Objective: To characterize salivary gland pathology and neoplasms associated with CYLD cutaneous syndrome, including location, risk of malignancy, and management practices.
Data sources: MEDLINE Ovid, Embase, Cochrane CENTRAL, and Scopus.
Review methods: Following Preferred Reporting Items for Systematic Review and Meta-analyses (PRISMA) guidelines, a database search was performed to identify articles describing salivary gland lesions in the setting of CYLD cutaneous syndrome.
Results: Twenty-six articles meeting the inclusion criteria were identified, describing 31 patients with CYLD cutaneous syndrome who presented with salivary gland neoplasms. Mean age was 61.0 years (range 37-76). Sixteen (51.6%) patients were male, 12 (38.7%) female, and 3 (9.7%) did not specify patient sex. Neoplasms were located in the parotid gland in 27 (87.1%) patients, both the parotid and submandibular glands in 2 (6.5%) patients, the submandibular gland in 1 (3.2%) patient, and minor salivary glands in 1 (3.2%) patient. Fifteen (48.4%) neoplasms were reported as multifocal, and 9 (29.0%) patients presented with salivary gland malignancy during their disease course. Eight (25.8%) cases reported recurrence at a mean of 3.8 (range 1-10) years.
Conclusion: Salivary gland lesions presenting in the setting of CYLD cutaneous syndrome occur predominantly in the parotid gland. Tumors frequently present with multifocal and malignant pathology, and warrant definitive surgical excision with parotidectomy.
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