447 research outputs found

    Management of Childhood Malignant Peripheral Nerve Sheath Tumor

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    Abstract Malignant peripheral nerve sheath tumor (MPNST) is rare, but is one of the most frequent non-rhabdomyosarcoma soft-tissue sarcomas in the pediatric population. These tumors occur most frequently at axial sites and are characterized by local aggressiveness and a propensity to metastasize. They are often associated with neurofibromatosis type 1 (NF-1): the lifetime risk of patients with NF-1 developing MPNST has been estimated at 8-13%, compared with 0.001% in the general population. Because of the rarity of this tumor, little information is available on its clinical management, particularly in the pediatric age group. In a recent report on the clinical findings and treatment outcomes from a large number of children and adolescents with MPNST in an Italian and German series, less satisfactory overall outcomes than those for other pediatric sarcomas were described. Therefore, the approach to the treatment of patients with MPNST should be aggressive and risk adapted, and is necessarily complex. Patients should be referred to selected institutions with adequate experience in treating soft-tissue sarcomas, and with the multidisciplinary skills for enrolling patients in clinical trials. Surgical resection represents the mainstay of treatment, while the role of adjuvant treatment is not yet clear. Post-operative radiotherapy seems to have a role in improving local control, although the potential morbidity of irradiation should be taken into account, particularly when treating children. Although lack of local control is the major cause of treatment failure, MPNST may give rise to distant metastases. These tumors are usually considered as having uncertain chemosensitivity, but recent evidence suggests that there may be a role for chemotherapy in patients with a high-grade histology. For the near future, our hopes lie in the development of novel tailored therapies directed specifically against the molecular targets of the neoplastic cells: soft-tissue sarcomas seem particularly promising candidates for targeted therapy

    Ifosfamide in pediatric solid tumors.

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    Phase II studies conducted in Europe and the USA on pediatric solid tumors have shown that ifosfamide, as a single agent, is an active drug against a variety of neoplasms - rhabdomyosarcoma (RMS), some non-RMS soft tissue sarcomas, Wilms' tumor, bone sarcomas and neuroblastoma. Furthermore, an increase in tumor response rate has been observed when ifosfamide has been used in combination with other drugs. The usual dose of ifosfamide varies from 1.8 to 3 g/m(2)/day for 2-5 days according to the different regimens. Some controversies still exist on the modality of drug administration and more precisely on the time of infusion, however in pediatric practice, short infusion (e.g. 3 h) is usually preferred because of the reduced neurotoxicity in comparison to lengthier administration (e.g. 24 h). Ifosfamide is currently included in the standard therapy of pediatric bone and soft tissue sarcomas. It is also used in a selected high-risk group of patients with Wilms' tumor, neuroblastoma and germ cell tumors. Copyright 2003 S. Karger AG, Base

    Preparation of aloe-emodin derivatives and their use in the treatment of neoplasias

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    The invention relates to prepn. of aloe-emodin (AE) derivs. such as I [R = satd. or unsatd. linear or branched aliph. polycarboxylic acid, arylic polycarboxylic acid, amino acid, acetal of an amino sugar, inorg. acid], for their use as anticancer drugs. Thus, reaction between aloe-emodin with Boc-L-Ala-OSu provided II (R = Boc), which upon deprotection and treatment with concd. trifluoroacetic acid, afforded II [R = H.CF3CO2H (III)]. I show a specific cytotoxicity to tumor cells, also of neuroectodermal origin, to which they may in particular act as aloe-emodin prodrugs. Pharmaceutical compns. contg. I may be used in the treatment of neoplasias. It has surprisingly been found that aloe-emodin derivs. in position 3' (bearing either a pos. or neg. charge) exhibit improved soly. properties and, at the same time, in vitro show cytotoxicity to tumor cells, also of neuroectodermal origin. III exhibited cytotoxicity [EC50 = 6.7 μM, EC50 = 6.7 μM, and EC50 = 5 μM against neurolastoma (S-J-R-KP), neurolastoma (IMR-5), and adenocarcinoma (LoVo 109), resp]
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