Liver involvement in neuroblastoma: the Memorial Sloan-Kettering Experience supports treatment reduction in young patients.

Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York, United States
Pediatric Blood & Cancer (Impact Factor: 2.39). 03/2006; 46(3):278-84. DOI: 10.1002/pbc.20564
Source: PubMed


We reviewed clinical and biologic findings in a series of infants with neuroblastoma (NB) in liver. The aim was to gain insights into improving therapy.
Among 19 newly or recently diagnosed infants with NB in liver, 1987-2002, those with stage 4 involving bone received chemotherapy, while those without bone or extensive bone marrow (BM) involvement were observed or received limited treatment if NB caused life-threatening symptoms. We assessed results in the context of NB treatment risk stratification, which is based on age, stage, and selected biologic features (MYCN, ploidy, histology).
Six of eight infants with bone involvement became long-term event-free survivors including 1/2 with MYCN amplification and four who received only 4-6 cycles of chemotherapy; at the end of treatment, four infants had abnormalities in liver +/- the primary site, but these resolved. All 11 infants without bone lesions became long-term survivors with either no cytotoxic therapy or only one cycle of chemotherapy (+/- radiotherapy to liver), including four who had stage 4 and one stage 4S patient who still had NB in BM at age 15 months.
Treatment reduction should be considered for subsets of infants with non-MYCN-amplified widespread NB: stage 4 without bone or extensive BM involvement may not require cytotoxic therapy, stage 4S with symptomatic hepatomegaly may not require multiple cycles of chemotherapy, and classic stage 4 may do well with limited chemotherapy. Persistent liver abnormalities post-treatment may not require continued therapy to achieve a radiologic complete remission.

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    • "The presence of liver metastases in stage 4S neuroblastoma did not influence the outcome [7]. This concurs with the findings in 19 infants with liver involvement [1]. After a mean follow-up of more than 10 years of stage 4S patients, ultrasound of the liver was normal in 13 of 25; 6 had mild coarsening and 3 had moderate coarsened texture of the liver; in three, the left lobe was atrophic. "
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    ABSTRACT: Three patients with stage 4S neuroblastoma without MYC-N amplification who progressed to stage 4 with persistent liver involvement, were treated with iodine 131-meta-iodobenzylguanidine therapy, chemotherapy, and surgery. Successive histologic examination of the liver showed differentiation of the tumor in 2 patients and fibrosis in the third. One patient died of brain metastases at the age of 30 months. The other 2 patients are alive at 50 and 44 months. Diffuse liver involvement in patients with stage 4 progression of previous stage 4S without MYC-N amplification may differentiate after treatment. The aim of this report is to draw attention to major liver surgery that it may not be necessary in tumors without MYC-N amplification, despite the persistence of lesions in the liver.
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    ABSTRACT: Cancer in children has a low incidence. The type of malignancies differs substantially from tumors in adults. Since long-term event-free survival rates above 80% are noted in several tumors, it is estimated that in the future 1 in 750 adults will be a cancer survivor. Data on late effects are emerging and indicate that a large proportion of ex-patients has several and sometimes severe treatment sequelae. Minimizing treatment without loss of effectiveness in good risk cases and improving therapy in poor cases should be main goals in the future. In this review an update is given on tumor biology and treatment for several tumors, i.e. neuroblastoma, nephroblastoma, rhabdomyosarcoma, Ewing sarcoma, osteosarcoma, hepatoblastoma and germ cell tumors.
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    ABSTRACT: Neuroblastoma is a malignancy of childhood derived from neural crest cells appearing anywhere along the distribution of the sympathetic nervous system. It is the most common extra-cranial solid tumor of infancy, with most cases occurring under 1 year of age. The term neuroblastoma first originated in 1910 and was used by Dr. James Wright to describe a new group of tumors [1]. These neuroblastic tumors run a spectrum from poorly differentiated and malignant (neuroblastoma), to partially differentiated (ganglioneuroblastoma), and ending with completely differentiated and benign ganglioneuroma [2].
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