eMedicine Specialties > Orthopedic Surgery > Neoplasms

Telangiectatic Osteosarcoma

Author: Nirag C Jhala, MD, MBBS, MIAC, Associate Professor, Department of Pathology, University of Alabama School of Medicine; Consulting Staff, Department of Pathology, University of Alabama Hospitals and Birmingham Veterans Affairs Medical Center
Coauthor(s): Gene P Siegal, MD, PhD, Director, Division of Anatomic Pathology, Professor, Departments of Pathology and Surgery, University of Alabama at Birmingham; Donald A Hackbarth Jr, MD, FACS, Professor of Clinical Orthopedic Surgery, Division Chief, Musculoskeletal Oncology, Department of Orthopedic Surgery, Medical College of Wisconsin; Stuart Wong, MD, Assistant Professor, Department of Medicine, Section of Hematology/Oncology, Froedert Memorial Lutheran Hospital; Vinod B Shidham, MD, FRCPath, FIAC,, Professor, Director of Cytopathology Fellowship Training Program, FNAB Service, and International Cytopathology Fellowship, Department of Pathology, Medical College of Wisconsin; Co-Editor-in-Chief and Executive Editor, CytoJournal
Contributor Information and Disclosures

Updated: Jan 18, 2008

Introduction

Osteosarcoma is the most common primary malignant bone tumor in children and adolescents. When first recognized, telangiectatic osteosarcoma was proposed to be a distinct clinical and pathologic entity.1 On the basis of subsequent findings that provided a better understanding of the condition's presentation and prognostic implications, telangiectatic osteosarcoma should be considered a variant of osteosarcoma.

Telangiectatic osteosarcoma appears as a painful, radiographically lytic mass lesion in the metaphyseal portion of the long bones. It is characterized by dilated, blood-filled vascular spaces lined by malignant osteoblasts. These osteoblasts are separated by fibrous septa, which contain the malignant cells, multinucleated giant cells, and tumor osteoid.

Frequency

As an uncommon variant of osteosarcoma, telangiectatic osteosarcoma accounts for 0.4-12% of all osteosarcomas.2,3,4,5

Etiology

Because of the rarity of this lesion, the etiologic factors that promote malignant transformation have not been extensively investigated. Telangiectatic osteosarcomas are presumed to originate from transformed osteoblasts or from stem cells that are of mesenchymal derivation.

Results from transmission electron microscopic examination show that, in addition to undifferentiated osteoblastlike and fibroblastlike tumor cells, angiosarcomatous elements may be observed in this malignant bone tumor. Endothelial, cell-like structures, including pinocytotic vesicles, tight intercellular junctions, fine fibrils, and Weibel-Palade bodies, are seen in the cytoplasm of these cells.6 Such observations suggest that telangiectatic osteosarcoma may be derived from multipotential mesenchymal cells, with possible differentiation along various pathways.7

Familial occurrence has been reported at least once.8 In all cases of telangiectatic osteosarcoma, familial genetic changes may be responsible, but other causes are more likely. Molecular and cytogenetic studies are necessary to resolve these issues.9

Presentation

The clinical presentation of telangiectatic osteosarcoma closely resembles that of conventional osteosarcoma. However, local pain, soft-tissue masses, and fractures are the most common presenting symptoms and signs.5,10

Patient sex and age

Telangiectatic osteosarcoma occurs in a male-to-female ratio of 2:1. Although disease can be found in patients aged 3-71 years, it rarely occurs in persons older than 25 years. Most patients present when they are aged 10-20 years.

Site of lesions

Telangiectatic osteosarcoma lesions are usually osseous, but extraosseous lesions have been reported.

