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Osteosarcomas are rare, highly malignant, bone tumors defined by the presence of malignant mesenchymal cells producing osteoid or immature bone. Osteosarcomas of the jaws are extremely rare, representing about 7% of all osteosarcomas and 1% of all head and neck malignancies. An accurate diagnosis, usually facilitated by chemotherapy (CT), MRI and b...
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Simple Summary
Osteosarcoma is an aggressive cancer of the bone that can present in children or adults. It has historically been thought that adults have worse clinical outcomes than children. However, current treatment strategies for adults are largely extrapolated from pediatric studies since the majority of clinical trials for osteosarcoma treat...
Background:
Artificial intelligence has an emerging progress in diagnostic pathology. A large number of studies of applying deep learning models to histopathological images have been published in recent years. While many studies claim high accuracies, they may fall into the pitfalls of overfitting and lack of generalization due to the high variabi...
Osteosarcoma is a mesenchymally derived, high-grade bone sarcoma that is the most frequently diagnosed primary malignant bone tumor. Today, chemoprevention is regarded as a promising and realistic approach in the prevention of human cancer. Previous studies have suggested ellagic acid (EA) and Sennoside B have potential in this regard. The aim of t...
Osteosarcoma (OS) is a highly aggressive and lethal malignant bone tumor that primarily afflicts children, adolescents, and young adults. However, the molecular mechanisms underlying OS pathogenesis remain obscure. Mounting evidence implicates dysregulated long non-coding RNAs (lncRNAs) in tumorigenesis and progression. These lncRNAs play a pivotal...
Artificial intelligence (AI) has an emerging progress in diagnostic pathology. A large number of studies of applying deep learning models to histopathological images have been published in recent years. While many studies claim high accuracies, they may fall into the pitfalls of overfitting and lack of generalization due to the high variability of...
Citations
... As a rare form of OS, osteosarcoma of the jaw accounts for 7% of all osteosarcomas and 1% of all malignant diseases of the head and neck. Without the appropriate treatment, this malignancy has an unfavorable prognosis [19]. Surgical treatment is one of the main therapeutic options, but it can be a challenging process due to the complex anatomy of the maxillo-facial region. ...
Cancer is a significant health problem worldwide; consequently, new therapeutic alternatives are being investigated, including those found in the vegetable kingdom. Eugenol (Eug) has attracted attention for its therapeutic properties, especially in stomatology. The purpose of this study was to investigate the cytotoxicity of Eug, in vitro, on osteosarcoma (SAOS-2) and oropharyngeal squamous cancer (Detroit-562) cells, as well as its potential irritant effect in ovo at the level of the chorioallantoic membrane (CAM). The data obtained following a 72 h Eug treatment highlighted the reduction in cell viability up to 41% in SAOS-2 cells and up to 37% in Detroit-562 cells, respectively. The apoptotic-like effect of Eug was indicated by the changes in cell morphology and nuclear aspect; the increase in caspase-3/7, -8 and -9 activity; the elevated expression of Bax and Bad genes; and the increase in luminescence signal (indicating phosphatidylserine externalization) that preceded the increase in fluorescence signal (indicating the compromise of membrane integrity). Regarding the vascular effects, slight signs of coagulation and vascular lysis were observed, with an irritation score of 1.69 for Eug 1 mM. Based on these results, the efficiency of Eug in cancer treatment is yet to be clarified.
... The peak incidence of osteosarcoma is second to fourth decades with predilection for male. It occurs most 1 commonly in fast growing bones. The clinical behavior of osteosarcoma of long bone versus jaw bone differs slightly. ...
Osteosarcoma of maxilla in a sero-positive patient : rare case report
... Based on histology, it can be a central (intramedullary) or peripheral (surface) subtype. Based on the predominant type of extracellular matrix, these can have osteoblastic, chondroblastic or fibroblastic differentiation [22]. Mardinger et al. [23] reported the highest prevalence for chondroblastic osteosarcoma and the least for the fibroblastic variant. ...
Purpose of review:
There is no clear consensus guideline that specifies the optimum course of treatment for adult head and neck osteosarcoma (HNO) because of its rarity. The review's goal is to examine the most recent research on the presentation, diagnosis, prognosis, and therapy of head and neck osteosarcoma.
