Background: Smooth muscle tumour (SMT) composed of leiomyoma and leiomyosarcoma recently has been described in many HIV-infected children. Leiomyosarcoma has become the second most frequent malignancy in children with HIV infection or other immunodeficiency diseases in the United States. Although leiomyosarcoma accounts for only 2% to 4% of childhood soft tissue sarcomas, the prognosis is poor in HIV-infected compared with non-infected patients. The development of Epstein-Barr virus (EBV)-associated SMT in children with acquired immunodeficiency virus (AIDS) decreases health, reduces quality of life, and often results in death. Some researchers, therefore, ascribe cause of death to SMT in the majority of these cases, not to AIDS. Currently, the optimal therapeutic strategy is controversial and there is a need to identify the efficacy and safety of different interventions for AIDS-associated SMT on overall survival and disease-free survival in children.
Objectives: To assess the effectiveness of current therapeutic interventions for previously untreated children with AIDS-associated leiomyoma and leiomyosarcoma
Search strategy: We searched the following electronic databases by subject headings and text words:Cochrane HIV/AIDS Group trials register (November 2009); Cochrane Central Register of Controlled Trials on Cochrane Library (Issue 4, 2009); MEDLINE (January 1966 to November 2009); EMBASE (January 1985 to November 2009); NLMGateway database and AEGIS; Chinese Biomedical Disc (CBMDisc 1978 to November 2009); VIP (1989 to present); and China National Knowledge Infrastructure (CNKI 1994 to 2009). We also searched physicians data query protocols, proceedings, and abstracts from AIDS and cancer conferences, and the reference lists from identified trials for unidentified trials to discover any unpublished or currently on-going relevant trials. All the trials were searched by comprehensive electronic databases or hand searching. The search was not limited by language.
Selection criteria: We searched for published or unpublished randomised controlled trials (RCTs) or controlled clinical trials (CCTs) of therapy for leiomyosarcoma and leiomyoma in children with AIDS.
Data collection and analysis: Two authors screened the results of the search independently to select relevant studies. The full text of all potentially relevant studies was retrieved and the qualities were assessed by the two authors using predetermined criteria. No eligible RCTs or CCTs were identified.
Main results: We were unable to find any RCTs or CCTs of interventions for treating AIDS-associated SMT in children.
Implications for clinical practice: We could not find any RCTs or CCTs of intervention for treating AIDS-associated SMT in children with HIV infection, and currently, the clinical practice of treating SMT in HIV-infected children is based on descriptive studies and simply situational analyses. Thus there is insufficient evidence to establish the efficacy and acceptability of these interventions, and we recommend a case-by-case treatment of patients until evidence becomes available.
Implications for research: In future, high-quality RCTs are urgently needed before any final conclusion can be drawn. Rigorously designed, multicenter, randomised, double-blind controlled trials are required to evaluate these interventions as a way of improving the survival and decreasing mortality in that population. Policy makers and researchers should prioritise funding for these trials to increase the quantity and quality of such studies and provide strong evidence for the effectiveness of therapies for AIDS-associated SMTs. Meanwhile, safety and adverse events should be critically assessed by standardized monitoring or an effective self-report system, and attention should be paid to long-term adverse effects in children with HIV infection.