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Osteosarcoma is the most common primary malignancy of the jaws and is treated by radical surgical resection. Early detection is crucial since removing the lesion with clean margins contributes most to outcome. We present three cases of osteosarcoma occurring in the interradicular region of the mandible, two of which were thought to represent non-malignant processes upon initial presentation. All three demonstrated early but significant clinical and radiographic features indicative of the malignant disease process. Radiographically, all of these cases had in common unilateral periodontal ligament (PDL) space widening, displacement of teeth, and growth of the crestal bone. Clinically, all three cases presented with a painless intraoral swelling. Case 1: A 20 year old African American male presented with a swelling and loose teeth of 1.5 months duration. Histopathological diagnosis of osteoblastic osteosarcoma, high grade was made. Case 2: A 75 year old Caucasian male presented with a 2 × 2 cm expansile lesion of two months duration. Histopathological diagnosis of chondroblastic osteosarcoma was made. Case 3: A 63 year old Caucasian female presented with a 5-6 mm lesion for at least 1 month duration. Histopathological diagnosis of chondroblastic osteosarcoma was made. All three cases were treated with wide surgical resection.

The internet and social media give our patients extraordinary access to information; in these unprecedented times of the COVID-19 pandemic, where so much of life takes place online, patients and professionals alike look to the internet more and more for information and (self-)diagnosis. This article details the treatment journey for a patient with a high-grade chondroblastic osteosarcoma of the right maxilla, paranasal area and zygoma, from misdiagnosis through to resection and full rehabilitation with free flap surgery and implant reconstruction. Uniquely, the article details the patient's treatment, in parallel with her own perception of the treatment, as shared on social media., Key points Alerts the reader to the power of social media as a tool to inform and educate the patient and the dental team.Informs the reader of the power of social media as a means for the patient to describe and share their experiences - both positive and negative.Describes contemporary management of an advanced oral malignancy, from the patient's and the surgeon's perspective.

A 76-year-old woman with uterine cervical cancer 20 years ago received right total hip replacement 3 months ago for right hip avascular necrosis without specific intraoperative finding. She reported persistent right hip pain after falling from bed. Pelvic x-ray showed right pubic ramus fracture. To evaluate prosthesis loosening, 99mTc-MDP 3-phase bone scan was arranged, showing diffusely and heterogeneously increased vascularity and tracer perfusion over the right hip, with intensely and heterogeneously increased metabolism in the right iliac bone and hip. SPECT/CT showed nearby swelling of calcified muscles. After debridement and synovectomy, the pathologic report showed chondroblastic osteosarcoma.

Background The establishment of precise and personalized prediction systems for chondroblastic osteosarcoma patients is important for guiding the treatment. Methods The univariate logrank test and multivariate Cox regression analysis were performed to identify independent prognostic factors for overall survival (OS) and cancer-specific survival (CSS). Nomograms were constructed to estimate the OS and CSS based on these factors. Internal and external validation was performed. The predictive power of the nomograms was determined by C-index and calibration plots. Results A total of 401 chondroblastic osteosarcoma cases were identified. Univariate and multivariate analysis revealed that age at diagnosis, histological grade, tumor size, Surveillance, Epidemiology, and End Results stage, and surgical resection were independent prognostic factors for OS and CSS. The five factors were incorporated to construct the nomograms for estimating the 3- and 5-year OS and CSS. The C-index values for the internal validation of the OS and CSS nomogram were 0.732 and 0.746, respectively, and for the external validation were 0.780 and 0.808, respectively. The calibration curves revealed that the predicted OS and CSS could well match the actual survival rate. Conclusions The nomograms for predicting 3- and 5-year OS and CSS were constructed and were proved to be accurate and reliable by the internal and external validation.

