Seminars in Oncology
Volume 36, Issue 4 , Pages 347-357 , August 2009

Epithelioid Soft Tissue Tumors

  • A. Paolo Dei Tos

      Affiliations

    • Department of Anatomic Pathology, General Hospital of Treviso, Treviso, Italy
    • These authors contributed equally to this work.
    • Corresponding Author InformationAddress correspondence to Angelo Paolo Dei Tos, MD, Director of Anatomic Pathology, General Hospital of Treviso, Piazza Ospedale 1, 31100 Treviso, Italy
    • ,
  • Andrew J. Wagner

      Affiliations

    • Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Boston, MA
    • These authors contributed equally to this work.
    • ,
  • Piergiorgio Modena

      Affiliations

    • Unit of Molecular Cytogenetics–UO5, Fondazione IRCCS, Istituto Nazionale dei Tumori, Milano, Italy
    • These authors contributed equally to this work.
    • ,
  • Alessandro Comandone

      Affiliations

    • SC Oncologia Ospedale Gradenigo, Torino, Italy
    • These authors contributed equally to this work.
    • ,
  • Serge Leyvraz

      Affiliations

    • Centre Pluridisciplinaire d'Oncologie, University Hospital, Lausanne, Switzerland
    • These authors contributed equally to this work.

References 

  1. Enzinger FM. Epitheloid sarcoma (A sarcoma simulating a granuloma or a carcinoma). Cancer. 1970;26:1029–1041
  2. Kodet R, Smelhaus V, Newton WA, et al. Epithelioid sarcoma in childhood: an immunohistochemical, electron microscopic, and clinicopathologic study of 11 cases under 15 years of age and review of the literature. Pediatr Pathol. 1994;14:433–451
  3. Chase DR, Enzinger FM. Epithelioid sarcoma (Diagnosis, prognostic indicators, and treatment). Am J Surg Pathol. 1985;9:241–263
  4. Bos GD, Pritchard DJ, Reiman HM, et al. Epithelioid sarcoma (An analysis of fifty-one cases). J Bone Joint Surg Am. 1988;70:862–870
  5. Chase DR, Enzinger FM, Weiss SW, et al. Keratin in epithelioid sarcoma (An immunohistochemical study). Am J Surg Pathol. 1984;8:435–441
  6. Evans HL, Baer SC. Epithelioid sarcoma: a clinicopathologic and prognostic study of 26 cases. Semin Diagn Pathol. 1993;10:286–291
  7. de Visscher SA, van Ginkel RJ, Wobbes T, et al. Epithelioid sarcoma: still an only surgically curable disease. Cancer. 2006;107:606–612
  8. Miettinen M, Fanburg-Smith JC, Virolainen M, et al. Epithelioid sarcoma: an immunohistochemical analysis of 112 classical and variant cases and a discussion of the differential diagnosis. Hum Pathol. 1999;30:934–942
  9. Arber DA, Kandalaft PL, Mehta P, et al. Vimentin-negative epithelioid sarcoma (The value of an immunohistochemical panel that includes CD34). Am J Surg Pathol. 1993;17:302–307
  10. Wick MR, Manivel JC. Epithelioid sarcoma and isolated necrobiotic granuloma: a comparative immunocytochemical study. J Cutan Pathol. 1986;13:253–260
  11. Mirra JM, Kessler S, Bhuta S, et al. The fibroma-like variant of epithelioid sarcoma (A fibrohistiocytic/myoid cell lesion often confused with benign and malignant spindle cell tumors). Cancer. 1992;69:1382–1395
  12. Guillou L, Wadden C, Coindre JM, et al. “Proximal-type” epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features (Clinicopathologic, immunohistochemical, and ultrastructural study of a series). Am J Surg Pathol. 1997;21:130–146
  13. Bittesini L, Dei Tos AP, Fletcher CD. Metastatic malignant melanoma showing a rhabdoid phenotype: further evidence of a non-specific histological pattern. Histopathology. 1992;20:167–170
