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.
To assess the effectiveness of current therapeutic interventions for previously untreated children with AIDS-associated leiomyoma and leiomyosarcoma
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AIDS and cancer conferences, and the reference lists from identified trials for unidentified trials to discover any unpublished or currently on-going relevant trials.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.
We searched for published or unpublished randomised controlled trials (RCTs) or controlled clinical trials (CCTs) of therapy for leiomyosarcoma and leiomyoma in children with AIDS.
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.
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.
"Although it accounts for only 2–4% of childhood soft tissue sarcomas, it is now the second most frequent malignancy in children infected with HIV or other immunodeficiency diseases in the United States . The prognosis is worst in HIV-infected children compared with non-infected patients, and there is currently a lack of uniform and effective therapies  "
[Show abstract][Hide abstract] ABSTRACT: Highly Active Antiretroviral Therapy (HAART) changed the natural history of pediatric HIV infection. This review focuses on trends of HIV-associated cancers in childhood in the HAART era and analyses potential pathogenetic mechanisms. HAART reduced AIDS-defined malignancies (ADM), but incidence of several non-ADM is increasing. HIV-associated immune activation and inflammation, promoting tumorigenesis, can only partially be reduced by HAART. In addition, HIV-infected children may undergo accelerated immune senescence that favors cancer development. How HAART affects this condition is an open question. Lastly, there is no evidence that prenatal exposure to HAART increases the risk of cancer in childhood, but long-term studies are needed.
Cancer letters 02/2014; 347(1). DOI:10.1016/j.canlet.2014.02.002 · 5.62 Impact Factor
"Yin et al. recently reviewed the literature regarding therapeutic strategies for treating leiomyoma and LMS in children with AIDS. Based on the fact that there are no randomized or clinical controlled trials and most of the data is based on case reports and small series, they conclude that it is likely best to treat each patient with a case-by-case manner until larger, more rigorous studies are carried out . In some cases, conservative management and simple observation alone may be appropriate for some of these extremely ill patients [27, 32]. "
[Show abstract][Hide abstract] ABSTRACT: The number of reported cases of smooth muscle tumor (SMT) arising in patients with AIDS has been increasing since the mid-1990s. The aim of this study is to characterize the epidemiology, clinical manifestations, pathologic features, prognosis and, management of Epstein-Barr virus-related SMT (EBV-SMT) in patients with AIDS. An English language literature search identified 53 articles including 64 reported cases of EBV-SMT. The majority of these reports involved patients who were young, severely immunosuppressed, and had multifocal tumors. The central nervous system was the most common site to be involved. Histologically, tumors had smooth muscle features and were immunoreactive for muscle markers and all but two tumors demonstrated the presence of EBV by either immunohistochemistry, in situ hybridization, and/or PCR. While mitoses and/or necrosis were used to separate leiomyoma from leiomyosarcoma, these features did not correlate with clinical outcome. Treatment included primarily resection, and less often radiotherapy, chemotherapy and highly active antiretroviral therapy (HAART). Overall, EBV-SMTs appear to have variable aggressiveness and clinical outcome and may exhibit a more favorable prognosis compared to conventional leiomyosarcoma. Tumor-related death from EBV-SMT occurred in only 4 of 51 patients.
Pathology Research International 03/2011; 2011:561548. DOI:10.4061/2011/561548
[Show abstract][Hide abstract] ABSTRACT: EBV-associated smooth muscle tumors are found in immunocompromised patients, most commonly HIV/AIDS. We present a 12-year-old girl with the first documented case of EBV-related smooth muscle tumors in the presence of a rare classic NK cell deficiency. This sheds light on the role of NK cells in controlling EBV-related smooth muscle tumors.
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