Further insights into the natural history and management of primary XXXX .

@entity1 @entity3852 @entity5 @entity3003 @entity2879 @entity3
PURPOSE: Primary @entity3852 is a rare @entity5 with aggressive behavior but potential for response to radiotherapy and chemotherapy. Optimal treatment regimens are evolving based on reports of case series and a growing understanding of the natural history. METHODS AND MATERIALS: A retrospective analysis of 34 cases treated at two Australian Institutions over 13 years is presented, focusing on clinical features and response to therapy. RESULTS: The aggressive nature of this @entity5 is confirmed by the local recurrence rate of 22% following surgical excision, the development of regional node @entity3 in 76%, and of distant @entity3 in 70%. Overall median survival was 24 months with 65% of @entity1 succumbing to metastatic disease. An association with B cell @entity5 and immunosuppressive therapy is noted, with these @entity1 having a poorer outcome, and one spontaneous remission was observed. Radiation therapy produced responses in 21 of 30 measurable sites (11 complete, 10 partial), and in 11 sites irradiated prophylactically there was only one infield relapse (9%). Responses to chemotherapy were observed in 8 of 20 applications (40%), particularly @entity3003 and @entity2879 given in the setting of regional node disease. CONCLUSION: In this poor prognosis @entity5 , further investigation of adjuvant radiotherapy and chemotherapy is warranted, as responsiveness of recurrent disease is confirmed. Immunological factors appear important in the natural history, and their manipulation may offer additional therapeutic options.