In the long bones, these tumors usually occur in the metaphyseal region within the medullary cavity. As the tumors expand and destroy the cortex, blowout fractures may occur. These lesions may also occur in a diaphyseal location.11

The distribution pattern of telangiectatic osteosarcomas in the long bones is as follows12 :
Distal femur - 48%;
Proximal humerus - 12%;
Proximal tibia - 10%;
Proximal femur - 8%;
Fibula - 5%;
Midfemur - 2%;
Midhumerus - 2%

These tumors also occur in the mandible.13

Potentially, telangiectatic osteosarcomas can arise in bones involved with Paget disease,14,15 where it can mimic other forms of conventional osteosarcoma.16

Telangiectatic osteosarcomatous differentiation has been reported in parosteal osteosarcoma,17 in dedifferentiated chondrosarcoma arising in the background of osteochondroma,18 in association with aneurysmal bone cysts,19,20 and in osteitis deformans.

Telangiectatic osteosarcoma also has been noted to arise in extraosseous soft tissues in the forearm, thigh, and popliteal fossa.21 Although rare, telangiectatic osteosarcomatous differentiation has been seen in cases of malignant phyllodes tumor of the breast22 and in cases of ovarian sarcoma.23

More on Telangiectatic Osteosarcoma

Overview: Telangiectatic Osteosarcoma
Workup: Telangiectatic Osteosarcoma
Treatment: Telangiectatic Osteosarcoma
Follow-up: Telangiectatic Osteosarcoma
Multimedia: Telangiectatic Osteosarcoma
References
Further Reading
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References

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  2. Huvos AG, Rosen G, Bretsky SS. Telangiectatic osteogenic sarcoma: a clinicopathologic study of 124 patients. Cancer. Apr 15 1982;49(8):1679-89. [Medline].

  3. Larsson SE, Lorentzon R, Wedren H, et al. Osteosarcoma. A multifactorial clinical and histopathological study with special regard to therapy and survival. Acta Orthop Scand. Dec 1978;49(6):571-81. [Medline].

  4. Larsson SE, Lorentzon R, Boquist L. Telangiectatic osteosarcoma. Acta Orthop Scand. Dec 1978;49(6):589-94. [Medline].

  5. Mervak TR, Unni KK, Pritchard DJ, et al. Telangiectatic osteosarcoma. Clin Orthop Relat Res. Sep 1991;(270):135-9. [Medline].

  6. Metcalf DJ, Nightingale TD, Zenner HL, et al. Formation and function of Weibel-Palade bodies. J Cell Sci. Jan 1 2008;121(Pt 1):19-27. [Medline].

  7. Roessner A, Hobik HP, Immenkamp M, et al. Ultrastructure of telangiectatic osteosarcoma. J Cancer Res Clin Oncol. Oct 1979;95(2):197-207. [Medline].

  8. Nishida J, Abe M, Shiraishi H, et al. Familial occurrence of telangiectatic osteosarcoma: cousin cases. J Pediatr Orthop. Jan-Feb 1994;14(1):119-22. [Medline].

  9. Bell W, Siegal GP. Osteosarcoma. In: Cullinane C, Burchill S, Squire J, et al, eds. Molecular Biology and Pathology of Paediatric Cancer. London, England: Oxford University Press; 2003.

  10. Papagelopoulos PJ, Mavrogenis AF, Savvidou OD, et al. Pathological fractures in primary bone sarcomas. Injury. Nov 29 2007;[Medline].

  11. Brown MJ, Logan PM, O'Connell JX. Diaphyseal telangiectatic osteosarcoma as a second tumor after bilateral retinoblastomas. Skeletal Radiol. Oct 1996;25(7):685-8. [Medline].

  12. Fechner R, Mills S. Tumors of the bone and joints. In: Rosai J, Sobin L, eds. Atlas of Tumor Pathology. Washington, DC: Armed Forces Institute of Pathology; 1993:51-4.

  13. Chan CW, Kung TM, Ma L. Telangiectatic osteosarcoma of the mandible. Cancer. Nov 1 1986;58(9):2110-5. [Medline].

  14. Donato G, Lavano A, Volpentesta G. Telangiectatic osteosarcoma of the skull. A post-Paget case. Clin Neuropathol. Jul-Aug 1997;16(4):201-3. [Medline].

  15. Merino S, Arrazola J, Saiz A, et al. Post-Paget telangiectatic osteosarcoma of the skull. Skeletal Radiol. Aug 1999;28(8):470-2. [Medline].

