Skip to main content

Soft-Tissue Sarcomas: Etiology, Pathology, and Clinical Features

  • Chapter
Manual of Soft-Tissue Tumor Surgery

Abstract

Soft-tissue sarcomas are uncommon cancers representing only 1% of all malignant neoplasms (excluding skin) in adults. The total number of soft-tissue sarcomas diagnosed each year in the United States is about 5000. In view of this, few surgeons have had enough experience with these lesions to appreciate the many variations in their clinical presentation or natural history. Also, owing to this limited incidence of sarcomas, few controlled clinical trials have been carried out to determine optimal treatment programs. Accordingly, many of the observations in this book will be based on our personal clinical experience, rather than thorough scientific study, but the available data will be reviewed.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 74.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Abbas JS, Holyoke ED, Moore R, Karakousis CP: The surgical treatment and outcome of soft-tissue sarcoma. Arch Surg 116: 765–769, 1981.

    PubMed  CAS  Google Scholar 

  2. Akwari OE, Dozois RR, Weiland LH, Beahrs OH: Leiomyosarcoma of the small and large bowel. Cancer 42: 1375–1384, 1978.

    Article  PubMed  CAS  Google Scholar 

  3. Alvira MM, Mandybur TI, Menefee MG: Light microscopic and ultrastructural observations of a metastasizing malignant epithelioid schwannoma. Cancer 38: 1977–1982, 1976.

    Article  PubMed  CAS  Google Scholar 

  4. Appleman HD, Helwig EB: Gastric epithelioid leiomyoma and leiomyosarcoma (leiomyoblastoma). Cancer 38: 708–728, 1976.

    Article  PubMed  CAS  Google Scholar 

  5. Ariel IM, Briceno M: Rhabdomyosarcoma of the extremities and trunk: analysis of 150 patients treated by surgical resection. J Surg Oncol 7: 269–287, 1975.

    Article  PubMed  CAS  Google Scholar 

  6. Axelson O, Sundell I, Andersson K, Edling C, Hodstedt C, Kling H: Herbicide exposure and tumour mortality. An updated epidemiological investigation on Swedish railroad workers. Scand J Work Environ Health 6: 73–79, 1980.

    PubMed  CAS  Google Scholar 

  7. Beck A: Zur Frage des Roentgensarkoms “Zugleich ein Beitrag zur Pathogenese der Sarkoms.” Munchen Med Wschr 69: 623–624, 1922.

    Google Scholar 

  8. Binder SC, Katz B, Sheridan MFB: Retroperitoneal liposarcoma. Ann Surg 187: 257–261, 1978.

    Article  PubMed  CAS  Google Scholar 

  9. Cadman CL, Soule EH, Kelly PJ: Synovial sarcoma: an analysis of 134 tumors. Cancer 18: 613–627, 1965.

    Article  PubMed  CAS  Google Scholar 

  10. Cahan WG, Woodard HQ, Higinbotham NL, Stewart FW, Coley BL: Sarcoma arising in irradiated bone. Report of eleven cases. Cancer 1: 3–29, 1948.

    Article  PubMed  CAS  Google Scholar 

  11. Cameron HU, Kostuik JP: A long-term follow-up of synovial sarcoma. J Bone Joint Surg 56-B: 613–617, 1974.

    Google Scholar 

  12. Celik C, Karakousis CP, Moore R, Holyoke ED: Liposarcomas: prognosis and management. J Surg Oncol 14: 245–249, 1980.

    Article  PubMed  CAS  Google Scholar 

  13. Chen KTK, Kirkegaard DD, Bocian JJ: Angiosarcoma of the breast. Cancer 46: 368–371, 1980.

    Article  PubMed  CAS  Google Scholar 

  14. Chiotasso PJP, Fazio VW: Prognostic factors of 28 leiomyosarcomas of the small intestine. Surg Gynecol Obstet 155: 197–202, 1982.

    PubMed  CAS  Google Scholar 

  15. Cody HS, Turnbull AD, Fortner JG, Hajdu SI: The continuing challenge of retroperitoneal sarcomas. Cancer 47: 2147–2152, 1981.

    Article  PubMed  Google Scholar 

  16. Coggon D, Acheson ED: Do phenoxy herbicides cause cancer in man? Lancet 1: 1057–1059, 1982.

    Article  PubMed  CAS  Google Scholar 

  17. Coia LR, Fazekas JT, Kramer S: Postirradiation sarcoma of the head and neck: a report of three late sarcomas following therapeutic irradiation for primary malignancies of the paranasal sinus, nasal cavity, and larynx. Cancer 46: 1982–1985, 1980.

