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The Iowa Orthopaedic Journal 2016Extraskeletal osteosarcoma is a rare tumor with a poor prognosis. The purpose of this study is to examine the oncologic outcomes of this disease as they relate to... (Review)
Review
BACKGROUND
Extraskeletal osteosarcoma is a rare tumor with a poor prognosis. The purpose of this study is to examine the oncologic outcomes of this disease as they relate to surgical treatment and use of adjuvant therapies.
METHODS
We retrospectively analyzed all patients treated at our institution for high-grade extraskeletal osteosarcoma of the limb or chest wall. We recorded demographic data, presenting stage, surgical margin, use of adjuvant chemotherapy or radiation, incidence of local recurrence, metastases, and death. Overall and event-free survival were calculated using Kaplan-Meier survival methods.
RESULTS
There were 12 patients treated with primary wide resection or re-excision of a previously operated tumor bed. Four patients presented with metastases. Seven patients received chemotherapy and four patients received radiation therapy. There were two local recurrences, six patients developed new metastases, and nine patients died. There was no difference in overall survival in patients who received chemotherapy. There was, however, a trend towards increased length of survival in patients who received chemotherapy compared to those who did not (16.4 months vs. 9.3 months, p=0.16).
CONCLUSIONS
Despite no difference in overall survival, patients treated with adjuvant chemotherapy have a trend towards increased length of survival. We suggest that extraskeletal osteosarcoma be treated with standard osteosarcoma chemotherapy regimens in addition to wide resection.
Topics: Adult; Aged; Aged, 80 and over; Antineoplastic Agents; Chemotherapy, Adjuvant; Combined Modality Therapy; Female; Humans; Male; Middle Aged; Osteosarcoma; Prognosis; Radiotherapy, Adjuvant; Soft Tissue Neoplasms; Survival Rate; Thigh; Thoracic Wall; Treatment Outcome
PubMed: 27528844
DOI: No ID Found -
Hand (New York, N.Y.) Dec 2015Extraskeletal osteosarcoma of the hand is rare, and its optimal modality of local control is not currently known. (Review)
Review
BACKGROUND
Extraskeletal osteosarcoma of the hand is rare, and its optimal modality of local control is not currently known.
METHODS
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.
RESULTS
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.
CONCLUSION
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.
PubMed: 26568711
DOI: 10.1007/s11552-015-9760-0 -
Molecular and Clinical Oncology May 2020Extraskeletal osteosarcoma is a rare soft tissue sarcoma and typically appears as a solid mass with variable mineralization. A few cases of extraskeletal osteosarcoma...
Extraskeletal osteosarcoma is a rare soft tissue sarcoma and typically appears as a solid mass with variable mineralization. A few cases of extraskeletal osteosarcoma have shown extensive hemorrhagic changes or bleeding due to its high-grade malignancy; however, to the best of our knowledge, no previous reports have described the pathological characteristics of tumors with non-hemorrhagic cystic change. The present report discusses three cases involving cystic lesions with a solid area at the periphery that arose in soft tissues. The large cystic spaces contained only yellowish-brown fluid and little or no blood, with no clear pathological necrotic tissue. The solid component comprised a focal area of osteoid production by highly anaplastic sarcomatous cells. All of the cases showed high-grade malignancy histologically, and the average maximum diameter was approximately 175 mm. The tumors were located at the adductor muscles in two cases and at the intermuscular region of the hamstring muscles in one case. All of the patients consulted a doctor after more than half a year had passed since the recognition of swelling or a mass on their affected extremities. Surgical treatment was performed for all patients. One patient had lymph node metastasis, and another had lymphoedema after surgery, but no histological invasion to the lymphatic ducts was observed in the excised specimen. Degenerative changes in the fluid content over a long time-course as a result of tumor bleeding or necrosis, rather than lymphorrhea by lymphatic channel invasion of the tumor, might have contributed to the formation of the cystic lesion. Only 1 patient who underwent chemotherapy remained clinically disease-free over 10 years after surgery. Chemotherapy regimens for osteosarcoma rather than those for soft tissue sarcoma are mostly effective for extraskeletal osteosarcoma, so the diagnosis by a biopsy is essential. It is important to consider extraskeletal osteosarcoma in the differential diagnosis of soft tissue tumor with cystic form and calcification and a long clinical course before consulting a doctor.