  16. Wick MR, Siegal GP, Unni KK, et al. Sarcomas of bone complicating osteitis deformans (Paget''s disease): fifty years'' experience. Am J Surg Pathol. Jan 1981;5(1):47-59. [Medline].

  17. Wines A, Bonar F, Lam P. Telangiectatic dedifferentiation of a parosteal osteosarcoma. Skeletal Radiol. Oct 2000;29(10):597-600. [Medline].

  18. Radhi JM, Loewy J. Dedifferentiated chondrosarcoma with features of telangiectatic osteosarcoma. Pathology. Nov 1999;31(4):428-30. [Medline].

  19. Adler CP. Case report 111. Skeletal Radiol. Feb 1980;5(1):56-60. [Medline].

  20. Kyriakos M, Hardy D. Malignant transformation of aneurysmal bone cyst, with an analysis of the literature. Cancer. Oct 15 1991;68(8):1770-80. [Medline].

  21. Mirra JM, Fain JS, Ward WG, et al. Extraskeletal telangiectatic osteosarcoma. Cancer. May 15 1993;71(10):3014-9. [Medline].

  22. Graadt van Roggen JF, Zonderland HM, Welvaart K, et al. Local recurrence of a phyllodes tumour of the breast presenting with widespread differentiation to a telangiectatic osteosarcoma. J Clin Pathol. Sep 1998;51(9):706-8. [Medline]. [Full Text].

  23. Hirakawa T, Tsuneyoshi M, Enjoji M, et al. Ovarian sarcoma with histologic features of telangiectatic osteosarcoma of the bone. Am J Surg Pathol. Jul 1988;12(7):567-72. [Medline].

  24. White VA, Fanning CV, Ayala AG, et al. Osteosarcoma and the role of fine-needle aspiration. A study of 51 cases. Cancer. Sep 15 1988;62(6):1238-46. [Medline].

  25. Vanel D, Tcheng S, Contesso G. The radiological appearances of telangiectatic osteosarcoma. A study of 14 cases. Skeletal Radiol. 1987;16(3):196-200. [Medline].

  26. Khuu H, Moore D, Young S. Examination of tumor and tumor-like conditions of bone. Ann Diagn Pathol. Dec 1999;3(6):364-9. [Medline].

  27. Bacci G, Picci P, Ferrari S. Primary chemotherapy and delayed surgery for non-metastatic telangiectatic osteosarcoma of the extremities. Results in 28 patients. Eur J Cancer. 1994;30A(5):620-6. [Medline].

  28. Rosen G, Huvos AG, Marcove R, et al. Telangiectatic osteogenic sarcoma. Improved survival with combination chemotherapy. Clin Orthop Relat Res. Jun 1986;(207):164-73. [Medline].

  29. Weiss A, Khoury JD, Hoffer FA, et al. Telangiectatic osteosarcoma: the St. Jude Children's Research Hospital's experience. Cancer. Apr 15 2007;109(8):1627-37. [Medline].

  30. Rosen G, Caparros B, Huvos AG. Preoperative chemotherapy for osteogenic sarcoma: selection of postoperative adjuvant chemotherapy based on the response of the primary tumor to preoperative chemotherapy. Cancer. Mar 15 1982;49(6):1221-30. [Medline].

  31. Basaran M, Bavbek ES, Saglam S, et al. A phase II study of cisplatin, ifosfamide and epirubicin combination chemotherapy in adults with nonmetastatic and extremity osteosarcomas. Oncology. Jan 10 2008;72(3-4):255-260. [Medline].

  32. Ferguson WS, Harris MB, Goorin AM, et al. Presurgical window of carboplatin and surgery and multidrug chemotherapy for the treatment of newly diagnosed metastatic or unresectable osteosarcoma: Pediatric Oncology Group Trial. J Pediatr Hematol Oncol. Aug-Sep 2001;23(6):340-8. [Medline].

