A rare association of parotid oncocytoma, lipoma, multinodular goitre and uterine adenomysosis

Abstract

Objective: To demonstrate the syndromic association of cutaneous lesions, multinodal goitre, lipoma and uterine adenomyosis in a patient with parotid oncocytoma. Case report: Oncocytoma is a rare tumor of the parotid gland. We describe a patient who presented with parotid oncocytoma in association with multiple cutaneous lesions, multinodular goitre, lipoma and uterine adenomyosis.The constellation of above lesions suggested a possibility of Birt-Hogg-Dube syndrome (BHDS). BHDS is a rare syndrome presenting with peculiar cutaneous lesions in varying combinations with multiple tumors. A review of literature did not reveal any previous reports on presence of uterine adenomyosis in a patient with BHDS. Conclusion: Patients of BHDS may present with various head and neck manifestations. This makes its awareness important so that a complete systems review may direct appropriate age and gender-specific screening for malignancies. We also propose to add uterine adenomyosis to the list of tumors observed to be associated with BHDS.