Intensive chemotherapy for children and young adults with metastatic primitive neuroectodermal tumors of the soft tissue

Abstract

Summary:The MMT4 study was designed to explore an intensive chemotherapy regimen (MMT4-89) and the role of high-dose melphalan (MMT4-91) in children with metastatic soft tissue sarcoma, including extraosseous peripheral neuroectodermal tumor (PNET). Thirtyone patients with PNET were treated between 1989 and 1995 (11 according to MMT4-89 and 20 according to MMT4-91). Chemotherapy consisted of four CEVAIE cycles, each including three 3-week courses: CEV (carboplatin 500 mg/m 2 , epirubicin 150 mg/m 2 , vincristine 1.5 mg/m 2 ), IVA ifosfamide 9 g/m 2 , actinomycin 1.5 mg/m 2 , vincristine 1.5 mg/m 2 ), IVE (ifosfamide 9 g/m 2 , etoposide 600 mg/m 2 , vincristine 1.5 mg/m 2 ). In MMT4-91 the fourth CEVAIE was replaced with melphalan 200 mg/m 2 with stem cell rescue. The CEV combination was evaluated as a window study. Surgery followed the second cycle. Radiotherapy was administered to post-surgical residual disease. The response rate was 55% after CEV, rising to 80% after the first CEVAIE. Twenty-five patients achieved complete remission (CR). Overall, the 5-year EFS was 22.6%: 36.4% and 15% for patients treated according to MMT4-89 and MMT4-91, respectively (P = 0.3). Local control was achieved in 77% of irradiated patients vs 45% of non-irradiated. Age Ͼ10 years was associated with significantly poorer outcome (P = 0.04). In conclusion, despite the high CR rate, intensive chemotherapy with or without high-dose melphalan appeared to have little impact on the survival of patients with metastatic extraosseus PNET.

SYNOVIAL SARCOMA OF THE NECK IN A CHILD: A Multidisciplinary Approach

“…In week 9 no response was documented by CT scan (the mass showed a slight increase of both diameters) and the child was shifted to the intensified chemotherapy regimen CEVAIE [22]: CEV (carboplatin 500 mg/m 2 day 1 + epirubicine 150 mg/m 2 day 1 + vincristine 1.5 mg/m 2 day 1) in week 10. Local radiotherapy was associated: hyperfractionated 6-MeV photon therapy, 1.6 Gy × 2/daily up to a cumulative dose of 38.4 Gy; the tumor region was boosted up to 51.2 Gy.…”

Primitive neuroectodermal adrenal gland tumour

“…13 Most chemotherapy regimens for adult-onset PNET are akin to those for ES in children as both share the same origin. 4,14 Chemotherapeutic agents such as cyclophosphamide, adriamycin, vincristine, ifosfamide, or etoposide have been used. 4,7,8 Recently, it has been noted that chemotherapy might be effective only for the first few cycles, and that the tumours develop resistance very quickly.…”