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

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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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Turban tumour with involvement of the parotid gland.

David M. Wagner, M Damm, M Jungehülsing (1999)

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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Parotid mass in a woman with multiple cutaneous cylindromas.

Daniel G. Deschler, William Faquin, R. Scott (2010)

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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Brooke-Spiegler syndrome with parotid gland involvement.

Despoina Kakagia, George Alexiadis, Anastasia kiziridou … (2015)

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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DOI:: 10.1155/2012/249583

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http://creativecommons.org/licenses/by/3.0/

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

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Most cited references 5

Turban tumour with involvement of the parotid gland.

Parotid mass in a woman with multiple cutaneous cylindromas.

Brooke-Spiegler syndrome with parotid gland involvement.

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