Development of metastatic inguinal squamous cell carcinoma in a patient with Netherton syndrome: A case report

Abstract

Introduction: Netherton syndrome (NS) is a rare multisystem disorder with both cutaneous and extra-cutaneous manifestations. The syndrome includes the presence of congenital ichthyosis, exhibited by hyperkeratosis and abnormal skin scaling over the entire body. In rare instances, NS has been associated with the development of squamous cell carcinoma (SCC). Case Report: A 54-year-old male with a history of NS and recurrent squamous cell carcinoma presented with an enlarging left inguinal mass. Biopsy of the mass was positive for a new invasive squamous cell carcinoma with regional lymph node involvement and invasion of the left femoral vein. The patient underwent wide local resection, superficial and deep regional lymph node dissection, and resection of the left femoral vein. His hospital course was further complicated by multiple infections and progression of his cancer. He eventually transitioned to hospice care before receiving any additional treatment for his malignancy. Conclusion: This case highlights a patient with NS and a history of recurrent inguinal squamous cell carcinoma. Further investigation is needed to better highlight the complexity of factors associated with the development of squamous cell carcinoma in patients with NS and other forms of ichthyoses.