CC BY
16
May 2016, Moscow, Russia
OF PEDIATRIC HEMATOLOGY and ONCOLOGY
OTHER SOLID NON-CNS TUMOURS
ABSTRACT NO.: PP-346
The nasopharyngeal cancer in children: Epidemiologic aspect and scalable
Sabah Sal
Residanat
Key words: chemosensitivity, radiocurabilite, nasopharyngeal, cancer, epithelial
Introduction. Malignant neoplasm of nasopharynx are mainly represented by the nasopharyngeal carcinoma (NPC) which is a tumor of epithelial origin. The most common historical-clinical entity is undifferentiated type nasopharyngeal carcinoma, the most common Carcinoma of the child who has a particular geographical distribution: Algeria - MAGHREB, the relationship EBV-nasopharyngeal carcinoma is highlighted. Aim. Through this work we studied the epidemiological, clinical and therapeutic characteristics in maghreb area.
Materials and methods. This work consists of a retrospective study of the epidemiological profile of the nasopharyngeal carcinoma in childhood at the level of Department oo
of Medical Oncology Centre Pierre and Marie Curie, on a sample of 43 cases a period of 2009-2015. 3
Among the studied cases of nasopharyngeal cancer, 65 % were male and 32 % of female. We were 9 deaths due to such cancer among which 11.6 % were boys and 9.3 % of the girls. O
At the level of the sample, the average age of children with nasopharyngeal carcinoma was 12 ± 3 years. Almost all children have received neoadjuvant chemotherapy and radio-concomitant chemotherapy, the frequency of locally advanced stages was 25.58 % for girls and 37.2 % for boys. i—
Results. Rates of remission have been obtained; 23 % among girls and 55.8 % among boys, progressions well led post-treatment: 11.6 % among girls and 13.95 % among boys. There were deaths: 9.3 % girls and 11.6 % boys. y
Conclusion. The nasopharyngeal carcinoma is frequent in Algeria, it is lymphophilie and has a high metastatic potential. ^
It characterized by its radiocurabilite and its chemosensitivity, but the diagnostic delay exposes local and systemic dissemination. 2
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AoSTcACT disease oriented
SOCltTt INTERNATIONALE D'ONCOLOGIE PEHATRIQUE
SIOP ASIA CONGRESS
ABSTRACT NO.: P-384
Aggresive fibromatosis in childhood at the oncologic pediatric service of National Oncology Institute of Cuba (2003-2013)
Migdalia Perez, Mariuska Forteza, Jose Alert, Jesus Reno
National institute of Oncology and Radiobiology of Cuba, Havana
Key words: fibromatosis, neoplasms
Introduction. Aggressive fibromatosis, also called desmoide-tumor is a rare benign tumor, which originates from structures deep musculo-fascial and/or the periosteum; and they are locally aggressive, prone to local recurrence. Not usually give distant metastases, but may infiltrate vital structures, and be life enfermo. 1,2 comprising 0.03 % of neoplasms in general and 3 % of all tumors parts blandas. 3,4 an incidence of 2-4 cases it is estimated per million inhabitants, and about 900 new cases are diagnosed annually in United States. 5 Its etiology is unknown, has been associated with surgery and / or previous trauma, pregnancy, estrogen therapy and of Gardner syndrome. The most common age of onset is during the first decade of life, but there are reports of all ages. The most common locations are: abdominal. Histological diagnosis and treatment is mainly the surgery.
Aim. To describe the clinical characteristics of and the treatment prescribed for patients with diagnosis of aggressive fibromatosis, who were treated at the oncologic pediatrics service of the National Institute of Oncology and Radiobiology.
Materials and methods. A descriptive, longitudinal and retrospective study. The universe consisted of all patients with malignant diseases in the INOR Oncopediatrics service; and the sample consisted of 9 patients with histologically aggressive fibromatosis, from 1 January 2003 to 31 December 2013, with prior approval of the informed consent of parents or guardians in each case.
