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 17 - GASTROINTESTINAL STROMAL TUMOR
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
Gastrointestinal stromal tumor (GIST) is defined as a specific mesenchymal tumor of the gastrointestinal (GI) tract that has a characteristic histologic spectrum, is generally KIT (CD117) positive, and is driven by oncogenic KIT or platelet-derived growth factor receptor α (PDGFRA) mutations. This definition of GIST excludes GI smooth muscle and nerve sheath tumors. Availability of KIT tyrosine kinase inhibitor drug therapy (imatinib mesylate and second-generation inhibitors) has greatly magnified the interest in GIST and vastly increased the importance of its specific diagnosis.
GISTs occur along the entire length of the GI tract, from the esophagus to the anus, and similar tumors can occur anywhere in the abdomen, usually representing metastases or tumors detached from their GI tract site of origin. GISTs have a wide clinical spectrum, ranging from benign, small incidentally detected nodules to massive, frankly malignant tumors that can fill the entire abdomen.
This chapter summarizes the clinical, pathologic, and molecular genetic features of GISTs. Although all GISTs share many features, tumors at each site have distinctive clinicopathologic and prognostic differences and are therefore reviewed site by site. Small intestinal GISTs in particular show malignant behavior more frequently than the gastric tumors do. The unique clinicopathologic features of pediatric GISTs, those occurring in Carney triad, neurofibromatosis 1, and familial GIST syndrome, are discussed separately.
Tumor size (preferably in three dimensions), mitotic rate per 50 HPFs, and tumor margin status should be included in the pathology report.
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- Chapter
- Information
- Modern Soft Tissue PathologyTumors and Non-Neoplastic Conditions, pp. 491 - 507Publisher: Cambridge University PressPrint publication year: 2010
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