Juxtacortical osteosarcoma. Diagnosis, differential diagnosis, treatment, and an analysis of eighty cases.
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Juxtacortical osteosarcomas, including periosteal and parosteal subtypes, are uncommon neoplasms of the jaws. In long bones, the biologic behavior of the periosteal variant is reported to be more aggressive than the parosteal tumor. Juxtacortical osteosarcoma of the jaws appears to have a slightly more favorable prognosis than in long bones, although a larger series of cases needs to be evaluated before a definitive conclusion concerning the behavior of juxtacortical osteosarcoma of the jaws can be made.
Periosteal osteosarcoma, a variant of juxtacortical osteosarcoma, is seldom seen in the jaws. The clinical, radiographic, and histopathologic features of a rare mandibular periosteal osteosarcoma presenting as a deep mass in the area of the parotid are reported. The clinicopathologic features that distinguish this lesion from the more common parosteal osteosarcoma are also discussed.
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Periosteal osteosarcoma is a distinct bone tumor entity with characteristic morphological features within the group of juxtacortical osteosarcoma. Periosteal osteosarcoma is predominantly located in the long tubular bones, especially in the tibia and femur and is situated on the outer circumference of the tumor-bearing bone (saucerization phenomenon). In contrast to parosteal osteosarcoma, periosteal osteosarcoma is less differentiated and is believed to have a worse prognosis. In this work the histological features are described with predominantly chondroblastic differentiation of 14 cases with periosteal osteosarcoma. A horizontal preparation technique of periosteal osteosarcoma specimens allows comparison with computed tomography and is the optimal method to detect an invasion of the medullary cavity. Further studies are necessary to clarify if neoadjuvant chemotherapy could improve the prognosis of certain patients.
The authors report two cases of periosteal osteosarcoma of the jaws, a tumour which is one of the two variants of juxtacortical osteosarcoma. Periosteal osteosarcoma involving the jaw bones is extremely rare. Because of its chondroblastic differentiation, sometimes predominant, this tumour may pose some problems of differential diagnosis with chondrosarcoma or pleomorphic adenoma: immunochemistry may be necessary. This localization does not change the clinical, radiologic, histologic and prognostic features of periosteal osteosarcoma.
Moloney murine sarcoma virus (M-MSV) was injected directly into the fetuses of Sprague-Dawley rats during the late stage of gestation and into the neonates within 24 hours after birth. Ninety rats developed 188 neoplastic lesions during the 8-week period of observation. Nearly all of the neoplasms were of mesenchymal derivation. Sixty percent of these neoplasms revealed more complex histologic features than those previously reported for neoplasms induced in rodents with M-MSV and were designated "malignant mesenchymoma" which developed preferentially in the proximal parts of the extremities, distant from the inoculation site. Rhabdomyosarcoma and osteosarcoma which developed in a pure form at the various sites were the next most common tumor type. Osteosarcoma developing in a pure form and as a component of malignant mesenchymoma in the humerus and femur was comparable to that of juxtacortical osteosarcoma of man; The development of excessive bones were observed in the forelimb and/or hind leg, suggesting a type of skeletal malformation. The reaction to M-MSV merits attention as a model for the study of an osteosarcoma and malignant mesenchymoma as well as rhabdomyosarcoma and also for the study of viral teratogenesis in man, as "rubella syndrome".
This article focuses on major clinical and imaging features that are of practical interest in the diagnosis and management of osteosarcoma, a malignant tumor arising from the osteogenic matrix. The current histologic classification of this tumor is also reported. Different types of osteosarcoma are described, each of them with a definite clinical and radiographic pattern. Conventional radiography is the keystone to diagnosis because it allows analysis of the patterns relevant to the different lesions (location, site, bone destruction, periostal reaction, soft tissue masses). The most common type of osteosarcoma is defined classic or conventional high grade (75%) and it typically involves the medullary cavity. Radiographically, it may be predominantly osteosclerotic or osteolytic, but more frequently it has a mixed (osteoslerotic/osteolytic) pattern. The teleangiectatic osteosarcoma is an aggressive form (5%) characterized by marked vascularization with large blood-filled cystic cavities; its typical radiographic pattern is purely osteolytic. Juxtacortical osteosarcoma (8-10%) indicates a group of osteosarcomas apparently arising on bone surface. The most common type is parosteal osteosarcoma which affects older subjects and has a better prognosis than the classic type. Radiography shows a heavily ossified mass with a broad base attached to the underlying cortex. CT and MRI are useful in the differential diagnosis of osteosarcoma and myositis ossificans or osteocondroma. Rare types of osteosarcoma include the periosteal and high-grade surface variants, as well as secondary and multifocal osteosarcoma (osteosarcomatosis). CT and MRI are the imaging procedures of choice in locoregional staging (intraosseous and extraosseous spread, skip metastases, growth plate and articular involvement). CT of the chest is a useful tool for detecting lung metastases. Also MRI has a role in monitoring the response to chemotherapy and in detecting recurrence. It permits a more accurate study of the tumor volume than other imaging techniques and clinical examination. MRI becomes even more useful when paramagnetic contrast agents are administered because dynamic MRI with contrast enhancement help differentiate postchemotherapy changes from viable tumor--the latter enhancing rapidly and the former slowly. Thus, dynamic MRI allows a precise mapping of any residual tumor activity.
