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A primary leiomyosarcoma of bone containing pseudoepithelial plexiform elements

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Abstract

A rare but distinctive variant of smooth muscle tumours that occurs almost exclusively in the uterus is characterised by the presence of plexiform tumourlets, which are composed of clumps and cords of tumour cells that form a discrete pseudoepithelial component. We report on a case of a primary leiomyosarcoma of the proximal humerus, which, in addition to characteristic histological and immunophenotypic features of leiomyosarcoma, contained plexiform tumourlets. Tumour cells in the plexiform component focally expressed muscle/smooth muscle actin, calponin and cytokeratin. Spindle-shaped and epithelioid smooth muscle tumour cells also expressed the above antigens. This is the first report of a plexiform smooth muscle tumour arising in bone. This case is remarkable, not only for being only the second reported case of a malignant plexiform smooth muscle tumour, but also for being one of very few examples of this type of tumour arising outside the uterus; it also is unique in having arisen in a male patient. This variant of primary leiomyosarcoma needs to be distinguished from other bone tumours containing epithelial elements, notably metastatic carcinoma.

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References

  1. Angervall L, Berlin O, Kindblom LG, Stener B. Primary leiomyosarcoma of bone: a study of five cases. Cancer 1980; 46: 1270–1279.

    Article  PubMed  CAS  Google Scholar 

  2. Khoddami M, Bedard YC, Bell RS, Kandel RA. Primary leiomyosarcoma of bone: report of seven cases and review of the literature. Arch Pathol Lab Med 1996; 76: 904–946.

    Google Scholar 

  3. Antonescu CR, Erlandson RA, Huvos AG. Primary leiomyosarcoma of bone: a clinicopathologic, immunohistochemical, and ultrastructural study of 33 patients and a literature review. Am J Surg Pathol 1997; 21: 1281–1294.

    Article  PubMed  CAS  Google Scholar 

  4. Myers JL, Arocho J, Bernreuter W, Dunham W, Mazur MT. Leiomyosarcoma of bone. A clinico-pathologic, immunohistochemical, and ultrastructural study of five cases. Cancer 1997; 67: 1051–1056.

    Article  Google Scholar 

  5. Brown DC, Theaker JM, Banks PM, Gatter KC, Mason DY. Cytokeratin expression in smooth muscle and smooth muscle tumours. Histopathology 1987; 11: 477–486.

    Article  PubMed  CAS  Google Scholar 

  6. Iwata J, Fletcher CD. Immunohistochemical detection of cytokeratin and epithelial membrane antigen in leiomyosarcoma: a systematic study of 100 cases. Pathol Int 2000; 50: 7–14.

    Article  PubMed  CAS  Google Scholar 

  7. Young MP, Freemont AJ. Primary leiomyosarcoma of bone. Histopathology 1991; 19: 257–262.

    Article  PubMed  CAS  Google Scholar 

  8. Lopez-Barea F, Rodriguez-Peralto JL, Sanchez-Herrera S, Gonzalez-Lopez J, Burgos-Lizaldez E. Primary epithelioid leiomyosarcoma of bone: case report and literature review. Virchows Arch 1999; 434: 367–371.

    Article  PubMed  CAS  Google Scholar 

  9. Bader LV. Multicentric plexiform tumorlet of the uterus: Case report. Pathology 1971; 3: 167–170.

    PubMed  CAS  Google Scholar 

  10. Balaton AJ, Vuong PN, Vaury P, Baviera EE. Plexiform tumorlet of the uterus: immunohistochemical evidence for a smooth muscle origin. Histopathology 1986; 10: 749–754.

    Article  PubMed  CAS  Google Scholar 

  11. Cera PJ. Plexiform tumorlet of the uterus: report of two cases. Am J Clin Pathol 1973; 59: 263–266.

    PubMed  Google Scholar 

  12. Devaney K, Tavassoli FA. Immunohistochemistry as a diagnostic aid in the interpretation of unusual mesenchymal tumors of the uterus. Mod Pathol 1991; 4: 225–231.

    PubMed  CAS  Google Scholar 

  13. Goodhue WW, Susin M, Kramer EE. Smooth muscle origin of uterine plexiform tumors. Arch Pathol 1980; 97: 263–268.

    Google Scholar 

  14. Evans HL, Chawla SP, Simpson C, Finn KP. Smooth muscle neoplasms of the uterus other than ordinary leiomyoma. A study of 46 cases, with emphasis on diagnostic criteria and prognostic factors. Cancer 1988; 62: 2239–2247.

    Article  PubMed  CAS  Google Scholar 

  15. Higa S, Matsumoto M, Tamai O, et al. Plexiform leiomyoma of the esophagus: a peculiar gross variant simulating plexiform neurofibroma. J Gastroenterol 1996; 31: 100–104.

