Offering comprehensive yet practical coverage on diagnosis and differential diagnosis in one of the most challenging areas of surgical pathology, Enzinger and Weiss’s Soft Tissue Tumors, edited by Drs. Andrew L. Folpe and G. Petur Nielsen, continues the tradition of excellence in this fully revised 8th Edition. It provides clear guidance from cover to cover with detailed, well-written, logically organized information on the full spectrum of these complex tumors. Practicing and trainee pathologists will find complete coverage of diagnosis of tumors by microscopy, immunohistochemistry, and molecular genetics, while clinicians who most frequently see these diseases—dermatologists, orthopaedists, and oncologists—will also benefit from a considerable amount of clinically significant information of interest to their field.
1. Introduction - p. 102 2. Intermediate Filaments - p. 102 3. Epithelial Membrane Antigen - p. 108 4. Markers of Muscle Differentiation - p. 108 5. Markers of Nerve Sheath Differentiation - p. 119 6. “Neuroectodermal” Markers - p. 124 7. Markers of Melanocytic Differentiation - p. 128 8. Markers of Endothelial Differentiation - p. 132 9. Markers of Gastrointestinal Stromal Tumors - p. 137 10. Additional Markers of Cytolineage or Differentiation - p. 137 11. Immunohistochemistry as a Surrogate for Tumor-Specific Molecular Alterations - p. 141 12. Immunohistochemical Markers to Detect Components of Fusion Oncoproteins - p. 143 13. NUTM1 as a Marker of NUTM1-Rearranged Sarcoma - p. 154 14. RNA in situ Hybridization for Detecting Overexpression of Secreted Growth Factors - p. 155 15. Immunohistochemical Markers of Recurrent Fusion-Related Gene Expression Signatures - p. 156 16. Surrogate IHC Markers of Recurrent Nonfusion Molecular Alterations - p. 161 17. Prognostic Markers - p. 166 18. Application of Immunohistochemistry to Sarcoma Diagnosis: Clinical Scenarios - p. 167 19. Conclusion - p. 170 20. 7. Benign Fibroblastic/Myofibroblastic Neoplasms and Proliferations, Including Superficial Fibromatoses - p. 171 21. Classification of Benign Fibroblastic/Myofibroblastic Proliferations - p. 172 22. Nodular Fasciitis - p. 172 23. Proliferative Fasciitis - p. 183 24. Proliferative Myositis - p. 187 25. Organ-Associated Pseudosarcomatous Myofibroblastic Proliferations (Pseudosarcomatous Myofibroblastic Neoplasm) - p. 188 26. Ischemic Fasciitis (Atypical Decubital Fibroplasia) - p. 195 27. Fibroma of Tendon Sheath - p. 198 28. Pleomorphic Fibroma of the Skin - p. 201 29. Nuchal-Type Fibroma - p. 201 30. Gardner-Associated Fibroma - p. 202 31. Elastofibroma - p. 205 32. Sinonasal tract Angiofibroma - p. 208 33. Keloid - p. 211 34. Desmoplastic Fibroblastoma (Collagenous Fibroma) - p. 214 35. Intranodal Palisaded Myofibroblastoma - p. 215 36. Mammary-Type Myofibroblastoma - p. 216 37. Superficial Acral Fibromyxoma (Digital Fibromyxoma) - p. 220 38. Soft Tissue Angiofibroma - p. 224 39. Superficial Fibromatoses - p. 228 40. Palmar Fibromatosis (Dupuytren Disease) - p. 228 41. Plantar Fibromatosis (Ledderhose Disease) - p. 232 42. Penile Fibromatosis (Peyronie Disease) - p. 234 43. Knuckle Pads - p. 236 44. 8. Fibrous Tumors of Infancy and Childhood - p. 238 45. 9. Borderline and Malignant Fibroblastic/Myofibroblastic Tumors - p. 268 46. Deep (Desmoid-type) Fibromatoses - p. 268 47. Infantile Fibrosarcoma - p. 279 48. Inflammatory Myofibroblastic Tumor - p. 284 49. Fibrosarcomas - p. 290 50. Adult-type Fibrosarcoma - p. 293 51. Fibrosarcoma Variants - p. 297 52. 