Johannes E A Wolff

Alternative Therapies for the Treatment of Childhood Cancer

To the Editor: Recently, we have been deeply disturbed by the decision of several families to use alternative therapies exclusively as front-line treatment for their childrens cancer, or to use al...

Congenital extrarenal non-central nervous system malignant rhabdoid tumor

Malignant rhabdoid tumor (MRT) is a rare tumor occurring mostly in kidneys and central nervous system (CNS). Its prognosis is not good. Occasionally, MRTs are diagnosed at or immediately after birth. A female neonate presented with MRT in the chest wall, axilla, right elbow, and bone marrow. Chest wall lesion was resected completely. Although the masses in axilla and bone marrow responded rapidly to chemotherapy, the elbow lesion increased in size. Despite intense treatment, the tumor relapsed in lungs and the patient died 12 months after diagnosis. Review of the literature showed twenty additional congenital MRTs arising from sites outside of the kidney and central nervous system were published in the literature. Eighteen patients had disseminated disease at diagnosis. The median overall survival time for all (n = 21) patients was 2.0 months (0–24 months). The only patient who survived had a localized tumor at initial diagnosis. Congenital, extrarenal, non-CNS MRTs are aggressive tumors with poor outcome.

Bilateral perioptic nerve lesions in a patient with Langerhans-cell histiocytosis

Langerhans-cell histiocytosis (LCH) is a disease caused by proliferation of abnormal histiocytic cells of the Langerhans cell system. It includes the diseases formerly described as histiocytosis X: eosinophilic granuloma of bone, Hand-SchiillerChristian disease and Letterer-Siwe disease. 1 LCH predominantly affects children 1 to 4 years old, although it can affect persons of any age. The estimated incidence is 2 to 5 per million per year in the pediatric population. Lesions can involve a variety of organ systems, including bone, skin, lymph nodes, bone marrow, liver, spleen, lung, gastrointestinal tract, thymus, endocrine glands and the central nervous system (CNS). The morbidity and prognosis of LCH depend primarily on age and the number of organ systems involved. 1 Recently the results of the first randomized international clinical trial of the Histiocyte Society indicated another prognostic factor: patients with systemic LCH who do not respond to therapy within 6 weeks are at increased risk for treatment failure, up to two-thirds dying of this disease.2

Polyoxoanions are cytotoxic to malignant glioma cells.

We studied the cytotoxicity of five polyoxoanions on two human malignant glioma cell lines (T98G and 86HG39), a rat glioma cell line (C6) and a human flbroblast cell line (NIH-3T3) using MTT tests to measure the drug concentration killing 50% of the cells (LC50). Cisplatin was used as a reference agent. Cisplatin had the highest efficacy in three of the four cell lines. Only in T98G cells, one of the components (POA5) had a lower LC50 value (1.3x10-6mol/l) than cisplatin (2.5 x 10-6-6). POA5 was also the most cytotoxic polyoxoanion when the LC50 values of all four cell lines were averaged (6.6x10-6). Average LC50 values of the other compounds were 10.9, 12.6, 19.0 and 19.2x10-6 mol/l in POA1, POA2, POA3 and POA4, respectively. When the benign fibroblasts were used to calculate a therapeutic index as LC50 in fibroblasts divided by LC50 in glioma cells, POA5 was superior to cisplatin. These results indicate that polyoxoanions are cytotoxic for malignant glioma cells and that the most promising compound investigated here was POA5. [( 1998 Llpplncott Williams & Wllklns.