Book contents
- Frontmatter
- Contents
- CONTRIBUTORS
- PREFACE
- Chap 1 OVERVIEW OF SOFT TISSUE TUMORS
- Chap 2 RADIOLOGIC EVALUATION OF SOFT TISSUE TUMORS
- Chap 3 IMMUNOHISTOCHEMISTRY OF SOFT TISSUE TUMORS
- Chap 4 GENETICS OF SOFT TISSUE TUMORS
- Chap 5 MOLECULAR GENETICS OF SOFT TISSUE TUMORS
- Chap 6 FIBROBLAST BIOLOGY, FASCIITIS, RETROPERITONEAL FIBROSIS, AND KELOIDS
- Chap 7 FIBROMAS AND BENIGN FIBROUS HISTIOCYTOMAS
- Chap 8 FIBROMATOSES
- Chap 9 BENIGN FIBROBLASTIC AND MYOFIBROBLASTIC PROLIFERATIONS IN CHILDREN
- Chap 10 CHILDHOOD FIBROBLASTIC AND MYOFIBROBLASTIC PROLIFERATIONS OF VARIABLE BIOLOGIC POTENTIAL
- Chap 11 MYXOMAS AND OSSIFYING FIBROMYXOID TUMOR
- Chap 12 SOLITARY FIBROUS TUMOR, HEMANGIOPERICYTOMA, AND RELATED TUMORS
- Chap 13 FIBROBLASTIC AND MYOFIBROBLASTIC NEOPLASMS WITH MALIGNANT POTENTIAL
- Chap 14 LIPOMA VARIANTS AND CONDITIONS SIMULATING LIPOMATOUS TUMORS
- Chap 15 ATYPICAL LIPOMATOUS TUMOR AND LIPOSARCOMAS
- Chap 16 SMOOTH MUSCLE TUMORS
- Chap 17 GASTROINTESTINAL STROMAL TUMOR
- Chap 18 STROMAL TUMORS AND TUMOR-LIKE LESIONS OF THE FEMALE GENITAL TRACT
- Chap 19 ANGIOMYOLIPOMA AND RELATED TUMORS (PERIVASCULAR EPITHELIOID CELL TUMORS)
- Chap 20 RHABDOMYOMAS AND RHABDOMYOSARCOMAS
- Chap 21 HEMANGIOMAS, LYMPHANGIOMAS, AND REACTIVE VASCULAR PROLIFERATIONS
- Chap 22 HEMANGIOENDOTHELIOMAS, ANGIOSARCOMAS, AND KAPOSI'S SARCOMA
- Chap 23 GLOMUS TUMOR, SINONASAL HEMANGIOPERICYTOMA, AND MYOPERICYTOMA
- Chap 24 NERVE SHEATH TUMORS
- Chap 25 NEUROECTODERMAL TUMORS: MELANOCYTIC, GLIAL, AND MENINGEAL NEOPLASMS
- Chap 26 PARAGANGLIOMAS
- Chap 27 PRIMARY SOFT TISSUE TUMORS WITH EPITHELIAL DIFFERENTIATION
- Chap 28 MALIGNANT MESOTHELIOMA AND OTHER MESOTHELIAL PROLIFERATIONS
- Chap 29 MERKEL CELL CARCINOMA AND METASTATIC AND SARCOMATOID CARCINOMAS INVOLVING SOFT TISSUE
- Chap 30 CARTILAGE- AND BONE-FORMING TUMORS AND TUMOR-LIKE LESIONS
- Chap 31 SMALL ROUND CELL TUMORS
- Chap 32 ALVEOLAR SOFT PART SARCOMA
- Chap 33 PATHOLOGY OF SYNOVIA AND TENDONS
- Chap 34 MISCELLANEOUS TUMOR-LIKE LESIONS, AND HISTIOCYTIC AND FOREIGN BODY REACTIONS
- Chap 35 LYMPHOID, MYELOID, HISTIOCYTIC, AND DENDRITIC CELL PROLIFERATIONS IN SOFT TISSUES
- Chap 36 CYTOLOGY OF SOFT TISSUE LESIONS
- Chap 37 SURGICAL MANAGEMENT OF SOFT TISSUE SARCOMA: HISTOLOGIC TYPE AND GRADE GUIDE SURGICAL PLANNING AND INTEGRATION OF MULTIMODALITY THERAPY
- Chap 38 MEDICAL ONCOLOGY OF SOFT TISSUE SARCOMAS
- Index
- References
Chap 20 - RHABDOMYOMAS AND RHABDOMYOSARCOMAS
Published online by Cambridge University Press: 01 March 2011
- Frontmatter
- Contents
- CONTRIBUTORS
- PREFACE
- Chap 1 OVERVIEW OF SOFT TISSUE TUMORS
- Chap 2 RADIOLOGIC EVALUATION OF SOFT TISSUE TUMORS
- Chap 3 IMMUNOHISTOCHEMISTRY OF SOFT TISSUE TUMORS
- Chap 4 GENETICS OF SOFT TISSUE TUMORS
- Chap 5 MOLECULAR GENETICS OF SOFT TISSUE TUMORS
- Chap 6 FIBROBLAST BIOLOGY, FASCIITIS, RETROPERITONEAL FIBROSIS, AND KELOIDS
- Chap 7 FIBROMAS AND BENIGN FIBROUS HISTIOCYTOMAS
- Chap 8 FIBROMATOSES
- Chap 9 BENIGN FIBROBLASTIC AND MYOFIBROBLASTIC PROLIFERATIONS IN CHILDREN
- Chap 10 CHILDHOOD FIBROBLASTIC AND MYOFIBROBLASTIC PROLIFERATIONS OF VARIABLE BIOLOGIC POTENTIAL
- Chap 11 MYXOMAS AND OSSIFYING FIBROMYXOID TUMOR
- Chap 12 SOLITARY FIBROUS TUMOR, HEMANGIOPERICYTOMA, AND RELATED TUMORS
- Chap 13 FIBROBLASTIC AND MYOFIBROBLASTIC NEOPLASMS WITH MALIGNANT POTENTIAL
- Chap 14 LIPOMA VARIANTS AND CONDITIONS SIMULATING LIPOMATOUS TUMORS
- Chap 15 ATYPICAL LIPOMATOUS TUMOR AND LIPOSARCOMAS
- Chap 16 SMOOTH MUSCLE TUMORS