    Article  PubMed  CAS  Google Scholar 

  18. Cook RR: Dioxin, chloracne, and soft tissue sarcoma. Lancet 1: 618–619, 1981.

    Article  PubMed  CAS  Google Scholar 

  19. Deck KB, Silberman H: Leiomyosarcomas of the small intestine. Cancer 44: 323–325, 1979.

    Article  PubMed  CAS  Google Scholar 

  20. DiSant’agnese PA, Knowles DM: Extracardiac rhabdomyoma: a clinicopathologic study and review of the literature. Cancer 46: 780–789, 1980.

    Article  Google Scholar 

  21. Editorial: Phenoxy herbicides, trichlorophenols, and soft-tissue sarcomas. Lancet 1: 1051–1052, 1982.

    Google Scholar 

  22. Ekfors TO, Kalino H, Rantakokko V, Latvala M, Parvinen M: Alveolar soft part sarcoma: a report of two cases with some histochemical and ultrastructural observations. Cancer 43: 1672–1677, 1979.

    Article  PubMed  CAS  Google Scholar 

  23. Enzinger FM, Winslow DJ: Liposarcoma: a study of 103 cases. Virchows Arch Pathol Anat 335: 367–388, 1962.

    Article  CAS  Google Scholar 

  24. Eriksson M, Hardell L, Berg NO, Möller T, Axelson O: Soft tissue sarcomas and exposure to chemical substances: a case-referent study. Br J Indust Med 38: 27–33, 1981.

    CAS  Google Scholar 

  25. Erlandson RA, Woodruff JM: Peripheral nerve sheath tumors: an electron microscopic study of 43 cases. Cancer 49: 273–287, 1982.

    Article  PubMed  CAS  Google Scholar 

  26. Falk H, Popper H, Thomas LB, Ishak KG: Hepatic angiosarcoma associated with androgenic-anabolic steroids. Lancet 2: 1120–1123, 1979.

    Article  PubMed  CAS  Google Scholar 

  27. Ferrell HW, Frable WJ: Soft part sarcomas revisited: review and comparison of a second series. Cancer 30: 475–480, 1972.

    Article  PubMed  CAS  Google Scholar 

  28. Fields JP, Helwig EB: Leiomyosarcoma of the skin and subcutaneous tissue. Cancer 47: 156–169, 1981.

    Article  PubMed  CAS  Google Scholar 

  29. Gare NF, Lindup R, Strickland P, Bennett MH: Radiation induced fibrosarcoma. Br J Cancer 24: 705–711, 1970.

    Article  Google Scholar 

  30. Gerner GE, Moore GE: Synovial sarcoma. Ann Surg 181: 22–25, 1975.

    Article  PubMed  CAS  Google Scholar 

  31. Gerner RE, Moore GE, Pickren JW: Hemangiopericytoma. Ann Surg 179: 128–132, 1974.

    Article  PubMed  CAS  Google Scholar 

  32. Gerner RE, Moore GE, Pickren JW: Soft tissue sarcomas. Ann Surg 181: 803–808, 1975.

    Article  PubMed  CAS  Google Scholar 

  33. Ghosh BC, Ghosh L, Huvos AG, Fortner JG: Malignant schwannoma: a clinicopathologic study. Cancer 31: 184–190, 1973.

    Article  PubMed  CAS  Google Scholar 

  34. Hajdu SI: History and classification of soft tissue tumors. In: Pathology of Soft Tissue Tumors. Philadelphia, Lea & Febiger, 1979, p 44.

    Google Scholar 

  35. Hajdu SI, Lemos LB, Kozakewich H, Helson L, Beattie EJ: Growth pattern and differentiation of human soft tissue sarcomas in nude mice. Cancer 47: 90–98, 1981.

    Article  PubMed  CAS  Google Scholar 

  36. Hajdu SI, Shiu MH, Fortner JG: Tendosynovial sarcoma: a clinicopathological study of 136 cases. Cancer 39: 1201–1217, 1977.

    Article  PubMed  CAS  Google Scholar 

  37. Hardell L: Soft tissue sarcomas and exposure to phenoxyacetic acids—a clinical observation. Läkartidningen 74: 2753–2754, 1977.

    PubMed  CAS  Google Scholar 

  38. Hardell L, Sandström A: Case-control study: soft-tissue sarcomas and exposure to phenoxyacetic acids or chlorophenols. Br J Cancer 39: 711–717, 1979.

    Article  PubMed  CAS  Google Scholar 

  39. Hardy TJ, An T, Brown PW, Terz JJ: Postirradiation sarcoma (malignant fibrous histiocytoma) of axilla. Cancer 42: 118–124, 1978.