PubMed: 32257205
DOI: 10.3892/mco.2020.2015 -
European Journal of Cancer (Oxford,... Jan 2020The role of chemotherapy (CT) and radiotherapy (RT) for management of extraskeletal osteosarcoma (ESOS) remains controversial. We examined disease outcomes for ESOS...
PURPOSE
The role of chemotherapy (CT) and radiotherapy (RT) for management of extraskeletal osteosarcoma (ESOS) remains controversial. We examined disease outcomes for ESOS patients and investigated the association between CT/RT with recurrence and survival.
PATIENTS AND METHODS
Retrospective review at 25 international sarcoma centers identified patients ≥18 years old treated for ESOS from 1971 to 2016. Patient/tumour characteristics, treatment, local/systemic recurrence, and survival data were collected. Kaplan-Meier survival and Cox proportional-hazards regression and cumulative incidence competing risks analysis were performed.
RESULTS
370 patients with localized ESOS treated definitively with surgery presented with mainly deep tumours (n = 294, 80%). 122 patients underwent surgical resection alone, 96 (26%) also received CT, 70 (19%) RT and 82 (22%) both adjuvants. Five-year survival for patients with localized ESOS was 56% (95% CI 51%-62%). Almost half of patients (n = 173, 47%) developed recurrence: local 9% (35/370), distant 28% (102/370) or both 10% (36/370). Considering death as a competing event, there was no significant difference in cumulative incidence of local or systemic recurrence between patients who received CT, RT, both or neither (local p = 0.50, systemic p = 0.69). Multiple regression Cox analysis showed a significant association between RT and decreased local recurrence (HR 0.46 [95% CI 0.26-0.80], p = 0.01).
CONCLUSION
Although the use of RT significantly decreased local recurrences, CT did not decrease the risk of systemic recurrence, and neither CT, nor RT nor both were associated with improved survival in patients with localized ESOS. Our results do not support the use of CT; however, adjuvant RT demonstrates benefit in patients with locally resectable ESOS.
Topics: Adult; Aged; Aged, 80 and over; Female; Humans; Male; Middle Aged; Osteosarcoma; Prognosis; Retrospective Studies; Risk Factors; Survival Analysis; Young Adult
PubMed: 31806415
DOI: 10.1016/j.ejca.2019.07.029 -
BMC Ophthalmology Oct 2020Extraskeletal osteosarcoma is a malignant tumour composed of an osteoid and/or cartilaginous matrix; it arises in soft tissues without connection to the skeleton, and to... (Review)
Review
BACKGROUND
Extraskeletal osteosarcoma is a malignant tumour composed of an osteoid and/or cartilaginous matrix; it arises in soft tissues without connection to the skeleton, and to our knowledge, this type of tumour is extremely rare.
CASE PRESENTATION
The present study reports a 57-year-old man with primary orbital extraskeletal osteosarcoma who presented with a history of painful swelling in the left orbit that had occurred for 11 months. Imaging of the orbit showed an atypical, well-defined heterogeneous mass attached to the posterior globe of the left orbit. The patient underwent an anterior orbitotomy and complete excision of the tumour. The mass was originated from neither the globe nor the bony orbital wall but from the soft tissue. Histopathology demonstrated an extraskeletal osteosarcoma. After 13 months of follow-up, there was apparent recurrence of the tumour. The medical history showed no complaints of previous trauma or radiotherapy.
CONCLUSIONS
ESOS is a highly malignant tumour. Immunosuppression, trauma and adjuvant radiotherapy are possible predisposing factors in the development of this tumour. Prompt recognition and thorough treatment are essential for preventing orbital lesions and presence of metastasis from other organs.
Topics: Bone Neoplasms; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Orbit; Osteosarcoma; Surgical Flaps
PubMed: 33092564
DOI: 10.1186/s12886-020-01690-9 -
American Journal of Clinical Oncology Sep 2018Extraskeletal osteosarcoma (EO) is a malignant neoplasm that produces osteoid, bone, and chondroid material without direct attachment to bone or periosteum. Surgical...
OBJECTIVES
Extraskeletal osteosarcoma (EO) is a malignant neoplasm that produces osteoid, bone, and chondroid material without direct attachment to bone or periosteum. Surgical resection is the mainstay of treatment; the role of chemotherapy is not well defined. Therefore, we evaluated the impact of chemotherapy in the survival of patients with EO.
