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PubMed1997

Osteosarcoma of the meninges in association with glioblastoma.

Primary intracranial osteosarcoma not originating in the skull is a distinctly rare tumour, as is post-irradiation sarcoma of short latency. The authors report the case of a 56 year old caucasian male who underwent resection of a glioblastoma of the left temporal region and was subsequently administered partial field external beam radiation therapy (XRT) to a total dose of 5940 cGy. Seven months following the completion of XRT, an enhancing region adjacent to the surgical site was noted on followup magnetic resonance images (MRI), one which increased in size on serial studies. Initial biopsy of the dural lesion adjacent to the temporal resection site revealed a sarcoma with a suggestion of osseous differentiation. Subsequent reoperation with resection of the lesion showed it to be a primary meningeal tumour, and histological evaluation of the lesion demonstrated an osteosarcoma. Immunohistochemical staining for p53 protein performed on both the original glioblastoma and the subsequently resected osteosarcoma showed widespread nuclear positivity. The clinical, radiographic and pathologic features of this unusual case are discussed. Meningeal osteosarcoma should be included among the rare secondary sarcomas of the meninges which may be associated with malignant glioma.

Bone Neoplasms↗

PubMed2000

Leptin and apoptosis inhibitor soluble Fas antigen in the serum of patients with osteosarcoma and neuroectodermal bone tumors.

Serum contents of leptin and soluble Fas antigen were measured in 28 patients with osteosarcoma, 7 with neuroectodermal bone tumors, and 17 healthy subjects. The incidence and levels of soluble Fas antigen in patients with osteosarcoma and neuroectodermal bone tumors were higher than in healthy subjects and did not depend on sex and age in both healthy subjects and patients. Serum concentration of leptin in women was higher than in men (both in healthy controls and patients) and was lower in healthy subjects compared to patients with osteosarcoma and neuroectodermal bone tumors. A trend to a negative correlation between the concentrations of leptin and soluble Fas antigen in female patients with osteosarcoma and male patients with neuroectodermal bone tumors was revealed. The role of leptin and soluble Fas antigen in the pathogenesis of primary bone tumors is discussed.

Adolescent↗

PubMed1983

Cultivation of human osteosarcoma cell lines in serum-free hormone supplemented medium.

Two human osteosarcoma cell lines (TE-85, LM-1) were adapted to grow in serum-free hydrocortisone supplemented medium. Human osteosarcoma associated antigens were detected both on the cell membrane as well as in the concentrated culture medium. TE-85 cells produced bone specific alkaline phosphatase and LM-1 cells produced both bone specific and placental-like alkaline phosphatases when cultured in this medium. Successful proliferation of human osteosarcoma cell lines in serum-free medium is valuable in human osteosarcoma research.

Alkaline Phosphatase↗

PubMed2002

[Multimodal interdisciplinary therapy to improve function and long-term survival in patients with high-grade osteosarcoma of the extremities].

INTRODUCTION: The probability for survival of patients with highly malignant osteosarcoma of the extremities was essentially improved by (neo-)adjuvant chemotherapy. The goal was to further improve survival rates by introducing operative treatment of metastases. In the last 20 years, the percentage of limb-preserving operations has increased. PATIENTS AND METHODS: A consecutive series of 23 patients with localized highly malignant osteosarcoma of the extremities received (neo-) adjuvant chemotherapy according to the Cooperative Osteosarcoma Study (COSS) protocol. Local treatment was performed by wide (22 patients) or radical (1 patient) resection (17 limb salvage procedures, 5 amputations, and 1 rotationplasty). In four of seven patients who developed pulmonary metastases, the metastases (up to four) were resected. The patients did not receive salvage chemotherapy. RESULTS: The cumulative survival was 87% at 174 months. The four patients who underwent metastasectomy survived between 42 and 116 months without evidence of disease. One local recurrence developed outside the operative field and could be resected without influencing the functional or oncological outcome. After limb salvage procedures, the mean score according to the Musculoskeletal Tumor Society (MSTS) was 83%, and after rotationplasty 67%. CONCLUSIONS: In patients with osteosarcoma of the extremities, limb salvage is possible in a high percentage of cases with a low risk of local recurrence by an effective interdisciplinary cooperation between diagnostic radiologists, oncologists, and orthopedic surgeons. The midterm survival could be improved by the multimodal therapy from about 20% up to about 80%. Metastasectomy gives patients with a limited number of pulmonary metastases a realistic chance to survive.

Adolescent↗

PubMed2004

[Osteosarcoma of the pelvisTwo case reports and review of the literature].

