Klinika Detske Onkologie

Brno, Czech Republic

Klinika Detske Onkologie

Brno, Czech Republic
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Geryk E.,Odbor vedy a vyzkumu | Stampach R.,Masaryk University | Bajciova V.,Klinika detske onkologie | Horvath T.,Chirurgicka klinika
Onkologie (Czech Republic) | Year: 2014

The neoplasms following primary cancers of cervix (CC), uterus (CU), ovary (CO), and primary neoplasms before these gynaecological cancers (GC), were analyzed in a retrospective study in survived females aged 0-49 years, notified in the National Czech Cancer Registry between 1976 and 2010. A total 3,727 patients of these three diagnoses, presented 12.1 % of 30,794 newly registered GC, were associated with 4,160 other neoplasms. Their development was concerned with a) 1,339 females with primary CC, 715 with CU and 664 with CO (followed by 1,509 and 831, respectively 758 other neoplasms) and b) subsequent 263 CC, 212 CU and 534 CO (preceded by 274 and 223, respectively 565 primary neoplasms). The average interval of occurrence of subsequent neoplasms was 14.2 years after CC, 13.6 years after CU and 10.2 years after CO. The most frequent of 3.098 subsequent neoplasms were 21 % of digestive tract, 16 % breast, 13 % skin, 11.7 % female genital, 9.7 % respiratory and 7.8 % urinary organs; the most frequent during the first year after primary GC were those of the ovarial, endometrial, cervical and colorectal cancers. The most frequent of 1,062 primary neoplasms before GC were 26.7 % cancers of female genital organs, 24.3 % breast and 14.8 % digestive tract. During 35 years the number of females with primary GC has decreased, especially of their representation at the early clinical stages, the females with subsequent GC has increased, especially at the advanced stages. There were recorded early stages in 70.4 % females with primary GC and 54 % with subsequent GC, advanced stages in 29.5 % females with subsequent GC and 13.3 % with primary GC, the unknown stages included about 16.5 %. In our study are estimated about 1100 cases of advanced stages in females aged 49 years survived with GC in 1976-2010. Most of the evaluated criteria were unfavorable among females with ovarian cancers. Mainly the cases at advanced stages must be prevented by the dispensary guidelines and preventive interventions. Limited financial resources and persistent risks of lifestyle do not allow to slow down the increase of primary and subsequent neoplasms in the near future among cancer survivors not only in gynaecology.

Broad spektrum of hereditary cancer predisposition syndromes affecting pediatric population. Early genetic testing and diagnosis may assist in patient dispensarisation and management. Earlier diagnosis of the cancer and improve prognosis and overall survival. A general pediatrician is usually the first person to suspect some dyspmorhology and possible genetic syndrome.

Bajciova V.,Klinika detske onkologie
Onkologie (Switzerland) | Year: 2013

Melanoma is a rare malignancy in the pediatric population, but its incidence has risen very rapidly, specially in adolescent girls. We are presenting review of the current clinical and biological features of pediatric melanoma and problems with its diagnosis and management. Despite the differences between pediatric and adult melanoma survival rates are very similar. Age specific pediatric clinical studies are essential for management of metastatic pediatric malignant melanoma. Prevention and erly detection are also the point of interest.

Nursing care of patients who have undergone transplantation of bone marrow or peripheral stem cells is highly specialized care provided by specialized haematological-oncology centres. This care is provided not only to adult patients, but also to children. To provide immunocompromised patients with the most suitable conditions for their treatment. To ensure that these patients are as little threatened by external infections as possible and that the level of their social isolation from their family and the surroundings is as low as possible. The level of reverse-protective isolation is a subject of discussions by specialists all over the world.

There is a broad spectrum of hereditary cancer predisposition syndromes affecting pediatric population. Early genetic testing establishing the diagnosis may assist in patient dispensa-risation and management. Secondary prevention and prompt diagnosis of cancer improves the prognosis and overall survival. A centralised dispensarisation of pediatric patients with hereditary cancer predisposition syndromes on pediatric oncology department offers complex multidisciplinary care to the patient and his family.

