With great interest, we read the report by Skapek et al 1 in the September 1998 issue of the Journal of Clinical Oncology. This report detailed 10 cases of recurrent desmoid tumor in children treated with combination therapy of vinblastine and methotrexate. We report five additional cases successfully treated with a regimen similar to that of Skapek et al. Currently, none of our patients has evidence of regrowth of their tumor 7 to 76 months after elective cessation of chemotherapy.Between 1990 and 1997, five boys aged 7 to 17 years with biopsy-proven desmoid tumors were referred to us because the tumors were not amenable to surgical resection or because amputation was considered after multiple courses of surgery had failed. Although desmoid tumors do not show neoplastic features in histologic sections, the use of high-dose radiotherapy or aggressive polychemotherapy vinblastine [VBL], dactinomycin, and cyclophosphamide 2 is commonly advocated in these situations. Similar to Skapek et al, concerns about the toxicity profile and quality of life associated with these types of therapy, especially in prepubertal children, led us to a low-dose chemotherapeutic regimen of VBL and methotrexate (MTX), as previously reported in eight adults. 3 The apparent advantage of this regimen is the minimal risk for acute and long-term toxicities. We administered both VBL (6 mg/m 2 ) and MTX (30 mg/m 2 ) by intravenous push once a week. The total duration of treatment was 12 months, provided that the mass did not grow during that interval. Two patients experienced complete responses, one patient had a partial response, one patient had a minor response, and one patient had stable disease (Table 1). Follow-up after completion of therapy was 7 to 76 months. Tumor regression after VBL/MTX was slow (2 to 6 months) and was preceded by softening of the tumor, first notable after several weeks of treatment. These observations are consistent with the slow growth of the tumor; therefore, the presence of persistent disease immediately after VBL/MTX should not be misconstrued as an indicator of treatment failure. Side effects, including nausea, vomiting, and moderate hair loss, were minor and never required dosage reduction. Tumor recurrence observed in one patient (patient no. 4) was successfully re-treated with another course of VBL/MTX, and complete remission was achieved after 4 weeks.On the basis of our observations and those of Skapek et al, we agree that low-dose VBL/MTX should be considered in children with unresectable or multiply recurrent desmoid tumor who would otherwise require ablative surgery. At present, we do not know how many children will experience definitive cure with low-dose VBL/MTX chemotherapy. However, remission occurs for prolonged periods of time, and even recurrence can be successfully re-treated. In any event, the VBL/MTX combination offers the chance, as pointed out by Skapek et al, to treat young patients with minimal morbidity until they complete puberty. If recurrence occurs at that time, radiation can be expe...

The authors report a girl with high-grade intraosseous osteosarcoma of the left femur who lost one eye during early infancy for an unknown reason. Nine months after treatment, osteosarcoma in the stomach was detected and completely excised when the girl underwent endoscopy for Helicobacter pylori infection. Three years later osteosarcoma in the pulmonary interlobular space was noticed and completely removed. The girl is doing well and free of disease 26 months after the last resection.

A 10-month-old boy presented with a 6-week history of abdominal pain. The pain was due to a large, stage IV embryonal rhabdomyosarcoma of the urinary bladder. The rhabdomyosarcoma was found in association with neurofibromatosis 1 (NF1) manifesting multiple café au lait spots and bowing of the right calf. The diagnosis of NF1 had not been made before presentation. This case report is intended to heighten the awareness of the manifestations of NF1 and the possibility of developing a nonneuroectodermal tumor as a concomitant of NF1, and to emphasize the importance of timely diagnosis and treatment of such an NF1-associated malignancy. Reports of the epidemiologic evidence for rhabdomyosarcoma in children with NF1 are reviewed.

Hypogammaglobulinemia is a common symptom in different immunodeficiencies. It is, however, not usually associated with Epstein-Barr virus (EBV) infections. The X-linked lymphoproliferative disease (XLP) on the other hand shows immunological changes in response to the EBV. Here we report three previously healthy boys, all of which developed persistent hypogammaglobulinemia following severe acute infectious mononucleosis. All three patients revealed T-cell abnormalities including inverted CD4/CD8 and increased CD8(+) T-cell numbers. The number of IFN-gamma-producing T cells were markedly increased in the two patients studied so far. In addition, patient 2 showed mainly T cells, instead of B cells, to be infected with the EBV. Apart from an uncle of patient 3, who died of malignant lymphoma, family history was unremarkable in all cases. All three patients exhibited mutations in the SH2D1A gene, establishing the diagnosis of XLP. Protein expression was found on immunoblot analysis in one patient with a missense mutation. Development of persistent hypogammaglobulinemia after severe primary EBV infection seems to be a specific diagnostic sign for XLP even in males with unremarkable family history.

Background. Non‐Hodgkin lymphomas (NHL) account for approximately 50% of neoplasms in patients with ataxia teleangiectasia (AT). Prognosis is poor. Published data regarding the treatment of NHL in patients with AT suggested that these children respond poorly to therapy. The authors report on an infant with AT with mediastinal high‐grade T‐NHL who remained in continuous complete remission after chemotherapy. Diagnosis of AT was established after tumor diagnosis. Methods and Results. The 7‐month‐old boy was treated according to the acute lymphoblastic leukemia‐Berlin, Frankfurt, Münster 86 protocol. The therapeutic response was prompt, but therapy had to be stopped because of severe side effects. Surprisingly, the boy remained in a stable complete remission for 3½ years. Then tumors in both kidneys occurred and the child died a few months later. Postmortem examination demonstrated large tumors in both kidneys caused by a low‐grade malignant lymphoma of B‐cell lineage. Conclusion. Congenital immune deficiency should be ruled out at diagnosis of rare malignancies in respect to age. NHL in patients with AT can be cured, but poor tolerance to chemotherapy has to be considered. Patients whose disease has been cured may be at high risk for development of a second independent malignancy. Cancer 1994; 73:1522‐5.

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