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.^[2] It is an uncommon variant, accounting for 0.4-12% of all osteosarcomas.^{[3, 4, 5, 6, 7]}

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.^[8]

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.^[9]Such observations suggest that telangiectatic osteosarcoma may be derived from multipotential mesenchymal cells, with possible differentiation along various pathways.^[10]

Familial occurrence has been reported at least once.^[11]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.^[12]

Epidemiology

Telangiectatic osteosarcoma occurs in a male-to-female ratio of 2:1. Although disease can be found in patients as young as 3 years and as old as 71 years, it rarely occurs in persons older than 25 years. Most patients present between the ages of 10 and 20 years.^[13]

Prognosis

Historically, local recurrence has been found to be more common than metastasis.^{[14, 15, 6, 16]}With the use of preoperative chemotherapy, however, this observation is probably less accurate than it was. Telangiectatic osteosarcomas have vascular spread and therefore, similar to conventional osteosarcomas, metastasize to the bones and lungs.^{[14, 15]}

Clinical Presentation

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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

Background

Etiology

Epidemiology

Prognosis

References

Tables

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