Primary malignant neoplasms of the bone are rare. One of the most common bone tumors is osteosarcoma, most often localized in the metaphysis of adolescent long bones. The sacrum is concerned in only about 1%. Primary bone sarcomas of the pelvis are usually recognized after they have grown to a considerable size. Osteosarcoma patients from Germany, Austria, and Switzerland are treated by neoadjuvant chemotherapy combined with complete surgical excision according to the Cooperative Osteosarcoma Study Group (COSS). Based on a review of the literature, diagnosis, treatment, and complications of two cases of sacrum osteosarcoma are introduced.

Adult↗

PubMed2002

[Parosteal osteosarcoma (POS). Clinical and therapeutic aspects].

Parosteal osteosarcoma is a malignant bone-forming tumor, which is characterized by its superficial origin and its high structural differentiation. Because of the radiological and histological variability, finding the right diagnosis is a great challenge for physicians, radiologists, and pathologists, especially at the time of primary manifestation. Usually there is a low-grade malignancy. Often a benign tumor is imitated so that finding the correct diagnosis is indispensable. Wide resection with sufficient margin is the adequate therapy. High-grade parosteal osteosarcoma needs adjuvant chemotherapy. Our own experience with secondary dedifferentiation and the possibility of primary undergrading shows that regarding diagnostics, operative therapy, and follow-up parosteal osteosarcoma should be treated like conventional osteosarcoma.

Adolescent↗

PubMed2000

Accuracy of MR imaging for detecting epiphyseal extension of osteosarcoma.

BACKGROUND: Too few patients are receiving epiphyseal-sparing limb salvage procedures for osteosarcoma. OBJECTIVE: To determine how magnetic resonance (MR) imaging can best predict the epiphyseal extension of osteosarcoma. MATERIALS AND METHODS: Forty children underwent complete pretreatment static and dynamic contrast-enhanced MR imaging (DEMRI). Static MR images [T1-weighted and short tau inversion recovery (STIR)] of the epiphyses were read in three ways: (1) for suspicion of any abnormality (tumor or edema), (2) for suspicion of tumor, excluding suspected edema, and (3) validating the second method by using a scale to rate the likelihood of tumor. Presentation imaging was compared to histopathologic findings after chemotherapy and resection. The receiver operating characteristic (ROC) method was used to analyze the scaled ratings of static MR and DEMRI values. RESULTS: At delayed resection, 20 of 40 children with osteosarcoma had confirmed epiphyseal tumor; however, 32 epiphyses were abnormal on STIR and 28 abnormal on T1. Differentiating suspected tumor from edema increased the accuracy to an Az (area under the ROC curve) of 0.94 for both T1-weighted and STIR static sequences. T1-weighted MR had better specificity and STIR better sensitivity at any given rating. DEMRI was slightly less accurate (Az = 0.90). CONCLUSION: Static MR imaging most accurately detected epiphyseal extension of osteosarcoma when readers distinguished suspected tumor from edematous or normal tissue.

Adolescent↗

PubMed2002

Low-grade osteosarcoma of the maxillary sinus.

Low-grade osteosarcomas of the paranasal sinus are extremely rare. Most reported cases of maxillary sinus osteosarcoma are high-grade. We present a 51-year-old man with a low-grade osteosarcoma which was histologically difficult to diagnose in isolation since it had bland features. The presence of an aggressive destructive lesion on imaging influenced the final pathological diagnosis as often appears to be the case with this rare tumor. We briefly discuss paranasal sinus osteosarcomas.

Humans↗

PubMed2004

Osteosarcoma associated with hyperparathyroidism.

The fourth case in the literature is presented of a patient with the rare association of hyperparathyroidism and osteosarcoma. A 56-year-old woman presented with hyperparathyroidism and a lesion in the tibia. Initial diagnosis was brown tumor. Histology, however, revealed osteosarcoma, and the patient was treated accordingly. The experimental induction of osteosarcoma by parathormone in rodent studies makes this finding alarming, considering the increasing use of parathormone in the treatment of osteoporosis. The mechanism by which osteosarcoma is induced in humans cannot be explained based on current knowledge of mechanisms of action of parathyroid hormone.

Bone Neoplasms↗

PubMed2006

Current strategies of chemotherapy in osteosarcoma.

Osteosarcoma, the most common type of primary malignant tumour that develops in bone, can be classified into several different sub-types. While the majority of osteosarcomas are highly malignant, there are some low-grade variants. Since the introduction of chemotherapy into the multi-modal treatment regimen of high-grade osteosarcoma, its prognosis has impressively improved, with long-term survival being achieved in two-thirds of all patients. This review summarises current chemotherapeutic treatment strategies in classical osteosarcoma and also addresses the indication for chemotherapy in more unusual types and variants. It emphasises the need for treatment in specialised centres and within prospective, multi-institutional trials, amongst which EURAMOS1 and EURO-B.O.S.S are currently active in many European countries.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed2002

[Expression analysis of protein tyrosine kinases of the FAK (focal adhesion kinase) family in osteosarcoma].

