Hemangiopericytoma in pediatric ages: a report from the Italian and German Soft Tissue Sarcoma Cooperative Group.
ABSTRACT Hemangiopericytoma (HPC) is very uncommon in childhood and comprises two different clinical entities, the adult type and the infantile type, occurring in the first year of age. We report on a series of 27 pediatric patients treated from 1978 to 1999 by the Italian and German Soft Tissue Sarcoma Cooperative Group.
Seven patients had infantile HPC; complete resection was achieved in the tumors of five patients and chemotherapy was given to four patients. Twenty children had adult type HPC; nine received complete tumor resection (four patients at diagnosis and five at delayed surgery). Post-operative radiotherapy was administered to 15 patients, chemotherapy to 19.
Six of seven patients with infantile HPC were alive in first remission; one patient died of disease. Chemotherapy achieved an objective response in four of four patients. Among the adult type HPC cases, 5-year event free survival was 64% (median follow-up 125 months); 12 patients were alive in first remission, eight patients relapsed and died of disease. Seven of 10 evaluable patients showed good response to chemotherapy. Statistically significant differences in outcome were observed in relation to Intergroup Rhabdomyosarcoma Study grouping, size, local invasiveness, and gender.
Infantile HPC is a unique entity probably related to infantile myofibroblastic lesions and characterized by a high response to chemotherapy, which is required in case of unresectable, life-threatening tumors. In children over 1 year of age, HPC behaves like its adult counterpart; complete surgical resection remains the mainstay of treatment, but chemotherapy and radiotherapy seem effective and are recommended in all patients with incomplete tumor resection and/or locally invasive, large tumors.
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ABSTRACT: Hemangiopericytoma is a rare, vascular soft tissue tumour originating from the pericytes, the contractile cells that surround capillaries. A case of retrorectal hemangiopericytoma in a 49-year-old woman is reported. With a posterior sagittal approach, the tumour was completely removed. Pathological examination, including immunohistological stains, was consistent with a hemangiopericytoma. A step-by-step approach to a retrorectal tumour is exemplified, and a very rare pathological entity is added to the spectrum of retrorectal masses.Acta chirurgica Belgica 107(4):438-41. · 0.44 Impact Factor
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ABSTRACT: This review summarizes and comments on the major articles that have been published in English concerning pediatric soft-tissue sarcomas in the past 2 years. Studies of rhabdomyosarcoma and undifferentiated sarcoma, including late sequelae of treatment; nonrhabdomyosarcomatous soft-tissue sarcoma; and the pathology of soft-tissue sarcomas are included.Current Oncology Reports 08/2002; 4(4):291-8. DOI:10.1007/s11912-002-0004-4 · 2.87 Impact Factor
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ABSTRACT: A 55-year-old Japanese woman underwent extirpation of a malignant hemangiopericytoma in the pelvic cavity, followed by postoperative irradiation. An abdominal computed tomography scan 3 years later revealed a local recurrent tumor, 12 cm in diameter, in the pelvic cavity, for which transarterial embolization was done, followed by excision of the tumor employing Hartmann's procedure. Although an unresectable part of the recurrent tumor remained, postoperative irradiation reduced its size remarkably. The patient is still alive 7 years 2 months after her first operation, but with more recurrent tumors in the abdominal wall and around the bilateral iliac arteries. Because hemangiopericytoma often recurs or metastasizes after a prolonged disease-free interval, close long-term follow-up is necessary after the operation. Combined-modality therapy against the recurrent or unresectable disease may result in a good prognosis.Surgery Today 02/2003; 33(6):479-82. DOI:10.1007/s10595-002-2505-0 · 1.21 Impact Factor