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 but has similar epidemiology.
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.
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.
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 lesion.
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,
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.
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,
Vander Griend, RA, osteosarcoma and Its Variants, Orthopaedic Clinics
of North America, 27(3):575581, July, 1996.