Mycosis fungoides in the pediatric population: report from an international Childhood Registry of Cutaneous Lymphoma.

BACKGROUND/OBJECTIVES: There are limited data on the clinical presentation and progression of pediatric cutaneous lymphoma. This study focuses on the clinical characteristics of pediatric patients with mycosis fungoides (MF). MATERIALS AND METHODS: This descriptive study presents clinical characteristics of 22 pediatric patients with MF, enrolled in the international Childhood Registry for Cutaneous Lymphomas (CRCL). RESULTS: The mean ages at onset and at diagnosis were 7.5 (SD 3.8 years) years and 9.9 (SD 3.4) years, respectively. The most common MF presentation was patch stage (68%), followed by hypopigmentation (59%) and plaque stage disease (50%). Epidermotropism and lymphocytic atypia were the most common pathologic features, found in 89% and 85%, respectively. Cerebriform nuclei were noted in 42%, and Pautrier microabscesses were seen in 16% of cases. A cytotoxic pattern was more commonly seen (67% vs 33%), and clonality was detected in 21% (3 of 14) of patients. All patients presented with early-stage disease and received skin-directed therapy (topical steroids, 73%; light therapy, 54%; or combination therapy, 35%). CONCLUSIONS: Pediatric patients with MF present in the first decade of life, with early-stage disease and unusual forms such as hypopigmented variant. Further patient enrollment will provide information regarding natural history, treatment response, and overall prognosis of pediatric cutaneous T-cell lymphoma (CTCL).

Isolated central nervous system relapse in childhood acute promyelocytic leukemia.

Central nervous system (CNS) involvement is rare in acute promyelocytic leukemia (APL). The majority of CNS relapses occur in patients with hyperleukocytosis at presentation, and the optimal management of such patients is still controversial. We describe a 13-year-old boy with APL who developed an isolated CNS relapse after first-line treatment with all-trans retinoic acid and chemotherapy. A second remission was achieved with a regimen consisting of intrathecal chemotherapy, intravenous high-dose cytarabine, and oral 6-mercaptopurine. All-trans retinoic acid was avoided owing to severe complications during initial therapy. The patient remains in molecular remission at 9 months after autologous stem cell transplant. Prognostic factors of CNS relapse in children with APL are needed to define the indications for CNS prophylaxis in this group of patients.

Complementary and alternative (CAM) dietary therapies for cancer.

Complementary and alternative (CAM) therapies include a wide spectrum of dietary practices, some of which are claimed to cure cancer. Observational studies have shown consistently that predominantly plant-based diets reduce the risk for some adult type cancers such as breast cancer and prostate cancer. These studies form the basis of the American Cancer Society (ACS) nutritional guidelines. Many CAM diets prescribe a similar low fat, high fiber, high fruit and vegetable type diet, but also add detoxification and many different supplements to the basic diet which is then claimed to cure cancer. The potential advantages and disadvantages of CAM diets are discussed. Many aspects can be potentially harmful, particularly to the child with cancer. Advantages include involvement of the child and family in decision-making and care. There is no evidence to support the claims that CAM dietary therapies cure cancer.

Folic acid food fortification is associated with a decline in neuroblastoma.

BACKGROUND: Neuroblastoma, an embryonic tumor, is the second most common pediatric tumor and is the most prevalent extracranial solid tumor in children. Results of previous studies have suggested that maternal vitamin intake may decrease the risk of several childhood cancers. In January 1997, Canada began fortifying flour with folic acid for the prevention of neural tube defects. The effect of folic acid fortification on the rate of neuroblastoma in offspring is not known. METHODS: We investigated the rates of neuroblastoma (<1 year), acute lymphoblastic leukemia, and hepatoblastoma registered by the Pediatric Oncology Group of Ontario, which captures 95% of all pediatric cancers in Ontario, before and after the introduction of folate fortification. RESULTS: An interventional time series analysis showed that the incidence of neuroblastoma declined from 1.57 cases per 10,000 births before to 0.62 case per 10,000 births after folic acid fortification (P <.0001). The crude incidence rate ratio (0.40; 95% confidence interval, 0.25-0.64) remained significant after adjustment for both age and disease stage at diagnosis (adjusted incidence rate ratio, 0.38; 95% confidence interval, 0.23-0.62). In contrast, there was no significant change in the rate of infant acute lymphoblastic leukemia (incidence rate ratio, 0.97; 95% confidence interval, 0.41-2.27) or hepatoblastoma (incidence rate ratio, 0.81; 95% confidence interval, 0.35-1.89). CONCLUSIONS: Folic acid fortification was associated with a 60% reduction in neuroblastoma but was not associated with any change in the rate of infant acute lymphoblastic leukemia or hepatoblastoma. Further investigation is needed into the role of metabolism in the formation and prevention of neuroblastoma and other embryonically determined cancers.