Renal tumors occur accross the age. The age specific differences are described not only on histology type of tumor, but also on epidemiology, ethiology, pathogenesis, biology studies and clinical behavior. These age specific differences are noticed also in the same type of the tumor and have an effect on prognosis and survival of the patients. The remaining question is regarding current treatment guidelines - are these guidelines really suitable for all age groups or not.

In the Czech Republic, more than 60 000 new oncology patients are diagnosed each year, and approximately 300 of these are pediatric oncology patients. In developed countries including the Czech Republic, malignant neoplasms are the second most frequent cause of death in children, even if it is managed to return around 80 % of these pediatric patients to their normal lives. Care for these patients is highly specialized and demands a multidisciplinary team and approach. Specifics of pediatric oncology are by no means taught in detail during the qualification of general nurses. Nurses taking care of pediatric oncology patients in centers of complex pediatric oncology care complete their specialized education (pediatric nurse, nursing care in oncology) during their work practice. Work in pediatric oncology from the nurse's point of view is very demanding not only expertly and psychically, but also humanly.

Tuberous sclerosis complex is a neurocutaneous syndrome that results from a germline muta-tion in TSC1 or TSC2 genes. The pathogenic activation of mTORCI leads to the development of subependymal giant cell astrocytomas in patients with tuberous sclerosis complex. Blocking of the dysregulated pathway with mTOR inhibitors has the potential to reduce the volume of this low-grade brain tumor. This article reviews the current knowledge on the pharmacological treatment of subependymal giant cell astrocytomas. A long-term follow-up and early therapeutic intervention should lead to mortality and morbidity reduction and quality of life improvement in patients with tuberous sclerosis complex associated tumors.

Background: Malignant melanoma is a rare malignancy in the pediatric population. Etiology is usually unknown. Clinical symptoms are non-specific, clinical behavior and biology features may differ from those in an adult population. The most important prognostic factor is spread of disease. Surgical resection is treatment of choice for localized melanoma. Advanced and metastatic melanoma is still an incurable disease. Case: We are presenting an eight-year-old boy with metastatic malignant melanoma of unknown origin based on TP53 mutation (Li-Fraumeni syndrome). He underwent surgery and adjuvant chemotherapy (temozolomide as single agent). Complete remission was achieved at the end of treatment. Two years after the end of therapy (and 31 months from diagnosis) he developed metastatic progression to the lungs. He has received immunotherapy with ipilimumab, according to our knowledge as the first child under the age of 12 in Europe. He completed three courses of ipilimumab, with irAE (immune related adverse event) grade III during the first course of anti CTLA-4. Therefore, further doses of ipilimumab were given with corticoids and antihistamines as premedication. Also, asymptomatic thyreoiditis grade II has been confirmed. The best documented treatment response is stable disease. Performance status was excellent. Three years since the first progression, he developed further massive progression to the lungs. Second line immunotherapy with anti-PD-1 monoclonal antibody (pembrolizumab) is currently going on. So far, the overall survival of the patient is 74 months. Conclusion: The presented case study supports the administration of immunotherapy in children younger than 12 years. Therapeutic effect has led to significant overall survival with tolerable toxicity. The problem remains significantly limited number of pediatric clinical trials using immunotherapy.

Bajciova V.,Klinika Detske Onkologie
Klinicka Onkologie | Year: 2015

Background: Currently, most children with cancer can be cured with standard therapy (surgery, chemotherapy, radiotherapy). The only limiting factor is its severe acute toxicity and late adverse events. In pediatric oncology, immunotherapy has been delayed but the initial clinical trials of immunotherapy show a good tolerance and promising results, especially in the setting of refractory or recurrent high-risk tumors. In this article, we will discuss a current situation in pediatric oncology, what immunotherapies are being tested in the clinical practice, from monoclonal antibodies, checkpoint inhibitors to tumor vaccines, T-lymphocytes with chimeric antigen receptors, cytokines and innate immunity. Conclusion: Immunotherapy is a promising treatment modality for children and adolescents with recurrent high-risk cancer with potential to improve both survival and quality of life. The challenge of developing immunotherapies in pediatric oncology remains - age barriers for using new drugs and a limited number of pediatric clinical studies.

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