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The effect of sodium butyrate (NaB) on cell growth and expression of morphological and biochemical properties was examined in the human neuroblastoma cell lines AF8 and SJ-N-KP. The obtained data show that NaB induced a marked growth inhibition and morphological differentiation, while it was ineffective in inducing biochemical differentiation.

Background and Methods: B-cell acute lymphoblastic leukemia (B-ALL) presents FAB L3 morphology and surface Ig, CD19, CD20, CD24. This pattern may show some morphological and immunological heterogeneity. Seventeen pediatric cases of B-ALL at onset, treated in twelve AIEOP Centers (Italian Association for Pediatric Hematology and Oncology), are presented., Results and Conclusions: Clinical and hematological features were characterized by low WBC counts at presentation, high M/F ratio, older age and association with extramedullary involvement. The overall survival curve at 78 months is 40%. All patients showed blasts positive for surface Ig (sIg), DR, CD19, and CD24. Ten/17 cases presented the classical features of B-ALL: FAB L3 morphology, sIg+ restricted to light chains, CD20+, cytoplasm mu (c mu)-, CD10-, TdT-. The remainder showed some differences in this pattern, such as non-L3 morphology (3 cases), absence of CD20 (3 cases), CD10+ (4 cases), TdT+ (3 cases), c mu+ (1 case), lack of surface light chains (1 case). This rare ALL subset seems to be characterized by a high phenotype heterogeneity, indicating various degrees of differentiation.

Background: Granular acute lymphoblastic leukemia (ALL) is a rare morphological variant of ALL, characterized by cytoplasmic azurophil granules or inclusions, positive for aspecific esterase and acid phosphatase, with heterogeneous features at the ultrastructural level., Methods: In an attempt to determine whether the presence of granules or inclusions marks a biologically distinct variety of ALL with peculiar clinical features, a prospective morphological review was undertaken of children entering AIEOP protocols for ALL in the period from 1985 to 1989., Results: Of 531 cases examined, 16 (3%) were found to have greater than 1% granular bone marrow blasts, with 7 cases (1.3%) having greater than 10%. The presence of granules or inclusions was associated with the immunophenotype of "common" ALL. There was no clear association with FAB type L1 or L2 nor with particular clinical or hematological findings at presentation. Complete remission was achieved in all cases; one patient died of infection in remission at 3 months and 2 patients relapsed after 12 and 32 months, respectively, while the others are still in remission after a minimum follow-up of 24 months., Conclusions: In conclusion, granular morphology seems to have no prognostic importance in children ALL.

Co-expression of myeloid antigens on the leukaemic blast cells was evaluated in 532 children with a diagnosis of acute lymphoblastic leukaemia (ALL). Using a panel of monoclonal antibodies belonging to CD11b, CD13, CD14, CD15 and CD33 an overall incidence of 4.3% was found, with values ranging between 1.8% for CD14 and 6.1% for CD15. When the data were further dissected, a significantly higher incidence of co-expression was noted in null-ALL (15/70 cases = 21.4%), compared to cases expressing a more mature immunophenotype, i.e. common-ALL (7/394 cases = 1.7%) and T-ALL (1/68 cases = 1.4%) (P less than 0.001). In null-ALL, 9/15 patients were infants, five of whom with the t(4;11); two further children also had a t(4;11). The clinical outcome of the 23 cases which co-expressed myeloid antigens was unfavourable. Only two of the 15 null-ALL, two of the seven common-ALL and the unique case with T-ALL are in fact in persistent first remission between 19 and 93 months from diagnosis. Though the overall incidence of childhood ALL expressing myeloid antigens is low, the evidence that this co-expression may be related to an unfavourable clinical course and that it more frequently occurs in null-ALL, particularly in the first year of life, suggests that the routine assessment of myeloid antigens may allow to identify a subgroup of childhood ALL with a poor clinical outcome.

A continuous tumor cell line (LAP-35) was established from a primitive neuroectodermal tumor of bone from the right tibia of a 12-year-old female. The neural character of the cell line was documented by the spontaneous growth of neurites and by the presence of several neural markers, including neuron-specific enolase (NSE), S-100 protein, neurofilaments, chromogranin A, synaptophysin and positivity to monoclonal antibodies UJ127.11, UJ13A, UJ181.4. Cell-sorter analysis showed a high expression of nerve growth factor receptor (NGFr) and major histocompatibility complex class I-related molecules. A unique cytogenetic profile was observed, including a reciprocal chromosomal translocation (rct) 11:22 (q24;q12), typically associated with Ewing's sarcoma and neuroepithelioma, and deletion of the short arm of chromosome 1 (lp-), otherwise a feature of neuroblastoma. N-myc proto-oncogene was neither amplified nor expressed, whereas the expression of c-myc was documented by northern blot analysis. These features distinguish this new cell line from previously reported neuroectodermal cell lines, identifying LAP-35 as a unique model of a group of neural bone tumors that share characteristics of neuroblastoma as well as neuroepithelioma.

The effects of epirubicin and retinoic acid (RA) as differentiation inducing agents on human neuroblastoma cell lines were investigated. We have compared the response of neuroblastoma cells to epirubicin alone, to RA alone and to combined treatment, with respect to neuritic processes outgrowth, acethylcholinesterase activity, growth inhibition and antigenic expression. The obtained data indicate that the combination of the two agents is able to produce a synergistic effect on differentiation and on growth inhibition.