Recent findings:
Due to overlapping symptoms with various benign disorders of the lower jaw and midface bone, these patients present with a noticeable delay. The greatest results for these malignancies can be achieved with surgery with sufficient margins. However, it may not be able to achieve sufficient margins in tumours of the midface and skull base, and the significance of adjuvant radiation/chemotherapy needs to be investigated. The use of adjuvant radiation in instances with an advanced stage, poor prognostic indicators, and inadequate resection is supported by evidence. Nonetheless, there are divergent opinions regarding the advantages of chemotherapy in adjuvant and neoadjuvant conditions, and further multicentric randomized control trials are required to provide robust evidence.
Summary:
Multimodality treatments seem to yield better results for advanced HNO with adverse features and incomplete resections.
... Radiography shows a sclerotic lesion with thickening of the cortex and string sign. Approximately, 10-25% of parosteal osteosarcomas become dedifferentiated into high-grade osteosarcoma with worse survival outcomes [6,7,13]. Periosteal osteosarcoma develops from the inner cambium layer of the periosteum, which contains progenitor cells capable of differentiating into osteoblasts and chondroblasts. ...
... It was obtained from the patient for whom identifying information is uniquely included in this manuscript. a high-grade tumor, identical to conventional osteosarcoma [1,6,7,13]. ...
Introduction: Surface osteosarcomas represent around 3-6% of all osteosarcomas, which include the parosteal (low-grade), periosteal (intermediate-grade) and high-grade surface osteosarcomas. The classical location is the femur, followed by tibia and humerus. Parosteal osteosarcoma is the most common type of surface osteosarcoma. To date, 26 cases of parosteal osteosarcoma affecting the jaws have been reported, with most cases following an evolution time of several months to years, clinically favoring a benign osseous or fibro-osseous lesion. Methods: Here, we report a 39-year-old female who was referred presenting a maxillary tumoral mass 5 years ago, clinically diagnosed as osteoma. After clinical, imaginological and histopathological analysis, a diagnosis of parosteal osteosarcoma was made. Conclusion: Thus, parosteal osteosarcoma should also be considered in the differential diagnosis of benign-appearance, bone-forming nodular lesions affecting the jaws.
... • Werner syndrome: a rare autosomal disorder characterized by features of premature aging that appears in the third decade of life. This disorder is known to present with bilateral cataracts, short stature, graying and thinning of scalp hair, characteristic skin disorders, and increased incidence of specific tumors, including HNOS [13,14]. ...
... The incidence of osteosarcoma secondary to Paget's disease is not known, but it is estimated to be about 1%. This association accounts for about half of the osteosarcomas reported in elderly patients [13]. ...
... Additionally, HNOS has also been associated with trauma, bone infarcts, and chronic osteomyelitis [13]. ...
Osteosarcomas of the head and neck (HNOS) are an infrequent disease, representing less than 10% of all osteosarcomas and 1% of all head and neck cancers. However, they exhibit a different clinical behavior and natural history than extremities osteosarcomas (OS), therefore requiring a specific study and analysis. Specifically, in head and neck sites, OS have shown a different presentation age, reduced likelihood of distant metastases, and a severely higher local recurrence rates. This may be due to the difficulties in ensuring wide negative margins, given the multiple vital structures surrounding tumors in this particular region. This singular features render HNOS a different prognosis and prognostic factors, becoming a topic that should be assessed independently, as they may need a different treatment approach than osteosarcomas of the trunk or extremities.
... High-grade craniofacial osteosarcoma could be treated in the same way as high-grade osteosarcomas at other locations [III, B]. However, some authors defend different approaches, as to initiate with surgery, since the main risk in this location is related to local recurrence (Ferrari et al., 2017). There is not a clear reason to avoid chemotherapy in the context of high-grade craniofacial osteosarcoma, given the high-risk nature of any recurrence, local or distant. ...
Bone sarcoma are infrequent diseases, representing < 0.2% of all adult neoplasms. A multidisciplinary management within reference centers for sarcoma, with discussion of the diagnostic and therapeutic strategies within an expert multidisciplinary tumour board, is essential for these patients, given its heterogeneity and low frequency. This approach leads to an improvement in patient’s outcome, as demonstrated in several studies. The Sarcoma European Latin-American Network (SELNET), aims to improve clinical outcome in sarcoma care, with a special focus in Latin-American countries. These Clinical Practice Guidelines (CPG) have been developed and agreed by a multidisciplinary expert group (including medical and radiation oncologist, surgical oncologist, orthopaedic surgeons, radiologist, pathologist, molecular biologist and representatives of patients advocacy groups) of the SELNET consortium, and are conceived to provide the standard approach to diagnosis, treatment and follow-up of bone sarcoma patients in the Latin-American context.