    Article  PubMed  CAS  Google Scholar 

  40. Hare FF, Cerny MJ: Soft tissue sarcoma: a review of 200 cases. Cancer 16: 1332–1337, 1963.

    Article  PubMed  CAS  Google Scholar 

  41. Hatfield PM, Schulz MD: Postirradiation sarcoma, including 5 cases after x- ray therapy of breast carcinoma. Radiology 96: 593–602, 1970.

    PubMed  CAS  Google Scholar 

  42. Honchar PA, Halperin WE: 2,4,5-T,trichlorophenol and soft tissue sarcomas. Lancet 1: 268–269, 1981.

    Article  PubMed  CAS  Google Scholar 

  43. Iwasaki H, Hikuchi M, Takii M, Enjoji M: Benign and malignant fibrous histiocytomas of the soft tissues: functional characterization of the cultured cells. Cancer 50: 520–530, 1982.

    Article  PubMed  CAS  Google Scholar 

  44. Johnson FE, Kugler MA, Brown SM: Soft tissue sarcomas and chlorinated phenols. Lancet 2: 40, 1981.

    Article  PubMed  CAS  Google Scholar 

  45. Kearny MM, Soule EH, Ivins JC: Malignant fibrous histiocytoma: a retrospective study of 167 cases. Cancer 45: 167–178, 1980.

    Article  Google Scholar 

  46. Keyhani A, Booher RJ: Pleomorphic rhabdomyosarcoma. Cancer 5: 956–967, 1968.

    Article  Google Scholar 

  47. Kinne DW, Chu FCH, Huvos AG, Yagoda A, Fortner JG: Treatment of primary and recurrent retroperitoneal liposarcoma: twenty-five-year experience at Memorial Hospital. Cancer 31: 53–64, 1973.

    Article  PubMed  CAS  Google Scholar 

  48. Kyriakos M, Kempson RL: Inflammatory fibrous histiocytoma: an aggressive and lethal lesion. Cancer 37: 1584–1606, 1976.

    Article  PubMed  CAS  Google Scholar 

  49. Leborgne F, Falconi LM: Lymphangiosarcoma of the anterior abdominal wall: a case report. Cancer 46: 1228–1230, 1980.

    Article  PubMed  CAS  Google Scholar 

  50. Lehti PM, Moseley HS, Peetz ME, Fletcher WS: Liposarcoma of the leg. Am J Surg 144: 44–47, 1982.

    Article  PubMed  CAS  Google Scholar 

  51. Lieberman PH, Foote FW, Stewart FW, Berg JW: Alveolar soft-part sarcoma. JAMA 198: 1047–1051, 1966.

    Article  PubMed  CAS  Google Scholar 

  52. Lindsay PC, Ordonez N, Raaf JH: Gastric leiomyosarcoma: clinical and pathological review of fifty patients. J Surg Oncol 18: 399–421, 1981.

    Article  PubMed  CAS  Google Scholar 

  53. Linscheid RL, Soule EH, Henderson ED: Pleomorphic rhabdomyosarcomata of the extremities and limb girdles: a clinicopathological study. J Bone Joint Surg 47A: 715–726, 1965.

    PubMed  CAS  Google Scholar 

  54. Maddox JC, Evans HL: Angiosarcoma of skin and soft tissue: a study of forty- four cases. Cancer 48: 1907–1921, 1981.

    Article  PubMed  CAS  Google Scholar 

  55. Markhede G, Angervall L, Stoner B: A multivariate analysis of the prognosis after surgical treatment of malignant soft-tissue tumors. Cancer 49: 1721–1733, 1982.

    Article  PubMed  CAS  Google Scholar 

  56. Mathew T: Evidence supporting neural crest origin of an alveolar soft part sarcoma: an ultrastructural study. Cancer 50: 507–514, 1982.

    Article  PubMed  CAS  Google Scholar 

  57. McMaster MJ, Soule EH, Ivins JC: Hemangiopericytoma: a clinicopathologic study and long-term follow-up of 60 patients. Cancer 36: 2232–2244, 1975.

    Article  PubMed  CAS  Google Scholar 

  58. McSwain B, Whitehead W, Bennett L: Angiosarcoma: report of three cases of postmastectomy lymphangiosarcoma and one of hemangiosarcoma. South Med J 66: 102–106, 1973.

    Article  PubMed  CAS  Google Scholar 

  59. Mindell ER, Shah NK, Webster JH: Postradiation sarcoma of bone and soft tissues. Orthop Clin North Am 8: 821–834, 1977.

