Enzinger and Weiss's Soft Tissue Tumors: Expert Consult: Online and Print

Since its first publication more than 35 years ago, Enzinger and Weiss’s Soft Tissue Tumors has established itself as the most comprehensive and authoritative reference available on soft tissue pathology. The 7th Edition from Drs. John R. Goldblum, Andrew L. Folpe, and Sharon W. Weiss, continues this tradition with detailed, well-written, logically organized coverage of the full spectrum of these often difficult and challenging tumors. It offers clear guidance to practicing and trainee pathologists on diagnosis of tumors by microscopy, immunohistochemistry, and molecular genetics, as well as a significant amount of clinically significant information of interest to the clinicians who most frequently see these diseases – dermatologists, orthopaedists, and oncologists. Offers practical information on differential diagnosis of tumors of the skeletal muscles, connective tissue, fat, and related structures, helping you accurately diagnose and confidently sign out pathology reports on even the most challenging cases. Provides unsurpassed scope and depth in this complex area with microscopic findings correlated with the latest developments in molecular biology, cytogenetics, and immunohistochemistry, for a comprehensive and integrated approach to evaluation and diagnosis. Incorporates new knowledge on recently identified entities, next-generation sequencing (NGS), molecular diagnostic techniques, and immunohistochemical and genetic features of soft tissue tumors, providing up-to-date diagnostic and prognostic information that will inform day-to-day therapeutic decisions. Features nearly 2,000 high-quality images that clearly capture the clinical, macroscopic and microscopic features of benign and malignant conditions, helping you relate these characteristics to their specific classifications. Utilizes a logical, well-structured format including summary outlines at the beginning of each chapter, a color-coded page design, and a consistent approach to every entity, enabling you to navigate the text quickly, improve turnaround time when diagnosing a specimen, and clearly report on the prognosis and therapeutic management options. Includes abundant algorithms, tables, and graphs to facilitate rapid decision making. Enhanced eBook version included with purchase, which allows you to access all of the text, figures, and references from the book on a variety of devices Enzinger and Weiss's Soft Tissue Tumors, Seventh Edition Title Page Copyright Dedication Contributors Preface Preface to the First Edition 1 - General Considerations INCIDENCE OF SOFT TISSUE TUMORS PATHOGENESIS OF SOFT TISSUE TUMORS Environmental Factors Oncogenic Viruses Immunologic Factors Genetic Factors CLASSIFICATION OF SOFT TISSUE TUMORS GRADING AND STAGING SOFT TISSUE SARCOMAS Grading Systems Limitations of Grading Staging Systems STANDARDIZED REPORTING OF SOFT TISSUE SARCOMAS REFERENCES 2 - Clinical Evaluation and Treatment of Soft Tissue Tumors CLINICAL EVALUATION Clinical Presentation and Assessment Pretreatment Evaluation PROGNOSTIC FACTORS Clinicopathologic Factors Classification and Prognostic Significance of Surgical Margins Nomograms for Assessment of Individual Patient Prognosis TREATMENT OF LOCALIZED PRIMARY EXTREMITY SARCOMAS Surgery Management of Regional Lymph Nodes Radiotherapy Chemotherapy TREATMENT OF LOCALLY ADVANCED DISEASE Hyperthermic Isolated Limb Perfusion Radiation Alone MANAGEMENT OF LOCAL RECURRENCE KEY POINTS REFERENCES 3 - Radiologic Evaluation of Soft Tissue Tumors IMAGING MODALITIES Radiography Ultrasonography Computed Tomography Positron Emission Tomography–Computed Tomography Magnetic Resonance Imaging Positron Emission Tomography–Magnetic Resonance Imaging BENIGN SOFT TISSUE TUMORS Benign Lipomatous Tumors Neurogenic Tumors Benign Vascular Lesions Desmoid Tumors Myxomas Desmoplastic Fibroblastomas Leiomyomas Glomus Tumors Fibromas of Tendon Sheath Myofibromas Granular Cell Tumors Soft Tissue Chondromas or Osteochondromas MALIGNANT SOFT TISSUE TUMORS Soft Tissue Sarcomas TUMORLIKE CONDITIONS OF SOFT TISSUE Elastofibromas Hematomas Pigmented Villonodular Synovitis Synovial Chondromatosis Ganglia, Synovial Cysts, and Bursitis Postoperative Collections Abscesses Heterotopic Ossification Accessory or Hypertrophied Muscles Decubital Ischemic Fasciitis (Atypical Decubital Fibroplasia or Ischemic