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Low-grade central osteosarcoma (LGCO) is a rare subtype of osteosarcoma, constituting

Osteosarcoma is the classic malignant osteoid-forming bone tumor. The typical clinical presentation, histology, imaging findings, treatment, and prognosis for each subtype of osteosarcoma is provided. Particular emphasis is placed on more current magnetic resonance imaging and nuclear medicine imaging techniques that may soon improve the ability to determine the most appropriate therapy and ultimately improve patient survival.

Summary Aims Syndecan-4 (SDC4) and fibronectin (FN), which belong to the cell adhesion molecules, have been reported to correlate with tumour growth and invasion in various carcinomas. We aimed to investigate the prognostic value of these molecules in osteosarcoma. Methods Using immunohistochemistry, we compared the expression of these molecules in high grade osteosarcoma to low grade central osteosarcoma, osteoid osteoma and normal bone. Further, the expression of SDC4 and FN were analysed with prognostic factors of high grade osteosarcoma. Results In high grade osteosarcoma, SDC4 was expressed in 50 of the 65 samples; of these, 32 of 65 showed strong expression profiles. FN was expressed in 46 of 65 samples, and 29 of 65 had evidence of strong expression of this molecule. SDC4 and FN expression were increased in high grade osteosarcoma as compared to other tissues. Strong SDC4 expression was associated with the occurrence of distant metastasis and a large tumour size, and strong FN expression was associated with the occurrence of distant metastasis. Strong expression of SDC4 or FN was associated with significantly shorter overall survival, respectively. Conslusions Increased expression of SDC4 and FN may be underlying molecular alteration of osteosarcoma which accounts for more aggressive clinical behaviour.

Prosthetic reconstruction in two patients with malignant bone tumors of the distal tibia was conducted. The diagnoses were metastatic bone tumor in one patient and low grade central osteosarcoma in another. The mean duration of follow-up was 5.5 years (3 and 8 years). Reconstruction was achieved using custom-made prosthesis (JMM, Japan Medical Materials), which replaced the distal tibia. In the patient with metastasis, local recurrence occurred 8 months after the primary surgery and the recurrent tumor was resected. Both patients were free from neoplastic disease at the latest follow-up. The average functional scores according to the system of the Musculoskeletal Tumor Society were 25 and 23. Custom-made prostheses allow an early return to functional weight-bearing without major complications. This technique provides a safe and effective method of stabilization for properly selected malignant tumors of the distal tibia.

The ultrastructure of low-grade intraosseous osteosarcoma (LGOS) is not well documented in the literature. Four cases of LGOS are described with an emphasis on its distinguishing characteristics as well those it shares with other lesions. The predominant cells of LGOS are fibroblasts with well-developed rough endoplasmic reticulum and mild focal immunoreactivity to SMA and MSA. Few osteoblasts and myofibroblasts are present. Transition cells between fibroblasts and osteoblasts are also noted. The fibroblasts are closely related to osteoblasts from a histological and functional point of view, and phenotypically are probably modified osteoblasts. Comparative ultrastructural studies between LGOS and fibrous dysplasia (FD) reveal basic similarities, although the cells in LGOS are larger with more of an abundance of organelles. Therefore, accurate differentiation between these two lesions rests at the histological and radiological levels. LGOS and parosteal osteosarcomas (PO) also share similar ultrastructural features. In the case samples in this study, an unusual type of multilayered amorphous material was found in the osteoid matrix of a case of LGOS and one of PO. This probably emphasizes the morphologic similarities between these 2 tumors.

We report a case of low-grade central osteosarcoma of the rib, mimicking fibrous dysplasia protuberans or parosteal osteosarcoma. A 13-year-old boy presented with a densely ossified exophytic tumor in the left sixth rib. The surgically resected rib revealed an ossified tumor that had developed within bone and had formed an exophytic mass. Microscopic examination revealed parosteal osteosarcoma-like low-grade central osteosarcoma. Low-grade central osteosarcomas rarely affect the rib, and exophytic tumor formation is also rare. This tumor required differentiation from fibrous dysplasia protuberans and parosteal osteosarcoma because of its clinical presentation.