    PubMed  CAS  Google Scholar 

  60. Moses M, Selikoff IJ: Soft tissue sarcomas, phenoxy herbicides, and chlorinated phenols. Lancet 1: 1370, 1981.

    Article  PubMed  CAS  Google Scholar 

  61. Nelson WR, Morfit HM: Lymphangiosarcoma in the lymphedematous arm after radical mastectomy. Cancer 9: 1189–1194, 1956.

    Article  PubMed  Google Scholar 

  62. Ott MJ, Holder BB, Olson RD: A mortality analysis of employees engaged in the manufacture of 2,4,5-trichlorophenoxy acetic acid. J Occup Med 22: 47–50, 1980.

    Article  PubMed  CAS  Google Scholar 

  63. Pack GT, Pierson JC: Liposarcoma. Surgery 36: 687–712, 1954.

    PubMed  CAS  Google Scholar 

  64. Pack GT, Tabah EJ: Dermatofibrosarcoma protuberans: a report of thirty-nine cases. Arch Surg 62: 391–411, 1951.

    CAS  Google Scholar 

  65. Patchefsky AS, Soriano R, Kostianovsky M: Epithelioid sarcoma: ultrastructural similarity to nodular synovitis. Cancer 39: 143–152, 1977.

    Article  PubMed  CAS  Google Scholar 

  66. Phillips TL, Sheline GE: Bone sarcomas following radiation therapy. Radiology 81: 992–996, 1963.

    PubMed  CAS  Google Scholar 

  67. Pinkston JA, Sekine I: Postirradiation sarcoma (malignant fibrous histiocytoma) following cervix cancer. Cancer 49: 434–438, 1982.

    Article  PubMed  CAS  Google Scholar 

  68. Prat J, Woodruff JM, Marcove RC: Epithelioid sarcoma: an analysis of 22 cases indicating the prognostic significance of vascular invasion and regional lymph node metastasis. Cancer 41: 1472–1487, 1978.

    Article  PubMed  CAS  Google Scholar 

  69. Prioleau PG, Santa Cruz DJ: Lymphangioma circumscriptum following radical mastectomy and radiation therapy. Cancer 42: 1989–1991, 1978.

    Article  PubMed  CAS  Google Scholar 

  70. Pritchard DJ, Soule EH, Taylor WF, Ivins JC: Fibrosarcoma—a clinicopathologic and statistical study of 199 tumors of the soft tissues of the extremities and trunk. Cancer 33: 888–897, 1974.

    Article  PubMed  CAS  Google Scholar 

  71. Roth F: Arsen-Leber-Tumoren (Hámangioendotheliom). Z Krebsforsch 61: 468–503, 1957.

    Article  PubMed  CAS  Google Scholar 

  72. Schrieber H, Barry FM, Russell WC, Macon WL, Ponsky JL, Pories WJ: Stewart- Treves syndrome: a lethal complication of postmastectomy lymphedema and regional immune deficiency. Arch Surg 114: 82–85, 1979.

    Google Scholar 

  73. Sears HF, Hopson R, Inouye W, Rizzo T, Grotzinger PJ: Analysis of staging and management of patients with sarcoma: a ten-year experience. Ann Surg 191: 488–493, 1980.

    Article  PubMed  CAS  Google Scholar 

  74. Shieber W, Graham P: An experience with sarcomas of the soft tissues in adults. Surgery 52: 295–298, 1962.

    PubMed  CAS  Google Scholar 

  75. Shiu MH, Castro EB, Hajdu SI, Fortner JG: Surgical treatment of 297 soft tissue sarcomas of the lower extremity. Ann Surg 182: 597–602, 1975.

    Article  PubMed  CAS  Google Scholar 

  76. Shiu MH, Farr GH, Papachristou DN, Hajdu SI: Myosarcomas of the stomach: natural history, prognostic factors and management. Cancer 49: 177–187, 1982.

    Article  PubMed  CAS  Google Scholar 

  77. Shiu MH, McCormack PM, Hajdu, SI, Fortner JG: Surgical treatment of tendosynovial sarcoma. Cancer 43: 889–897, 1979.

    Article  PubMed  CAS  Google Scholar 

  78. Snover DC, Sumner HW, Dehner LP: Variability of histologic pattern in recurrent soft tissue sarcomas originally diagnosed as liposarcoma. Cancer 49: 1005–1015, 1982.