    Article  PubMed  CAS  Google Scholar 

  16. Kaminski PF, Tavassoli FA. Plexiform tumorlet: a clinical and pathologic study of 15 cases with ultrastructural observations. Int J Gynecol Pathol 1984; 3: 124–134.

    Article  PubMed  CAS  Google Scholar 

  17. Kuroda H, Konishi I, Nanbu K, et al. Malignant plexiform tumor of the uterus: an unusual variant of epithelioid leiomyosarcoma. Gynecol Oncol 1996; 63: 270–275.

    Article  PubMed  CAS  Google Scholar 

  18. Larbig GG, Clemmer JJ, Koss LG, Foote FW Jr. Plexiform tumorlet of endometrial stromal origin. Am J Clin Pathol 1965; 45: 32–35.

    Google Scholar 

  19. Mazur MT, Kraus FT. Histogenesis of morphologic variations in tumors of the uterine wall. Am J Surg Pathol 1980; 4: 59–74.

    Article  PubMed  CAS  Google Scholar 

  20. Patchefsky AS. Plexiform tumorlet of the uterus. Obstet Gynecol 1970; 35: 592–596.

    PubMed  CAS  Google Scholar 

  21. Seidman JD, Thomas RM. Multiple plexiform tumorlets of the uterus. Arch Pathol Lab Med 1993; 117: 1255–1256.

    PubMed  CAS  Google Scholar 

  22. Hiraga H, Nojima T, Isu K, Yamashiro K, Yamawaki S, Nagashima K. Histological and molecular evidence of synovial sarcoma of bone. A case report. J Bone Joint Surg 1999; 81A: 558–563.

    Google Scholar 

  23. Berlin O, Angervall L, Kindblom L-G, Berlin IC, Stener B. Primary leiomyosarcoma of bone. A clinical, radiographic, pathologic–anatomic, and prognostic study of 16 cases. Skeletal Radiol 1987; 16: 364–376.

    Article  PubMed  CAS  Google Scholar 

  24. Shen SH, Steinbach LS, Wang SF, Chen WY, Chen WM, Chang CY. Primary leiomyosarcoma of bone. Skeletal Radiol 2001; 30: 600–603.

    Article  PubMed  CAS  Google Scholar 

  25. Sundaram M, Akduman I, White LM, McDonald DJ, Kandel R, Janney C. Primary leiomyosarcoma of bone. AJR Am J Roentgenol 1999; 172: 771–776.

    PubMed  CAS  Google Scholar 

  26. Nielsen GP, Rosenberg AE, Koerner FC, Young RH, Scully RE. Smooth muscle tumours of the vulva. A clinicopathological study of 25 cases and review of the literature. Am J Surg Pathol 1996; 20: 779–793.

    Article  PubMed  CAS  Google Scholar 

  27. Weiss SW, Goldblum JR, Enzinger FM. Soft tissue tumours. Mosby; 2001.

  28. Quinlan RA, Schiller DL, Hatzfeld M, et al. Patterns of expression and organization of cytokeratin intermediate filaments. Ann N Y Acad Sci 1985; 455: 282–306.

    Article  PubMed  CAS  Google Scholar 

  29. Chu PG, Weiss LM. Keratin expression in human tissues and neoplasms. Histopathology 2002; 40: 403–439.

    Article  PubMed  CAS  Google Scholar 

  30. Wesche WA, Khare V, Rao BN, Bowman LC, Parham DM. Malignant peripheral nerve sheath tumor of bone in children and adolescents. Pediatr Dev Pathol 1999; 2: 159–167.

    Article  PubMed  CAS  Google Scholar 

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Acknowledgement

N.A.A. would like to thank Professor Cyril Fisher for his help in diagnosing this case.

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Authors and Affiliations

  1. Department of Radiology, Nuffield Orthopaedic Centre, Oxford, OX3 7LD, UK

    A. Rigopoulou, M. Vlychou & S. J. Ostlere

  2. Department of Orthopaedic Surgery, Nuffield Orthopaedic Centre, Oxford, OX3 7LD, UK

    C. L. M. H. Gibbons

  3. Department of Pathology, Nuffield Department of Orthopaedic Surgery, University of Oxford, Nuffield Orthopaedic Centre, Oxford, OX3 7LD, UK

    N. A. Athanasou

Authors
  1. A. Rigopoulou
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  2. M. Vlychou
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  3. S. J. Ostlere
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  4. C. L. M. H. Gibbons
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  5. N. A. Athanasou
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Correspondence to N. A. Athanasou.

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Rigopoulou, A., Vlychou, M., Ostlere, S.J. et al. A primary leiomyosarcoma of bone containing pseudoepithelial plexiform elements. Skeletal Radiol 36, 791–796 (2007). https://doi.org/10.1007/s00256-007-0301-y

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  • DOI: https://doi.org/10.1007/s00256-007-0301-y

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