Background Primary extraskeletal chondroblastic osteosarcoma has very poor prognosis, especially in the elderly. The pericardium is an extremely rare site for this tumour. Case summary A 67-year-old man presented with a large pericardial effusion and an intrapericardial mass. His past medical history of pulmonary tuberculosis led us to initially suspect tuberculous pericarditis. Primary extraskeletal chondroblastic osteosarcoma arising from the pericardium was diagnosed by the pathologist after surgery. The patient suffered severe intraoperative blood loss and surgical trauma. He went into shock and died a few hours after surgery. Discussion Extraskeletal chondroblastic osteosarcoma is a very rare tumour with a grim prognosis. Clinical manifestations frequently are not specific and can be explained by associated pathology. In this report, we describe an unusual case of primary extraskeletal chondroblastic osteosarcoma located in the pericardium and present a review of the literature.

There are three subtypes of undifferentiated human conventional osteosarcoma (HCOS): osteoblastic osteosarcoma (OOS), chondroblastic osteosarcoma (COS), and fibroblastic osteosarcoma (FOS). HCOS also exhibits heterogeneous pathological maldifferentiation in individual patients. Currently, the mechanism regulating HCOS differentiation remains unclear, and therapies are ineffective. Osteopontin (OPN) and osteocalcin (OCN) are markers of osteoblast maturation, and their expression is inhibited in HCOS. A previous study found that PLK2 inhibited TAp73 phosphorylation and consequent anti‐OS function of TAp73 in OS cells with enriched TAp73. TAp73 was also reported to regulate bone cell calcification. Here, OOS was found to have higher TAp73 levels and PLK2 expression than those in COS, which is correlated with HCOS maldifferentiation according to Spearman analysis and affects patient prognosis according to Kaplan‐Meier survival analysis. In the conventional OS cell‐line Saos2 and in patient‐derived xenograft OS (PDX‐OS) cells, increased PLK2 expression owing to abundant TAp73 levels affected OPN and OCN content as measured by RT‐PCR and Western blotting, and alizarin red staining showed that PLK2 affected calcium deposition in OS cells. In addition, PLK2 inhibition in PDX‐OS cells prohibited clone formation, as indicated by a clonogenic assay, and sensitized OS cells to cisplatin (CDDP) (which consequently limited proliferation), as shown by the CCK‐8 assay. In an established PDX animal model with abundant TAp73 levels, PLK2 inhibition or CDDP treatment prevented tumor growth and prolonged median survival. The combined therapeutic effect of PLK2 inhibition with CDDP treatment was better than that of either monotherapy. These results indicate that increased PLK2 levels due to enriched TAp73 affect osteogenic differentiation and maturation and OS prognosis. In conclusion, PLK2 is a potential target for differentiation therapy of OS with enriched TAp73., This study indicates that PLK2 modulation of highly abundant TAp73 to regulate the clonogenicity, proliferation, osteogenic differentiation, and maturation of OS cells. In addition, PLK2 is a potential therapeutic target in preclinical PDX hosts that can sensitize tumors to the antitumor effect of CDDP and prolong the medium survival of the hosts. In conclusion, PLK2 may be an ideal target for OS differentiation therapy.

INTRODUCTION: Chondroblastic osteosarcoma is an aggressive bone tumor characterized by chondroid matrix production with variable cellularity. This retrospective study is aimed to assess the demographic distribution, clinical behavior and oncological outcome of chondroblastic osteosarcoma. METHODS: 27 patients (14 males, 13 females) with chondroblastic osteosarcoma were included. The site of involvement included: proximal tibia (41%), distal femur (26%) and distal tibia (15%).The mean age of patients was 17 years (S.D: 6.9 years) (range 6–45 years). RESULTS: Painful swelling was the main complaint in all cases. Two patients were classified as Enneking III (lung) stage and the remaining patients had Enneking IIB stage. Post-neoadjuvant chemotherapy limb sparing surgery was performed in 70% of cases while in 30% of cases limb amputation was done. On histopathological examination of specimens mean necrosis was 64% (20–95%). Disease free survival (DFS) at 2 years was 46.4% and at 4 years was 27.9%. Median disease free survival was 24 ± 10.30 months. Overall survival (OS) at 2 years was 53.3% and at 4 years was 16.6%. Median overall survival is 28 ± 5.93 months. CONCLUSIONS: Chondroblastic osteosarcoma is associated with poor prognosis, high recurrence rate and metastatic potential with dismal long-term survival. Chondroblastic osteosarcoma is an aggressive histological variant of conventional osteosarcoma with frequent location around the knee joint, affecting young population. Chemotherapy with Methotrexate, Adriamycin, Cisplatin (MAP) regimen is a well-tolerated regimen, resulting in limb salvage surgery in majority of the cases.

