ABSTRACT
Angiosarcoma in children - still uncontrollable oncological problem. The report of the Polish Paediatric Rare Tumours StudyAngiosarcoma is a rare, highly malignant vascular neoplasm with little data available on its clinical course and management in children. Ten children with angiosarcoma (M/F: 6/4; aged 2, 3-16 years) registered in Polish Paediatric Rare Tumours and Soft Tissue Sarcomas Studies between 1992 and 2006. Primary tumour exceeded 5 cm in seven patients and affected mainly deep tissues (heart-2, head/neck, bladder, brain, liver and upper limb - one patient each). Four patients had regional and two metastatic diseases (lungs and bones). Three patients were initially misdiagnosed as haemangioma. Complete primary excision was unfeasible even in local stages. All patients received supplementing chemotherapy with no response in four. Radiotherapy was given to five children, including three after relapse. Three of five secondary tumour resections proved complete. Seven patients experienced relapses (mainly metastatic) and two continuous progression. Relapsed patients received chemotherapy +/- radiotherapy and surgery (three). Nine patients died of disease (overall survival 6-66 months), and one child after mutilating secondary resection is alive. Angiosarcoma in children is highly aggressive with an extremely poor prognosis. Complete primary excision is unfeasible, even in seemingly local stages. The response to chemotherapy is poor and the large number of metastatic recurrences suggests a need for systemic therapy modifications.
Subject(s)
Hemangiosarcoma/pathology , Hemangiosarcoma/therapy , Sarcoma/pathology , Sarcoma/therapy , Adolescent , Antineoplastic Agents/therapeutic use , Child , Child, Preschool , Disease Progression , Hemangiosarcoma/mortality , Humans , Male , Poland/epidemiology , Prognosis , Radiotherapy , Recurrence , Retrospective Studies , Sarcoma/mortality , Survival RateABSTRACT
Forty-seven children treated in various Polish centers between 1985 and 1995 for primary malignant liver tumors were retrospectively analyzed. Hepatoblastoma (HB) prevailed--it was found in 39 cases. There were 6 hepatocarcinoma (HCC) cases and 2 cases of undifferentiated sarcoma (UDS). In 44% of HB patients the tumor involved both liver lobes. 18% of children with HB presented with pulmonary metastases at diagnosis. Chemotherapy was applied in 92% of cases (preoperatively in 67%). Tumor resection was performed in 56% of HB patients. Overall survival of patients with hepatoblastoma was 43.6%, while it was 50% for hepatocarcinoma and 100% for undifferentiated sarcoma (2 cases only). Mean observation time was 58 months. The hepatoblastoma subgroup, being the largest (83% of all cases), was analyzed separately for prognostic factors. Completeness of tumor excision strongly influenced survival. Involvement of both lobes of the liver and multifocality of the tumor were other adverse prognostic factors.
Subject(s)
Carcinoma, Hepatocellular/surgery , Hepatoblastoma/surgery , Liver Neoplasms/surgery , Neoplasms, Germ Cell and Embryonal/surgery , Antineoplastic Agents/therapeutic use , Carcinoma, Hepatocellular/epidemiology , Child , Child, Preschool , Combined Modality Therapy , Female , Hepatoblastoma/drug therapy , Hepatoblastoma/epidemiology , Humans , Liver Neoplasms/drug therapy , Liver Neoplasms/epidemiology , Male , Neoplasms, Germ Cell and Embryonal/epidemiology , Poland/epidemiology , Retrospective Studies , Survival RateABSTRACT
7-year-old girl treated for acute lymphoblastic leukaemia since June 1995 after marrow relapse (June 1997) presented with painful oedema of the upper part of left hip and limping, 16 months after allogeneic bone marrow transplantation (BMT). Bone marrow examination excluded medullary relapse. Histopathological investigation of periosteum and bone scrapings revealed massive leukemic infiltration. Radiotherapy resulted in local arrest of the malignant process. However, bone marrow relapse was diagnosed in the child two months later with subsequent death after one month.
Subject(s)
Bone Marrow Transplantation , Hip Joint/pathology , Leukemic Infiltration/pathology , Precursor Cell Lymphoblastic Leukemia-Lymphoma/pathology , Child , Fatal Outcome , Female , Hip Joint/diagnostic imaging , Humans , Leukemic Infiltration/diagnostic imaging , Precursor Cell Lymphoblastic Leukemia-Lymphoma/surgery , Radiography , Recurrence , Transplantation, HomologousABSTRACT
43 patients aged between 4 days and 10 years 8 months with Wilms' tumor were treated according SIOP 92-01 protocol. Clinical stage, therapy results and adverse events were analysed. In presented group 9 complete recoveries were obtained in the period of over 30 months, in 94%--without clinical symptoms of disease the during therapy or during observation period shorter than 30 months. There were 14% of deceases. Neoadjuvant chemotherapy allowed to decrease the intraoperative stage of the disease progression. In analysed group stage II N (-) was the most common, what is higher in comparison with other European groups. The problem is the treatment of bilateral and generalized Wilms' tumour.
Subject(s)
Kidney Neoplasms/pathology , Kidney Neoplasms/therapy , Wilms Tumor/pathology , Wilms Tumor/therapy , Chemotherapy, Adjuvant , Child , Child, Preschool , Disease Progression , Female , Humans , Infant , Infant, Newborn , Kidney Neoplasms/mortality , Male , Neoplasm Staging , Radiotherapy, Adjuvant , Remission Induction , Survival Rate , Treatment Outcome , Wilms Tumor/mortalityABSTRACT
Retrospective analysis of chemotherapy results of children with nephroblastoma was performed in 220 patients aged from 1 yr to 14 yrs of live in 12 centers. Stage I nephroblastoma was documented in 24.5% but stage II--in 55.3%. Histologically 74.6% cases were diagnosed as medium malignant and 12.7%--high malignant. Therapy results were similar to observed in other centers.