AIMS: Expression analysis of the protein tyrosine kinases, focal adhesion kinase (FAK) and proline-rich tyrosine kinase2 (Pyk2) in high grade osteosarcomas. MATERIALS AND METHODS: Expression of the kinases was evaluated qualitatively by immunohistochemical staining and quantitatively by real-time PCR. RESULTS: Osteoblastic cells of high grade osteosarcomas show a distinct FAK expression but an overexpression at the transcriptional level could not be detected. The Pyk2-mRNA expression was decreased in osteosarcomas. CONCLUSION: An altered relationship of FAK and Pyk2 was observed for different tumors and could also be important for osteosarcoma development.

Adolescent↗

PubMed1994

[Osteosarcoma--apoptosis and proliferation. Study of bcl-2 expression].

The relationship between the growth of tumors and the expression of the protooncogene Bcl-2 could be shown in epithelial tumors. A bcl-2 expression leads to a prolonged cell survival due to an inhibition of apoptosis. The potential meaning of bcl-2 expression in mesenchymal tumors remains still unknown. The fact, that the heterogenous group of osteosarcoma is not sufficiently characterized at present, suggested to investigate the bcl-2 expression in osteosarcoma. Thus, immunohistochemistry was used to analyze 47 specimens of different osteosarcomas of 36 patients. Sixteen cases (46%) showed a strong expression of bcl-2 and 13 cases (35%) were moderately positive for bcl-2. Seven cases (19%) were negative for bcl-2. The heterogenous, negative up to strong expression of bcl-2 yield clues, that the Bcl-2 controlled regulation of programmed cell death could be an important factor of cellular kinetics. Additionally the cellular proliferation rate was determined with the monoclonal antibody MIB 1, directed against the Ki-67 epitope. The data of bcl-2 expression and cellular proliferation rate lead to a classification correlating with the histological classification. To verify the importance of apoptosis in the genesis of mesenchymal tumors and whether Bcl-2 may play an important role as a predictive factor for the prognosis of osteosarcoma, further investigations will be needed.

Adolescent↗

PubMed2001

Imaging recurrent parosteal osteosarcoma.

The aim of this study was to document the imaging features of recurrent parosteal osteosarcoma. The clinical and imaging records of 33 patients with a parosteal osteosarcoma referred to an orthopaedic oncology service over a 17-year period were retrospectively reviewed. The mode of identification of locally recurrent tumour was noted, together with the management and clinical outcome. Five patients developed a local recurrence of their parosteal osteosarcoma ranging from 6 months to 10 years after initial surgery. In 4 patients the recurrence was first suspected clinically due to the development of a mass. In the fifth patient recurrence was first detected on routine follow-up radiography. In 4 patients the recurrence could be identified on radiography as a mineralized mass. All the recurrences were readily identified on MR imaging, despite artefacts from prostheses. The recurrences were also evident in the 3 cases in which bone scintigraphy was performed. Local recurrence of parosteal osteosarcoma is adequately detected with a combination of clinical examination and conventional radiography. MR imaging is required to stage local recurrence or where radiography has failed to confirm clinically suspected recurrence. The routine use of MR imaging to follow-up patients is of doubtful value because of the frequently long time between initial surgery and relapse.

Adolescent↗

PubMed1999

Assessment of osteosarcoma response to neoadjuvant chemotherapy: comparative usefulness of dynamic gadolinium-enhanced spin-echo magnetic resonance imaging and technetium-99m skeletal angioscintigraphy.

The aim of this work was to study and compare the usefulness of dynamic contrast-enhanced spin-echo MR imaging with high temporal resolution hydroxymethylene diphosphonate technetium-99 m skeletal angioscintigraphy in predicting the osteosarcoma histological response to neoadjuvant chemotherapy. Twelve patients with resectable osteosarcoma were prospectively monitored with dynamic MR imaging and skeletal scintigraphy before start of neoadjuvant chemotherapy, after two cycles of therapy and before surgery. Neoplasm signal intensity and activity intensity were plotted against time, and slopes were calculated for percentage increase over baseline values in the first minute. Stability and increase in slope values during or after chemotherapy were defined as a "radiological non-response". Changes in slopes were compared with the "histological response" (Huvos grading). At midpoint of the chemotherapy, these two imaging modalities failed in predicting final histological response. After the completion of the chemotherapy, these imaging modalities allowed the prediction of histological response with the same accuracy (91 %). In this series, dynamic MR imaging and technetium skeletal scintigraphy provide similar results regarding the prediction of final histological response during neoadjuvant chemotherapy; these results cannot be used to modify the therapeutic protocol at midpoint of chemotherapy; these imaging tools predict accurately the histological response at the end of chemotherapy. These latter results may permit anticipation of the adjuvant chemotherapy strategy during decalcification procedures in resected osteosarcoma and thus to monitor chemotherapy in non-surgical osteosarcoma.

