell adenoma and adenocarcinoma of the parotid gland, with only 17 reported cases. Ours is the first CT demonstration of both the scalp and parotid gland findings in this uncommon situation.

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          Most cited references 5

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          Turban tumour with involvement of the parotid gland.

          Familial autosomal dominant cylindromatosis (FADC, turban tumour syndrome, Brooke-Spiegler-syndrome and many more, MacKusick catalogue numbers 123850, 313100) is a rare hereditary disease usually presenting in the second or third decade. With female preponderance dermal cylindromas predominantly arise in hairy areas of the body with approximately 90 per cent on the head and neck. Transformation to malignancy seems to be scarce. Although cylindromas of the skin resemble basal cell adenomas of the salivary gland, there is usually no salivary gland involvement. On the other hand, patients with basal cell adenomas of a salivary gland usually do not show dermal lesions. We report one of the rare cases of FADC combined with multiple basal cell adenomas of the parotid glands and present a review of the literature.
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            Brooke-Spiegler syndrome with parotid gland involvement.

            Salivary gland involvement in Brooke-Spiegler syndrome (BSS), an autosomal dominantly inherited disease, is known though not frequent. A case of familial cylindromatosis with parotid gland adenoma is herein reported. A 67-year-old lady presented with multiple scalp nodules and papular coalescent lesions over the nasolabial folds and the forehead. The clinical examination also revealed a left preauricular lump. Multiple biopsies of the scalp lesions and the nasolabial papules revealed cylindromas and trichoepitheliomas respectively. CT scan and FNA of the preauricular lump were suggestive of parotid gland adenoma. The patient underwent excision of the scalp cylindromas and total left parotidectomy. There is no evidence of recurrence after 4 years. The association of BSS with salivary gland tumours, emphasizes the necessity of thorough salivary gland examination in all patients with skin lesions. Knowledge of the genetic background of BSS allows for genetic counseling of patients.
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              Parotid mass in a woman with multiple cutaneous cylindromas.

              Familial autosomal dominant cylindromatosis (FADC) is a rare disease characterized by multiple cutaneous cylindromas, trichoepitheliomas, and spiradenomas. We present a case of a woman with scalp lesions and a parotid mass. Biopsy of a skin nodule demonstrated cylindroma, and fine-needle aspiration of the parotid mass suggested membranous basal cell adenoma, which was confirmed following superficial parotidectomy. The study includes case report and review of the literature. Eighteen cases of cylindromatosis with coexistent salivary gland membranous basal cell adenoma were reported. Seventeen cases involved the parotid gland; there are 2 reports of malignant transformation. FADC should be considered in anyone with coexistent dermal and salivary gland neoplasms. Membranous basal cell adenoma may be mistaken for adenoid cystic carcinoma on cytologic or histologic examination. Total parotidectomy with facial nerve preservation or superficial parotidectomy with close follow-up is recommended, as multifocality and malignant transformation exist.
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                Author and article information

                Journal
                Case Rep Radiol
                Case Rep Radiol
                CRIM.RADIOLOGY
                Case Reports in Radiology
                Hindawi Publishing Corporation
                2090-6862
                2090-6870
                2012
                12 February 2012
                : 2012
                Affiliations
                1Department of Radiology, Mayo Clinic, Rochester, MN 55905, USA
                2Department of Dermatology, Mayo Clinic, Rochester, MN 55905, USA
                Author notes

                Academic Editors: E. Bölke and P. D. Corr

                Article
                10.1155/2012/249583
                3350190
                22606564
                Copyright © 2012 P. Kalina and R. el-Azhary.

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                Categories
                Case Report

                Radiology & Imaging

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