Soft-tissue sarcomas of the diaphragm: A report from the intergroup Rhabdomyosarcoma Study Group from 1972 to 1997

Purpose: To describe clinical details and outcome of children and adolescen ts with primary sarcomas of the diaphragm treated on Intergroup Rhabdomyosa rcoma Studies (IRS) I through IV. Patients and Methods: We reviewed the records of 15 patients with sarcoma o f the diaphragm who were entered on IRS Group protocols between 1972 and 19 97. Patient ages at diagnosis ranged from 0.5 to 20 years (median, 13 yrs), and 10 were girls. Patients had chest pain, dyspnea, and/or coughing, decr eased breath sounds, and occasionally hepatomegaly. Results: Localized, gross residual disease after initial surgery was presen t in 10 patients, and five had metastases at diagnosis (pleura, 3; pericard ium, 1; lungs and bones, 1). Tumor subtypes were alveolar rhabdomyosarcoma (RMS) in five cases, embryonal RMS in three, undifferentiated sarcoma in th ree, extraosseous Ewing sarcoma in three, and unclassified sarcoma in one. Treatment consisted of radiation therapy to the primary tumor and metastase s when feasible, and combination chemotherapy with vincristine, actinomycin D, and cyclophosphamide with or without doxorubicin, ifosfamide, cisplatin , and etoposide. Ten patients achieved complete remission (67%), four obtai ned a partial remission, and one was improved. Five patients (33%) are cont inuously failure-flee and alive at a median of 8.8 years from diagnosis (ra nge, 1.1-15 yrs). However, the other 10 patients experienced relapse at 0.3 to 2 years from start of therapy (median, 1 yr). Sites of relapse were loc al in five, distant in three, and combined in two. Death after relapse occu rred at 0.39 to 2.6 years (median, 1.6 yrs) from diagnosis. Conclusions: Sarcomas of the diaphragm are generally deemed unresectable at diagnosis and/or are metastatic. Most of them are not embryonal rhabdomyos arcomas. Treatment with more effective primary chemotherapy to shrink the t umor, followed-up by surgical resection and radiation therapy, should impro ve the prognosis for patients with sarcomas arising in the diaphragm, espec ially for the majority who have localized tumors.