10. Benign Fibrohistiocytic Tumors - p. 313 53. 11. Fibrohistiocytic Tumors of Intermediate Malignancy - p. 358 54. 12. Undifferentiated Pleomorphic Sarcoma, Myxofibrosarcoma, and Atypical Fibroxanthoma/Pleomorphic Dermal Sarcoma - p. 388 55. 13. Benign Lipomatous Tumors - p. 409 56. White Fat - p. 409 57. Brown Fat - p. 410 58. Classification of Benign Lipomatous Tumors - p. 410 59. Lipoma - p. 410 60. Multiple Lipomas - p. 413 61. Angiolipoma - p. 415 62. Myolipoma - p. 420 63. Chondroid Lipoma - p. 421 64. Spindle Cell/Pleomorphic Lipoma - p. 424 65. Atypical Spindle Cell/Pleomorphic Lipomatous Tumor - p. 430 66. Lipoblastoma and Lipoblastomatosis - p. 434 67. Lipoblastoma-Like Tumor - p. 439 68. Angiomyolipoma - p. 442 69. Myelolipoma - p. 442 70. Intramuscular and Intermuscular Lipomas - p. 442 71. Lipomas of Tendon Sheaths and Joints - p. 443 72. Lumbosacral Lipoma - p. 443 73. Lipomatosis of Nerve (Lipofibromatous Hamartoma of Nerves) - p. 445 74. Diffuse Lipomatosis - p. 446 75. Symmetric Lipomatosis - p. 447 76. Pelvic Lipomatosis - p. 450 77. Steroid Lipomatosis - p. 450 78. Nevus Lipomatosus Superficialis (Nevus Lipomatosus Cutaneous Superficialis) - p. 450 79. Hibernoma - p. 451 80. 14. Liposarcoma - p. 457 81. 15. Benign Tumors Showing Smooth Muscle Differentiation - p. 506 82. 16. Benign Genital Stromal Tumors - p. 529 83. 17. Leiomyosarcoma - p. 555 84. 18. Gastrointestinal Stromal Tumor (GIST) and Other Specialized Gastrointestinal Mesenchymal Tumors - p. 577 85. 19. Skeletal Muscle Tumors: Benign and of Borderline Malignancy - p. 615 86. 20. Rhabdomyosarcoma - p. 635 87. 21. Benign Vascular Tumors and Malformations - p. 676 88. 22. Hemangioendothelioma: Vascular Tumors of Intermediate Malignancy - p. 729 89. 23. Angiosarcoma, Atypical Vascular Lesions Following Irradiation, and Kaposi Sarcoma - p. 766 90. 24. Tumors and Malformations of Lymphatic Vessels - p. 799 91. 25. Perivascular Tumors - p. 819 92. 26. Tumors and Tumor-Like Lesions of Synovial Tissue - p. 843 93. 27. Benign Tumors of Peripheral Nerves - p. 880 94. Normal Anatomy - p. 880 95. Traumatic (Amputation) Neuroma - p. 881 96. Mucosal Neuroma - p. 883 97. Pacinian Neuroma - p. 883 98. Solitary Circumscribed Neuroma (Palisaded Encapsulated Neuroma) - p. 884 99. Morton Neuroma (Morton Metatarsalgia) - p. 885 100. Nerve Sheath Ganglion Cysts - p. 886 101. Neuromuscular Choristoma (Neuromuscular Hamartoma, Benign Triton Tumor) - p. 887 102. Neurofibroma - p. 887 103. Neurofibromatosis Type-1 (NF1) - p. 891 104. Schwannoma - p. 901 105. Schwannomatosis - p. 926 106. Perineurioma - p. 927 107. Granular Cell Tumor - p. 934 108. Congenital (Gingival) Granular Cell Tumor - p. 941 109. Nerve Sheath Myxoma - p. 941 110. Extracranial Meningioma - p. 944 111. Glial Heterotopias - p. 945 112. 28. Malignant Peripheral Nerve Sheath Tumors - p. 953 113. 29. Peripheral Neuroblastic Tumors - p. 987 114. 30. Soft Tissue Tumors Showing Melanocytic Differentiation - p. 1006 115. 31. Osteocartilaginous Soft Tissue Tumors, Tumoral Calcinosis and Amyloidoma - p. 1055 116. Extraskeletal Chondroma (Chondroma of Soft Parts) - p. 1055 117. Calcified Chondroid Mesenchymal Neoplasms - p. 1060 118. Mesenchymal Chondrosarcoma - p. 1061 119. Fibrodysplasia (Myositis) Ossificans Progressiva - p. 1073 120. Extraskeletal Osteosarcoma - p. 1080 121. Ossifying Fibromyxoid Tumor of Soft Tissue - p. 1084 122. Phosphaturic Mesenchymal Tumor - p. 1097 123. Amyloid Tumor (Amyloidoma) - p. 1107 124. 