Extraskeletal Osteosarcoma Research Articles

Prognostic factors analysis of extraskeletal osteosarcoma: Updated results of an EMSOS study.

10526 Background: Extra Skeletal Osteosarcoma (ESO) account 1% among soft tissue sarcomas. There is no agreement on the best strategy of treatment. Methods: An EMSOS collaborative study was perform...

Extraskeletal osteosarcoma of the hand: the role of marginal excision and adjuvant radiation therapy.

Extraskeletal osteosarcoma of the hand is rare, and its optimal modality of local control is not currently known. A literature search was performed to identify studies that describe the treatment and outcomes of extraskeletal osteosarcoma. A second literature search was performed to identify studies that describe the treatment and outcomes of extraskeletal osteosarcoma of the hand specifically. The role of adjuvant radiation for extraskeletal osteosarcoma is not well defined. All cases in the literature describing treatment of extraskeletal osteosarcoma of the hand utilized amputation, and none of the patients described received radiation therapy. However, there are multiple reports showing excellent local control, minimal toxicity, and superior functional outcome with limb conservation and radiation rather than amputation of the hand in pediatric and adult soft tissue sarcoma. For extraskeletal osteosarcoma of the hand, we recommend a treatment approach with the goal of preservation of form and function using limb-sparing surgery and planned postoperative radiation.

Long-term response of gemcitabine plus docetaxel chemotherapy regimen for extraskeletal osteosarcoma: A case report.

Extraskeletal osteosarcomas (EOSs) are rare variants of primary osteosarcoma of the bone, and are defined as sarcomas located in the soft tissues and characterized by osteoid production. EOS exhibits distinctive demographic, imaging and prognostic features compared with osteosarcoma of bone origin. The available data are contradictory with regard to the use of chemotherapy regimens in the management of EOS. The present study describes a case of EOS that progressed following two lines of therapy oriented to soft-tissue and bone sarcoma histology, respectively. As a gemcitabine-docetaxel combination schedule has demonstrated synergistic activity against bone and soft-tissue sarcoma histologies, this chemotherapy regimen was selected as salvage therapy. The treatment was well-tolerated and induced a long lasting partial response for ~14 months. To the best of our knowledge, this is the first report involving the clinical use of this combination regimen for the treatment of EOS. Furthermore, as demonstrated in this report, EOS may maintain relative chemosensitivity, indicating the potential to control advanced disease in the long term and to plan subsequent chemotherapy regimens.

Outcome following treatment of soft tissue and visceral extraskeletal osteosarcoma in 33 dogs: 2008-2013.

Extraskeletal osteosarcoma (EOS) is a rare, highly malignant mesenchymal neoplasm arising from viscera or soft tissues characterised by the formation of osteoid in the absence of bone involvement. Owing to the rarity of these neoplasms very little information exists on treatment outcomes. The purpose of this study was to describe the outcome following surgical treatment of non-mammary and non-thyroidal soft tissue and visceral EOS in dogs. Thirty-three dogs were identified; the most common primary tumour site was the spleen. Dogs that had wide or radical tumour excision had longer survival times compared with dogs that had only marginal tumour excision performed [median survival time of 90 days (range: 0-458 days) versus median survival time of 13 days (range: 0-20 days)]. The use of surgery should be considered in the management of dogs with non-mammary and non-thyroidal soft tissue and visceral EOS.

Dentine matrix protein 1 (DMP-1) is a marker of bone formation and mineralisation in soft tissue tumours.

Dentine matrix protein 1 (DMP-1) is a non-collagenous matrix protein found in dentine and bone. It is highly expressed by osteocytes and has been identified in primary benign and malignant osteogenic bone tumours. Bone formation and matrix mineralisation are seen in a variety of benign and malignant soft tissue tumours and tumour-like lesions, and in this study, we analysed immunohistochemically the DMP-1 expression in a wide range of soft tissue lesions (n = 254) in order to assess whether DMP-1 expression is useful in the histological diagnosis of soft tissue tumours. Matrix staining of DMP-1 was seen in all cases of myositis ossificans, fibro-osseous tumour of the digits, extraskeletal soft tissue osteosarcoma and in most cases of ossifying fibromyxoid tumour. DMP-1 was also noted in dense collagenous connective tissue of mineralising soft tissue lesions such as tumoural calcinosis, dermatomyositis and calcific tendinitis. DMP-1 was expressed in areas of focal ossification and calcification in synovial sarcoma and other soft tissue tumours. With few exceptions, DMP-1 was not expressed in other benign and malignant soft tissue tumours. Our findings indicate that DMP-1 is a matrix marker of bone formation and mineralisation in soft tissue tumours. DMP-1 expression should be particularly useful in distinguishing extraskeletal osteosarcoma and ossifying fibromyxoid tumour from other sarcomas and in identifying areas of osteoid/bone formation and mineralisation in soft tissue tumours.

