Therapy results in pediatric Hodgkin lymphoma - does less mean better? Experience from a single children's oncology center.

Therapy results in pediatric Hodgkin lymphoma reflect remarkable progress in pediatric oncology. In the last decade, relevant development of new therapeutic options for children with refractory or relapsed disease has been made. In this study, we retrospectively analyzed therapy results and risk factors in children treated in a single oncology center according to five therapeutic protocols.

Lymphocyte Subpopulations in Patients With Neurofibromatosis Type 1-associated Optic Pathway Gliomas and Plexiform Neurofibromas.

Background/aim: Neurofibromatosis type 1 (NF1) is characterized by the occurrence of multisystem tumors, among which the most characteristic are optic pathway gliomas (OPGs) and plexiform neurofibromas (PNFs). With the development of new anticancer drugs targeting the immune system, it is important to examine the immunological status of patients with NF1. Furthermore, the immune system has been suggested as a probable modulator of NF1-associated phenotypes.

Coordinated medical care for children with neurofibromatosis type 1 and related RASopathies in Poland.

Coordinated medical care offered in Poland for patients suffering from neurofibromatosis type 1 and related RASopathies combines complex multispecialty consultation with permanent supervision and the patient's oriented longitudinal care. Neurofibromatosis type 1 is one of the most common single gene disorders in the global population, observed in 1 out of 2500-3000 live births. It is a primary neoplasia disease with 100% penetration of the gene mutation but remarkable age-dependent onset of different disease signs and symptoms, outstanding clinical heterogeneity between patients even in one family and lack of genotype-phenotype correlation, a high rate of spontaneous mutation exceeding 50%, and multiple comorbidities among which increased risk of malignancy is the most important.

Demographical Profile and Spectrum of Multiple Malignancies in Children and Adults with Neurocutaneous Disorders.

Background/aim: Neurocutaneous disorders, also referred as phacomatoses, are congenital disorders manifesting at different ages with central nervous system and cutaneous abnormalities. Analysis of the demographic and clinical profile of patients with phacomatoses in the context of the incidence and spectrum of malignancy.

Materials And Methods: This is a retrospective analysis of 20 years of data in a single-center study in Poland.

[Neurofibromatosis type 1--own experiences].

Purpose: Neurofibromatosis type 1 is one of the most common inherited syndromes. The aim of this study was to evaluate eye symptoms on this disease.

Material And Methods: 52 patients with neurofibromatosis type 1 were observed (28 males and 24 females), age between 3 and 49 years old (mean 21).

[Pathogenesis and the most frequent symptoms of neurofibromatosis type 1].

This study was to evaluate pathogenesis, symptoms, clinical course and possible treatment of neurofibromatosis type 1. Neurofibromatosis type 1, von Recklinghausen's disease, is one of the phacomatoses. It belongs to the most frequent inherited diseases in human population.