    Article  PubMed  CAS  Google Scholar 

  79. Sordillo PP, Chapman R, Hajdu SI, Magill GB, Golbey RB: Lymphangiosarcoma. Cancer 48: 1674–1679, 1981.

    Article  PubMed  CAS  Google Scholar 

  80. Sordillo PP, Helson L, Hajdu SI, Magill GB, Kosloff C, Golbey RB, Beattie EJ: Malignant schwannoma—clinical characteristics, survival, and response to therapy. Cancer 47: 2503–2509, 1981.

    Article  PubMed  CAS  Google Scholar 

  81. Soule EH, Geitz M, Henderson ED: Embryonal rhabdomyosarcoma of the limbs and limb girdles: a clinicopathologic study of 61 cases. Cancer 23: 1336–1346, 1969.

    Article  PubMed  CAS  Google Scholar 

  82. Stewart FW, Treves N: Lymphangiosarcoma in postmastectomy lymphedema: a report of six cases in elephantiasis chirurgica. Cancer 1: 64–81, 1948.

    Article  PubMed  CAS  Google Scholar 

  83. Storm FK, Eilber FR, Mirra J, Morton DL: Neurofibrosarcoma. Cancer 45: 126–129, 1980.

    Article  PubMed  CAS  Google Scholar 

  84. Stringel G, Shandling B, Mancer K, Ein EH: Lipoblastoma in infants and children. J Pediatr Surg 17: 277–280, 1982.

    Article  PubMed  CAS  Google Scholar 

  85. Taxy JB, Battifora H: Malignant fibrous histiocytoma: an electron microscopic study. Cancer 40: 254–267, 1977.

    Article  PubMed  CAS  Google Scholar 

  86. Thomas LB, Popper H, Berk PD, Selikoff I, Falk H: Vinyl-chloride-induced liver disease: from idiopathic portal hypertension (Banti’s syndrome) to angiosarcomas. N Engl J Med 292: 17–22, 1975.

    Article  PubMed  CAS  Google Scholar 

  87. Travis EL, Kreuther A, Young T, Gerald WL: Unusual postirradiation sarcoma of chest wall. Cancer 38: 2269–2273, 1976.

    Article  PubMed  CAS  Google Scholar 

  88. Trojanowski JQ, Kleinman GM, Proppe KH: Malignant tumors of nerve sheath origin. Cancer 46: 1202–1212, 1980.

    Article  PubMed  CAS  Google Scholar 

  89. Underwood JCE, Huck P: Thorotrast associated hepatic angiosarcoma with 36 years latency. Cancer 42: 2610–2612, 1978.

    Article  PubMed  CAS  Google Scholar 

  90. van Miller JP, Lalich JJ, Allen JR: Increased incidence of neoplasms in rats exposed to low levels of 2,3,7,8-tetrachlorodibenzo-p-dioxin. Chemosphere 6: 537–544, 1977.

    Article  Google Scholar 

  91. Visfeldt J, Poulson H: On the histopathology of liver and liver tumours in thorium- dioxide patients. Acta Pathol Microbiol Scand (A) 80: 97–108, 1972.

    CAS  Google Scholar 

  92. Weingrad DN, Rosenberg SA: Early lymphatic spread of osteogenic and soft- tissue sarcomas. Surgery 84: 231–240, 1978.

    PubMed  CAS  Google Scholar 

  93. Weiss SW, Enzinger FM: Malignant fibrous histiocytoma: an analysis of 200 cases. Cancer 41: 2250–2266, 1978.

    Article  PubMed  CAS  Google Scholar 

  94. Wile AG, Evans HL, Romsdahl MM: Leiomyosarcoma of soft tissue: a clinicopathologic study. Cancer 48: 1022–1032, 1981.

    Article  PubMed  CAS  Google Scholar 

  95. Yap B-S, Yap H-Y, McBride CM, Bodey GP: Chemotherapy for postmastectomy lymphangiosarcoma. Cancer 47: 853–856, 1981.

    Article  PubMed  CAS  Google Scholar 

  96. Zack J A, Suskind RS: The mortality experience of workers exposed to tetrachlorodibenzodioxin in a trichlorophenol process accident. J Occup Med 22: 11–14, 1980.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

Copyright information

© 1983 Springer-Verlag New York Inc.

About this chapter

Cite this chapter

Lawrence, W., Neifeld, J.P., Terz, J.J. (1983). Soft-Tissue Sarcomas: Etiology, Pathology, and Clinical Features. In: Manual of Soft-Tissue Tumor Surgery. Comprehensive Manuals of Surgical Specialties. Springer, New York, NY. https://doi.org/10.1007/978-1-4612-5556-7_1

Download citation

  • DOI: https://doi.org/10.1007/978-1-4612-5556-7_1

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4612-5558-1

  • Online ISBN: 978-1-4612-5556-7

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics