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The rarity of malignant and intermediate vascular tumors in children means that little is known about their clinical course, optimal treatment, and variables predicting survival. A total of 32 children with malignant vascular tumors (14 angiosarcomas [AS], 5 epithelioid hemangioendotheliomas, and 13 intermediate vascular tumors, including other hemangioendotheliomas plus adult-type hemangiopericytomas), registered in the German and Polish Paediatric Soft Tissue Sarcomas Study Groups, were treated following the Cooperative Weichteilsarkom Studiengruppe (CWS)-81, -86, -91, and -96 protocols. Male sex, AS histology, tumor size >5 cm, and T2 invasiveness were independent predictors of inferior 5-year overall survival, while AS histology and T2 invasiveness were predictors of inferior 5-year event-free survival. AS histology was the most important negative prognostic factor for overall survival and event-free survival. Completeness of primary tumor excision was a good prognostic factor for survival in univariate, but not multivariate, analysis. Local therapy (radiotherapy and delayed surgery) were provided to the minority of patients (28% and 38%, respectively) late in the course of disease (after a mean of 9 and 6 months, respectively) and did not prevent local relapses. Response to systemic treatment was poor (44%) and did not prevent local and distant relapses. The clinical course and outcome in childhood epithelioid HE seems to be similar to intravascular tumors and less aggressive than AS. RTX and delayed surgery should be performed more frequently and earlier in the disease course. An urgent need for modification of systemic therapy is needed because of the development of many metastatic and/or combined relapses and poor response to classic chemotherapy. The problem of effective therapy for childhood AS is the most appaling: 13 of 14 patients died of progression despite multimodal treatment.

Wstep i cele Miesaki tkanek miekkich, mimo ze stanowią heterogenną grupe nowotworow zlośliwych, ze wzgledu na wspolne cechy biologiczne, kliniczne i wrazliwośc na stosowane cytostatyki podlegają zblizonej strategii leczenia. W 11 ośrodkach onkohematologicznych w Polsce, w ramach Polskiej Pediatrycznej Grypy Guzow Litych (PPGGL) od lat prowadzone jest leczenia zgodne z miedzynarodowym protokolem CWS. Pacjenci i metody Analizą objeto 52 pacjentow w III stopniu zaawansowania choroby, zarejestrowanych i leczonych przez PPGGL, wg protokolu CWS-96 w latach 1997–2003. Wyniki W calej analizowanej grupie chorych stwierdzono 30% wznow miejscowych, natomiast w wyselekcjonowanej grupie pacjentow z RMS stwierdzono 24% wznow miejscowych. Stwierdzono zjawisko zwiekszenia liczby wznow miejscowych u chorych, u ktorych rozpoczeto napromienianie po 20 tygodniu od rozpoczecia leczenia. Roznica ta najwyraźniej wystąpila u dzieci z rozpoznaniem RMS zarodkowego (44% vs 12,5%, p = 0,047). Wznowa miejscowa wystąpila najcześciej u pacjentow z rozpoznaniem histopatologicznym pozakostnej postaci miesaka Ewinga (PNET/EES) – 8 wznow na 13 chorych (61%). Wnioski Opoźnienie napromieniania wplywa na zwiekszenie liczby wznow miejscowych, szczegolnie u pacjentow z rozpoznaniem RMS embryonale. Wysoki odsetek niepowodzen leczenia pacjentow z rozpoznaniem pozakostnej postaci miesaka Ewinga sugeruje rozwazenie u tych chorych modyfikacji stosowanego w protokole CWS96 sposobu terapii.

Background: Pediatric hemangiopericytoma (HPC) is an extremely rare vascular tumor with little data available on its clinical course and management. Methods: Fourteen children with HPC registered in Polish Pediatric Rare Tumors and Polish Pediatric Soft-tissue Sarcomas Studies between 1992 and 2002 are reported. Results: Seven patients (F/M: 5/2, age 2–10 months) had infantile HPC, four of whom had primary tumors affecting superficial tissues of the trunk and upper limbs. No child had initial nodal or organ metastases. Primary excision (PE) was performed only in three patients with superficial lesions. They received no supplemental treatment. The remaining four children responded well to chemotherapy (CHT), entering complete remission after CHT alone (n= 2) or after delayed resection (n= 2). No relapses occurred and all patients were alive 48–146 months after treatment. Seven patients (F/M: 2/5, age 3.2–16.5 years) had adult-type HPC, five of whom had tumors localized in superficial tissues of the lower limbs or head/neck. All patients presented with locally advanced disease. PE was performed in five children (complete in four); all patients were alive at follow up of 40–127 months. Three patients died of recurrence after incomplete PE despite supplemental chemo- and radiotherapy. Conclusions: Complete surgical excision remains the mainstay of treatment for both HPC types. In unresectable adult-type HPC adjuvant chemo- and radiotherapy should be administered in macro- and microscopic tumor residues, but the prognosis is poor despite supplemental treatment. High chemo-responsiveness of infantile-type HPC produces a favorable outcome even in cases of unresectable, life-threatening tumors.