Combination chemotherapy in the treatment of Kaposi sarcomain a child infected with human immunodeficiency virus

Abstract

Kaposi sarcoma (KS), a malignancy of the wall of blood vessels or the lymphatic system, remains the most commonly diagnosed neoplastic complication in persons infected with human immunodeficiency virus (HIV). Although KS commonly defines acquired immunodeficiency syndrome (AIDS) in adults, a small number of cases with KS in the pediatric population have been described. 1Mueller BU Pizzo PA Cancer in children with primary or secondary immunodeficiencies.J Pediatr. 1995; 126: 1-10Abstract Full Text Full Text PDF PubMed Scopus (120) Google Scholar In January 1996 a 7-year-old boy was admitted to the hospital with the recent onset of fever associated with weight loss, dyspnea, and systemic lymphadenopathy. The patient had no blood transfusion history and had resided in Zimbawe before moving to Italy 1 month before hospitalization. There was no previous history of opportunistic diseases. Physical examination showed oral thrush and bilateral tender cervical, supraclavicular, and inguinal lymph nodes. Lesions consistent with nodular KS were observed on the gingiva and the pubic area. Hepatosplenomegaly was noted. X-ray films of the thorax showed bilateral interstitial infiltrates with evidence of left pleural effusion. Numerous retroperitoneal lymph nodes, up to 2 cm in diameter, were present on abdominal sonograms. Biopsy specimens of the cervical lymph nodes confirmed diagnosis of KS. Laboratory data showed a leukocyte count of 4650 cells/mm 3 (normal value, 4800 to 8500 cells/mm 3 ), a CD4 + cell count of 458/μl (normal value, 530 to 1880 cells/μl), and a lactate dehydrogenase value of 573 U/L (normal value, 240 to 460 U/L). HIV antibodies were positively detected by enzyme-linked immunosorbent assay and Western blot. The patient was treated with orally administered fluconazole, 50 mg/day for 10 days; intravenously administered trimethoprim-sulfamethoxazole, 500 mg daily for 21 days; and intravenously administered bleomycin (6 mg), doxyrubicin (6 mg), and vincristine (0.6 mg). The patient received this combination chemotherapy every 2 weeks for three courses of treatment. No significant neurologic or cardiac toxic effects were seen. A transient moderate leukopenia was observed after the third chemotherapy cycle, and granulocyte colony-stimulating factor, 125 μg daily, was administered subcutaneously for 5 days. The patient responded well to anticancer therapy with a partial regression of neoplastic lesions and was discharged on hospital day 45 on a regimen of chemotherapy and granulocyte colony-stimulating factor every 2 weeks for 2 months. During the next 6 months his condition was stable. Children with AIDS are at an increased risk of having a malignancy during the course of their illness. Since the advent of the AIDS pandemic, KS and non-Hodgkin lymphoma have been associated with HIV infection. In HIV-infected pediatric patients, KS appears predominantly in a rapidly progressive lymphadenopathic form with frequent visceral involvement, whereas KS of the skin is much less common in children than in adults with AIDS. In recent years, several approaches to the treatment of patients with KS were evaluated in a number of trials, and many new experimental treatments are being investigated. Connor et al. 2Connor E Boccon-Gibod L Joshi V et al.Cutaneous acquired immunodeficiency syndrome–associated Kaposi's sarcoma in pediatric patients.Arch Dermatol. 1990; 126: 791-793Crossref PubMed Scopus (35) Google Scholar treated a 5 ½-year-old boy with cutaneous nodular KS lesions with 12 million U of interferon alfa per day. Although this patient tolerated therapy without an adverse reaction, treatment was discontinued after 5 weeks because new lesions continued to appear. Malekzadeh et al. 3Malekzadeh MH Chuch JA Siegel SE Mitchell WG Opas L Liliberman YE Human immunodeficiency virus–associated Kaposi's sarcoma in a pediatric renal transplant recipient.Nephron. 1987; 42: 62-65Crossref Scopus (28) Google Scholar treated with etoposide an 11-year-old boy in whom mucocutaneous KS developed 5 years after kidney transplantation; an excellent although transient regression of tumor ensued. Our experience supports the contention that prompt treatment with combination chemotherapy in association with supportive care may be benefical in the treatment of clinically aggressive lymphadenopathic KS in children infected with HIV.