Das niedrigmaligne zentrale Osteosarkom ist eine hochdifferenzierte Variante des Osteosarkoms, die haufig fur eine gutartige Knochengeschwulst gehalten wird. In unserer Studie wurden Manner und Frauen ungefahr zu gleichen Teilen betroffen. Als haufigste Lokalisation fanden sich Tibia und Femur. Anhand von 3 Fallberichten wird auf die Besonderheit dieses Tumors eingegangen. Unter Wurdigung der aktuellen Literatur werden die Therapiemasnahmen diskutiert. Eine intralasionale Resektion fuhrte in den meisten Fallen zu Rezidiven, eine weite Resektion fuhrte zu guten Resultaten mit rezidivfreien Verlaufen und muss als die Therapie der Wahl gelten.

Low-grade central osteosarcoma (LGCOS) is a rare subtype of osteosarcoma most commonly found in the long bones, with a predilection for the distal femur and proximal tibia. The radiographic features of low-grade central osteosarcoma are variable. Usually there is some evidence of aggressive behavior

Comparative enzyme immunoassay of matrix metalloproteinases (MMP-2, -7, -9) and their tissue inhibitor-1 (TIMP-1) in the sera of 26 healthy individuals and 54 patients with primary osteal tumors before therapy revealed elevated TIMP-1 levels in the patients with classical central and periosteal osteosarcomas in comparison with the control. In patients, the level of MMP-9 significantly decreased compared to that in healthy individuals, while the levels of MMP-2 and MMP-7 remained unchanged. No differences in serum levels of MMP and TIMP-1 associated with gender, age, primary osteal tumor location and size were detected. Overall 3-year survival of patients with classical central osteosarcoma with serum level of MMP-9 below its median was higher than that of patients with MMP-9 level equal to above the median (90.9 ± 8.7 and 50.8 ± 23%, respectively).

Low-grade central osteosarcoma is a rare variant of osteosarcoma which comprises less than 1-2% of all osteosarcomas. Most low-grade osteosarcomas involve long bones, most commonly distal femur, and proximal tibia. Histologically this tumor is difficult to diagnose, and an unusual location makes this diagnosis even more challenging. Here we report a case of low-grade osteosarcoma presenting as a chest wall mass involving the left 6th–8th ribs. This unusual site of presentation significantly added to the diagnostic difficulties of this rare tumor with challenging histologic features. To the best of our knowledge, only six cases of low-grade central osteosarcoma of the ribs have been reported in the English literature.

We report a case of a 32 year-old male, admitted for a lytic lesion of the distal femur. One month after the first X-ray, clinical and imaging deterioration was evident. Open biopsy revealed fibrous dysplasia. Three months later, the lytic lesion had spread to the whole distal third of the femur reaching the articular cartilage. The malignant clinical and imaging features necessitated excision of the lesion and reconstruction with a custom-made total knee arthroplasty. Intra-operatively, no obvious soft tissue infiltration was evident. Nevertheless, an excision of the distal 15.5 cm of the femur including 3.0 cm of the surrounding muscles was finally performed. The histological examination of the excised specimen revealed central low-grade osteosarcoma. Based on the morphological features of the excised tumor, allied to the clinical findings, the diagnosis of low-grade central osteosarcoma was finally made although characters of a fibrous dysplasia were apparent. Central low-grade osteosarcoma is a rare, well-differentiated sub-type of osteosarcoma, with clinical, imaging, and histological features similar to benign tumours. Thus, initial misdiagnosis is usual with the condition commonly mistaken for fibrous dysplasia. Central low-grade osteosarcoma is usually treated with surgery alone, with rare cases of distal metastases. However, regional recurrence is quite frequent after close margin excision.

Low-grade osteosarcoma, including low-grade central osteosarcoma and parosteal osteosarcoma, is an extremely rare variant, and the diagnosis is occasionally difficult. In this article we present cases of low-grade osteosarcomas that should be reviewed by a clinical oncologist.Nine cases of histologically diagnosed Broder grade 1 osteosarcoma were retrospectively reviewed. The pathological diagnoses included parosteal osteosarcoma, low-grade central osteosarcoma, and low-grade chondroblastic osteosarcoma in four, four, and one cases, respectively.Duration from initial surgical intervention including biopsy to final diagnosis as low-grade osteosarcoma was a mean of 9.4 months. The initial benign diagnoses on biopsy specimens included fibrous dysplasia in three cases, chondroblastoma in one case, and a giant cell tumor in one case. The average number of histological examinations was 1.8. Low-grade osteosarcomas are well suited for biological reconstruction: seven cases were reconstructed by frozen autografts, distraction osteogenesis, or vascularized bone grafts.Low-grade osteosarcomas can be misdiagnosed as benign lesions, especially fibrous dysplasia. If the diagnosis of a low-grade osteosarcoma is not established on the basis of radiologic findings, care should be exercised, even when a biopsy suggests a benign lesion. Low-grade osteosarcomas should be treated with wide excision, even after an intralesional excision. Biological reconstruction might be a better option for low-grade osteosarcomas.

