Abstract
Background: Primary leiomyosarcoma of bone is a very rare malignant tumor with uncertain pathogenicity.
Methods: The authors studied five cases of surgically treated primary leiomyosarcoma of bone. Clinical histories and radiographic findings were recorded. Regular clinical and radiographic controls were obtained postoperatively. In all cases, immunohistochemical studies were used to confirm the diagnosis. Molecular biologic examinations, using the polymerase chain reaction technique with microsatellite DNA markers from regions of tumor-relevant genes, were performed to determine the stability of the genome or to detect some typical genomic changes.
Results: The study included three women and two men, with an average age of 42 years. The tumor was located in the pelvis in two patients, in the femur in two patients, and in the proximal tibia in one patient. All tumors were classified as high-grade tumors (four stage IIB, one stage IIA). Radiographically, all tumors appear as purely osteolytic lesions, with a geographic or moth-eaten appearance and without any sclerotic margin.
Three patients underwent limb salvage surgery followed by endoprosthetic replacement. The other two patients required amputation. The mean follow-up was 19 months (range, 8–29 months). Three patients died of disease, with a mean postoperative survival period of 18 months (range, 6–27 months). Four patients developed diffuse pulmonary metastases after an average of 10.5 months. One of those patients responded well to chemotherapy. In all cases, immunohistochemistry showed strong reactivity of the tumor cells for α-SMA and vimentin. Molecular biologic investigations revealed a high rate of genomic instabilities in all of the stage IIB tumors.
Conclusion: Clinical follow-up suggests that primary osseous leiomyosarcoma has an aggressive biologic behavior. The immunohistochemical studies are useful tools and suggest that osseous leiomyosarcoma arise from the vascular smooth muscle cells within the bone. The molecular biologic findings of a high rate of genomic instability confirm the hypothesis that this rare entity is of an aggressive nature.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Abdelwahab IF, Hermann G, Kenan S, Klein MJ, Lewis MM. Case report 794.Skeletal Radiol 1993;22:379–81.
Angervall L, Berlin Ö, Kindblom L-G, Stener B. Primary leiomyosarcoma of bone: a study of five cases.Cancer 1980;46:1270–9.
Berlin Ö, 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;16:374–6.
Meister P, Konrad E, Gokel JM, Remberger K. Case report.Skeletal Radiol 1978;2:265–7.
Meltzer CC, Fishman EK, Scott WW Jr. Computed tomography appearance of bone metastases of leiomyosarcoma.Skeletal Radiol 1992;31:445–7.
Krishnan V, Miyaji CM, Mainous EG. Leiomyosarcoma of the mandible: a case report.J Oral Maxillofac Surg 1991;49:652–5.
Martin-Hirsch DP, Habashi S, Benbow EW, Farrington WT. Post-irradiation leiomyosarcoma of the maxilla.J Laryngol Otol 1991;105:1068–71.
Wang TY, Erlandson RA, Marcove RC, Huvos AG. Primary leiomyosarcoma of bone.Arch Pathol Lab Med 1980;104:100–4.
Enzinger FM, Weiss SW.Soft Tissue Tumors. St. Louis: CV Mosby, 1988.
Hajdu SI.Pathology of Soft Tissue Tumors. Philadelphia: Lea & Febiger, 1979.
Hochstetter von AR, Eberle H, Rüttner JR. Primary leiomyosarcoma of extragnathic bones: case report and review of literature.Cancer 1984;53:2194–2200.
Jundt G, Moll C, Nidecker A, Schild R, Remagen W. Primary leiomyosarcoma of bone: report of eight cases.Hum Pathol 1994;25:1205–12.
Myers JL, Arocho J, Bernreuter W, Dunham W, Mazur MT. Leiomyosarcoma of bone: a clinicopathologic, immunohistochemical and ultrastructural study of five cases.Cancer 1991;67:1051–6.
Overgaard J, Frederiksen P, Helmig O, Jensen OM. Primary leiomyosarcoma of bone.Cancer 1977;39:1664–71.
Sanarkin NG. Primary leiomyosarcoma of the bone and its comparison with fibrosarcoma: a cytological, histological and ultrastructural study.Cancer 1979;44:1375–87.
Evans D, Sanarkin NG. Primary leiomyosarcoma of bone.J Pathol Bacteriol 1965;90:348–50.
Eady JL, McKinney JD, McDonald EC. Primary leiomyosarcoma of bone. A case report and review of the literature.J Bone Joint Surg 1987;69A:287–9.
Kawai T, Suzuki M, Mukai M, Hiroshima K, Shinmei M. Primary leiomyosarcoma of bone: an immunohistochemical and ultrastructural study.Arch Pathol Lab Med 1983;107:433–7.
Enneking WF.Musculoskeletal Tumor Surgery. New York: Churchill Livingstone, 1983.
Shamsuddin AK, Reyes F, Harvey JW, Toller C. Primary leiomyosarcoma of bone.Hum Pathol 1980;11:581–3.
Bago J, Madurell J, Nardi J, Suso S, Velasco J, Vicente P. Leiomyo-sarcoma osseux du bassin.Acta Orthop Belgica 1993;49:279–81.
Marymont JV, Clanton TO. Leiomyosarcoma of the os calcis.Foot Ankle 1990;10:239–42.
Shapiro S. Myelopathy secondary to leiomyosarcoma of the spine.Spine 1992;17:249–51.
Takami KM, Ishida T, Ieguchi M, Kuniyoshi Y, Wakasa K, Sakurai M. Primary leiomyosarcoma or leiomyoma of the pubic bone.Int Orthopaedics 1994;18:248–51.
Wulfeck DW, Sakow NK, Williams TE. Scintigraphic appearance of primary skeletal leiomyosarcoma.Clin Nucl Med 1994;19:350–2.
Quade BJ. Pathology, cytogenetics and molecular biology of uterine leiomyomas and other smooth muscle lesions.Curr Opin Obstet Gynecol 1995;7:35–42.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Wirbel, R.J., Verelst, S., Hanselmann, R. et al. Primary leiomyosarcoma of bone: Clinicopathologic, immunohistochemical, and molecular biologic aspects. Annals of Surgical Oncology 5, 635–641 (1998). https://doi.org/10.1007/BF02303834
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF02303834