Chondrosarcomas of the spine are rare tumors and represent

Purpose Non-epithelial tumors of the breast are extremely rare and have an incidence of less than 1%. The most common non-epithelial breast tumor is the phyllodes tumor (PT), which accounts for 61%. Primary osteosarcomas of the breast contribute up to only 12.5% of all breast sarcomas. In young females, osteosarcomas are extremely rare, especially in those without a previous history of primary bone osteosarcoma. A case of a 16-year old female with primary osteosarcoma of the breast (POB) with extensive chondroid matrix involving the left breast is herein presented. Case report This report describes a 16-year old female with neither a previous history of bone osteosarcoma nor family history of breast cancer who was diagnosed with a primary chondroblastic osteosarcoma of the left breast. The mass was shining, warm, firm, and slightly fixed. The excisional biopsy showed a large tumor measuring 11x9x7 cm which was encapsulated, grayish-white, and nodular. Conclusion Primary osteosarcomas of the breast carry a poor prognosis by being triple negative and because of being the rarest tumors, they pose a challenge in managing the patients due to lack of established treatment modalities.

Osteosarcoma of the jaw only represents 0.5-1% of tumors of the facial mass. Due to its rarity, clinical diagnosis is thus difficult. The guidelines for this pathology remain unclear, and the need for neoadjuvant chemotherapy is still debated. This case report aims to describe a rare case of chondroblastic osteosarcoma in a 50-year-old woman on risedronate treated by neoadjuvant chemotherapy. The tumor extended from the mandibular left first premolar to the mandibular right canine. An excisional biopsy was performed, leading to a diagnosis of chondroblastic osteosarcoma. Neoadjuvant chemotherapy was ineffective, as it did not result in the shrinkage of the tumor. A pelvi-mandibulectomy with fibula free flap reconstruction of the mandible was subsequently successfully performed followed by radiotherapy.

Head and neck osteosarcomas (HNOS) account for less than 1% of all head and neck cancers and makeup 6-10% of all primary osteosarcomas. Mandibular osteosarcomas are the second most common subtype of HNOS. They demonstrate higher recurrence rates; however, are amenable to surgery. An 18-year-old male presented with a 2 cm x 3 cm x 2 cm intraoral mass for two months. Biopsy revealed chondroblastic osteosarcoma. Computed tomography revealed extension into the left internal jugular vein. Composite resection of the left mandible, floor of the mouth, ventral tongue, submandibular gland, and modified radical neck dissection with fibular flap repair was performed. Adjuvant chemotherapy and palliative radiotherapy were added. Unfortunately, progressive metastasis to the contralateral mandible and entire spinal cord ensued. We report the first case of head and neck osteosarcoma with intravascular invasion into the internal jugular vein.

Key Clinical Message Osteosarcoma is the most common malignancy of mesenchymal cells mostly originating within long bones, but rarely in the jaws. This report illustrates a case of chondroblastic osteosarcoma in the region of anterior maxilla in a 58‐year‐old male patient previously treated for ossifying fibroma of the same site., Osteosarcoma is the most common malignancy of mesenchymal cells mostly originating within long bones, but rarely in the jaws. This report illustrates a case of chondroblastic osteosarcoma in the region of anterior maxilla in a 58‐year‐old male patient previously treated for ossifying fibroma of the same site.