Fasciitis) Nodular Fasciitis Lipomatosis of Nerve Subcutaneous Granuloma Annulare Massive Localized Lymphedema REFERENCES 4 - Molecular Genetic Pathology of Soft Tissue Tumors ORGANIZATION OF THE HUMAN GENOME: IMPLICATIONS FOR MOLECULAR GENETIC PATHOLOGY CONSTITUTIONAL MUTATIONS PREDISPOSING TO SOFT TISSUE TUMORS SOMATIC MUTATIONS IN SOFT TISSUE TUMORS: GENERAL CONCEPTS Small Genetic Variants Chromosomal Imbalances Gene Fusions GENETIC TECHNIQUES Chromosome Banding Analysis Genomic Arrays Gene Expression Profiling Fluorescence In Situ Hybridization Reverse-Transcriptase Polymerase Chain Reaction Massively Parallel Sequencing CHARACTERISTIC GENETIC CHANGES IN SOFT TISSUE TUMORS Fibroblastic/Myofibroblastic Tumors Smooth Muscle Tumors Gastrointestinal Stromal Tumors Skeletal Muscle Tumors Vascular and Perivascular Tumors Nerve Sheath Tumors Tumors of Uncertain Differentiation Ewing Sarcoma and Undifferentiated Sarcomas CONCLUSION Acknowledgments REFERENCES 5 - Approach to the Diagnosis of Soft Tissue Tumors CLINICAL INFORMATION BIOPSY DIAGNOSIS FROZEN SECTION DIAGNOSIS EVALUATION OF RESECTION SPECIMENS MICROSCOPIC EXAMINATION IMMUNOHISTOCHEMISTRY MOLECULAR TESTS DIAGNOSTIC NOMENCLATURE REFERENCES 6 - Immunohistochemistry for Analysis of Soft Tissue Tumors INTERMEDIATE FILAMENTS Vimentin Keratins EPITHELIAL MEMBRANE ANTIGEN MARKERS OF MUSCLE DIFFERENTIATION Desmin Actin Caldesmon Myogenic Transcription Factors Myoglobin and Other Less Commonly Used Markers Recommendations for Use of Muscle Markers MARKERS OF NERVE SHEATH DIFFERENTIATION S-100 Protein SOX10 Claudin-1 GLUT-1 “NEUROECTODERMAL” MARKERS CD99 CD56 (Neural Cell Adhesion Molecule) Synaptophysin and Chromogranin A NKX2.2 MARKERS OF MELANOCYTIC DIFFERENTIATION HMB-45 Melan-A Microphthalmia Transcription Factor PNL2 Tyrosinase MARKERS OF ENDOTHELIAL DIFFERENTIATION Von Willebrand Factor (Factor VIII-Related Antigen) CD34 (Human Hematopoietic Progenitor Cell Antigen) CD31 (Platelet Endothelial Cell Adhesion Molecule-1) FLI-1 and ERG proteins Markers of Lymphatic Endothelial Differentiation Human Herpesvirus 8 (HHV8) Latency-Associated Nuclear Antigen (LANA) Recommendations for Use of Vascular Markers MARKERS OF GASTROINTESTINAL STROMAL TUMORS CD117 (c-kit) Anoctamin-1 (ANO1, DOG1, TMEM16A) IMMUNOHISTOCHEMISTRY AS SURROGATE FOR TUMOR-SPECIFIC MOLECULAR ALTERATIONS FLI-1 and ERG as Markers of Ewing Sarcoma WT-1 as Marker of t(11;22)(13;q24) Translocation of Desmoplastic Small Round Cell Tumor and CIC-Rearranged Sarcomas TFE3 as Marker of Alveolar Soft Part Sarcoma and TFE3-Rearranged Perivascular Epithelioid Cell Tumors SMARCB1 (INI1) and SMARCA4 (BRG1) Expression Loss as Markers of Aberrations in SWI/SNF Chromatin-Remodeling Complex Anaplastic Lymphoma Kinase in ALK-Rearranged Tumors ETV4 in CIC-Rearranged Primitive Sarcomas BCOR and CCNB3 as Markers of Primitive Sarcomas Showing BCOR Gene Rearrangements and Internal Tandem Duplications CAMTA1 as Marker of Epithelioid Hemangioendothelioma–Defining WWTR1-CAMTA1 Fusion FOSB as Marker of Epithelioid Sarcoma–Like (Pseudomyogenic) Hemangioendothelioma and Epithelioid Hemangioma STAT6 as Marker of NAB2-STAT6 Fusion in Solitary Fibrous Tumors H3K27me3 Loss as Marker of Malignant Peripheral Nerve Sheath Tumors OTHER MARKERS CD68 and CD163 as Markers of Histiocytes TLE1 MUC4 β-Catenin MDM2 and CDK4 Bcl-2 Type IV Collagen IgG4 SATB2 Cathepsin K PRKAR1A PROGNOSTIC MARKERS Ki-67 p53 p21WAF1 p16 and p27kip PD-L1 (Programmed Death Ligand-1) APPLICATION OF IMMUNOHISTOCHEMISTRY TO SARCOMA DIAGNOSIS: CLINICAL SCENARIOS The Undifferentiated Round Cell Tumor The Monomorphic Spindle Cell Tumor The Poorly Differentiated Epithelioid Tumor “Orphan Sarcomas” CONCLUSION REFERENCES 7 - Benign Fibroblastic/Myofibroblastic Proliferations, Including Superficial Fibromatoses CLASSIFICATION OF BENIGN FIBROBLASTIC/MYOFIBROBLASTIC PROLIFERATIONS NODULAR FASCIITIS Clinical Findings Gross Findings Microscopic Findings Ossifying Fasciitis Intravascular Fasciitis Cranial Fasciitis Immunohistochemical Findings Cytogenetic and Molecular Genetic Features Differential Diagnosis Discussion PROLIFERATIVE FASCIITIS Clinical Findings Pathologic Findings PROLIFERATIVE MYOSITIS Clinical Findings Pathologic Findings Differential Diagnosis Discussion ORGAN-ASSOCIATED PSEUDOSARCOMATOUS MYOFIBROBLASTIC PROLIFERATIONS Clinical Findings Pathologic Findings Immunohistochemical Findings Differential