Добавил:
Upload Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз: Предмет: Файл:
Pediatric_Oncology_A_Comprehensive_Guide.pdf
Скачиваний:
29
Добавлен:
10.02.2016
Размер:
2 Мб
Скачать
☆

12 Soft Tissue Sarcoma

153

 

 

12.8Hemangiopericytoma

12.8.1 Incidence

• Three percent of all soft tissue sarcomas in childhood

12.8.2 Location

• Predominantly in (lower) extremities, in the retroperitoneal area and in head and neck; also paraspinal area

• Infants: tongue and sublingual area

12.8.3 Pathology and Cytogenetics

•Originates from vascular pericytes

•Histologically, difficult differentiation between benign, intermediate, or malignant forms

•Metastatic spread: mainly in lung and bone

•Cytogenetics: translocation t(12;19)(q13;q13.3), t(13;22)(q22;q11)

12.8.4 Therapy

• Radical resection

• Chemoand radiotherapy according to rhabdomyosarcoma (see above)

12.8.5 Prognosis

• In general, 30–70% 5-year survival

• Prognosis depends on extent of disease

• In children, predominance of favorable prognosis with hemangiopericytoma

12.8.6 Congenital Hemangiopericytoma Variant

•Location in subcutis

•Therapeutically surgical excision is sufficient

•Prognosis: more favorable than hemangiopericytoma in childhood

Соседние файлы в предмете [НЕСОРТИРОВАННОЕ]