Abstract
Ehlers-Danlos syndrome (EDS) is a rare hereditary disease characterized by joint hypermobility, skin hyperextensibility, and vascular fragility. The disorder is classified into 13 subtypes, of which vascular EDS is the most severe subtype involving high risks of artery dissection, arteriorrhexis, and rupture of the intestines.
To the best of our knowledge, there are no reports describing oral cancer occurring in an EDS patient. We present an extremely rare case of tongue carcinoma that occurred in a patient suffering from vascular EDS. A 48-year-old Japanese woman was referred to our hospital for examination of a white lesion on the left edge of the tongue, which was highly suspicious of malignancy. We performed an excisional biopsy under intravenous sedation to prevent severe complications caused by circulatory fluctuation during general anesthesia. No general complications or abnormal bleeding occurred during the operation with stable circulatory dynamics. The histopathological diagnosis was well-differentiated squamous cell carcinoma (pT1N0M0, stage Ⅰ). Wound healing was delayed, but there has been no recurrence in the 13 months after surgery.