BACKGROUNDA Phase II trial in children with advanced neuroblastoma was carried out in five Italian institutions to evaluate the antitumor activity and tolerability of topotecan followed by vincristine and doxorubicin.METHODSChildren older than age 1 year with Stage III or Stage IV neuroblastoma, all of whom had been treated previously with chemotherapy and were diagnosed with either refractory or recurrent disease, were treated with topotecan at an intravenous dose of 1.5 mg/m2 (the dose was 0.75 mg/m2 for patients who were treated within 1 year of previous megatherapy) per day for 5 days followed by 48‐hour intravenous infusions of 2 mg/m2 vincristine and 45 mg/m2 doxorubicin. Cycles of therapy were repeated every 3 weeks.RESULTSTwenty‐five patients (2 with Stage III disease and 23 with Stage IV disease; 19 with refractory disease and 6 with recurrent disease) were treated with a total of 115 cycles. Four patients had complete responses, 12 patients had partial responses, 4 patients had minor responses or stable disease, and 5 patients had tumor progression. The overall response rate (including complete and partial responses) was 64% (95% confidence interval, 43–82%). Fifteen patients were alive at the time of the current report and were progression free at 4–16 months (median, 9 months) after the first course of this treatment. Toxicity generally was limited to the hematopoietic system. Dose‐limiting toxicity was observed in only 1 patient (Grade 4 liver toxicity). There were no deaths due to infectious or toxic causes.CONCLUSIONSThe topotecan‐vincristine‐doxorubicin combination was active and well tolerated in previously treated patients with advanced neuroblastoma. Cancer 2003. © 2003 American Cancer Society.
Scheduled follow-up programs failed to detect relapses in 50% of cases presented here. Survival after relapse is not affected by whether relapse was detected at a scheduled or an unscheduled visit.
A 16-year-old youth was referred to our hospital for evaluation of a scalp lesion associated with cervical lymphadenopathy. Six months previously he had presented elsewhere with painful cervical lymphadenopathy and fever. A biopsy of the lymph node was inconclusive. On presentation to us he had a bald scalp area over a deeply infiltrating tumour mass. Cranial magnetic resonance imaging showed a densely enhancing, mushroom-like soft-tissue mass involving the pericranium, the aponeurotic coverings (left) and the dura mater (left). The external cerebral surface and the adipose layer of the scalp were apparently intact. The tumoral tissue had grown through a large osteolytic area of the left parietal bone, which could easily be seen using three-dimensional computerized tomography (right).A biopsy from the parietal lesion showed an anaplastic large cell lymphoma (ALCL). Malignant cells on immunohistochemical investigation were positive for CD30, epithelial membrane antigen, ALK and BH9. A study of the t(2;5)(p23;q35) investigated using reverse transcription-polymerase chain reaction was negative. Extensive staging investigations found no other evidence of disease. Primary osseous ALCL was diagnosed and polychemotherapy was started. Clinical response was complete and is still ongoing 1 year after diagnosis.Anaplastic large cell lymphoma, mainly affecting adolescents and young adults, represents approximately 10-15% of childhood lymphomas. Presentation can be varied and in some patients spontaneous regression followed by rapid progression is observed before a diagnosis is made. Combination chemotherapy is effective but skin involvement with nodal disease, visceral or mediastinal involvement and high lactate dehydrogenase levels are associated with an increased risk of failure.
A 7-year-old girl was admitted to another hospital with paraplegia and a 6-month history of hip pain and progressive weakness and numbness of the lower extremities. This was then diagnosed as rheumatic disease and treated with penicillin. Magnetic resonance imaging (MRI) revealed an extradural mass between the L3 and L5 vertebral bodies. On T2-weighted images, a high signal intensity was noted at L4, suggesting a bone tumor originating in the vertebral body (Fig. 1). Decompressive laminectomy was performed. The post-operative period was uneventful, with clear motor de®cit recovery of the lower extremities.Histological examination showed an in¯ammatory process with no diagnostic characteristics. The child was then referred to our hospital. On admission, she was in fairly good general conditions, with a fever but no evidence of an infectious focus. Laboratory blood examinations showed only moderate hypochromic anemia, while the erythrocyte sedimentation rate and C-reactive protein levels were high. Bone scintigraphy showed two areas of increased uptake on L4 and on the left iliac bone. A computed tomography (CT) scan of the lumbar column carried out to check the status after laminectomy and extended to the pelvic bones revealed an osteolytic process of the left iliac bone with adjacent soft-tissue mass (Fig. 2). A biopsy was performed on this area.The diagnosis was a nodular sclerosing variant of osseous Hodgkin lymphoma. Review of the material obtained by laminectomy con®rmed this.Staging procedures (abdominal and chest CT scans, abdominal ultrasound and bone marrow biopsy) ruled out other sites of disease, and the diagnosis became stage 4B Hodgkin lymphoma (HL) solely aecting the skeletal system (vertebra L4 and left iliac bone). Six weeks after laminectomy, we started chemotherapy with COPP/ABVD [2]. On the whole, the therapy was well tolerated, and after 5 courses of chemotherapy, imaging revealed the disappearance of soft-tissue masses and a healing process of the iliac bone.Radiotherapy (2960 cGy over 16 fractions) was then delivered to the lumbar vertebrae (L3±5) and left iliac bone.One year later, the patient is fully ambulatory, and physical examination is normal. Follow-up imaging is stable. DiscussionBenign or functional diseases such as discopathies, which are frequently seen in adults, have never been reported in children or adolescents. Therefore, in cases of repeated back pain, particularly if associated with limping and diculty in walking, a speci®c work-up is mandatory and urgent. Investigation must be extensive, not only at the site of pain but also throughout the whole body to determine where the biopsy should be carried out, and whenever possible, aggressive approaches such as laminectomy should be avoided.Clinical evidence of spinal cord compression in children with newly diagnosed tumors is a rather uncommon event. Neuroblastoma and Ewing sarcoma are most often involved, but the dierential diagnosis also includes non-Hodgkin lymphomas, leukemia, metastatic lesions, Langerhans cell histiocytosi...
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