  33. Rashid M, Hafeez S, Zia Ul Islam M, et al. Limb salvage in malignant tumours of the upper limb using vascularised fibula. J Plast Reconstr Aesthet Surg. Dec 22 2007;[Medline].

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Further Reading

See also the following topics in eMedicine:
Giant Cell Tumor [Orthopedic Surgery]
Giant Cell Tumor [Radiology]
Osteosarcoma [Orthopedic Surgery]
Osteosarcoma [Pediatrics: General Medicine]
Osteosarcoma, Classic
Osteosarcoma, Variants
Aneurysmal Bone Cyst [Orthopedic Surgery]
Aneurysmal Bone Cyst [Radiology]
Resource Center Biologic Therapies in Cancer

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Keywords

primary malignant bone tumor, osteosarcoma variant, lytic mass lesion in long bone metaphysis

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Contributor Information and Disclosures

Author

Nirag C Jhala, MD, MBBS, MIAC, Associate Professor, Department of Pathology, University of Alabama School of Medicine; Consulting Staff, Department of Pathology, University of Alabama Hospitals and Birmingham Veterans Affairs Medical Center
Nirag C Jhala, MD, MBBS, MIAC is a member of the following medical societies: American Society of Cytopathology, Biomedical Engineering Society, College of American Pathologists, International Academy of Cytology, and United States and Canadian Academy of Pathology
Disclosure: Nothing to disclose.

Coauthor(s)

Gene P Siegal, MD, PhD, Director, Division of Anatomic Pathology, Professor, Departments of Pathology and Surgery, University of Alabama at Birmingham
Gene P Siegal, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Medical Association, American Society for Clinical Pathology, American Society for Investigative Pathology, College of American Pathologists, International Academy of Pathology, International Skeletal Society, New York County Medical Society, Royal Society of Medicine, Sigma Xi, and United States and Canadian Academy of Pathology
Disclosure: Nothing to disclose.

Donald A Hackbarth Jr, MD, FACS, Professor of Clinical Orthopedic Surgery, Division Chief, Musculoskeletal Oncology, Department of Orthopedic Surgery, Medical College of Wisconsin
Donald A Hackbarth Jr, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Association of Tissue Banks, American College of Surgeons, Children's Oncology Group, Christian Medical & Dental Society, Clinical Orthopaedic Society, and Wisconsin Medical Society
Disclosure: Musculoskeletal Transplant Foundation Honoraria Board membership

Stuart Wong, MD, Assistant Professor, Department of Medicine, Section of Hematology/Oncology, Froedert Memorial Lutheran Hospital
Disclosure: Nothing to disclose.

Vinod B Shidham, MD, FRCPath, FIAC,, Professor, Director of Cytopathology Fellowship Training Program, FNAB Service, and International Cytopathology Fellowship, Department of Pathology, Medical College of Wisconsin; Co-Editor-in-Chief and Executive Editor, CytoJournal
Vinod B Shidham, MD, FRCPath, FIAC, is a member of the following medical societies: American Association for Cancer Research, American Society of Cytopathology, College of American Pathologists, International Academy of Cytology, Royal College of Pathologists, and United States and Canadian Academy of Pathology
Disclosure: Nothing to disclose.

Medical Editor

Miguel A Schmitz, MD, Consulting Surgeon, Department of Orthopedics, Klamath Orthopedic and Sports Medicine Clinic
Miguel A Schmitz, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Orthopaedic Society for Sports Medicine, and Arthroscopy Association of North America
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Sean P Scully, MD, PhD, Professor, Department of Orthopedics, University of Miami
Sean P Scully, MD, PhD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, International Society on Thrombosis and Haemostasis, and Society of Surgical Oncology
Disclosure: Nothing to disclose.

CME Editor

Dinesh Patel, MD, FACS, Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital
Dinesh Patel, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Association of Physicians of Indian Origin, American College of International Physicians, and American College of Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Harris Gellman, MD, Consulting Surgeon, Broward Hand Center; Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami School of Medicine
Harris Gellman, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, and Arkansas Medical Society
Disclosure: Nothing to disclose.

 
 
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