Telangiectatic Osteosarcoma 

  • Author: Nirag C Jhala, MD, MBBS, MIAC; Chief Editor: Harris Gellman, MD   more...
 
Updated: Apr 5, 2010
 

Background

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.

An image depicting findings in an aneurysmal bone cyst can be seen below.

Large blood lakes seen at a low magnification are Large blood lakes seen at a low magnification are reminiscent of findings in an aneurysmal bone cyst (hematoxylin and eosin, original magnification X4).
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Epidemiology

Frequency

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

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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]

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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, 11, 12]

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.[13]

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.[14]

The distribution pattern of telangiectatic osteosarcomas in the long bones is as follows[15] :

  • 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.[16]

Potentially, telangiectatic osteosarcomas can arise in bones involved with Paget disease,[17, 18] where it can mimic other forms of conventional osteosarcoma.[19]

Telangiectatic osteosarcomatous differentiation has been reported in parosteal osteosarcoma,[20] in dedifferentiated chondrosarcoma arising in the background of osteochondroma,[21] in association with aneurysmal bone cysts,[22, 23] and in osteitis deformans.

Telangiectatic osteosarcoma also has been noted to arise in extraosseous soft tissues in the forearm, thigh, and popliteal fossa.[24] Although rare, telangiectatic osteosarcomatous differentiation has been seen in cases of malignant phyllodes tumor of the breast[25] and in cases of ovarian sarcoma.[26]

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Contributor Information and Disclosures
Author

Nirag C Jhala, MD, MBBS, MIAC  Director of Cytopathology, Perelman Center for Advanced Medicine, Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania

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)

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

Vinod B Shidham, MD, FRCPath  Professor, Vice-chair-AP, and Director of Cytopathology, Department of Pathology, Wayne State University School of Medicine, Karmanos Cancer Center & Detroit Medical Center; Co-Editor-in-Chief and Executive Editor, CytoJournal

Vinod B Shidham, MD, FRCPath 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.

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.

Stuart Wong, MD  Assistant Professor, Department of Medicine, Section of Hematology/Oncology, Froedert Memorial Lutheran Hospital

Disclosure: Nothing to disclose.

Specialty Editor Board

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, Arthroscopy Association of North America, and North American Spine Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

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.

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

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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Large blood lakes seen at a low magnification are reminiscent of findings in an aneurysmal bone cyst (hematoxylin and eosin, original magnification X4).
Careful examination of the lining of blood-filled lakes shows overt malignant cells. Atypical tripolar mitosis is noted in the field (hematoxylin and eosin, original magnification X20).
Numerous giant cells may be noted in the tumor, which mimics a giant, cell–rich osteosarcoma (hematoxylin and eosin, original magnification X20).
Areas of necrosis with persistent tumor cells are present after neoadjuvant chemotherapy (hematoxylin and eosin, original magnification X10).
Anteroposterior radiograph of the distal femur shows a large, aggressive lytic lesion replacing the distal femur with no appreciable intralesional matrix. Courtesy of Robert Lopez-Ben, MD.
Lateral radiograph of the distal femur shows a large, aggressive lytic lesion that replaces the distal femur with no appreciable intralesional matrix. Courtesy of Robert Lopez-Ben, MD.
Table 1. Initial Enneking Clinical Staging System for Primary Malignant Bone Tumors
StageGradeLocationMetastasis
IALow grade, G1T1M0, intracompartmental
IBLow grade, G1T2M0, intracompartmental
IIAHigh grade, G2T1M0, intracompartmental
IIBHigh grade, G2T2M0, extracompartmental
IIIALow or high grade, G1 or G2T1M1, intracompartmental with metastasis
IIIBLow or high grade, G1 or G2T2M1, extracompartmental with metastasis
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