32. Myxoid Soft Tissue Tumors: Benign, Borderline and Malignant - p. 1110 125. 33. Soft Tissue Tumors Showing Epithelial, Myoepithelial and Notochordal Differentiation - p. 1150 126. Introduction - p. 1150 127. Epithelioid Sarcoma - p. 1150 128. Malignant Extrarenal Rhabdoid Tumor - p. 1163 129. Synovial Sarcoma - p. 1167 130. Low Risk for Metastasis - p. 1186 131. High Risk for Metastasis - p. 1186 132. Myoepithelioma/Mixed Tumor of Soft Tissue - p. 1186 133. Extraaxial Chordoma (Chordoma Periphericum) - p. 1196 134. Xanthogranulomatous Epithelial Tumor/Keratin-Positive Giant Cell-Rich Tumor of Soft Tissue and Bone - p. 1202 135. 34. Fusion-Driven Round Cell Sarcomas - p. 1212 136. 35. Other Important Soft Tissue Tumors - p. 1246 137. Introduction - p. 1246 138. Solitary Fibrous Tumor - p. 1246 139. Angiomatoid Fibrous Histiocytoma - p. 1260 140. Biphenotypic Sinonasal Sarcoma - p. 1270 141. Extraskeletal Myxoid Chondrosarcoma - p. 1274 142. Clinical Findings - p. 1274 143. Gross and Microscopic Findings - p. 1275 144. Outcome - p. 1282 145. Differential Diagnosis - p. 1283 146. Alveolar Soft Part Sarcoma - p. 1284 147. Clinical Findings - p. 1284 148. Gross and Microscopic Findings - p. 1284 149. Immunohistochemical Findings - p. 1285 150. Ultrastructural Findings - p. 1287 151. Cytogenetic and Molecular Genetic Findings - p. 1287 152. Outcome - p. 1287 153. Differential Diagnosis - p. 1289 154. Follicular Dendritic Cell Sarcoma - p. 1289 155. Clinical Findings - p. 1291 156. Gross and Microscopic Findings - p. 1291 157. Cytogenetic and Molecular Genetic Findings - p. 1294 158. Outcome - p. 1294 159. Differential Diagnosis - p. 1294 160. 36. Emerging Entities - p. 1300 161. Index - p. 1335
Offering comprehensive yet practical coverage on diagnosis and differential diagnosis in one of the most challenging areas of surgical pathology, Enzinger and Weiss’s Soft Tissue Tumors, edited by Drs. Andrew L. Folpe and G. Petur Nielsen, continues the tradition of excellence in this fully revised 8th Edition. It provides clear guidance from cover to cover with detailed, well-written, logically organized information on the full spectrum of these complex tumors. Practicing and trainee pathologists will find complete coverage of diagnosis of tumors by microscopy, immunohistochemistry, and molecular genetics, while clinicians who most frequently see these diseases—dermatologists, orthopaedists, and oncologists—will also benefit from a considerable amount of clinically significant information of interest to their field.
1. Introduction - p. 102 2. Intermediate Filaments - p. 102 3. Epithelial Membrane Antigen - p. 108 4. Markers of Muscle Differentiation - p. 108 5. Markers of Nerve Sheath Differentiation - p. 119 6. “Neuroectodermal” Markers - p. 124 7. Markers of Melanocytic Differentiation - p. 128 8. Markers of Endothelial Differentiation - p. 132 9. Markers of Gastrointestinal Stromal Tumors - p. 137 10. Additional Markers of Cytolineage or Differentiation - p. 137 11. Immunohistochemistry as a Surrogate for Tumor-Specific Molecular Alterations - p. 141 12. Immunohistochemical Markers to Detect Components of Fusion Oncoproteins - p. 143 13. NUTM1 as a Marker of NUTM1-Rearranged Sarcoma - p. 154 14. RNA in situ Hybridization for Detecting Overexpression of Secreted Growth Factors - p. 155 15. Immunohistochemical Markers of Recurrent Fusion-Related Gene Expression Signatures - p. 156 16. Surrogate IHC Markers of Recurrent Nonfusion Molecular Alterations - p. 161 17. Prognostic Markers - p. 166 18. Application of Immunohistochemistry to Sarcoma Diagnosis: Clinical Scenarios - p. 167 19. Conclusion - p. 170 20. 