Malignant bone-forming tumors that arise from the surfaces of long bones are far less common than those that arise from within bone. These surface osteosarcomas are clinically and radiographically similar, yet histologically they are quite distinct. In reviewing the literature, we classified the tumors according to three subgroups: parosteal (juxtacortical) osteosarcoma, periosteal osteosarcoma, and high-grade surface osteosarcoma. We also studied the clinical, radiographic, and histological findings in eighty patients who had been treated for an osteosarcoma over a forty-four-year period. Adequate follow-up data existed for forty-eight of the eighty patients. The duration of follow-up ranged from two to fifteen years after the initial operation (amputation or resection). Patients who had a parosteal osteosarcoma had the best prognosis; those who had a periosteal osteosarcoma, the next best; and those who had a high-grade surface osteosarcoma, the poorest. Because of these widely varying prognoses, the lesions require different treatment.
The appearance of a sarcoma of bone is a well-recognized complication of Paget's disease. The most common type of such a sarcoma is osteosarcoma. Much less common are soft tissue lesions adjoining the pagetic bone that clinically and radiologically simulate sarcoma but histologically represent exaggerated periosteal bone formation as a manifestation of the basic pathologic process. We present a case of a bulky juxtacortical soft tissue mass in the thigh arising from a pagetic femur in a 62-year-old patient with polyostotic Paget's disease that was clinically and radiologically suspected to be a juxtacortical osteosarcoma. Microscopically, the lesion showed features of florid Paget's disease without any evidence of sarcomatous growth. It is important to be aware of this rare manifestation of Paget's disease to avoid unnecessary overtreatment.
A patient with juxtacortical osteosarcoma of the upper extremity of the left tibia which encased the popliteal neurovascular bundle was treated by monobloc resection of the superior extremity of the tibia and the tibial nerve. The knee joint was replaced by a Guepar prosthetic knee. Arterial and venous continuity was reestablished by a double bypass using the contralateral greater saphenous vein. One year later the patient had good lower limb function and was free of local or general disease. This observation confirms that preservation of lower limb function is possible using reconstruction techniques of bone, nerves, and vessels in sarcoma of the musculoskeletal system.
Five cases of calcifying tendinitis of the femur are presented. Diagnosis were proved histologically or catamnestically. The calcifying tendinitis occurred secondary to localized regressive changes in 3 patients; in 2 cases systemic inflammatory disease had to be assumed. In patients with unclear femoral pain and radiologically visible calcifications, especially at the linea aspera, one should include a calcifying tendinitis in the differential diagnosis besides juxtacortical osteosarcoma and myositis ossificans. This entity can sometimes be a symptom of systemic disease such as psoriasis and palmoplantar pustulosis.
Seven samples from seven patients with juxtacortical osteosarcomas, and 27 samples from 19 patients with conventional high-grade osteosarcomas were investigated for a possible correlation between telomerase activity and clinicopathological features such as age, sex, and response to chemotherapy. Of seven juxtacortical osteosarcomas, telomerase activity was weakly positive in three parosteal osteosarcomas, and highly positive in one parosteal osteosarcoma. In contrast, of 27 conventional high-grade osteosarcomas, telomerase activity was weakly positive in eight tumors and highly positive in three. Of all samples, 44.1% of the osteosarcomas showed telomerase activity (57.1% of juxtacortical and 40.7% of conventional osteosarcomas). The majority of poor responders to chemotherapy showed no telomerase activity (nine of 11), whereas five of seven good responders showed strong or weak telomerase activity. There was a significant correlation between telomerase activity and the response to chemotherapy (P<0.05). Telomerase activity was not correlated with MIB-1 proliferation index, age at the time of surgery, or sex. These findings suggest that telomerase activation occurs early in the oncogenesis of osteoblastic tumors without having an effect on the progression of these tumors. In malignant osteoblastic tumors, the biological significance of telomerase activation is different from that described for most epithelial cancers.
Two cases of osteosarcoma of the jaws in children are reported. One patient was a 13-year-old girl whose first symptoms included nasal and maxillary sinus congestion, followed by epistaxis. She was found to have chondroblastic osteosarcoma extending through the left maxillary alveolar process and sinus. Following surgery and chemotherapy, the patient has been free of disease for 7 years. The second patient, an 8-year-old boy, was diagnosed with juxtacortical (parosteal) osteosarcoma of the mandible, which is a less aggressive variant of the neoplasm. It is believed that this is the youngest patient reported to date with juxtacortical osteosarcoma of the jaws. He was treated by block resection of the right side of the mandible and is free of disease 3(1/2) years later.