Adolescent↗

PubMed2000

Human leukocyte antigen (HLA) phenotypes in siblings with osteosarcoma.

Osteosarcoma was detected in two siblings. Their human leukocyte antigen (HLA) phenotypes were completely identical, although they were different from those of osteosarcoma patients in previous reports. Despite an extensive search of family and past history, no significant background related to the induction of cancer could be found. These cases suggest that genetic similarity may influence the development of osteosarcoma. Ascertainment of the HLA phenotypes in siblings with osteosarcoma might be a useful strategy to facilitate the early diagnosis of this tumor.

Adolescent↗

PubMed2004

Primary testicular osteosarcoma with hydrocele.

Primary testicular osteosarcoma is an extremely rare malignancy. To date, only two cases have been reported. Here, we report a third case of primary testicular osteosarcoma complicated with hydrocele. A 78-year-old man presented with right scrotal swelling. Ultrasonography revealed hydrocele and a testicular heterogeneous solid mass with focal calcification in the right testis. Right inguinal orchiectomy revealed a pure intratesticular osteosarcoma. Retroperitoneal lymph-node dissection revealed no metastasis. Thorough sampling of the tumor failed to show any additional histological components. It is unlikely that our case arose from teratoma or mixed sex-cord/stromal tumor, because no other neoplastic elements were identified in whole sampling of the tumor. He remained well without evidence of disease 44 months after operation. This case illustrates that primary pure testicular osteosarcoma may be associated with a favorable prognosis.

Aged↗

PubMed1998

The operative management of a malignant proximal humerus tumor represented by secondary Paget's osteosarcoma.

PURPOSE: Malignant tumors at the proximal humerus are an operative challenge. Radical removal is a principal of tumor surgery but as much functionality as possible should be retained. These conditions often conflict so a compromise has to be reached. This paper proposes a solution to this dilemma, introducing an operative approach, with a new modular prosthesis, to a secondary Paget's osteosarcoma in the proximal humerus. METHODS AND RESULTS: A recently developed humerus modular prosthesis is described, which has been implanted into a patient with Paget's osteosarcoma. In these, mostly elderly, patients, successful operative therapy should combine radical removal with early mobilization. Paget's osteosarcoma is a recognized complication of the disease, and its prognosis is poor. In our patient, the implanted humerus prosthesis allowed a limb-saving procedure to be combined with radical removal of the tumor and postoperative early mobilization. A 3-month follow-up yielded good results with no recurrence of the disease and the patient had satisfactory movement. She was able to resume normal daily life shortly after the operation. CONCLUSION: Implantation of a modular prosthesis of the humerus may allow radical removal of a malignant tumor in that area while achieving early motion. In the literature, amputation is often advocated, as radical treatment with chemotherapy is not a successful option in this elderly patient group. We think the alternative use of a modular prosthesis of the humerus is possible in selected cases. We have encountered no other case in the literature where a limb-saving procedure attempts to preserve as much functionality as possible in Paget's osteosarcoma.

Aged↗

PubMed1999

Expression of CD44 variants in osteosarcoma.

The standard form of CD44 (CD44H) is a transmembranous glycoprotein, widely distributed on a variety of human lymphoid cells, epithelial cells and tumours. CD44 has many variant forms, which are generated by alternative splicing. In recent years, CD44 has been reported to be related to the degree of tumour differentiation, tumour cell invasion, and metastasis. We investigated 44 tumour specimens in 39 patients with osteosarcoma immunochemically to analyse the expression of CD44 standard (CD44H) and variant exon-encoded gene products (CD44v3, v4, v5, v6, v7, v9, and v10). Furthermore, the relationship between CD44 expression and the clinical outcome of patients with osteosarcoma was analysed. Membrane accentuation and exclusive cytoplasmic reactivity were analysed as separate staining patterns. Tumour cells and some multinucleated giant cells were markedly stained. CD44H, v3, v4, v5, v6, v7, v9, and v10 were expressed in 85%, 49%, 54%, 59%, 46%, 5%, 28%, and 10% of the specimens respectively. The cumulative 5-year metastasis-free survival was 58% in CD44v6-negative cases and 24% in CD44v6-positive cases (P=0.046). However, the cumulative 5-year metastasis-free survival was not significantly different between cases positive and negative for other variants of CD44. Multivariate analysis (Cox proportional-hazard model) with CD44v6 expression (positive or negative), chemotherapy (intensive or non-intensive), tumour site (proximal or distal), and age (at least 30 years or less than 30 years) showed that expression of CD44v6 and chemotherapy were important prognostic factors in patients with osteosarcoma. Overexpression of CD44 isoforms containing variant v6 is correlated with poor prognosis in patients with osteosarcoma.

Adolescent↗