7. Benign Fibroblastic/Myofibroblastic Neoplasms and Proliferations, Including Superficial Fibromatoses - p. 171 21. Classification of Benign Fibroblastic/Myofibroblastic Proliferations - p. 172 22. Nodular Fasciitis - p. 172 23. Proliferative Fasciitis - p. 183 24. Proliferative Myositis - p. 187 25. Organ-Associated Pseudosarcomatous Myofibroblastic Proliferations (Pseudosarcomatous Myofibroblastic Neoplasm) - p. 188 26. Ischemic Fasciitis (Atypical Decubital Fibroplasia) - p. 195 27. Fibroma of Tendon Sheath - p. 198 28. Pleomorphic Fibroma of the Skin - p. 201 29. Nuchal-Type Fibroma - p. 201 30. Gardner-Associated Fibroma - p. 202 31. Elastofibroma - p. 205 32. Sinonasal tract Angiofibroma - p. 208 33. Keloid - p. 211 34. Desmoplastic Fibroblastoma (Collagenous Fibroma) - p. 214 35. Intranodal Palisaded Myofibroblastoma - p. 215 36. Mammary-Type Myofibroblastoma - p. 216 37. Superficial Acral Fibromyxoma (Digital Fibromyxoma) - p. 220 38. Soft Tissue Angiofibroma - p. 224 39. Superficial Fibromatoses - p. 228 40. Palmar Fibromatosis (Dupuytren Disease) - p. 228 41. Plantar Fibromatosis (Ledderhose Disease) - p. 232 42. Penile Fibromatosis (Peyronie Disease) - p. 234 43. Knuckle Pads - p. 236 44. 8. Fibrous Tumors of Infancy and Childhood - p. 238 45. 9. Borderline and Malignant Fibroblastic/Myofibroblastic Tumors - p. 268 46. Deep (Desmoid-type) Fibromatoses - p. 268 47. Infantile Fibrosarcoma - p. 279 48. Inflammatory Myofibroblastic Tumor - p. 284 49. Fibrosarcomas - p. 290 50. Adult-type Fibrosarcoma - p. 293 51. Fibrosarcoma Variants - p. 297 52. 10. Benign Fibrohistiocytic Tumors - p. 313 53. 11. Fibrohistiocytic Tumors of Intermediate Malignancy - p. 358 54. 12. Undifferentiated Pleomorphic Sarcoma, Myxofibrosarcoma, and Atypical Fibroxanthoma/Pleomorphic Dermal Sarcoma - p. 388 55. 13. Benign Lipomatous Tumors - p. 409 56. White Fat - p. 409 57. Brown Fat - p. 410 58. Classification of Benign Lipomatous Tumors - p. 410 59. Lipoma - p. 410 60. Multiple Lipomas - p. 413 61. Angiolipoma - p. 415 62. Myolipoma - p. 420 63. Chondroid Lipoma - p. 421 64. Spindle Cell/Pleomorphic Lipoma - p. 424 65. Atypical Spindle Cell/Pleomorphic Lipomatous Tumor - p. 430 66. Lipoblastoma and Lipoblastomatosis - p. 434 67. Lipoblastoma-Like Tumor - p. 439 68. Angiomyolipoma - p. 442 69. Myelolipoma - p. 442 70. Intramuscular and Intermuscular Lipomas - p. 442 71. Lipomas of Tendon Sheaths and Joints - p. 443 72. Lumbosacral Lipoma - p. 443 73. Lipomatosis of Nerve (Lipofibromatous Hamartoma of Nerves) - p. 445 74. Diffuse Lipomatosis - p. 446 75. Symmetric Lipomatosis - p. 447 76. Pelvic Lipomatosis - p. 450 77. Steroid Lipomatosis - p. 450 78. Nevus Lipomatosus Superficialis (Nevus Lipomatosus Cutaneous Superficialis) - p. 450 79. Hibernoma - p. 451 80. 14. Liposarcoma - p. 457 81. 15. Benign Tumors Showing Smooth Muscle Differentiation - p. 506 82. 16. Benign Genital Stromal Tumors - p. 529 83. 17. Leiomyosarcoma - p. 555 84. 18. Gastrointestinal Stromal Tumor (GIST) and Other Specialized Gastrointestinal Mesenchymal Tumors - p. 577 85. 19. Skeletal Muscle Tumors: Benign and of Borderline Malignancy - p. 615 86. 20. Rhabdomyosarcoma - p. 635 87. 21. Benign Vascular Tumors and Malformations - p. 676 88. 22. Hemangioendothelioma: Vascular Tumors of Intermediate Malignancy - p. 729 89. 