Aims Clear cell chondrosarcomas are known to occasionally contain areas of low-grade conventional chondrosarcoma; however, the opposite phenomenon has not yet been described. We identified five cases of conventional chondrosarcoma alongside clear cell chondrosarcoma. Here, we report on their clinicopathological and molecular characteristics, and investigate whether these hybrid lesions should be considered to be a collision tumour, conventional chondrosarcoma with clear cell change, or clear cell chondrosarcoma with extensive areas of conventional chondrosarcoma, as this has clinical implications. Methods and results Clinicohistopathological features were characterised, immunohistochemistry was performed for H3 histone family member 3B (H3F3B), histone H3 trimethylated on lysine 27 (H3K27me3), and p53, and genetic alterations of IDH1 (encoding isocitrate dehydrogenase 1), IDH2 (encoding isocitrate dehydrogenase 2), TP53 and H3F3B were evaluated. All five chondrosarcomas consisted predominantly of areas with conventional chondrosarcoma. Different grades were found [grade I (n = 1), grade II (n = 2), and grade III (n = 2)]. Up to 20% of the tumour consisted of classic features of clear cell chondrosarcoma. Gradual merging between both components was observed. Molecular analysis of conventional chondrosarcoma components revealed an IDH1 c.395G>T, p.(Arg132Leu) mutation in two cases, and an IDH1 c.394C>T, p.(Arg132Cys) mutation in one case, with identical IDH mutations in the clear cell chondrosarcoma counterpart (100%). Two cases were IDH wild-type. In all cases, none of the components harboured H3F3B mutations. High-grade tumours had an aggressive course, as three patients died of the disease. Conclusion On the basis of clinicopathological characterisation and genetic alterations, it is suggested that these lesions should be considered to be conventional chondrosarcoma, with clear cell change. Pathologists should be aware of their existence to avoid confusion with clear cell chondrosarcoma, dedifferentiated chondrosarcoma, or chondroblastic osteosarcoma.

Osteosarcoma is the most common primary malignant bone tumor, with a higher prevalence among children and young adults. Its most frequent localization is in the distal femur, followed by the proximal humerus and the proximal femur. In this article, we will describe the case of a 25-year-old male patient who presented to our medical facility, accusing spontaneous pain and swelling, both originating from the left knee. The X-ray of the distal femur showed a heterogeneous mass around the left femoral metaphysis with massive periosteal reaction. The computerized tomography (CT) analysis revealed cortical destruction, with extra-compartmental localization. The patient has later undergone to an incisional biopsy. The histopathology report showed the presence of a chondroblastic osteosarcoma. According to the multidisciplinary diagnosis, the tumor was finallyclassified as type IIB on the Enneking classification. In such cases, the elective surgical procedure is reconstructive surgery, consisting of wide resection of the distal femur, followed by modular reconstruction. After the surgery, the patient’s condition improved significantly and the functional outcome of the reconstructed joint was also satisfying.

This is the fourteenth case of an intracranial and dural-based osteosarcoma in the literature to date. The case involves a forty-year old woman with a previous history of a brain tumour, which was treated with surgery, chemotherapy and radiotherapy six years previously. The hypothesis is that this rare malignancy is secondary to the radiation exposure given the growth of the lesion in the previous radiation field

A 10-year-old boy presented with continuous reports of pain located to the left knee. Imaging revealed a sclerotic process in the left distal femur, and biopsies were consistent with chondroblastic osteosarcoma. As part of standard treatment the patient underwent neoadjuvant chemotherapy followed by limb sparring surgery and adjuvant chemotherapy. The entire tumour was excised and femoral bone reconstruction was performed with a double barrel free vascularised fibular graft. Bone mineral density (BMD) can be decreased in childhood survivors of cancer. The patient was followed for 7 years with dual-energy X-ray absorptiometry scans in order to assess BMD and graft adaption. Despite two accidental fractures to the graft region local and global BMD underwent an overall increase. Approximately 7 years after tumour resection the patient had a global Z-score of 0.2, which is considered within normal range.