Radiological Features of a Fibro-Osseous Pseudotumor in the Digit: A Case Report

A fibro-osseous pseudotumor is an extremely rare benign ossifying subcutaneous mass, which usually presents as an ill-defined soft tissue mass with focal calcification, particularly in the proximal phalanx of the digit. It usually occurs in young adults, with a slight predominance in females, and presents as a painful and localized soft tissue swelling of the finger (1, 2). This lesion has been considered to be the subcutaneous counterpart of myositis ossificans, and the clinical and histological features thereof can be confused with those of a malignant tumor, particularly an extraskeletal osteosarcoma. Accurate interpretation of clinical, radiological, and histological features is very important to avoid misdiagnosis and unnecessary amputation (3, 4). A fibro-osseous tumor is benign, and has an excellent prognosis without local reccurence, after complete excision (4, 5). To date, only a few case reports have described the magnetic resonance imaging (MRI) findings of this rare lesion (6, 7), thus less is known about the MRI features of fibro-osseous pseudotumor. Therefore, here, we describe the radiological features, including MRI findings, of a case of a fibro-osseous pseudotumor that involved the subcutaneous tissue of the proximal phalanx of the digit, in a 19-year-old female.

胸壁軟部組織原発骨外性骨肉腫の1例

胸壁軟部組織原発骨外性骨肉腫の1例

経横隔膜裂孔的に急速に胸腔内にまで進展した後腹膜原発骨外性骨肉腫の1切除例

経横隔膜裂孔的に急速に胸腔内にまで進展した後腹膜原発骨外性骨肉腫の1切除例

Gastric wall implantation metastasis of retroperitoneal extraskeletal osteosarcoma: A case report and review of the literature.

Retroperitoneal extraskeletal osteosarcoma (ESOS) is a rare and highly invasive tumor that is usually diagnosed at an advanced stage due to the insidious onset. The present study analyses a case of retroperitoneal ESOS and its clinical, radiological and therapeutic conditions, and also provides a review of the literature. A 52-year-old male was diagnosed with retroperitoneal ESOS. The patient succumbed to the condition one year after the initial surgery. During treatment, the patient underwent two additional surgeries and two courses of chemotherapy. In the present case, a peritoneal metastatic lesion of ESOS was shed from the peritoneum and implanted into the outer membrane of the stomach and metastasis was identified, this has rarely been reported in the literature. Retroperitoneal ESOS should be considered in the differential diagnosis of a retroperitoneal mass in order to facilitate the management of surgery and help determine the appropriate treatment of the disease.

Immunohistochemical and FISH analysis of MDM2 and CDK4 in a dedifferentiated extraskeletal osteosarcoma arising in the vastus lateralis muscle: Differential diagnosis and diagnostic algorithm

Extraskeletal osteosarcoma is a rare neoplasia within the broad differential diagnostic spectrum of calcifying intramuscular lesions. We present a case of a slowly increasing mass within the left vastus lateralis muscle. At first presentation the patient showed a partially calcified well defined mass with a diameter of 5cm and with no direct contact to the femur. A biopsy from the periphery revealed an ossifying lesion compatible with myositis ossificans. The patient returned 18 months later with the lesion having increased to a diameter of 25cm. The resection specimen revealed a well delimitated tumor with a central core of partially necrotic neoplastic bone. Besides, histology showed high mitotic areas with pleomorphic spindle cells and regions with cartilaginous differentiation. Immunohistochemistry demonstrated: vimentin+, CD34−, desmin−, actin−, EMA− and pancytokeratin− with focal S100 protein positivity and a Ki-67 index of 20%. Comparative genomic hybridization (CGH) revealed a gain of chromosomal material on 12q; FISH analyses for the CDK4 and MDM2 region showed high level amplifications. Consequently, a high-grade dedifferentiated extraskeletal osteosarcoma was diagnosed. In conclusion, analysis of the MDM2 and CDK4 status is a powerful and discriminating diagnostic tool to distinguish dedifferentiated extraskeletal osteosarcoma from other benign/malignant ossifying lesions in the skeletal muscle.

Clinical features, outcomes, and prognostic factors in patients with extraskeletal osteosarcoma.

10565 Background: Extraskeletal osteosarcoma (ESOS) is a rare subtype of osteosarcoma. We investigated patient characteristics, overall survival for ESOS as compared to skeletal osteosarcoma, and p...

