Telangiectatic Osteosarcoma

Abstract

Osteosarcoma is one of the most common primary malignant bone tumors in children and adolescents. Telangiectatic osteosarcoma is an unusual variant of osteosarcoma, forming 3% to 10% of all osteosarcomas. Radiographically, these tumors appear as purely lytic destructive lesions located in the metaphyses of long bones. The location and x-ray appearance of telangiectatic osteosarcomas are reminiscent of an aneurysmal bone cyst and can test the acumen of a diagnostic radiologist. Distinguishing between the two entities microscopically can also be quite challenging. Telangiectatic osteosarcoma shows dilated blood-filled spaces lined or traversed by septa containing atypical stromal cells, with or without production of a lacelike osteoid matrix. This review highlights the diagnostic features of telangiectatic osteosarcoma and discusses differential diagnostic considerations, treatment options, and prognostic implications.

Cystic bone lesions: histopathological spectrum and diagnostic challenges

“…repeat biopsy has been performed in 5 of our patients because of this possibility. Cellular atypia, atypical and especially tripolar-tetrapolar mitoses, and the presence of irregular, lace-like osteoid are the most important features that favor telangiectatic osteosarcoma (2,11). However, one should bear in mind that osteoid formation may be prominent in ABC as well.…”

Telangiectatic osteosarcoma, a rare complication of Paget's disease of bone

“…5,7 Telangiectatic osteosarcoma, a rare histological variant of osteosarcoma, is described in 2-12% of all osteosarcomas. 5,9,10 Historically, first recognised by Paget in 1853 and classified as a distinct variant of osteosarcoma by Ewing in 1922, telangiectatic osteosarcoma predominantly affects male patients (male to female ratio of 2:1), and is seen more often in younger patients. [10][11][12] When considering the clinical, radiological and histopathological features of telangiectatic osteosarcoma, it is important to differentiate it from an aneurysmal bone cyst and giant cell tumours of the bone.…”

“…12,13 Distinguishing pathological features of this case were the destructive growth pattern (cortical destruction and infiltration of soft tissue), gross appearance (predominantly cystic lesion filled with blood), and the presence of malignant cells on microscopic examination. [9][10][11][12] Despite advances in neoadjuvant chemotherapy, the prognosis remains poor in the setting of all secondary osteosarcomas arising in PDB (a five-year survival rate of 10%). The poor outcome of osteosarcoma in this clinical setting suggests a different biological potential which may relate to underlying genetic susceptibility or environmental factors.…”

“…However an unusual photopenic area was localised to the left iliac bone. This phenomenon was described as the "doughnut sign" by Sangle and Layfield et al 9 It proved to be decisive in supporting the eventual diagnosis ( Figure 2). Magnetic resonance imaging delineated the extent of the tumour and showed the hallmarks of fluid-fluid levels, in keeping with the final histopathological diagnosis (Figures 3-4).…”

Neglected telangiectatic osteosarcoma of the femur presenting as surgical emergency

“…Pathologic fracture is a clinical feature of this malignant bone-forming tumor [1]. This tumor resembles aneurysmal bone cyst radiologically and microscopically with large cavity containing blood filled cystic spaces separated by septa [4,5]. There is usually invasion of soft tissues and formation of a large palpable mass resembling a soft tissue tumor at clinical examination.…”