Multiple Eyelid Neoplasms in Cowden Syndrome
Sweeney AR, Shetlar DJ
Read MoreA 74-year-old male with a history of Cowden syndrome and multiple bodily skin lesions presented for several year history of bilateral worsening epiphora and eye irritation. Twenty years prior, the patient had numerous eyelid lesions biopsied—all were consistent with trichilemmoma on pathology. On examination, there was an elevated tear lake in both eyes, an ulcerative lesion on the right lower eyelid, and multiple large pedunculated, fleshy lesions on all eyelids, most markedly in the medial canthi blocking the tear drainage pathways (Fig. A, B). Biopsy revealed bilateral 10 to 13 mm pedunculated lesions growing through the puncta and into the canaliculi. Pathology demonstrated squamous cell carcinoma of the right lower eyelid ulcerative lesion (Fig. C) and squamous papilloma of all the pedunculated lesions (Fig. D). Cowden syndrome is an autosomal dominant with a mutation of the tumor suppressor gene phosphatase and tensin homolog. Patients develop numerous skin hamartomas and are also at risk of various malignancies including breast adenocarcinoma, thyroid adenocarcinoma, and squamous cell carcinoma. Patients classically develop trichilemmomas; however, careful surveillance should be directed at lesions with concerning features of carcinoma. The collection and evaluation of patient information for this report was compliant under the Health Insurance Portability and Accountability Act of 1996.
Journal : OPRS
Ref : Eyelid tumour Oculoplastics