History and Physical Examination

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.^{[6, 17, 18, 19]}

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.^[20]

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 have also been known to occur in the mandible^{[21, 22]} and, rarely, the rib.^[23]

Potentially, telangiectatic osteosarcomas can arise in bones involved with Paget disease,^{[24, 25]}where it can mimic other forms of conventional osteosarcoma.^[26]

Telangiectatic osteosarcomatous differentiation has been reported in parosteal osteosarcoma,^[27]in dedifferentiated chondrosarcoma arising in the background of osteochondroma,^[28]in association with aneurysmal bone cysts,^{[29, 30, 31]}and in osteitis deformans.

Telangiectatic osteosarcoma also has been noted to arise in extraosseous soft tissues in the forearm, thigh, and popliteal fossa.^[32]Although rare, telangiectatic osteosarcomatous differentiation has been seen in cases of malignant phyllodes tumor of the breast^[33]and in cases of ovarian sarcoma.^[34]

Differential Diagnoses

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Media Gallery

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.

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Author

Nirag C Jhala, MD, MBBS Director of Anatomic Pathology, Director of Cytopathology, Temple University Hospital; Professor of Pathology and Laboratory Medicine, Temple University School of Medicine

Nirag C Jhala, MD, MBBS is a member of the following medical societies: American Society of Cytopathology, Biomedical Engineering Society, College of American Pathologists, International Academy of Cytology, 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, Christian Medical and Dental Associations, Clinical Orthopaedic Society, Children's Oncology Group, Wisconsin Medical Society

Disclosure: Received honoraria from Musculoskeletal Transplant Foundation for 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 and 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, 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 Investigative Pathology, American Society for Clinical Pathology, College of American Pathologists, International Academy of Pathology, International Skeletal Society, New York County Medical Society, Royal Society of Medicine, Sigma Xi, The Scientific Research Honor Society, 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

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Omohodion (Odion) Binitie, MD Medical Director, Assistant Member, Department of Sarcoma, Section Head, Orthopedics, Adolescent/Young Adult and Pediatric Orthopedic Oncology, Medical Director, Physical Therapy, Speech Therapy, and Rehabilitation Services, Assistant Fellowship Program Director, Musculoskeletal Oncology, Moffitt Cancer Center; Assistant Professor, Department of Oncologic Sciences, Department of Ortho and Sports Medicine, University of South Florida Morsani College of Medicine

Omohodion (Odion) Binitie, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, Children's Oncology Group, Florida Orthopaedic Society, Musculoskeletal Tumor Society

Disclosure: Nothing to disclose.

Stage	Grade	Location	Metastasis
IA	Low grade, G1	T1	M0, intracompartmental
IB	Low grade, G1	T2	M0, intracompartmental
IIA	High grade, G2	T1	M0, intracompartmental
IIB	High grade, G2	T2	M0, extracompartmental
IIIA	Low or high grade, G1 or G2	T1	M1, intracompartmental with metastasis
IIIB	Low or high grade, G1 or G2	T2	M1, extracompartmental with metastasis

Telangiectatic Osteosarcoma Clinical Presentation

History and Physical Examination

References

Tables

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