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Telangectatic osteosarcoma

Summary

Telangiectatic osteosarcoma is a rare and aggressive variant of osteosarcoma. It accounts for 5% of osteosarcoma' and arises in the metaphysis and diaphysis.

It presents more commonly as a pathological fracture than conventional osteosarcoma.
Telangiectatic osteosarcoma is often entirely osteolytic on plain x-ray.
Complete Information on this Tumor
Introduction and Definition: 

Telangiectatic osteosarcoma is a rare and aggressive variant of osteosarcoma. It accounts for 5% of osteosarcoma' and arises in the metaphysis and diaphysis.

Symptoms and Presentation: 

It presents more commonly as a pathological fracture than conventional osteosarcoma but has similar epidemiology.

X-Ray Appearance and Advanced Imaging Findings: 
Telangiectatic osteosarcoma is often entirely osteolytic on plain x-ray. Bone and cortex destruction are present as well as periosteal reaction and Codman's triangles. On T1 weighted MRI the lesion has high intensity and demonstrates fluid-fluid levels.
Histopathology findings: 
The characteristic gross appearance of telangiectatic osteosarcoma is a multi-cystic "bag of blood".Microscopically, telangiectatic osteosarcoma has large blood filled spaces and thin septation. Within the septa there is scanty osteoid production by the pleomorphic malignant cells of this high grade tumor.
Treatment Options for this Tumor: 
Telangiectatic osteosarcoma (TOS) has a similar prognosis to conventional osteosarcoma. It appears to be responsive to conventional chemotherapeutic protocols. There appears to be a higher-than anticipated amputation rate, perhaps due to the frequent occurence of pathological fracture in this lesio
Special and Unusual Features: 
This recent review a CORR article detailing the results of 17 patients with TOS is found in the Wheeless' Textbook of Orthopedic Surgery, and on-line resource: Pignatti-G; Bacci-G; Picci-P; Dallari-D; Bertoni-F; Bacchini-P; Capanna-R Department of Internal Medicine, Istituto Ortopedico Rizzoli, Bologna, Italy. Clin-Orthop. 1991 Sep(270): 99-106 Seventeen patients with telangiectatic osteogenic sarcoma (TOS) of the extremities were treated with neoadjuvant chemotherapy, according to two different protocols. Preoperatively, the patients received high-dose methotrexate (HD-MTX) and cisplatinum (CPD) (HD-MTX/CPD) and doxorubicin. CPD was delivered intraarterially, the other drugs intravenously. Limb-salvage surgery was performed in 12 instances, and five patients were treated with amputation. Postoperative chemotherapy was tailored according to the grade of chemotherapy-induced necrosis. In 13 cases (86%), the resultant grade of necrosis was greater than 90% (good responders). The mean follow-up interval was 3.5 years with a range of 18 to 78 months. Fourteen patients (82%) remained continuously disease-free, while three patients developed lung metastases. Two of these died because of uncontrolled disease, whereas the third patient is alive and disease-free after metastasectomy. No local recurrences were observed. These results are better than those observed in 215 contemporary cases of conventional osteosarcoma treated with the same protocols. This study confirms that TOS is not a uniformly lethal tumor as suggested by prior reports. By employing neoadjuvant chemotherapy, a high percentage of patients with TOS can be cured, and in most of them, a limb-sparing surgery is possible and safe.
Suggested Reading and Reference: 
Bloem, JL and HM Kroon, Osseous Lesions, Radiologic Clinics of North America, 31(2):261-277, March 1993. Bullough, Peter, Orthopaedic Patholovv (third edition), Times Mirror International Publishers Limited, London, 1997. Huvos, Andrew, Bone Tumors: Diagnosis. Treatment and Prognosis, W.B. Saunders, Co., 1991. Vander Griend, RA, osteosarcoma and Its Variants, Orthopaedic Clinics of North America, 27(3):575581, July, 1996.