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Pediatric_Oncology_A_Comprehensive_Guide.pdf
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136

 

 

P. Imbach

 

 

 

12.5

Liposarcoma.................................................................................................................

149

 

12.5.1

Incidence ........................................................................................................

149

 

12.5.2

Pathology and Cytogenetics...........................................................................

149

 

12.5.3

Clinical Manifestations ..................................................................................

150

 

12.5.4

Therapy ..........................................................................................................

150

 

12.5.5

Prognosis........................................................................................................

150

12.6 Malignant Peripheral Nerve Sheath Tumor..................................................................

150

 

12.6.1

Incidence ........................................................................................................

150

 

12.6.2

Location .........................................................................................................

151

 

12.6.3

Pathology and Cytogenetics...........................................................................

151

 

12.6.4

Clinical Manifestations ..................................................................................

151

 

12.6.5

Therapy ..........................................................................................................

151

12.7

Leiomyosarcoma..........................................................................................................

151

 

12.7.1

Incidence ........................................................................................................

151

 

12.7.2

Location .........................................................................................................

152

 

12.7.3

Pathology .......................................................................................................

152

 

12.7.4

Clinical Manifestations ..................................................................................

152

 

12.7.5

Therapy ..........................................................................................................

152

 

12.7.6

Prognosis........................................................................................................

152

12.8

Hemangiopericytoma ...................................................................................................

153

 

12.8.1

Incidence ........................................................................................................

153

 

12.8.2

Location .........................................................................................................

153

 

12.8.3

Pathology and Cytogenetics...........................................................................

153

 

12.8.4

Therapy ..........................................................................................................

153

 

12.8.5

Prognosis........................................................................................................

153

 

12.8.6

Congenital Hemangiopericytoma Variant......................................................

153

12.9

Malignant Fibrohistiocytoma.......................................................................................

154

12.1Overview

12.1.1 Definition

Soft tissue sarcomas (STS) are a heterogeneous group of malignant tumors which stem from muscles, vessels, lymphatic tissue, connective tissue, synovial tissue, or primitive mesenchymal cells. The STS are also divided in rhabdomyosarcoma RMS and non-RMS-STS = NRSTS

Subtypes

Tissue of origin

Tumor

Mesenchymal

Myxoma, mesenchymoma

Striated muscle

Rhabdomyosarcoma

Smooth muscle

Leiomyosarcoma

Fatty tissue

Liposarcoma

Connective tissue

Fibrosarcoma

 

Malignant fibrohistiosarcoma

Synovial tissue

Synovial sarcoma

Blood vessels

Angiosarcoma, hemangiopericytoma

Lymphatic vessels

Lymphangiosarcoma

Nerve sheath

Neurofibrosarcoma (malignant schwannoma)

 

Malignant peripheral nerve sheath tumor

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