Squamous cell carcinoma of the tongue in von Recklinghausen's disease: A case report

Abstract

von Recklinghausen's disease (vRD) is an autosomal dominant neurogenetic disorder characterized by symptoms of neurofibroma and café-au-lait plaques. Although some nonepithelial tumors, such as malignant peripheral nerve sheath tumors and optic nerve gliomas, are closely associated with vRD, carcinomas are quite rare.This study reports a case of squamous cell carcinoma of the tongue in a patient with vRD. A 54-year-old woman was referred to our hospital because of a painful mass on her tongue. Initial clinical examinations revealed a 12 mm-sized mass with indurated margins on the right lateral border of the tongue, with no metastasis observed. The cytological diagnosis was class V, which strongly suggested squamous cell carcinoma (cT1N0M0); therefore, partial glossectomy was performed. Histopathological examination confirmed the diagnosis of squamous cell carcinoma. Three months after the surgery, secondary metastatic foci were observed in the right cervical lymph nodes, and subsequent right neck dissection was performed. Histopathological examination confirmed three lymph node metastases out of the total resected lymph nodes. The patient was strictly followed up for two years without any evidence of disease.