  14. Parham DM, Weeks DA, Beckwith JB. The clinicopathologic spectrum of putative extrarenal rhabdoid tumors (An analysis of 42 cases studied with immunohistochemistry or electron microscopy). Am J Surg Pathol. 1994;18:1010–1029
  15. Wick MR, Ritter JH, Dehner LP. Malignant rhabdoid tumors: a clinicopathologic review and conceptual discussion. Semin Diagn Pathol. 1995;12:233–248
  16. Chase DR. Do “rhabdoid features” impart a poorer prognosis to proximal-type epithelioid sarcomas?. Adv Anat Pathol. 1997;4:293–299
  17. Hoot AC, Russo P, Judkins AR, et al. Immunohistochemical analysis of hSNF5/INI1 distinguishes renal and extra-renal malignant rhabdoid tumors from other pediatric soft tissue tumors. Am J Surg Pathol. 2004;28:1485–1491
  18. Modena P, Lualdi E, Facchinetti F, et al. SMARCB1/INI1 tumor suppressor gene is frequently inactivated in epithelioid sarcomas. Cancer Res. 2005;65:4012–4019
  19. Kohashi K, Izumi T, Oda Y, et al. Infrequent SMARCB1/INI1 gene alteration in epithelioid sarcoma: a useful tool in distinguishing epithelioid sarcoma from malignant rhabdoid tumor. Hum Pathol. 2009;40:349–355
  20. Chbani L, Guillou L, Terrier P, et al. Epithelioid sarcoma: a clinicopathologic and immunohistochemical analysis of 106 cases from the French sarcoma group. Am J Clin Pathol. 2009;131:222–227
  21. Baratti D, Pennacchioli E, Casali PG, et al. Epithelioid sarcoma: prognostic factors and survival in a series of patients treated at a single institution. Ann Surg Oncol. 2007;14:3542–3551
  22. Callister MD, Ballo MT, Pisters PW, et al. Epithelioid sarcoma: results of conservative surgery and radiotherapy. Int J Radiat Oncol Biol Phys. 2001;51:384–391
  23. Jawad MU, Extein J, Min ES, Scully SP. Progostic factors for survival in patients with epithelioid sarcoma. Clin Orthop Relat Res. 2009;Epub ahead of print DOI 10.1007/s11999-009-0749-2
  24. Wolf PS, Flum DR, Tanas MR, et al. Epithelioid sarcoma: the University of Washington experience. Am J Surg. 2008;196:407–412
  25. Christopherson WM, Foote FW, Stewart FW. Alveolar soft-part sarcomas; structurally characteristic tumors of uncertain histogenesis. Cancer. 1952;5:100–111
  26. Lieberman PH, Brennan MF, Kimmel M, et al. Alveolar soft-part sarcoma (A clinico-pathologic study of half a century). Cancer. 1989;63:1–13
  27. Portera CA, Ho V, Patel SR, et al. Alveolar soft part sarcoma: clinical course and patterns of metastasis in 70 patients treated at a single institution. Cancer. 2001;91:585–591
  28. Ladanyi M, Lui MY, Antonescu CR, et al. The der(17)t(X;17)(p11;q25) of human alveolar soft part sarcoma fuses the TFE3 transcription factor gene to ASPL, a novel gene at 17q25. Oncogene. 2001;20:48–57
  29. Argani P, Antonescu CR, Illei PB, et al. Primary renal neoplasms with the ASPL-TFE3 gene fusion of alveolar soft part sarcoma: a distinctive tumor entity previously included among renal cell carcinomas of children and adolescents. Am J Pathol. 2001;159:179–192
  30. Argani P, Lal P, Hutchinson B, et al. Aberrant nuclear immunoreactivity for TFE3 in neoplasms with TFE3 gene fusions: a sensitive and specific immunohistochemical assay. Am J Surg Pathol. 2003;27:750–761
  31. Aulmann S, Longerich T, Schirmacher P, et al. Detection of the ASPSCR1-TFE3 gene fusion in paraffin-embedded alveolar soft part sarcomas. Histopathology. 2007;50:881–886
  32. Tsuda M, Davis IJ, Argani P, et al. TFE3 fusions activate MET signaling by transcriptional up-regulation, defining another class of tumors as candidates for therapeutic MET inhibition. Cancer Res. 2007;67:919–929
