Perineal chordoma cutis, a rare localization for a rare pathology: a case report and consideration of the literature

Abstract

The chordoma is a very rare notochordal tumor, the cutaneous localization (chordoma cutis) is even rarer and generally follows a local invasion of the integument even a metastasis. The clinical presentation is non-specific and the diagnosis is often late. It is confirmed by the immunohistochemical characteristics of the tumor. Total surgical excision is necessary in order to avoid the local recurrence which represents a frequent character of this tumor. We report the case of a young patient with perineal chordoma evolving from childhood. To our knowledge, our patient represents the first case of chordoma cutis of the perineum without a history of chordoma.

Keywords

• chordoma
• extra axial chordoma
• perineal chordoma
• chordoma cutis
• tumor resection