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Retrospective multi-institutional study on hemangiopericytoma in Polish children
Ewa Bien1, Teresa Stachowicz-Stencel, Jan Godzinski
1Department of Pediatrics, Hematology, Oncology and Endocrinology, Medical University of Gdansk, Poland. ebien@amg.gda.pl
Insights
Pediatric hemangiopericytoma (HPC) management varies by type. Infantile HPC shows high chemo-responsiveness for a favorable outcome, while adult-type HPC requires complete surgical excision, with guarded prognosis despite adjuvant therapies.
Area of Science:
- Pediatric Oncology
- Vascular Tumors
- Rare Cancers
Background:
- Pediatric hemangiopericytoma (HPC) is an exceptionally rare vascular tumor in children.
- Limited clinical data exists regarding its course and optimal management strategies.
Purpose of the Study:
- To report on the clinical course and management of pediatric hemangiopericytoma.
- To analyze treatment outcomes for infantile and adult-type HPC in children.
Main Methods:
- Retrospective analysis of 14 children with HPC.
- Data collected from Polish Pediatric Rare Tumors and Soft-tissue Sarcomas Studies (1992-2002).
Main Results:
- Infantile HPC (7 patients): 4 superficial, no metastases. Complete remission achieved with chemotherapy alone or delayed resection in all cases. All survivors.
- Adult-type HPC (7 patients): 5 superficial, locally advanced. Complete surgical excision achieved in 4/5. 3 deaths due to recurrence after incomplete excision despite adjuvant therapy.
Conclusions:
- Complete surgical excision is the primary treatment for both infantile and adult-type HPC.
- Adjuvant therapy for unresectable adult-type HPC has a poor prognosis.
- Infantile HPC's chemo-responsiveness leads to favorable outcomes, even in aggressive cases.
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.