Head and neck liposarcoma. A histopathologic reevaluation of reported cases

Head and neck liposarcoma is an extremely rare tumor. As with all rare lesions there is a void in the literature regarding tumor activity and treatment response. It is difficult to make rational treatment plans and advise patients as to probable treatment response and prognosis. To obtain as much information as possible from available data we have reviewed the world literature and reevaluated the descriptive histopathologic findings and treatment response of reported cases. Seventy-six cases have been reported since 1911. We added another case and then reclassified the previously reported lesions according to a current histopathologic system (well-differentiated, myxoid, round-cell, and pleomorphic lesions). We assessed tumor activity and treatment response by histopathologic tumor type. Paralleling tumor activity in other anatomic regions, all patients with myxoid and well-differentiated tumors did well; all were alive at the end of follow-up, eight of eight and 11 of 11, respectively (average follow-up, 5.7 and 4.8 years). This is compared with a 50% mortality rate in the round-cell and pleomorphic groups by the end of 2 years, (two of three and six of 11 patients alive, respectively). The likelihood of recurrence correlated with extent of tumor removal, and metastatic disease was identified almost exclusively with the tumor of the round-cell and pleomorphic variants. Surgical excision remains the primary treatment modality, while radiation and chemotherapy appear to be of limited utility.