-
Anna Balcerska,
Jan Godziński,
Ewa Bień,
Teresa Stachowicz-Stencel,
Wojciech Sulka, Małgorzata Rapała,
Ewa Izycka-Swieszewska,
Marta Perek-Polnik,
Monika Drogosiewicz,
Danuta Perek, [......],
Jerzy Bodalski,
Agnieszka Pobudejska,
Danuta Sońta-Jakimczyk,
Jarosław Peregud-Pogorzelski,
Janusz Fydryk,
Sylwia Lapińska-Szumczyk,
Janusz Emerich,
Przemysław Mańkowski,
Andrzej Jankowski,
Wojciech Woźniak
[show abstract]
[hide abstract]
ABSTRACT: Clear statement that pediatric neoplasms are really rare is not easy. Thus the incidence of rare tumours in children has not been defined so far. The paper efforts to assess the topic of rare tumours of childhood in the Polish population. Following two categories are proposed: tumours typical for adults, but possible in children (neoplasms of epithelial origin--mainly carcinomas, melanomas, carcinoids) and paediatric tumours consisting less than 10% of cases in corresponding clinical groups according to the ICCC classification. Data on 317 patients aged 0-18 years treated in centres associated in the Polish Paediatric Group for Solid Tumours (PPGST) were analysed. Classical adult malignancies were registered in 130 patients: carcinomas in 90 (mean age 12.6 +/- 4.5 years), melanomas in 25 (mean age 9.4 +/- 4.9) and carcinoids in 9 (mean age 14.5 +/- 1.2 years). Non epithelial neoplasms were registered in 187 patients (mean age 10.4 +/- 5.5). That group included rare tumours of soft tissue, CNS, bones and other organs. Treatments of certain groups were specified by separate therapeutic protocols within PPGST. Rare malignancies of adult-type among children under 18 years of age in Poland comprised 1.5% of all pediatric neoplasms. The incidence of adult-type neoplasms increased with age until 14 years. In patients over 15 years of age the number of registered cases decreased. It may suggest a first peak of incidence in early adolescence or an underestimation of number of patients with carcinoma aged over 15 years. In the analyzed group, the mean age of patients with carcinomas and other epithelial and unspecified tumours significantly exceeded the age of children with rare neoplasms of non-epithelial origin (12.1 +/- 4.7 vs 10.4 +/- 5.5 years; p<0.05). A very young age at diagnosis of malignant melanomas (mean 9.4 years) and numerous cases of carcinomas affecting the digestive tract (n=24; 27% of all carcinomas), especially those located in colorectal region (n=10), seem surprising. The preliminary analysis of the collected data on rare neoplasms in Poland encourage to undertake a prospective study, meant to link the epidemiology and characteristics of rare epithelial tumours in childhood with diagnostic and therapeutic suggestions for these types that are not coordinated within Polish Paediatric Group of Solid Tumours.
Przegla̧d lekarski 02/2004; 61 Suppl 2:57-61.
-
[show abstract]
[hide abstract]
ABSTRACT: Ewing's sarcoma and PNET are more and more frequently submitted to surgical treatment, as this method of local therapy seems to offer a better chance for cure. Which method surgery or radiotherapy (RTx) is more mutilating in childhood, should be carefully considered. The aim of the report is to review the possibility of local resection and reconstruction in various sites of the tumour. PATIENTS: 12 patients with Ewing's sarcoma / PNET were submitted to the site-specific surgical procedures and assessed for local control and function. All were pre-treated with CHT and continued that treatment after surgery. Four patients had fibular Ewing's sarcoma and all underwent subtotal fibulectomy. Long term complete remission (CR) was achieved in all. Two patients had Askin tumours; both developed pleural relapses distant to the primary tumour site. One patient had total scapulectomy (CR for 7 years). One patient had hemipelvectomy supplemented with RTx because of micro residue (CR for 10 years). Two patients were amputated: one for PNET of tibia invading nerves and vessels, another one for Ewing's sarcoma of femur. The first one developed pulmonary metastases and died one year later, the second one has been alive for 13 years but has badly accepted the prosthesis. Two patients with very extensive tumours of pelvis could never be operated on, they never achieved CR despite chemo- and radiotherapy and died. SUMMARY: Of 10 patients submitted to surgery 7 patients are alive and 4 have an excellent functional result. Of the 2 patients in whom the tumour was inoperable neither is alive. It seems justified to recommend surgical resection of Ewing's sarcoma / PNET; satisfying reconstruction is usually feasible. RTx seems to have value rather as a method supplementing minor failures of surgery than as the only local treatment.
