Fibromyxoid sarcoma of maxilla

A recurrent mass in the nose can pose diagnostic and therapeutic challenges. Even more so, if it exhibits rapid growth, displays unusual clinical features, is a rare histopathological diagnosis or if the patient is unavailable for regular follow-up. We present a case of a middle-aged retrovirus-positive man who underwent surgical removal of a recurrent nasal mass. Histopathology revealed a diagnosis of low-grade fibromyxoid sarcoma, a rare entity in the sinonasal tract. Immunohistochemistry plays a crucial role in clinching the diagnosis, especially for lesions with fibrous and myxoid components. Early and complete surgical resection diminishes the likelihood of recurrence and metastasis.