Low-grade central osteosarcoma (LGCO) is a rare variant of osteosarcoma which is difficult to diagnose. If not treated appropriately, the tumour can recur with higher-grade disease. We reviewed our experience of this condition to try and identify factors that could improve both diagnosis and outcome. 18 patients out of 1540 osteosarcoma cases (over 25 years) had LGCO (1.2%). Only 11 patients (61%) were direct primary referrals. Almost 40% (7 of 18) cases were referred after treatment elsewhere when the diagnosis had not been made initially and all presented with local recurrence. Of the 11 who presented primarily, the first biopsy was diagnostic in only 6 (55%) cases. Of the remaining cases, up to three separate biopsies were required before a definitive diagnosis was made. Overall survivorship at 5 years was 90%. 17 patients were treated with limb salvage procedures, and one patient had an amputation. The diagnosis of LGCO remains challenging due to the relatively nonspecific radiological and histological findings. Since treatment of LGCO is so different to a benign lesion, accurate diagnosis is essential. Any difficult or nondiagnostic biopsies of solitary bone lesions should be referred to specialist tumour units for a second opinion.

Low-grade central osteosarcoma is a rare type of osteosarcoma, representing fewer than 2% of all osteosarcomas. Approximately 80% of these tumors are located in the long bones, and they are particularly common in the distal and proximal tibia. However, they are rare in the craniofacial bones, and this tumor type has never been reported to occur in the orbit. In this report, a case of low-grade central osteosarcoma arising from the orbit is presented. The differential diagnoses of this tumor and the surgical results of radical resection and reconstruction are also presented.

To determine the age and gender distribution and imaging features of low-grade central osteosarcoma (LGCOS) of the long bones and pelvis and to discuss our findings in the context of lesions for which LGCOS has been mistaken.We reviewed 99 cases of LGCOS collected between 1919 and 2002 from our institution and pathology consultation files. Adequate imaging was available in 70 cases (36 radiographs only, 17 radiographs/CT, 12 radiographs/MRI, 2 radiographs/CT/MRI, 2 CT only, 1 MRI only, 5 bone scans).Patient average age was 30.1+/-14.2 years, with a slight female predominance. The femur and tibia were the most common long bones involved (29 and 20 each) with the majority of these tumors arising around the knee, followed by the fibula, radius, humerus and ulna (four, three, two and one case each). Flat bones were involved in six cases (three pelvis, one rib, two scapulae). Short tubular bones were involved in five cases (two metatarsal, two phalanges, one clavicle). The lesion extended to the end of the affected long bone in 22 of 59 cases. Lesions were large at presentation (mean 7.9+/-4.6 cm, range 2-24). Four radiographic patterns were identified: lytic with varying amounts of thick and coarse trabeculation ( n=22), predominantly lytic with few thin, incomplete trabecula ( n=21), densely sclerotic ( n=17) and mixed lytic and sclerotic ( n=10). Lesions were benign-appearing overall with focally aggressive features. CT or MRI demonstrated cortical breech or extension into the soft tissues in all cases.LGCOS has a variable appearance on radiographs. A frequent pattern is a slow-growing large intracompartmental fibro-osseous lesion with varying amounts of septal ossification associated with focal areas of aggression. A homogeneously sclerotic pattern was also noted. Imaging with CT or MRI was helpful in every instance in our series in identifying areas of soft tissue extension or cortical disruption suggestive of a low-grade malignancy.