Oncology

The April 2014 Oncology Roundup360 looks at: Eyeball as good as microscope for tumour margins; when is best to stabilise femoral metastases?; fluorine does not cause bone tumours; whether giant cell tumour of the proximal femur ever successfully managed; extraskeletal osteosarcoma; modular lower limb tumour reconstruction; and observational studies the basis for most bone tumour treatment.

Small Cell Extraskeletal Osteosarcoma: A Rare Case Report

Extraskeletal osteosarcoma is a rare malignant mesenchymal neoplasm and its small cell variant is one among the rarest variant. This article describes a 60-year-old woman presenting with a large, lobulated, painful mass in left thigh with associated history of trauma since 18 months. Her magnetic resonance imaging showed a variegated mixed intensity lesion with associated cystic degeneration, necrosis and matrix arborizing nearby muscles. Fine needle aspiration cytology showed a small cell lesion with very scant osteoid. Tumor was excised and histopathological diagnosis was small cell osteosarcoma involving adjacent muscles and fat with sparing of lymph nodes. The aim of this article is to present the clinical, radiological, cyto-histological and immunohistochemical features of this extremely rare lesion.

An Unusual Case of Extraskeletal Retroperitoneal Osteosarcoma Incidentally Detected by (18)F-FDG PET/CT.

A 20-year-old male patient previously treated with chemotherapy 5 years before for Hodgkin lymphoma underwent fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) for surveillance. After fasting for at least 6 h, the patient underwent intravenous injection of 270 MBq of 18F-FDG. Before injection, the glucose blood levels corresponded to 95 mg/dl. PET images were acquired 1 h after the radiopharmaceutical injection and were interpreted visually and semi-quantitatively by using the maximal standardized uptake value (SUVmax). 18F-FDG PET/CT showed an area of increased radiopharmaceutical uptake corresponding to a 4 cm retroperitoneal calcified lesion (SUVmax, 4.9). No other areas of abnormal 18F-FDG uptake were detected in the rest of the body (Fig. 1). Fig. 1 Whole-body maximum intensity projection (MIP) 18F-FDG PET image (a), axial CT with bone (b) and abdominal (c) windows, and fused PET/CT images in axial (d), sagittal (e) and coronal (f) projections showing an area of increased 18F-FDG uptake (SUVmax, ... Based on this PET/CT finding, the patient underwent biopsy of the retroperitoneal lesion. Histology showed the presence of an extraskeletal retroperitoneal osteosarcoma. Radiologic imaging excluded other sites of disease in the skeleton, but the retroperitoneal lesion infiltrated the inferior vena cava, therefore the patient was referred to chemotherapy. Extraskeletal osteosarcomas are rare and aggressive mesenchymal tumors accounting for less than 4 % of all osteosarcomas and usually affecting individuals beyond the 5th decade of life [1–3]. The diagnosis of these tumors can be very challenging in cases of abdominal or pelvic lesions, often requiring histological confirmation. Such tumors usually presenting as calcified masses may reach enormous sizes before detection because the enlarging mass may not be associated with pain [1–3]. 18F-FDG PET/CT has been demonstrated to be a useful imaging method in the evaluation of patients with osteosarcoma [4]. A previous case reported the usefulness of 18F-FDG PET in the staging of a 58-year-old patient with extraskeletal retroperitoneal osteosarcoma [5]. In our case, 18F-FDG PET/CT has been useful in the incidental detection of an extraskeletal retroperitoneal osteosarcoma in a young adult patient.

SPECT/CT Imaging of Retroperitoneal Extraskeletal Osteosarcoma

The condition of a 52-year-old man was diagnosed with unresectable retroperitoneal extraskeletal osteosarcoma. SPECT/CT demonstrated a retroperitoneal extraskeletal osteosarcoma adjacent to the right upper ureter with large calcifications and increased Tc-MDP accumulation. Multiple hepatic capsular, renal fascial, and peritoneal metastases were noted. Four months later, multiple peritoneal metastases were noted at the second-look surgery. A histopathologic examination confirmed osteosarcoma.

Clinical Features and Outcomes Differ between Skeletal and Extraskeletal Osteosarcoma.

Background. Extraskeletal osteosarcoma (ESOS) is a rare subtype of osteosarcoma. We investigated patient characteristics, overall survival, and prognostic factors in ESOS. Methods. We identified cases of high-grade osteosarcoma with known tissue of origin in the Surveillance, Epidemiology, and End Results database from 1973 to 2009. Demographics were compared using univariate tests. Overall survival was compared with log-rank tests and multivariate analysis using Cox proportional hazards methods. Results. 256/4,173 (6%) patients with high-grade osteosarcoma had ESOS. Patients with ESOS were older, were more likely to have an axial tumor and regional lymph node involvement, and were female. Multivariate analysis showed ESOS to be favorable after controlling for stage, age, tumor site, gender, and year of diagnosis [hazard ratio 0.75 (95% CI 0.62 to 0.90); p = 0.002]. There was an interaction between age and tissue of origin such that older patients with ESOS had superior outcomes compared to older patients with skeletal osteosarcoma. Adverse prognostic factors in ESOS included metastatic disease, larger tumor size, older age, and axial tumor site. Conclusion. Patients with ESOS have distinct clinical features but similar prognostic factors compared to skeletal osteosarcoma. Older patients with ESOS have superior outcomes compared to older patients with skeletal osteosarcoma.

