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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

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