Medycyna wieku rozwojowego 8(4 Pt 2):1063-9.
-
[show abstract]
[hide abstract]
ABSTRACT: To analyze the perioperative period in children who required surgery anticipated in the protocol and suffered from prolonged bone marrow suppression.
The surgical treatment of 15 children with prolonged bone marrow suppression, resistant to GM-CSF for 5-15 weeks (M=10), was retrospectively analyzed. Criteria to submit a patient to the group were: neutropenia <1000/ml or/and thrombocytopenia <70,000/ml. Four had thrombocytopenia, 8 neutropenia and 3 cytopenia of both cell lines. Neutropenic patients were divided into two groups: severe neutropenia--<500/ml (2), and moderate--500-1000/ml (7). Children with thrombocytopenia were divided into two groups: with thrombocytes <30,000/ml (2) and between 30,000-70,000/ml (5).
Complications (pneumonia and sepsis) were noted in 2 out of 4 children with severe neutropenia, remaining patients had uncomplicated perioperative period. Patients with moderate neutropenia did not show any alarming signs after surgery. As a major haemorrhage we assumed loosing > 30 percent of circulating blood. This was observed only in 1 patient (with thrombocytes <30,000/ml). 13 patients were discharged from our Department between 3-10 days after surgery in good general condition. Two had to be treated for a longer time because of advanced cancers (18 and 21 days).
Neutropenia does not seem to be an absolute contraindication for surgical procedures needed in complex cure of malignancies. In our opinion thrombocyte level > 30,000/ml appears to be safe enough to conduct surgery.
Medycyna wieku rozwojowego 10(3 Pt 1):793-801.
-
Ewa Bień,
Jan Godziński,
Anna Balcerska, Małgorzata Rapała,
Ewa Izycka-Swieszewska,
Teresa Stachowicz-Stencel,
Wojciech Sulka,
Bernarda Kazanowska,
Adam Reich,
Alicja Chybicka, [......],
Andrzej Jankowski,
Joanna Nurzyńska-Flak,
Jerzy Kowalczyk,
Andrzej Kurylak,
Mariusz Wysocki,
Aleksandra Rybczyńska,
Jacek Wachowiak,
Beata Zalewska-Szewczyk,
Jerzy Bodalski,
Kazimierz Jaśkiewicz
[show abstract]
[hide abstract]
ABSTRACT: Malignant vascular tumours represent a rare group of neoplasms, usually treated according to protocols for soft tissue sarcomas.
To assess the clinical characteristics, disease course and outcome in Polish children with malignant vascular neoplasms.
The Polish Paediatric Rare Tumours Study retrospectively analysed multicentre data on 32 children with vascular tumours, registered between 1992 and 2002. On the basis of the histological type of the neoplasm these patients have been divided into three groups: group I -- 10 patients with angiosarcoma (ASA), group II -- 7 children with haemangioendothelioma (HE) and group III- 15 patients with haemangiopericytoma (HP), of both infantile (7 children) and adult-types (8 patients).
Group I: 5 patients presented with local, 3 with systemic and 2 with regional disease. Primary complete resection (PRC) was performed in 7 patients, not resulting in local control in any of them. Radiotherapy (RTX) was administered in 5 patients, chemotherapy (CHT) in all. 7 patients relapsed, 3 never entered clinical remission. 9 of the 10 patients of group I, died of disease progression. Group II: PRC was performed in 5 patients and led to local control in 2. Adjuvant RTX was used in 2 and CHT in 4 patients. 2 children relapsed and died of the disease. Infantile HP: PRC was performed in 3 children, remaining 4 patients received adjuvant CHT All children entered complete remission and are disease-free. Adult-type HP: PRC was performed in 5 patients, resulting in local control in 4. Five children were given adjuvant RTX and six CHT Three patients relapsed and died of disease progression.
The effectiveness of primary complete resection in all groups was doubtful. The high rate of metastatic relapses suggests that the currently given systemic therapy is not satisfactory. The only tumour with excellent prognosis was infantile type HP (all patients are alive and free of disease). Adequate treatment for children with angiosarcoma remains still unknown -- 9 of 10 patients died of disease progression. Prognosis in patients with haemangioendothelioma is intermediate, however the role of immunotherapy should be further investigated.
Medycyna wieku rozwojowego 8(2 Pt 1):145-58.
