Telangiectatic osteosarcoma: The St. Jude Children's Research Hospital's experience

Abstract

BACKGROUND. Telangiectatic osteosarcoma (TOS) isRESULTS. Among 323 patients with OS, 22 patients (6.8%) had TOS. Two additional patients who were treated in Chile on a recent OS trial were included. The median age at diagnosis of the 24 patients was 15.7 years. Four patients (17%) had metastatic disease, and 9 of 21 patients (43%) had pathologic fractures. Only 5 patients (who were treated after 1994) underwent limb-salvage surgery.Estimates of 5-year event-free survival (58.3% AE 11.9%) and overall survival (66.8% AE 11.6%) were similar to those for patients with other OS subtypes (P ! .85). The absence of local disease progression and chemotherapy with !3 agents that were active against OS were correlated with improved outcome (P .005).The presence of a pathologic fracture was not associated with surgery type or patient outcome.CONCLUSIONS. TOS was associated with a high rate of pathologic fracture. With multimodality therapy, the outcome of patients with TOS was similar to that of patients with other high-grade OS subtypes. The absence of local disease progression and chemotherapy with !3 active agents were associated with a favorable outcome.

Telangiectatic osteosarcoma of the spine: a case report

“…Telangiectatic osteosarcoma is primarily a tumour of the long tubular bones [9,14], the femur is involved most frequently, followed in frequency by the tibia and humerus [9],a distribution similar to that of conventional osteosarcoma. In these bones the metaphysis is the usual site of origin [9,10,15].…”

“…The awareness and recognition of the unique histological and radiological features of spine TOS is crucial for making an early and accurate diagnosis [14].It stands without saying that delaying the correct diagnosis and initiation of proper therapy may have a negative impact on survival of these patients [14].…”

“…Fluid-fluid levels are suggestive of haemorrhage. The anterior portions are bright,reflecting the supernatant whereas the posterior portions are dark representing the clot [14]. Fluid-fluid levels in blood filled cavities are a characteristic although nonspecific finding in aneurysmal bone cysts [2], giant cell tumours and telangiectatic osteosarcomas.…”

“…The dilemmas associated with the diagnosis of TOS has been emphasized in literature [10,14].In particular an ABC is almost indistinguishable from TOS on radiographs [10] and hence the correct diagnosis rests on a careful histological examination [14].The low power appearance of TOS is identical to that of ABC. TOS is characterized by multiple aneurysmal dilated blood filled cavities with high grade, sarcomatous cells in the peripheral rim and septa [6].…”

“…The distinction must ultimately rest on the finding of these obviously anaplastic cells [10], atypical mitotic figure and irregular closely packed lace like osteoid [1]. Therefore a combined modality approach, including the use of strict pathologic and radiological criteria, is crucial in diagnosing telangiectatic osteosarcoma [14]. These criteria as defined in the World Health Organization Classification [13] are: (1) predominantly lytic bone mass with minimal sclerosis on radiographs, (2) grossly cystic medullary mass with no or minimal solid or sclerotic component and (3) histological features consisting of bone forming tumour with notable blood filled spaces separate by septae lined by and/ or containing malignant tumour cells with prominent nuclear atypia and limited osteoid deposition.…”