Cases of LLS are extremely rare. Because the histologic changes are frequently subtle, LLS can be easily mistaken for a benign tumor. As a result, the diagnosis requires a high index of suspicion and diligence in examining biopsy specimens. Computed tomography and magnetic resonance imaging can assist in the diagnosis and surgical approach. Genetic and immunostaining analysis techniques may also prove to have valuable prognostic, diagnostic, and therapeutic implications for this disease. Wide surgical excision is the mainstay of treatment. The use of radiotherapy and chemotherapy in treating this cancer remains experimental, but might be considered on a case-to-case basis for palliation or to treat aggressive variants of the disease.