목적: 케모카인 수용체인 CXCR4는 암에서 발현되며 암의 전이에 관여한다고 알려져 있다. 저자들은 통상적인 조직학적으로 고등급인 골육종과 저등급 중심부 골육종에서 CXCR4 발현을 비교하고 CXCR4 발현과 예후 간에 연관성이 있는지 살펴보았다. 대상 및 방법: 총 63명의 골육종 환자에서 CXCR4에 대한 면역조직화학검사를 시행하였고 임상적, 병리학적 인자 및 전체적인 생존율과의 연관성을 살펴보았다. 결과: CXCR4는 통상적인 고등급(조직학적 등급 3 및 4) 골육종의 76.3%에서 발현된 반면 저등급(조직학적 등급 1 및 2) 중심부 골육종의 36%에서 발현되었다. 또한 고등급 골육종의 47.4%에서 미만성으로 발현된 반면 모든 저등급 골육종은 병소에 국한되어 발현되었다. CXCR4 발현은 조직학적 등급과 통계학적으로 유의한 상관관계를 나타내었다(p 【Purpose: The chemokine receptor CXCR4 has been reported to be aberrantly expressed in human cancer and has been shown to participate in cancer metastasis. We compared the expression of CXCR4 in conventional high-grade and low-grade central osteosarcomas, and determined if an association between CXCR4 expression and prognosis could be made. Materials and Methods: We performed the immunohistochemistry for CXCR4 in a total of 63 patients with osteosarcoma and determined the relationships according to the clinicopathologic variables and overall survival rates. Results: CXCR4 was detected in 76.3% of conventional high-grade osteosarcoma patients and in 36% of low-grade central osteosarcomas. Diffuse expression was noted in 47.4% of the high-grade osteosarcomas and all low-grade cases were focal positive. CXCR4 expression was significantly correlated with histologic grade (p】

Osteoblastoma (OB) is a rare benign osteogenic tumour formed by interwoven with one another osteal trabeculae, which are surrounded by a lot of osteoblasts. The purpose of the present work was to elucidate the nomenclature and main clinical-visualizing pathohistologic features of OB as a true osteogenic tumor, as well as some aspects of its differential diagnosis with other osteogenic tumors and tumor-like bone lesions. Forty-three cases of biopsies obtained from 39 patients with OB served as the material for this research. The article contains data about clinical-visualizing features of OB (X-ray and CT), its macropathology, histopathology and differential diagnosis, as well as clinical-morphological gradations of osteogenic tumours. It was found out that OB passed clinically as an active or aggressive form with signs of a local destructive growth and inclined to relapsing in case of its incomplete removal, but very seldom it subjected to malignancy. OB often becomes an object of complicated differential-diagnostic solutions, which are possible only with application of both clinical-visualizing and histological methods with priority of the latter ones. The nosological units for differential diagnosis of OB should include the following ones: osteoid-osteoma, ossifying periostitis, chronic osteomyelitis (corticolitis-periostitis), osteoblastoma-like central osteosarcoma with a low grade of malignancy.

Background High-grade foci (grade 3 according to Broder’s grading system) are sometimes detected in low-grade (grade 1 and 2) central osteosarcoma. The aim of this study was to retrospectively evaluate the clinical outcome in patients upgraded to high grade (grade 3) after a first diagnosis of low-grade osteosarcoma, following the detection of high-grade areas (grade 3) in the resected specimen. Methods Of the 132 patients with a diagnosis of low-grade central osteosarcoma at surgical biopsy at our Institute, 33 patients were considered eligible for the study. Results Median age was 37 (range 13–58 years). Location was in an extremity in 29 patients (88 %). Post-operative chemotherapy was given in 22 (67 %) patients. Follow-up data were available for all patients, with a median observation time of 115 months (range 4–322 months). After histological revision, areas of high-grade (grade 3) osteosarcoma accounting for less than 50 % of the tumor were found in 20 (61 %) patients, whereas the majority of the tumor was composed of a high-grade (grade 3) component in 13 (39 %) patients. In the 20 cases of low-grade osteosarcoma with high-grade foci (grade 3) in less than 50 % of the tumor, 9 patients did not receive adjuvant chemotherapy; only one of them died, of unrelated causes. In the adjuvant chemotherapy group (11 out of 20 patients), one patient developed multiple lung metastases and died of disease 39 months after the first diagnosis. In the other 13 cases of low-grade osteosarcoma with high-grade foci (grade 3) in more than 50 % of the tumor, 12 patients received adjuvant chemotherapy: 2 had recurrence, 4 developed multiple lung metastases and 3 died of disease. The only patient who did not receive chemotherapy is alive without disease 232 months after complete surgical remission. Conclusion Our data indicate that patients with a diagnosis of low-grade osteosarcoma where the high-grade (grade 3) component is lower than 50 % of the resected specimen, may not require chemotherapy, achieving high survival rates by means of complete surgical resection only.