METHODS
All EO patients seen at Mayo Clinic between 1990 and 2014 were assessed. Forty-three patients were included after all archived pathology slides were reviewed to confirm the diagnosis of EO.
RESULTS
Of 43 patients, 37 patients had localized disease and 6 patients had metastatic disease at diagnosis. Chemotherapy was used in 73% and 75% of patients, respectively. Chemotherapy was predominantly anthracycline based, and included platinum in 22 patients (84%).Median overall survival (OS) and progression-free survival (PFS) were 50 months (95% confidence interval, 25-99), and 21 months (95% confidence interval, 13-not reached), respectively. There was a trend towards longer OS and PFS in patients who received chemotherapy. Those who received platinum-based therapy had remarkably prolonged OS (median, 182 vs. 18 mo; 5-year, 61% vs. 0%; P=0.01) and PFS (median, not reached vs. 10 mo; 5-year, 56% vs. 0%; P=0.005). Baseline characteristics were similar in the platinum and nonplatinum group.In patients who received chemotherapy, relapse/recurrence rate was lower in the platinum-based group (41%) as opposed to the nonplatinum-based group (100%; P=0.02). In the neoadjuvant setting, the overall response rate of platinum-containing regimens was 27%.
CONCLUSIONS
Our results suggest a clinical benefit when platinum-based chemotherapy is incorporated in the management of patients with EO. We plan to validate this further with an expanded multicenter analysis.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Antineoplastic Combined Chemotherapy Protocols; Bone Neoplasms; Chemotherapy, Adjuvant; Child; Female; Follow-Up Studies; Humans; Male; Middle Aged; Neoadjuvant Therapy; Neoplasm Metastasis; Neoplasm Recurrence, Local; Osteosarcoma; Prognosis; Retrospective Studies; Survival Rate; Young Adult
PubMed: 28622153
DOI: 10.1097/COC.0000000000000397 -
BMC Cancer Jan 2024The aim of this study was to investigate the clinical, imaging and pathological features of extraskeletal osteosarcoma (EOS) and to improve the understanding of this...
OBJECTIVE
The aim of this study was to investigate the clinical, imaging and pathological features of extraskeletal osteosarcoma (EOS) and to improve the understanding of this disease and other similar lesions.
METHODS
The data for 11 patients with pathologically confirmed extraosseous osteosarcoma, including tumour site and size and imaging and clinical manifestations, were analysed retrospectively.
RESULTS
Six patients were male (60%), and 5 were female (40%); patient age ranged from 23 to 76 years (average age 47.1 years). Among the 11 patients, 7 had clear calcifications or ossification with different morphologies, and 2 patients showed a massive mature bone tumour. MRI showed a mixed-signal mass with slightly longer T1 and T2 signals in the tumour parenchyma. Enhanced CT and MRI scans showed enhancement in the parenchyma. Ten patients had different degrees of necrosis and cystic degeneration in the mass, 2 of whom were complicated with haemorrhage, and MRI showed "fluid‒fluid level" signs. Of the 11 patients, five patients survived after surgery, and no obvious recurrence or metastasis was found on imaging examination. One patient died of lung metastasis after surgery, and 2 patients with open biopsy died of disease progression. One patient died of respiratory failure 2 months after operation. 2 patients had positive surgical margins, and 1 had lung metastasis 6 months after operation and died 19 months after operation. Another patient had recurrence 2 months after surgery.
CONCLUSION
The diagnosis of EOS requires a combination of clinical, imaging and histological examinations. Cystic degeneration and necrosis; mineralization is common, especially thick and lumpy mineralization. Extended resection is still the first choice for localized lesions. For patients with positive surgical margins or metastases, adjuvant chemoradiotherapy is needed.
Topics: Humans; Male; Female; Middle Aged; Young Adult; Adult; Aged; Diagnosis, Differential; Margins of Excision; Retrospective Studies; Soft Tissue Neoplasms; Magnetic Resonance Imaging; Osteosarcoma; Lung Neoplasms; Bone Neoplasms; Necrosis
PubMed: 38166700
DOI: 10.1186/s12885-023-11731-3 -
Sarcoma 2014Background. Extraskeletal osteosarcoma (ESOS) is a rare subtype of osteosarcoma. We investigated patient characteristics, overall survival, and prognostic factors in...
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.
PubMed: 25294959
DOI: 10.1155/2014/902620