Stachowicz-Stencel Teresa, Bień Ewa, Zawitkowska-Klaczyńska Joanna, Dudkiewicz Ewa, Drozyńska Elzbieta, Katski Krzysztof, Polczyńska Katarzyna, Sierota Danuta, Stefanowicz Joanna, Szołkiewicz Anna, Kaczorowska-Hać Barbara, Czauderna Piotr, Stoba Czesław, Kosiak Wojciech, Czarniak Piotr, Balcerska Anna, Kowalczyk Jerzy R
Klinika Pediatrii, Hematologii, Onkologii i Endokrynologii, Akademia Medyczna, Debinki 7, 80-211 Gdańsk, Poland.
Med Wieku Rozwoj. 2004 Apr-Jun;8(2 Pt 1):159-68.
Extensive diagnostic and therapeutic dilemmas appear in children With primary malignant neoplasms located in the minor pelvis.
To evaluate the clinical symptoms, disease course and the results of treatment in patients with malignant pelvic neoplasms.
The study included 31 children (13 boys and 18 girls; aged 2 months to 16 years; mean age -- 8 years) treated in the Departments of Paediatric Oncology and Haematology in Gdansk and Lublin during the period of 1992-2003. The group comprised 17 patients with soft tissue sarcomas (MTM) (55%), 12 with germinal tumours (TGM) (39%) and tow. with neuroblastoma (NBL) (6%). The great majority of children (90%) presented with highly advanced disease (stages III + IV -- in 28 out of 31 patients).
with data analysis we were able to distinguish two categories of patients with different prognosis: with MTM and TGM. Most of he MTM tumours (11/17 - 65%) were localized in the urinary tract, the remaining six developed within pelvic muscles. Ten out of twelve TGM tumours (83%) were located in the ovaries. Radical tumour resection, especially primary resection, was shown to play the key role in both groups. Among TGM patients it was performed in 75% while in MTM patients -- in only 12%. All of these patients entered clinical remission and remain disease free. After adjuvant chemo- and/or radiotherapy secondary tumour resection was done in 17% of TGM and 41% of MTM patients.
in patients, who were not able to undergo radical tumour resection (mainly MTM patients), the disease progressed and led to death.