Bilateral adrenal neuroblastoma.

BACKGROUND: Bilateral adrenal neuroblastoma is extremely rare. To date, 45 cases have been reported in the literature. PROCEDURES: We retrospectively identified and reviewed 15 cases of bilateral adrenal neuroblastoma, treated between 1988 and 2004, by the French Society of Pediatric Oncology. We then compared our cohort to the 45 cases reported in literature. RESULTS: Median age at diagnosis was 4 months in our cohort whereas it was 3 months in the literature. The same percentage of infants was found in both series (86.6%). Disease had generally been detected due to metastasis-related symptoms. Ten out of 15 patients in our cohort and 18/39 cases in the literature were classified as stage 4S according to the International Neuroblastoma Staging System (INSS). The incidence of stage 4S was significantly higher than that described in other neuroblastoma cohorts (P = 10(-4)). Five-year overall survival was 90% for stage 4S. In our series, bilateral neuroblastoma was neither associated with familial cases nor with any risk factors. CONCLUSIONS: The majority of bilateral neuroblastoma carry a favorable prognosis. Exceptional cases exhibiting risk factors, such as amplified MYCN, are comparable to high-risk unilateral neuroblastoma cases with the same poor prognostic features. The therapeutic strategy could be similar to that used against unilateral neuroblastoma, except for surgery. However, the low incidence of relapse and the risk of adrenal failure if radical surgery is performed, argue against an aggressive surgical approach.

[New insights into acute lymphoblastic leukemia]. / Que savons-nous de la cellule leucémique?

Acute lymphoblastic leukemia is a malignant disorder of lymphoid progenitor cells. Advances in our understanding of lymphoblastic leukemia have mainly come from new molecular technologies and genomics. This article describes recent advances in our understanding of maturation arrest of leukemic cells, initial and subsequent gene defects and rearrangements, the role of chemokines, and lymphoid cell homing. These advances point to new ways of targeting leukemic cells.

Clinical disease caused by Klebsiella in 2 unrelated patients with interleukin 12 receptor beta1 deficiency.

Patients with interleukin 12 (IL-12)p40 or IL-12 receptor ß1 (IL12Rß1) deficiencies are prone to develop infections caused by mycobacteria and salmonella; other infections have only been rarely observed. In this report we describe 2 unrelated patients with complete autosomal recessive IL12Rß1 deficiency who suffered from sepsis attributable to Klebsiella pneumoniae. A Mexican boy suffered from disseminated bacille Calmette-Guérin disease and infections caused by K pneumoniae and Candida albicans and had a fatal outcome. A Turkish girl living in France suffered from disseminated Nocardia nova infection and K pneumoniae sepsis. Therefore, Klebsiella infections should be considered in patients with IL12Rß1 deficiency. Conversely, IL12Rß1 deficiency should be considered in patients with unexplained klebsiellosis.