Multiple Rare Skin Cancers in One Patient: Case Study
Posted: Wednesday, March 24, 2021
A case study presented in the Polish Archives of Internal Medicine reported the coexistence of epidermodysplasia verruciformis, a rare genodermatosis, and Merkel cell carcinoma in a single patient. Anna Wojas-Pelc, MD, PhD, of Jagiellonian University Medical College in Poland, and colleagues detailed the appearance and progression of these rare skin cancers in a 58-year-old woman.
The patient presented with epidermodysplasia verruciformis, of which she had experienced symptoms since age 17, and a history of multiple basal and squamous cell carcinomas and Bowen disease. An advanced, ulcerative basal cell carcinoma was observed on her left ear, for which she refused treatment. A firm, stationary tumor, present on her left cheek, was accompanied by enlarged cervical and submandibular lymph nodes. The tumor was confirmed through histopathologic examination to be Merkel cell carcinoma, with leukocyte antigen, nonspecific enolase positivity, and cytokeratin 20.
At a 3-week follow-up, the cheek tumor was substantially larger and included upper and lower lid edema on the left eye. Though she received a referral for surgery, the patient ultimately underwent chemotherapy due to the size of the Merkel cell carcinoma and the involvement of her lymph nodes. A few months following the appearance of the disease, the patient died of multiple metastases.
Epidermodysplasia verruciformis is associated with TMC6/EVER1 and TMC6/EVER2 gene mutation. Typically manifesting during childhood as flat warts or discolored patches throughout the body, it usually presents as skin cancers after age 30. Squamous cell carcinoma and Bowen disease commonly develop in patients with this skin condition, yet basal cell carcinoma is less common. Merkel cell carcinoma, which is aggressive prone to metastasis, is often associated with advanced age. Almost half of Merkel cell carcinoma cases include the neck or head.
Disclosure: The study authors reported no conflicts of interest.