- Chap 17 GASTROINTESTINAL STROMAL TUMOR
- Chap 18 STROMAL TUMORS AND TUMOR-LIKE LESIONS OF THE FEMALE GENITAL TRACT
- Chap 19 ANGIOMYOLIPOMA AND RELATED TUMORS (PERIVASCULAR EPITHELIOID CELL TUMORS)
- Chap 20 RHABDOMYOMAS AND RHABDOMYOSARCOMAS
- Chap 21 HEMANGIOMAS, LYMPHANGIOMAS, AND REACTIVE VASCULAR PROLIFERATIONS
- Chap 22 HEMANGIOENDOTHELIOMAS, ANGIOSARCOMAS, AND KAPOSI'S SARCOMA
- Chap 23 GLOMUS TUMOR, SINONASAL HEMANGIOPERICYTOMA, AND MYOPERICYTOMA
- Chap 24 NERVE SHEATH TUMORS
- Chap 25 NEUROECTODERMAL TUMORS: MELANOCYTIC, GLIAL, AND MENINGEAL NEOPLASMS
- Chap 26 PARAGANGLIOMAS
- Chap 27 PRIMARY SOFT TISSUE TUMORS WITH EPITHELIAL DIFFERENTIATION
- Chap 28 MALIGNANT MESOTHELIOMA AND OTHER MESOTHELIAL PROLIFERATIONS
- Chap 29 MERKEL CELL CARCINOMA AND METASTATIC AND SARCOMATOID CARCINOMAS INVOLVING SOFT TISSUE
- Chap 30 CARTILAGE- AND BONE-FORMING TUMORS AND TUMOR-LIKE LESIONS
- Chap 31 SMALL ROUND CELL TUMORS
- Chap 32 ALVEOLAR SOFT PART SARCOMA
- Chap 33 PATHOLOGY OF SYNOVIA AND TENDONS
- Chap 34 MISCELLANEOUS TUMOR-LIKE LESIONS, AND HISTIOCYTIC AND FOREIGN BODY REACTIONS
- Chap 35 LYMPHOID, MYELOID, HISTIOCYTIC, AND DENDRITIC CELL PROLIFERATIONS IN SOFT TISSUES
- Chap 36 CYTOLOGY OF SOFT TISSUE LESIONS
- Chap 37 SURGICAL MANAGEMENT OF SOFT TISSUE SARCOMA: HISTOLOGIC TYPE AND GRADE GUIDE SURGICAL PLANNING AND INTEGRATION OF MULTIMODALITY THERAPY
- Chap 38 MEDICAL ONCOLOGY OF SOFT TISSUE SARCOMAS
- Index
- References
Summary
Tumors composed of skeletal muscle are the most common soft tissue sarcomas in the pediatric age group but make up only a small percentage of sarcomas in adults. Multicenter studies by the Intergroup Rhabdomyosarcoma Study/Children's Oncology Group and others have made it possible to study these tumors extensively. The focus of this chapter is benign and malignant soft tissue tumors that have skeletal muscle differentiation as a principal component (Table 20.1). There are several other tumors that display heterologous skeletal muscle differentiation. For this reason, when one encounters rhabdomyosarcomatous differentiation, these diagnoses must be ruled out, especially in adults.
Unlike tumors of adipose tissue and blood vessels, and fibrous lesions, malignant tumors with skeletal muscle differentiation are more common than benign ones. A prevalent theory is that an undifferentiated mesenchymal cell becomes malignant and then differentiates along several tissue pathways, giving rise to sarcomas having various histologic characteristics. Some evidence exists for the origination of some rhabdomyosarcomas (RMSs) from differentiated myocytes, however. Preexisting skeletal muscle is not a prerequisite for their development, however, as is proved by RMSs arising in sites such as the urinary bladder.
The reason for the predominance of RMS in childhood and teenage years and their rarity in adults remains a mystery. During fetal development, normal myogenesis is complete by early in the second trimester. The association of RMS with younger age groups suggests that primitive myogenic stem cells are perturbed by proliferation factors, genetic associations, and tissue differentiation processes rather than external carcinogenic factors.
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- Information
- Modern Soft Tissue PathologyTumors and Non-Neoplastic Conditions, pp. 546 - 573Publisher: Cambridge University PressPrint publication year: 2010
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