  33. Reichardt P, Lindner T, Pink D, et al. Chemotherapy in alveolar soft part sarcomas (What do we know?). Eur J Cancer. 2003;39:1511–1516
  34. Ogose A, Yazawa Y, Ueda T, et al. Alveolar soft part sarcoma in Japan: multi-institutional study of 57 patients from the Japanese Musculoskeletal Oncology Group. Oncology. 2003;65:7–13
  35. Gardner K, Leahy M, Alvarez-Gutierrez M, et al. Activity of the VEGFR/KIT tyrosine kinase inhibitor cediranib (AZD2171) in alveolar soft part sarcoma. [abstract] Proc Connective Tissue Oncol Soc. 2008;14:34936
  36. Antonescu CR, Rosenblum MK, Pereira P, et al. Sclerosing epithelioid fibrosarcoma: a study of 16 cases and confirmation of a clinicopathologically distinct tumor. Am J Surg Pathol. 2001;25:699–709
  37. Meis-Kindblom JM, Kindblom LG, Enzinger FM. Sclerosing epithelioid fibrosarcoma (A variant of fibrosarcoma simulating carcinoma). Am J Surg Pathol. 1995;19:979–993
  38. Guillou L, Benhattar J, Gengler C, et al. Translocation-positive low-grade fibromyxoid sarcoma: clinicopathologic and molecular analysis of a series expanding the morphologic spectrum and suggesting potential relationship to sclerosing epithelioid fibrosarcoma: a study from the French Sarcoma Group. Am J Surg Pathol. 2007;31:1387–1402
  39. Ossendorf C, Studer GM, Bode B, et al. Sclerosing epithelioid fibrosarcoma: case presentation and a systematic review. Clin Orthop Relat Res. 2008;466:1485–1491
  40. Rosai J. Angiolymphoid hyperplasia with eosinophilia of the skin (Its nosological position in the spectrum of histiocytoid hemangioma). Am J Dermatopathol. 1982;4:175–184
  41. Rosai J, Gold J, Landy R. The histiocytoid hemangiomas (A unifying concept embracing several previously described entities of skin, soft tissue, large vessels, bone, and heart). Hum Pathol. 1979;10:707–730
  42. Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer. 1982;50:970–981
  43. Fletcher CD, Beham A, Bekir S, et al. Epithelioid angiosarcoma of deep soft tissue: a distinctive tumor readily mistaken for an epithelial neoplasm. Am J Surg Pathol. 1991;15:915–924
  44. Mentzel T, Beham A, Calonje E, et al. Epithelioid hemangioendothelioma of skin and soft tissues: clinicopathologic and immunohistochemical study of 30 cases. Am J Surg Pathol. 1997;21:363–374
  45. Weitz J, Klimstra DS, Cymes K, et al. Management of primary liver sarcomas. Cancer. 2007;109:1391–1396
  46. Rossi S, Orvieto E, Furlanetto A, et al. Utility of the immunohistochemical detection of FLI-1 expression in round cell and vascular neoplasm using a monoclonal antibody. Mod Pathol. 2004;17:547–552
  47. Billings SD, Folpe AL, Weiss SW. Epithelioid sarcoma-like hemangioendothelioma. Am J Surg Pathol. 2003;27:48–57
  48. Mosoia L, Mabrut JY, Adham M, et al. Hepatic epithelioid hemangioendothelioma: long-term results of surgical management. J Surg Oncol. 2008;98:432–437
  49. Nudo CG, Yoshida EM, Bain VG, et al. Liver transplantation for hepatic epithelioid hemangioendothelioma: the Canadian multicentre experience. Can J Gastroenterol. 2008;22:821–824
  50. Bagan P, Hassan M, Le Pimpec Barthes F, et al. Prognostic factors and surgical indications of pulmonary epithelioid hemangioendothelioma: a review of the literature. Ann Thorac Surg. 2006;82:2010–2013
  51. Meis-Kindblom JM, Kindblom LG. Angiosarcoma of soft tissue: a study of 80 cases. Am J Surg Pathol. 1998;22:683–697
  52. Eusebi V, Carcangiu ML, Dina R, et al. Keratin-positive epithelioid angiosarcoma of thyroid (A report of four cases). Am J Surg Pathol. 1990;14:737–747
