Clinical Research Of Children With Rare Malignant Tumors

Objectives: The aim of this study was to analyze the clinical characteristics and prognosis of some rare types of non-Hodgkin lymphoma(NHL)and soft tissue sarcomas in childhood,and also to discuss the progress in treatment and review related literatures.Methods: Clinical data of 14 patients with rare types of NHL at Shanghai Children's Medical Center between January 2004 and December 2014 as well as clinical data of 51 patients with rare types of soft tissue sarcomas diagnosed at Shanghai Children's Medical Center from June 1998 to December 2013,were retrospectively analyzed respectively.Their clinical features,treatment and prognosis were discussed.Results: As for the rare NHL part,6 cases of Subcutaneous panniculitis-like T-cell lymphoma(SPTCL),3 cases of Extranodal NK/T-cell lymphoma,nasal type(ENKTL),3 cases of Hydroa vacciniforme-like cutaneous lymphoma(HVLL)and 2cases of Pediatric follicular lymphoma(PFL)were included.10 patients(71.4%)primarily presented with skin lesions.10 patients(71.4%)were accompanied by fever and 7 patients(50%)were accompanied by hepatosplenomegaly.No evidence of bone marrow and central invasion was observed in all cases.Since there are no standard treatment guidelines for this group of patients,most of them received CHOP and/or chemotherapy similar to mature B-cell NHL regimens.Half of them were treated with interferon in addition.10 patients(71.4%)reached complete remission(CR).The2-year OS and EFS rates was 83.6% and 57.1% respectively,after a median follow-up of 36 months(range from 24 to 64 months).As for the rare soft tissue sarcoma part,17 fibrosarcoma,10 synovial sarcoma,9 extrarenal rhabdoid tumor,6 alveolar soft part sarcoma,3 desmoplastic small round cell tumor and 6 undifferentiated sarcoma were enrolled.The mean age at initial diagnosis was 5 years(range from 1 month to13.5 years).The most common primary site was limbs,followed by the head and neck,accounting for 41.2% and 19.6% respectively.12 cases presented distant tissue or organ involvements in which bone metastases occupied the first place.The median follow-up was 36 months(range from 1 month to 123 months).44 patients(accounting for 86.3%)achieved complete remission and 3 patients achieved partial remission after initial treatment,the overall response rate was 92.2%.Subsequent follow-up showed 29 patients remained relapse-free while 13 patients relapsed.OS and EFS at 2 years was 87.8% and 56.9% respectively.Complete resection was the only major prognosis factor in this group of patients.Conclutions: Pediatric rare types of NHL in this study show atypical clinical manifestation,low incidence of CNS/BM infiltration.It is hard to make pathological diagnosis and differentiation.No standard treatment has been found by now.We presume that SPTCL,HVLL and PFL have relatively good outcomes when treated with mature B-cell NHL chemotherapy added with interferon therapy.These 6 types of soft tissue sarcomas are rare in children and adolescents.The tumor can occur anywhere in the body but commonly presents on the extremities.Complete resection of tumor has a primary role in the treatment and is directly related to the prognosis.