The variables studied were: age, sex, location, clinical presentation, imaging studies (plain radiographs and computed tomography (CT) and/or magnetic resonance imaging (MRI)) of the affected area as well as diagnostic biopsy immunohistochemical study all cases and treatment modality.
The analysis of contingency tables was used for the studied variables using Fisher's exact test, with a confidence interval of 95 %. The results were presented in tables and figures. Data were processed in Excel and SPSS version 15.0.
Results. A total of 510 pediatric patients with malignant disease were evaluated in the selected period; of these, 9 had a diagnosis of aggressive fibromatosis, with a slight predominance of males (56 %) with a mean age of 9 years (range between 0 and 9), and only 22 % were older than 11 years.
The locations were: head and neck, accounting for 55 %, followed by gluteal and lower limbs. All patients underwent imaging studies. The diagnosis was made by incisional biopsy of the lesion in all patients. The exclusive surgery was used only in 3 patients, and in other cases surgery radiotherapy (RTP) and chemotherapy (QTP) combined. Right now we have a 100% survival.
Conclusion. The most common age of onset of aggressive fibromatosis is under 10 years, with a slight predominance in males. The palpable tumor mass is the main clinical manifestation, with a higher location in the head and neck. The main treatment is surgery, however, other therapeutic modalities should be included to achieve local control of the disease.
ABSTRACT NO.: P-392
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Results of investigation of main complex of HLA-phenotype at patients with solid malignant tumours
Yu.Yu. Kozel, S.A. Kuznetsov, M.V. Starzeckaya, G.A. Mkrtchyan
O Rostov Research Oncology Institute, Rostov-on-Don, Russia
Key words: solid malignant tumours, main complex of histocompatibility
O Introduction. Nowadays there is a theory that disease is a result of intercourse of environment and genetic factors. Among genetic factors the important place is taken by the main
cc complex of histocompatibility nowadays. There is a big interest now in investigation of correlation between tumours and HLA-specificity. These investigations are important for early
^^ diagnosing of oncological diseases.
Aim. Genetic typing of blood of children with solid malignant tumours for determination of prognosis of risk and forming of the risk groups.
Materials and methods. One hundred and thirty patients at age 1 to 18 years old treated in department of pediatric oncology of RROI with different solid malignant tumours were investigated: 21 (18.6 %) patients with neuroblastoma, 21 (18.6 %) with nephroblastoma, 20 (17.7 %) with osteosarcoma, 20 (17.7 %) with Ewing sarcoma, 31 (27.4 %) with germinal tumours. Molecular typing of HLA-gene DRB1 performed with the help of polymerase-chain reaction, allowed to reveal 13 groups of alleles HLA-DRB. Statistical difference was estimated with the help of x2 criterion with Yates allowance for continuity for the small selection.
Results. Results of analysis of children with solid tumours in comparison with healthy donors revealed the significant increasing of frequency of the following HLA-specificities: A26 - 20 % (control - 5.35 %) (P < 0.02), B51 - 30 % (control - 12.35 %) (P < 0.05 %) - at Ewing sarcoma; A24 - 38.09 % (control - 16.46 %) (P < 0.02), B13 - 38.09 % (control - 11.6 %) (P < 0.01), DRB 1*04 - 42.85 % (control - 18.75 %) (P < 0.05) - at neuroblastoma; DRB 1*13 - 57.14 % (control - 29.68 %) (P < 0.05) - at nephroblastoma; A26 - 16.12 % (control - 5.35 %) (P < 0.05) - at germinal tumours.
Conclusion. Thus, in case of revealing the following alleles the child should be considered as a patient at a risk of Ewing sarcoma (A26, B51) (P < 0.05), neuroblastoma (A24, B13, B44, DRB 1*04) (P < 0.01), nephroblastoma (DRB 1*13) (P < 0.05) and germinal tumours (A26) (P < 0.05).
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