... Multimodality treatment is recommended for the management of osteosarcomas, including surgery and adjuvant or neoadjuvant chemotherapy with or 13 without radiotherapy . JGCRO, just like GCRO of the temporal bone were treated in the same manner 7,12 as other osteosarcomas . ...
Giant cell-rich osteosarcoma of the jaw (JGCRO) is very rare but it is diagnostically important because of its poor prognosis compared to other variants of osteosarcoma. We report a rare case of recurrent JGCRO initially diagnosed as an ossifying fibroma. The clinical presentation, radiologic and histologic features and the intermediate outcome of treatment of the case are highlighted.
... Multimodality treatment is recommended for the management of osteosarcomas, including surgery and adjuvant or neoadjuvant chemotherapy with or 13 without radiotherapy . JGCRO, just like GCRO of the temporal bone were treated in the same manner 7,12 as other osteosarcomas . ...
Giant cell-rich osteosarcoma of the jaw (JGCRO) is very rare but it is diagnostically important because of its poor prognosis compared to other variants of osteosarcoma. We report a rare case of recurrent JGCRO initially diagnosed as an ossifying fibroma. The clinical presentation, radiologic and histologic features and the intermediate outcome of treatment of the case are highlighted.
... Besides obtaining adequate clean margins being the first goal of surgery, resection of head and neck osteosarcomas requires a careful balance between effective surgery and function-sparing procedures. While the use of preoperative and adjuvant chemotherapy has become the standard of care in long bone osteosarcomas, its role in osteosarcomas of the jaw is still controversial 28 . Now, chemotherapy following wide excision is the recommended treatment protocol for recurrent cases and those exhibiting highly malignant features or dedifferentiation on histological examination [5][6][7]15 . ...
Parosteal osteosarcoma of craniofacial location was first described in 1961 and since then, less than 20 cases have been reported in the English literature. This rare tumor of the jaws most commonly affects middle aged men, is considered a low grade, well‐differentiated neoplasm, characterized by spindle cell stroma with minimal atypia and rare mitotic figures, separating irregular well‐developed trabeculae of woven bone. It is considered to have a favorable prognosis and low metastatic potential if treated by radical excision, compared to conventional and periosteal osteosarcomas. Clinically and radiographically it can be mistaken for a benign lesion, therefore high clinical suspicion is advised. Herein we report a case of parosteal osteosarcoma located at the posterior maxilla in a 14 year old male and review of the literature with respect to clinical, radiographic and histopathological features of these lesions.
... Why MFOS appeared so resistant to neo-CT and different from other locations remained a partially unsettled question [46]. Some large series clearly underlined biologic differences between MFOS and OS of other localization [33] reflecting the molecular heterogeneity of human osteosarcoma [47]. ...
Introduction:
Neoadjuvant chemotherapy (neo-CT) for osteosarcomas is the standard of care. Management of maxillo-facial osteosarcomas (MFOS) is challenging. In this rare disease, we collected a large cohort of patients with the aim to report the histological and radiological local response rates to neo-CT.
Patients and methods:
All consecutive adult patients treated between 2001 and 2016 in two French sarcoma referral centers (Pitié-Salpêtrière Hospital, APHP, RESAP France and Gustave Roussy Institute France), for a histologically proved MFOS were included. Clinical, histological and radiological data were independently reviewed. Tumor response to neo-CT was assessed clinically, radiologically with independent review using RECIST v1.1 criterion and pathologically (percentage of necrosis). Multivariate analysis was done for outcomes, tumor response and disease-free survival (DFS).
Results:
A total of 35 high grade MFOS were collected. The clinical tumor response was 4% (1/24 receiving neo-CT), the radiological response was 0% (0/18 with available data) and the pathological response was 5% (1/20 with available data). Three patients (12.5%) initially resectable became unresectable due to clinical and radiological progression during neo-CT. Tumor size and R0 (clear margins) surgical resections were significantly associated with DFS.
Conclusion:
MFOS is a rare disease. This large retrospective cohort of MFOS indicates the lack of benefit and potentially deleterious effects of neo-CT. We suggest privileging primary surgery in initially localized resectable MFOS. The benefit of adjuvant chemotherapy should be prospectively studied.