Analysis of Outcomes in Extraskeletal Osteosarcoma

Extraskeletal osteosarcoma is a rare soft-tissue sarcoma about which little is known. The objectives of this study were to describe the clinical features and natural history of extraskeletal osteosarcoma and to investigate factors affecting outcomes. A retrospective review of a prospectively maintained database of patients diagnosed with soft-tissue sarcoma was conducted. Patients with pathologically confirmed extraskeletal osteosarcoma from 1982 to 2012 were identified and were included in the analysis. Medical records were reviewed for clinical features, treatment, and outcomes. Fifty-three patients were identified from the database: forty-two presented with localized disease, two presented with metastatic disease, and nine presented with recurrent (local and/or distant) disease. The median patient age at diagnosis was sixty-four years, with a median follow-up time of thirty-four months (range, one to 290 months) for survivors. Of the fifty-three patients who were identified, forty-one had lesions in the extremities, fifty-one had high-grade lesions, forty had lesions >5 cm, and forty-two had deep lesions. For patients presenting with localized disease, the median survival was 45.8 months with a three-year cumulative incidence of death due to disease of 39%. All patients with localized disease were managed with surgical resection of the primary tumor: nineteen with surgery only, ten with adjuvant radiation, five with adjuvant chemotherapy, and eight with both radiation and chemotherapy. Eighteen patients relapsed: two patients had local recurrences, ten patients had distant metastases, and six patients had local recurrences and distant metastases. In log-rank analysis, patients with superficial tumors and negative margins at resection had a higher three-year event-free survival. No significant association of disease-specific or event-free survival was found with the addition of radiation, chemotherapy, or both to surgery. For patients presenting with localized extraskeletal osteosarcoma, three-year event-free survival was higher for patients with superficial tumors and negative margins at resection. Radiation and chemotherapeutic treatment were not associated with a lower incidence of death due to disease or a longer event-free survival.

A Case of Extraskeletal Osteosarcoma of the External Ear

Extraskeletal osteosarcoma (ESOS) is an extremely rare malignancy arising from the soft tissue. The predominant site of origin of ESOS is the lower limbs, the thigh. ESOS arising in the head and neck region is extremely rare. This is the first report of ESOS of the ear, to the best of our knowledge. We describe a case of ESOS arising from the subcutaneous tissue in the cartilaginous external acoustic meatus. A 53-year-old man consulted our ENT department in the end of May 2009, complaining of inability to insert a cotton bud into the external acoustic meatus. Examination revealed an elastic-hard tumor arising from the posterior wall of the meatus, occluding the cartilaginous meatus. The eardrum was intact. Plain CT of the temporal bone showed calcification of the lesion arising from the meatus. There was no evidence of bone involvement. Biopsy performed in the middle of July revealed suspected chondrosarcoma. At the beginning of August, an operation was performed; the tumor was removed en bloc with the surrounding tissues including a part of the auricular cartilage, the temporal periosteum, and the meatus skin. Pathological examination confirmed the diagnosis of ESOS. At present, the patient is still alive with no evidence of recurrence of the disease.

Intraokulare Manifestation eines Osteosarkoms bei einem Hund

The present case describes the diagnostic and therapeutic procedure of a dog with an intraocular osteosarcoma. According to the results of the diagnostic imaging studies, the tentative diagnosis of an intraocular neoplasm with perforation of the globe and orbital invasion of the tumour was made and an orbital exenteration was performed. The histopathological diagnosis of the extracted organ implied an intraocular, extraskeletal osteosarcoma. Seventy-seven days later the patient displayed an acute paraparesis. Clinical and diagnostic reevaluation using magnetic resonance imaging (MRI) was performed and the dog was euthanized at the owner's request. By means of MRI and necropsy, an additional axial osteosarcoma of the 6th lumbar vertebra and a malignant melanoma of the right tonsil were diagnosed.

Primary duodenal extraskeletal osteosarcoma-a case report

Abstract Extraskeletal osteosarcoma is a rare malignant soft tissue tumour, which represents about 4% of all osteosarcomas and 1% of all soft tissue sarcomas. We present a case of extraskeletal osteosarcoma, albeit at a hitherto