23. Angiosarcoma, Atypical Vascular Lesions Following Irradiation, and Kaposi Sarcoma - p. 766 90. 24. Tumors and Malformations of Lymphatic Vessels - p. 799 91. 25. Perivascular Tumors - p. 819 92. 26. Tumors and Tumor-Like Lesions of Synovial Tissue - p. 843 93. 27. Benign Tumors of Peripheral Nerves - p. 880 94. Normal Anatomy - p. 880 95. Traumatic (Amputation) Neuroma - p. 881 96. Mucosal Neuroma - p. 883 97. Pacinian Neuroma - p. 883 98. Solitary Circumscribed Neuroma (Palisaded Encapsulated Neuroma) - p. 884 99. Morton Neuroma (Morton Metatarsalgia) - p. 885 100. Nerve Sheath Ganglion Cysts - p. 886 101. Neuromuscular Choristoma (Neuromuscular Hamartoma, Benign Triton Tumor) - p. 887 102. Neurofibroma - p. 887 103. Neurofibromatosis Type-1 (NF1) - p. 891 104. Schwannoma - p. 901 105. Schwannomatosis - p. 926 106. Perineurioma - p. 927 107. Granular Cell Tumor - p. 934 108. Congenital (Gingival) Granular Cell Tumor - p. 941 109. Nerve Sheath Myxoma - p. 941 110. Extracranial Meningioma - p. 944 111. Glial Heterotopias - p. 945 112. 28. Malignant Peripheral Nerve Sheath Tumors - p. 953 113. 29. Peripheral Neuroblastic Tumors - p. 987 114. 30. Soft Tissue Tumors Showing Melanocytic Differentiation - p. 1006 115. 31. Osteocartilaginous Soft Tissue Tumors, Tumoral Calcinosis and Amyloidoma - p. 1055 116. Extraskeletal Chondroma (Chondroma of Soft Parts) - p. 1055 117. Calcified Chondroid Mesenchymal Neoplasms - p. 1060 118. Mesenchymal Chondrosarcoma - p. 1061 119. Fibrodysplasia (Myositis) Ossificans Progressiva - p. 1073 120. Extraskeletal Osteosarcoma - p. 1080 121. Ossifying Fibromyxoid Tumor of Soft Tissue - p. 1084 122. Phosphaturic Mesenchymal Tumor - p. 1097 123. Amyloid Tumor (Amyloidoma) - p. 1107 124. 32. Myxoid Soft Tissue Tumors: Benign, Borderline and Malignant - p. 1110 125. 33. Soft Tissue Tumors Showing Epithelial, Myoepithelial and Notochordal Differentiation - p. 1150 126. Introduction - p. 1150 127. Epithelioid Sarcoma - p. 1150 128. Malignant Extrarenal Rhabdoid Tumor - p. 1163 129. Synovial Sarcoma - p. 1167 130. Low Risk for Metastasis - p. 1186 131. High Risk for Metastasis - p. 1186 132. Myoepithelioma/Mixed Tumor of Soft Tissue - p. 1186 133. Extraaxial Chordoma (Chordoma Periphericum) - p. 1196 134. Xanthogranulomatous Epithelial Tumor/Keratin-Positive Giant Cell-Rich Tumor of Soft Tissue and Bone - p. 1202 135. 34. Fusion-Driven Round Cell Sarcomas - p. 1212 136. 35. Other Important Soft Tissue Tumors - p. 1246 137. Introduction - p. 1246 138. Solitary Fibrous Tumor - p. 1246 139. Angiomatoid Fibrous Histiocytoma - p. 1260 140. Biphenotypic Sinonasal Sarcoma - p. 1270 141. Extraskeletal Myxoid Chondrosarcoma - p. 1274 142. Clinical Findings - p. 1274 143. Gross and Microscopic Findings - p. 1275 144. Outcome - p. 1282 145. Differential Diagnosis - p. 1283 146. Alveolar Soft Part Sarcoma - p. 1284 147. Clinical Findings - p. 1284 148. Gross and Microscopic Findings - p. 1284 149. Immunohistochemical Findings - p. 1285 150. Ultrastructural Findings - p. 1287 151. Cytogenetic and Molecular Genetic Findings - p. 1287 152. Outcome - p. 1287 153. Differential Diagnosis - p. 1289 154. Follicular Dendritic Cell Sarcoma - p. 1289 155. Clinical Findings - p. 1291 156. Gross and Microscopic Findings - p. 1291 157. Cytogenetic and Molecular Genetic Findings - p. 1294 158. Outcome - p. 1294 159. Differential Diagnosis - p. 1294 160. 36. Emerging Entities - p. 1300 161. Index - p. 1335