  53. Rosai J, Sumner HW, Kostianovsky M, et al. Angiosarcoma of the skin (A clinicopathologic and fine structural study). Hum Pathol. 1976;7:83–109
  54. Nagano T, Yamada Y, Ikeda T, et al. Docetaxel: a therapeutic option in the treatment of cutaneous angiosarcoma: report of 9 patients. Cancer. 2007;110:648–651
  55. Fata F, O'Reilly E, Ilson D, et al. Paclitaxel in the treatment of patients with angiosarcoma of the scalp or face. Cancer. 1999;86:2034–2037
  56. Fury MG, Antonescu CR, Van Zee KJ, et al. A 14-year retrospective review of angiosarcoma: clinical characteristics, prognostic factors, and treatment outcomes with surgery and chemotherapy. Cancer J. 2005;11:241–247
  57. Penel N, Bui BN, Bay JO, et al. Phase II trial of weekly paclitaxel for unresectable angiosarcoma: the ANGIOTAX study. J Clin Oncol. 2008;26:5269–5274
  58. Pasquier E, Carre M, Pourroy B, et al. Antiangiogenic activity of paclitaxel is associated with its cytostatic effect, mediated by the initiation but not completion of a mitochondrial apoptotic signaling pathway. Mol Cancer Ther. 2004;3:1301–1310
  59. Laskin WB, Weiss SW, Bratthauer GL. Epithelioid variant of malignant peripheral nerve sheath tumor (malignant epithelioid schwannoma). Am J Surg Pathol. 1991;15:1136–1145
  60. McMenamin ME, Fletcher CD. Expanding the spectrum of malignant change in schwannomas: epithelioid malignant change, epithelioid malignant peripheral nerve sheath tumor, and epithelioid angiosarcoma: a study of 17 cases. Am J Surg Pathol. 2001;25:13–25
  61. Prayson RA, Goldblum JR, Hart WR. Epithelioid smooth-muscle tumors of the uterus: a clinicopathologic study of 18 patients. Am J Surg Pathol. 1999;21:383–391
  62. Miettinen M, Enzinger FM. Epithelioid variant of pleomorphic liposarcoma: a study of 12 cases of a distinctive variant of high-grade liposarcoma. Mod Pathol. 1999;12:722–728
  63. Maki RG, Wathen JK, Patel SR, et al. Randomized phase II study of gemcitabine and docetaxel compared with gemcitabine alone in patients with metastatic soft tissue sarcomas: results of sarcoma alliance for research through collaboration study 002. J Clin Oncol. 2007;25:2755–2763
  64. Segal NH, Pavlidis P, Antonescu CR, et al. Classification and subtype prediction of adult soft tissue sarcoma by functional genomics. Am J Pathol. 2003;163:691–700
  65. Palmer NF, Sutow W. Clinical aspects of the rhabdoid tumor of the kidney: a report of the National Wilms' Tumor Study Group. Med Pediatr Oncol. 1983;11:242–245
  66. Bonnin JM, Rubinstein LJ, Palmer NF, et al. The association of embryonal tumors originating in the kidney and in the brain (A report of seven cases). Cancer. 1984;54:2137–2146
  67. Rousseau-Merck MF, Nogues C, Roth A, et al. Hypercalcemic infantile renal tumors: morphological, clinical, and biological heterogeneity. Pediatr Pathol. 1985;3:155–164
  68. Rousseau-Merck MF, Versteege I, Legrand I, et al. hSNF5/INI1 inactivation is mainly associated with homozygous deletions and mitotic recombinations in rhabdoid tumors. Cancer Res. 1999;59:3152–3156
  69. Versteege I, Sevenet N, Lange J, et al. Truncating mutations of hSNF5/INI1 in aggressive paediatric cancer. Nature. 1998;394:203–206
  70. Hornick JL, Dal Cin P, Fletcher CD. Loss of INI1 expression is characteristic of both conventional and proximal-type epithelioid sarcoma. Am J Surg Pathol. 2009;33:542–550

PII: S0093-7754(09)00105-5

doi: 10.1053/j.seminoncol.2009.06.005

Seminars in Oncology
Volume 36, Issue 4 , Pages 347-357 , August 2009

Article Tools

* Image Usage & Resolution