Diagnosis Discussion ISCHEMIC FASCIITIS (ATYPICAL DECUBITAL FIBROPLASIA) Pathologic Findings Differential Diagnosis Discussion FIBROMA OF TENDON SHEATH Pathologic Findings Immunohistochemical Findings Differential Diagnosis Discussion PLEOMORPHIC FIBROMA OF THE SKIN NUCHAL-TYPE FIBROMA GARDNER-ASSOCIATED FIBROMA Clinical Findings Pathologic Findings Immunohistochemical Findings Discussion ELASTOFIBROMA Clinical Findings Pathologic Findings Differential Diagnosis Discussion NASOPHARYNGEAL ANGIOFIBROMA Clinical Findings Pathologic Findings Immunohistochemical Findings Discussion KELOID Clinical Findings Pathologic Findings Differential Diagnosis Discussion DESMOPLASTIC FIBROBLASTOMA (COLLAGENOUS FIBROMA) Pathologic Findings Differential Diagnosis Discussion INTRANODAL PALISADED MYOFIBROBLASTOMA Pathologic Findings Discussion MAMMARY-TYPE MYOFIBROBLASTOMA Clinical Findings Pathologic Findings Immunohistochemical Findings Cytogenetic and Molecular Genetic Findings Differential Diagnosis Discussion SUPERFICIAL ACRAL FIBROMYXOMA (DIGITAL FIBROMYXOMA) Clinical Findings Pathologic Findings Immunohistochemical Findings Differential Diagnosis Discussion SOFT TISSUE ANGIOFIBROMA Clinical Findings Pathologic Findings Immunohistochemical Findings Cytogenetic and Molecular Genetic Findings Differential Diagnosis Discussion SUPERFICIAL FIBROMATOSES PALMAR FIBROMATOSIS (DUPUYTREN DISEASE) Clinical Findings Concurrent Diseases Pathologic Findings Immunohistochemical Findings Cytogenetic and Molecular Genetic Findings Differential Diagnosis Discussion PLANTAR FIBROMATOSIS (LEDDERHOSE DISEASE) Clinical Findings Pathologic Findings Discussion PENILE FIBROMATOSIS (PEYRONIE DISEASE) Clinical Findings Pathologic Findings Discussion KNUCKLE PADS REFERENCES 8 - Fibrous Tumors of Infancy and Childhood FIBROUS HAMARTOMA OF INFANCY Clinical Findings Pathologic Findings Immunohistochemical and Molecular Genetic Findings Differential Diagnosis Discussion INCLUSION BODY FIBROMATOSIS (INFANTILE DIGITAL FIBROMATOSIS) Clinical Findings Pathologic Findings Immunohistochemical Findings Discussion JUVENILE HYALINE FIBROMATOSIS/INFANTILE SYSTEMIC HYALINOSIS Clinical Findings Pathologic Findings Cytogenetic and Molecular Genetic Findings Differential Diagnosis Discussion GINGIVAL FIBROMATOSIS Clinical Findings Pathologic Findings Differential Diagnosis Discussion FIBROMATOSIS COLLI Clinical Findings Pathologic Findings Discussion LIPOFIBROMATOSIS (INFANTILE FIBROMATOSIS) Clinical Findings Pathologic Findings Differential Diagnosis Discussion CALCIFYING APONEUROTIC FIBROMA Clinical Findings Pathologic Findings Differential Diagnosis Discussion CEREBRIFORM FIBROUS PROLIFERATION (PROTEUS SYNDROME) CALCIFYING FIBROUS TUMOR REFERENCES 9 - Borderline and Malignant Fibroblastic/Myofibroblastic Tumors DEEP (DESMOID-TYPE) FIBROMATOSES Extraabdominal Fibromatosis Abdominal Fibromatosis Pelvic Fibromatosis Mesenteric Fibromatosis Pathologic Findings Ancillary Findings Etiology Clinical Behavior Treatment Differential Diagnosis CONGENITAL/INFANTILE FIBROSARCOMA Clinical Findings Pathologic Findings Immunohistochemical Findings Cytogenic and Molecular Genetic Findings Differential Diagnosis Discussion INFLAMMATORY MYOFIBROBLASTIC TUMOR Clinical Findings Pathologic Findings Immunohistochemical Findings Cytogenetic and Molecular Genetic Findings Differential Diagnosis Discussion ADULT-TYPE FIBROSARCOMA Clinical Findings Pathologic Findings Immunohistochemical Findings Cytogenetic and Molecular Genetic Findings Differential Diagnosis Discussion FIBROSARCOMA VARIANTS Low-Grade Fibromyxoid Sarcoma Sclerosing Epithelioid Fibrosarcoma Myxofibrosarcoma Fibrosarcomatous Change Arising in Dermatofibrosarcoma Protuberans Postradiation Fibrosarcoma Fibrosarcoma Arising in Burn Scars Low-Grade Myofibroblastic Sarcoma (Myofibroblastic Sarcoma) Myxoinflammatory Fibroblastic Sarcoma REFERENCES 10 - Benign Fibrohistiocytic Tumors FIBROUS HISTIOCYTOMA Clinical Findings Microscopic Findings VARIANTS OF BENIGN FIBROUS HISTIOCYTOMA Cellular Fibrous Histiocytoma Aneurysmal Fibrous Histiocytoma Atypical Fibrous Histiocytoma Minor Histologic Variants Immunohistochemical findings Genetic Findings Outcome Metastasizing Fibrous Histiocytomas Differential Diagnosis DERMATOMYOFIBROMA EPITHELIOID FIBROUS HISTIOCYTOMA Clinical and Morphologic Features Immunohistochemical and Genetic Features Differential Diagnosis NEUROTHEKEOMA (CELLULAR NEUROTHEKEOMA) Clinical Features Pathologic Features Immunohistochemistry Outcome Differential Diagnosis SOLITARY (JUVENILE) XANTHOGRANULOMA Clinical Findings and Gross Appearance Microscopic Findings Differential Diagnosis Clinical Behavior SOLITARY RETICULOHISTIOCYTOMA MULTICENTRIC RETICULOHISTIOCYTOSIS Pathologic Findings Discussion XANTHOMA Clinical Findings and Gross Appearance Microscopic Findings Discussion MISCELLANEOUS HISTIOCYTIC REACTIONS RESEMBLING A NEOPLASM Infectious Disease Malacoplakia Extranodal (Soft Tissue) Rosai-Dorfman disease Histiocytic Reactions to Endogenous and Exogenous Material REFERENCES 11 - Fibrohistiocytic Tumors of Intermediate Malignancy DERMATOFIBROSARCOMA PROTUBERANS Clinical Findings Gross Findings Microscopic Findings Immunohistochemical Findings Cytogenetic and Molecular Genetic Features Differential Diagnosis Discussion SARCOMA ARISING IN DERMATOFIBROSARCOMA PROTUBERANS (FIBROSARCOMATOUS VARIANT OF DERMATOFIBROSARCOMA PROTUBERANS) BEDNAR TUMOR (PIGMENTED DERMATOFIBROSARCOMA PROTUBERANS, STORIFORM NEUROFIBROMA) GIANT CELL FIBROBLASTOMA Clinical Findings Pathologic Findings Differential Diagnosis Discussion PLEXIFORM FIBROHISTIOCYTIC TUMOR Clinical Findings Gross and Microscopic Findings Ancillary Studies Differential Diagnosis Discussion SOFT TISSUE GIANT CELL TUMOR (OF LOW MALIGNANT POTENTIAL) Clinical and Pathologic Features Genetic Findings Differential Diagnosis Clinical Behavior REFERENCES 12 - Undifferentiated Pleomorphic Sarcoma PLEOMORPHIC SARCOMA WITH A SPECIFIC LINE OF DIFFERENTIATION ATYPICAL FIBROXANTHOMA Clinical Findings Pathologic Findings Immunohistochemical and Molecular Genetic Findings Differential Diagnosis Discussion UNDIFFERENTIATED PLEOMORPHIC SARCOMA Clinical Findings Etiologic Factors Gross Findings Microscopic Findings Immunohistochemical Findings Cytogenetic and Molecular Genetic Findings Differential Diagnosis Discussion REFERENCES 13 - Benign Lipomatous Tumors WHITE FAT BROWN FAT MOLECULAR BIOLOGY AND IMMUNOHISTOCHEMISTRY OF BENIGN LIPOMATOUS TUMORS CLASSIFICATION OF BENIGN LIPOMATOUS TUMORS LIPOMA Age and Gender Incidence Localization Clinical Findings Gross Findings Microscopic Findings Cytogenetic and Molecular Findings Behavior and Treatment Discussion MULTIPLE LIPOMAS ANGIOLIPOMA MYOLIPOMA CHONDROID LIPOMA Clinical Findings Pathologic Findings Cytogenetic and Molecular Genetic Findings Differential Diagnosis Discussion SPINDLE CELL/PLEOMORPHIC LIPOMA Clinical Findings Pathologic Findings Immunohistochemical Findings Cytogenetic and Molecular Genetic Findings Differential Diagnosis Discussion LIPOBLASTOMA AND LIPOBLASTOMATOSIS Clinical Findings Pathologic Findings Cytogenetic and Molecular Genetic Findings Differential Diagnosis Discussion LIPOBLASTOMA-LIKE TUMOR OF THE VULVA ANGIOMYOLIPOMA MYELOLIPOMA INTRAMUSCULAR AND INTERMUSCULAR LIPOMAS LIPOMAS OF TENDON SHEATHS AND JOINTS LUMBOSACRAL LIPOMA NEURAL FIBROLIPOMA (LIPOFIBROMATOUS HAMARTOMA OF NERVES) DIFFUSE LIPOMATOSIS SYMMETRIC LIPOMATOSIS PELVIC LIPOMATOSIS STEROID LIPOMATOSIS NEVUS LIPOMATOSUS CUTANEOUS SUPERFICIALIS HIBERNOMA Clinical Findings Pathologic Findings Immunohistochemical Findings Cytogenetic and Molecular Genetic Findings Differential Diagnosis Discussion REFERENCES 14 - Liposarcoma CRITERIA AND IMPORTANCE OF LIPOBLASTS ATYPICAL LIPOMATOUS TUMOR (ALT)/WELL-DIFFERENTIATED LIPOSARCOMA (WDL) Clinical Findings Gross and Microscopic Features Differential Diagnosis Cytogenetic and Molecular Findings Immunohistochemical and Molecular Diagnosis of ALT/WDL and DL Clinical Behavior DEDIFFERENTIATED LIPOSARCOMA Clinical Features Gross and Microscopic Features Differential Diagnosis Cytogenetic and Molecular Findings Clinical Behavior MYXOID LIPOSARCOMA Clinical Features Gross and Microscopic Features Differential Diagnosis Cytogenetic and Molecular Findings Clinical Course PLEOMORPHIC LIPOSARCOMA SPINDLE CELL LIPOSARCOMA (FIBROSARCOMA-LIKE LIPOMATOUS NEOPLASM, ATYPICAL SPINDLE CELL LIPOMATOUS TUMOR) LIPOSARCOMA OF MIXED OR UNCLASSIFIABLE TYPE LIPOSARCOMA IN CHILDREN SO-CALLED MULTICENTRIC LIPOSARCOMA REFERENCES 15 - Benign Tumors of Smooth Muscle STRUCTURE AND FUNCTION OF SMOOTH MUSCLE CELLS CUTANEOUS LEIOMYOMA (LEIOMYOMA CUTIS) Leiomyoma of Pilar Arrector Origin, Including Those Associated with Hereditary Leiomyomatosis and Renal Cell Cancer Syndrome (Re... Genital Leiomyomas ANGIOMYOMA (VASCULAR LEIOMYOMA) LEIOMYOMA OF DEEP SOFT TISSUE LEIOMYOMATOSIS PERITONEALIS DISSEMINATA Microscopic Findings Behavior and Treatment BENIGN GENITAL STROMAL TUMORS Angiomyofibroblastoma Cellular Angiofibroma (Angiomyofibroblastoma-Like Tumor of Male Genital Tract) Deep Angiomyxoma (“Aggressive Angiomyxoma”) Differential Diagnosis of Benign Genital Stromal Tumors MISCELLANEOUS LESIONS CONFUSED WITH LEIOMYOMAS REFERENCES 16 - Leiomyosarcoma RETROPERITONEAL/ABDOMINAL LEIOMYOSARCOMAS Gross and Microscopic Findings Histologic Variants of Leiomyosarcoma Immunohistochemical Findings Criteria of Malignancy Differential Diagnosis Genetic Findings Clinical Behavior LEIOMYOSARCOMAS OF SOMATIC SOFT TISSUE LEIOMYOSARCOMAS OF VASCULAR ORIGIN Clinical Findings Gross and Microscopic Findings Clinical Behavior ATYPICAL INTRADERMAL SMOOTH MUSCLE NEOPLASMS (CUTANEOUS LEIOMYOSARCOMAS) Microscopic, Immunohistochemical, and Genetic Findings Clinical Behavior MISCELLANEOUS SARCOMAS OF VASCULAR ORIGIN EPSTEIN-BARR VIRUS–ASSOCIATED SMOOTH MUSCLE TUMORS REFERENCES 17 - Gastrointestinal Stromal Tumor (GIST) and Extragastrointestinal GIST EPIDEMIOLOGY AND CLINICAL FINDINGS MACROSCOPIC FINDINGS MICROSCOPIC FINDINGS IMMUNOHISTOCHEMICAL FINDINGS ULTRASTRUCTURAL FINDINGS GENETIC FINDINGS SUCCINATE DEHYDROGENASE–DEFICIENT GIST PEDIATRIC GASTROINTESTINAL STROMAL TUMORS GASTROINTESTINAL STROMAL TUMOR SYNDROMES BEHAVIOR OF GIST TREATMENT OF GIST HISTOLOGIC ASSESSMENT OF TREATED GIST ALGORITHM FOR DIAGNOSIS AND TREATMENT OF GIST REFERENCES 18 - Rhabdomyoma STRIATED MUSCLE TISSUE: DEVELOPMENT AND STRUCTURE CLASSIFICATION OF RHABDOMYOMAS CARDIAC RHABDOMYOMA ADULT RHABDOMYOMA Pathologic Findings Differential Diagnosis Prognosis and Therapy FETAL RHABDOMYOMA Clinical Features Pathologic Findings Differential Diagnosis Prognosis and Therapy GENITAL RHABDOMYOMA RHABDOMYOMATOUS MESENCHYMAL HAMARTOMA OF THE SKIN MISCELLANEOUS LESIONS MIMICKING BENIGN STRIATED MUSCLE TUMORS REFERENCES 19 - Rhabdomyosarcoma INCIDENCE HISTOLOGIC CLASSIFICATION AGE AND GENDER DISTRIBUTION CLINICAL FEATURES GROSS FINDINGS RHABDOMYOSARCOMA SUBTYPES Embryonal Rhabdomyosarcoma Embryonal Rhabdomyosarcoma, Botryoid Type Alveolar Rhabdomyosarcoma Pleomorphic Rhabdomyosarcoma Sclerosing/Spindle Cell Rhabdomyosarcoma Epithelioid Rhabdomyosarcoma SPECIAL DIAGNOSTIC PROCEDURES FOR RHABDOMYOSARCOMA Special Stains Immunohistochemical Findings DIFFERENTIAL DIAGNOSIS OF RHABDOMYOSARCOMA PROGNOSIS FOR RHABDOMYOSARCOMA RECURRENCE AND METASTASIS OF RHABDOMYOSARCOMA REFERENCES 20 - Benign Vascular Tumors and Malformations HEMANGIOMAS Cherry Angioma (Senile Angioma, Campbell de Morgan Spots) Infantile Hemangioma Congenital Hemangiomas Pyogenic Granuloma (Lobular Capillary Hemangioma) Intramuscular Hemangioma, Capillary Type Epithelioid Hemangioma (Angiolymphoid Hyperplasia with Eosinophilia) Hobnail Hemangioma (Targetoid Hemosiderotic Hemangioma) Sinusoidal Hemangioma Anastomosing Hemangioma Acquired Tufted Angioma (Angioblastoma of Nakagawa) Spindle Cell Hemangioma VASCULAR MALFORMATIONS Cutaneous Capillary Malformations Venous Malformation (Venous Hemangioma) PTEN Hamartoma of Soft Tissue Arteriovenous Malformation (Arteriovenous Hemangioma) Hereditary Hemorrhagic Telangiectasia (Osler-Weber-Rendu disease) Angiomatosis Verrucous Venous Malformation Synovial Venous Malformation (Synovial Hemangioma) Vascular Anomalies Involving Peripheral Nerves REACTIVE VASCULAR PROLIFERATIONS Papillary Endothelial Hyperplasia Vascular Transformation of Lymph Nodes Glomeruloid Hemangioma Bacillary (Epithelioid) Angiomatosis Florid Vascular Proliferation of Colon Secondary to Intussusception and Prolapse Diffuse Dermal Angiomatosis (Reactive Angioendotheliomatosis) REFERENCES 21 - Hemangioendothelioma: Vascular Tumors of Intermediate Malignancy VASCULAR TUMORS OF INTERMEDIATE MALIGNANCY EPITHELIOID HEMANGIOENDOTHELIOMA Microscopic Features Differential Diagnosis Behavior and Treatment Genetics Epithelioid Hemangioendotheliomas in Other Sites KAPOSIFORM HEMANGIOENDOTHELIOMA KAPOSIFORM LYMPHANGIOMATOSIS HOBNAIL (DABSKA-RETIFORM) HEMANGIOENDOTHELIOMA Clinical Features Microscopic Features Immunohistochemical Findings Discussion PSEUDOMYOGENIC (EPITHELIOID SARCOMA-LIKE) HEMANGIOENDOTHELIOMA COMPOSITE HEMANGIOENDOTHELIOMA REFERENCES 22 - Malignant Vascular Tumors ANGIOSARCOMA Incidence Etiologic Factors Molecular Genetic Findings Clinical Subtypes Prognostic Factors and Clinical Behavior ATYPICAL VASCULAR LESION KAPOSI SARCOMA Clinical Findings Microscopic Findings Immunohistochemical Findings Differential Diagnosis Behavior and Treatment REFERENCES 23 - Tumors and Malformations of Lymphatic Vessels LYMPHATIC MALFORMATIONS (LYMPHANGIOMA, CYSTIC HYGROMA) Clinical Findings Lymphatic Malformations of the Head and Neck Intraabdominal Lymphatic Malformations Cutaneous Lymphatic Malformations Gross and Microscopic Findings Behavior and Treatment LYMPHANGIOMATOSIS Kaposiform Lymphangiomatosis LOCALIZED MASSIVE LYMPHEDEMA REFERENCES 24 - Perivascular Tumors CLASSIC (SPORADIC) GLOMUS TUMOR Clinical Findings Gross Findings Immunohistochemical Findings Genetic Findings Behavior and Treatment GLOMUVENOUS MALFORMATION (GLOMANGIOMA, FAMILIAL GLOMANGIOMA) GLOMANGIOMYOMA GLOMANGIOMATOSIS (DIFFUSE GLOMUS TUMOR) ATYPICAL AND MALIGNANT GLOMUS TUMORS Malignant Glomus Tumor Glomus Tumor of Uncertain Malignant Potential Symplastic Glomus Tumor MYOPERICYTOMA MYOFIBROMA AND MYOFIBROMATOSIS Clinical Findings Pathologic Findings Immunohistochemical Findings Genetic Findings Differential Diagnosis Discussion SINONASAL GLOMANGIOPERICYTOMA (SINONASAL HEMANGIOPERICYTOMA-LIKE TUMOR) REFERENCES 25 - Benign Tumors and Tumorlike Lesions of Synovial Tissue TENOSYNOVIAL GIANT CELL TUMOR, LOCALIZED TYPE Clinical Findings Gross Findings Microscopic Findings Differential Diagnosis Immunohistochemical Findings Cytogenetic and Molecular Genetic Findings Discussion TENOSYNOVIAL GIANT CELL TUMOR, DIFFUSE TYPE (EXTRAARTICULAR PIGMENTED VILLONODULAR SYNOVITIS) Differential Diagnosis Behavior and Treatment MALIGNANT TENOSYNOVIAL GIANT CELL TUMOR (MALIGNANT PIGMENTED VILLONODULAR SYNOVITIS) Clinical Features Pathologic Features Immunohistochemical and Molecular Genetic Features Behavior MISCELLANEOUS CONDITIONS RESEMBLING DIFFUSE GIANT CELL TUMOR REFERENCES 26 - Benign Tumors of Peripheral Nerves NORMAL ANATOMY TRAUMATIC (AMPUTATION) NEUROMA MUCOSAL NEUROMA PACINIAN NEUROMA SOLITARY CIRCUMSCRIBED NEUROMA (PALISADED ENCAPSULATED NEUROMA) MORTON NEUROMA (MORTON METATARSALGIA) NERVE SHEATH GANGLION CYSTS NEUROMUSCULAR CHORISTOMA (NEUROMUSCULAR HAMARTOMA, BENIGN TRITON TUMOR) NEUROFIBROMA Localized (Sporadic) Neurofibroma Microscopic Findings NEUROFIBROMATOSIS 1 (NF1) Clinical Findings Variants of NF1 Pathologic Findings Malignant Change in Neurofibromas Discussion SCHWANNOMA Clinical Findings Gross Findings Microscopic Findings Immunohistochemical Findings Discussion Schwannoma with Degenerative Change (Ancient Schwannoma) Cellular Schwannoma Plexiform Schwannoma Epithelioid Schwannoma NEUROFIBROMATOSIS 2 (NF2; BILATERAL VESTIBULAR SCHWANNOMAS) SCHWANNOMATOSIS PERINEURIOMA Intraneural Perineurioma Soft Tissue (Extraneural) Perineurioma Sclerosing Perineurioma Hybrid Benign Peripheral Nerve Sheath Tumors (Hybrid Perineurioma-Schwannoma, Hybrid Schwannoma-Neurofibroma, Hybrid Neurofibrom... GRANULAR CELL TUMOR Pathologic Findings Differential Diagnosis Discussion Malignant Granular Cell Tumor CONGENITAL (GINGIVAL) GRANULAR CELL TUMOR NERVE SHEATH MYXOMA EXTRACRANIAL MENINGIOMA GLIAL HETEROTOPIAS REFERENCES 27 - Malignant Peripheral Nerve Sheath Tumors MALIGNANT PERIPHERAL NERVE SHEATH TUMOR Clinical Findings Gross Findings Microscopic Findings Immunohistochemical Findings Differential Diagnosis Molecular and Cytogenetic Findings Clinical Behavior MALIGNANT PERIPHERAL NERVE SHEATH TUMOR WITH RHABDOMYOBLASTIC DIFFERENTIATION (MALIGNANT TRITON TUMOR) MALIGNANT PERIPHERAL NERVE SHEATH TUMOR WITH GLANDS (GLANDULAR MALIGNANT SCHWANNOMA) MALIGNANT PERIPHERAL NERVE SHEATH TUMOR WITH ANGIOSARCOMA EPITHELIOID MALIGNANT PERIPHERAL NERVE SHEATH TUMOR PERINEURIAL MALIGNANT PERIPHERAL NERVE SHEATH TUMOR (MALIGNANT PERINEURIOMA) EXTRASPINAL (SOFT TISSUE) EPENDYMOMA REFERENCES 28 - Peripheral Neuroblastic Tumors ETIOLOGIC AND GENETIC FACTORS CLINICAL FINDINGS RADIOGRAPHIC FINDINGS LABORATORY FINDINGS GROSS AND MICROSCOPIC FINDINGS Neuroblastoma (Schwannian Stroma-Poor) Ganglioneuroblastoma, Intermixed (Schwannian Stroma-Rich) Ganglioneuroma (Schwannian Stroma-Dominant) Ganglioneuroblastoma, Nodular (Composite, Schwannian Stroma-Rich/Stroma-Dominant and Stroma-Poor) IMMUNOHISTOCHEMICAL FINDINGS DIFFERENTIAL DIAGNOSIS PROGNOSTIC FACTORS AND RISK GROUPINGS Age at Diagnosis Clinical Staging Molecular/Genomic Prognostic Factors Histopathologic Classification International Neuroblastoma Risk Group Relationship between INRG and INPC Risk Classification and Therapy Stratification of High-Risk/Unfavorable Histology Neuroblastoma Proposed Subgrouping of Unfavorable Histology Neuroblastomas PERIPHERAL NEUROBLASTIC TUMORS IN ADULTS AND OTHER DISEASES/CONDITIONS REFERENCES 29 - Soft Tissue Tumors Showing Melanocytic Differentiation MALIGNANT MELANOTIC SCHWANNIAN TUMOR (MELANOTIC SCHWANNOMA) MELANOTIC NEUROECTODERMAL TUMOR OF INFANCY (RETINAL ANLAGE TUMOR, MELANOTIC PROGONOMA) Clinical Findings Gross and Microscopic Findings Discussion CLEAR CELL SARCOMA OF TENDON AND APONEUROSIS Clinical Findings Pathologic Findings Differential Diagnosis Discussion MALIGNANT GASTROINTESTINAL NEUROECTODERMAL TUMOR (CLEAR CELL SARCOMA-LIKE TUMOR OF GASTROINTESTINAL TRACT) Clinical Features Pathologic Features Cytogenetic and Molecular Genetic Features Discussion Differential Diagnosis PERIVASCULAR EPITHELIOID CELL FAMILY OF TUMORS Angiomyolipoma Lymphangiomyoma and Lymphangiomyomatosis PEComa (Excluding AML, LAM, and Pulmonary CCST) REFERENCES 30 - Cartilaginous and Osseous Soft Tissue Tumors EXTRASKELETAL CHONDROMA (CHONDROMA OF SOFT PARTS) Clinical Findings Pathologic Findings Differential Diagnosis Discussion MESENCHYMAL CHONDROSARCOMA Clinical Findings Pathologic Findings Cytogenetic and Molecular Genetic Findings Differential Diagnosis Discussion NONNEOPLASTIC HETEROTOPIC OSSIFICATIONS Myositis Ossificans Fibroosseous Pseudotumor of the Digits FIBRODYSPLASIA (MYOSITIS) OSSIFICANS PROGRESSIVA Clinical Findings Pathologic Findings Genetic Findings Discussion EXTRASKELETAL OSTEOSARCOMA Clinical Findings Pathogenesis Radiation-Induced Extraskeletal Osteosarcoma Pathologic Findings Immunohistochemical Findings Differential Diagnosis Discussion REFERENCES 31 - Miscellaneous Benign Soft Tissue Tumors and Pseudotumors TUMORAL CALCINOSIS Clinical Findings Pathologic Findings Differential Diagnosis Discussion INTRAMUSCULAR MYXOMA Clinical Findings Multiple Intramuscular Myxomas and Fibrous Dysplasia Pathologic Findings Differential Diagnosis Discussion JUXTA-ARTICULAR MYXOMA CUTANEOUS MYXOMA (SUPERFICIAL ANGIOMYXOMA) Pathologic Findings Differential Diagnosis Discussion CARNEY COMPLEX GANGLION (GANGLION CYST) AMYLOID TUMOR (AMYLOIDOMA) REFERENCES 32 - Soft Tissue Tumors of Intermediate Malignancy of Uncertain Type OSSIFYING FIBROMYXOID TUMOR OF SOFT TISSUE Clinical Findings Pathologic Findings Immunohistochemical Findings Genetic Findings Differential Diagnosis Discussion Line of Differentiation HEMOSIDEROTIC FIBROLIPOMATOUS TUMOR AND PLEOMORPHIC HYALINIZING ANGIECTATIC TUMOR Hemosiderotic Fibrolipomatous Tumor Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts Differential Diagnosis of HFLT and PHAT HFLT and PHAT Showing Progression to Myxoid Sarcoma MYOEPITHELIOMA/MIXED TUMOR OF SOFT TISSUE (PARACHORDOMA) Clinical Findings Pathologic Findings Immunohistochemical Findings Cytogenetic and Molecular Genetic Findings Differential Diagnosis Discussion SOLITARY FIBROUS TUMOR (HEMANGIOPERICYTOMA) Clinical Findings Pathologic Findings Immunohistochemical Findings Genetic Findings Differential Diagnosis Discussion ANGIOMATOID FIBROUS HISTIOCYTOMA Clinical Findings Pathologic Findings Immunohistochemical Findings Cytogenetic and Molecular Genetic Findings Discussion PHOSPHATURIC MESENCHYMAL TUMOR Clinical Findings Pathologic Findings Immunohistochemical Findings Genetic Findings Differential Diagnosis SUPERFICIAL CD34-POSITIVE FIBROBLASTIC TUMOR Clinical Findings Pathologic Findings Immunohistochemical Findings Genetic Findings Differential Diagnosis BIPHENOTYPIC SINONASAL SARCOMA Clinical Findings Pathologic Findings Immunohistochemical Findings Genetic Findings Differential Diagnosis REFERENCES 33 - Other Malignant Soft Tissue Tumors, Including Those of Uncertain Type EWING SARCOMA Clinical Features Pathologic Findings Immunohistochemical Findings Cytogenetic and Molecular Genetic Findings Clinical Behavior and Therapy CIC-REARRANGED SARCOMA Clinical Findings Pathologic Findings Immunohistochemical Findings Cytogenetic and Molecular Genetic Findings Discussion BCOR-REARRANGED SARCOMA Clinical Findings Pathologic Findings Immunohistochemical Findings Molecular Genetic Findings Discussion Differential Diagnosis of Ewing Sarcoma and Ewing-Like Sarcomas Harboring CIC and BCOR Rearrangements. In addition to being dist... EXTRASKELETAL MYXOID CHONDROSARCOMA Clinical Findings Pathologic Findings Immunohistochemical Findings Cytogenetic and Molecular Genetic Findings Differential Diagnosis Discussion SYNOVIAL SARCOMA Clinical Findings Anatomic Location. Synovial sarcomas occur predominantly in the extremities, where they tend to arise in the vicinity of large j... Radiographic Findings. Radiographic studies may be extremely helpful for suggesting a preoperative diagnosis of synovial sarcoma... Gross Findings Microscopic Findings Biphasic Synovial Sarcoma. The classic synovial sarcoma—the biphasic type—is generally readily recognizable by the coexistence o... Monophasic Fibrous Synovial Sarcoma. The monophasic fibrous synovial sarcoma is a relatively common neoplasm and is much more co... Epithelial-Predominant Synovial Sarcoma (so-called Monophasic Epithelial Synovial Sarcoma). Monophasic epithelial synovial sarco... Poorly Differentiated Synovial Sarcoma. Poorly differentiated synovial sarcoma (PDSS) can be seen as a form of tumor progression... Immunohistochemical Findings Cytogenetic and Molecular Genetic Findings Differential Diagnosis Differential Diagnosis of Biphasic Synovial Sarcoma. In general, biphasic synovial sarcoma causes few diagnostic problems, espec... Differential Diagnosis of Monophasic Fibrous Synovial Sarcoma. The monophasic fibrous synovial sarcoma may resemble a number of ... Differential Diagnosis of Epithelial-Predominant Synovial Sarcoma. Distinction of largely epithelial form "Since its first publication more than 35 years ago, Enzinger and Weiss's Soft Tissue Tumors has established itself as the most comprehensive and authoritative reference available on soft tissue pathology. The 7th Edition from Drs. John R. Goldblum, Andrew L. Folpe, and Sharon W. Weiss, continues this tradition with detailed, well-written, logically organized coverage of the full spectrum of these often difficult and challenging tumors. It offers clear guidance to practicing and trainee pathologists on diagnosis of tumors by microscopy, immunohistochemistry, and molecular genetics, as well as a significant amount of clinically significant information of interest to the clinicians who most frequently see these diseases - dermatologists, orthopaedists, and oncologists. Offers practical information on differential diagnoses of tumors of the skeletal muscles, connective tissue, fat, and related structures, helping you accurately diagnose and confidently sign out pathology reports on even the most challenging cases. Provides unsurpassed scope and depth in this complex area, with microscopic findings correlated with the latest developments in molecular biology, cytogenetics, and immunohistochemistry, for a comprehensive and integrated approach to evaluation and diagnosis. Incorporates new knowledge on recently identified entities, next-generation sequencing (NGS), molecular diagnostic techniques, and immunohistochemical and genetic features of soft tissue tumors, providing up-to-date diagnostic and prognostic information that will inform day-to-day therapeutic decisions. Features over 2000 high-quality images that clearly capture the clinical, macroscopic, and microscopic features of benign and malignant conditions, helping you relate these characteristics to their specific classifications. Utilizes a logical, well-structured format including chapter outlines and a consistent approach to every topic, enabling you to navigate the text quickly, improve turnaround time when diagnosing a specimen, and clearly report on prognosis and therapeutic management options. Includes abundant algorithms, tables, and graphs to facilitate rapid decision making."--Provided by publisher Since its first publication more than 35 years ago, __Enzinger and Weiss’s Soft Tissue Tumors__ has established itself as **the most comprehensive and authoritative reference available on soft tissue pathology.** The 7th Edition from Drs. John R. Goldblum, Andrew L. Folpe, and Sharon W. Weiss, continues this tradition with detailed, well-written, logically organized coverage of the full spectrum of these often difficult and challenging tumors. It offers **clear guidance to practicing and trainee pathologists** on diagnosis of tumors by microscopy, immunohistochemistry, and molecular genetics, as well as a significant amount of clinically significant information of interest to the clinicians who most frequently see these diseases – dermatologists, orthopaedists, and oncologists.