The present study aimed to develop nomograms to predict survival in patients with chondroblastic osteosarcoma (COS). An analysis was conducted of 320 cases of COS collected from the surveillance, epidemiology, and end results (SEER) database between 2004 and 2015. Independent prognostic factors were screened using univariate and multivariate Cox analyses. Subsequently, nomograms were established to predict the patients’ cancer-specific survival (CSS) and overall survival (OS) rates. The prediction accuracy and discriminative ability of the nomograms were examined using calibration curves and the concordance index (C-index). As revealed in the univariate and multivariate Cox regression analysis, age, tumor size, the primary site, the presence of metastasis, a history of having undergone surgery, and a history of having received radiotherapy were found to be independent prognostic factors associated with survival in patients with COS (all P 39 years, the presence of distant metastasis, no history of having undergone any surgery, and tumor size >103 mm were found to be associated with poor prognosis in patients, while the primary site of the mandible and no history of having undergone radiotherapy showed associations with a more favorable prognosis in patients. Next, nomograms were constructed to predict the OS and CSS in patients with COS. We constructed nomograms that can provide accurate survival predictions in patients with chondroblastic osteosarcoma. These nomograms can help surgeons customize the treatment strategies for patients with chondroblastic osteosarcoma.

Background Retinoblastoma (Rb) is a malignant tumor of the developing retina that affects children before the age of five years in association with inherited or early germline mutations of the RB1 gene. The genetic predisposition is also related with second primary malignancies arising de novo, or following radiotherapy which have become the leading cause of death in retinoblastoma survivors. Procedure We describe a retinoblastoma case with a novel RB1 and a synchronous MET aberration. Our goal was to identify all germline and somatic genetic alterations in available tissue samples from different time periods and to reconstruct their clonal relations using next generation sequencing (NGS). We also used structural and functional prediction of the mutant RB and MET proteins to find interactions between the defected proteins with potential causative role in the development of this uniqe form of retinoblastoma. Results In this study we detected a retinoblastoma case of non-parental origin with a novel RB1 c.2548C>T;p.(Gln850Ter) and a synchronous MET c.3029C>T;p.(Thr1010Ile) germline mutations. Following bilateral retinoblastoma the boy further developed at least four different manifestations of two independent osteosarcomas. Both histopathology and NGS findings supported the independent nature of a chondroblastic osteosarcoma of the irradiated facial bone followed by an osteoblastic sarcoma of the leg (tibia). Conclusions Because of the expanding number of registered Rb cases, the novel rare cases publication is very important to understand the molecular mechanism of this malignancy. We reported a novel form of Rb and consequential chondroblastic and osteoblastic osteosarcoma, the latter one developing pulmonary metastatses.

Intravascular tumour extension invading the intracardiac space is rarely seen with osteosarcoma. We present a patient with a history of previously resected pelvic osteosarcoma who was later found to have a local recurrence with continuous intravascular extension from the right femoral vein to the right atrium. Preoperative imaging studies initially described extensive thrombus burden, and a multidisciplinary approach involving open and percutaneous thrombectomy was planned. Intraoperative inspection and pathological analysis revealed unresectable malignant solid tumour rather than thrombus. Though exceedingly rare, the possibility of metastatic tumour must be considered when planning treatment strategies for these patients.

A 57-year-old woman with metastatic chondroblastic osteosarcoma developed sudden-onset right-sided weakness and aphasia. She was found to have an occluded left middle cerebral artery (M1 segment) and underwent endovascular stroke intervention with return to a normal neurological exam within 24 h. Histologic analysis of the embolus was consistent with chondroblastic osteosarcoma.

Patients diagnosed with metastatic sarcoma have limited options for achieving both local and distant tumor control. While SBRT can achieve local control, distant response rates remain low. There is limited evidence demonstrating the safety and efficacy for combining SBRT with concurrent PD-1 checkpoint blockade in metastatic sarcoma. In this prospective case-series, we examined five patients with metastatic sarcoma on pembrolizumab treated concurrently with SBRT from July 1, 2016-October 30, 2018. Acute and chronic toxicity were recorded using Common Terminology Criteria for Adverse Events (CTCAE, version 5.0). SBRT-treated tumor control was assessed using Response Evaluation Criteria in Solid Tumors (RECIST version 1.1). With median follow-up of 14.9 months, three patients with undifferentiated pleomorphic sarcoma, one with intimal, and one with chondroblastic osteosarcoma received SBRT with concurrent pembrolizumab to 10 sites of metastatic disease. No grade 5 toxicities were observed. There was a single incidence of transient grade 4 lymphopenia which resolved without intervention. Grade 3 toxicities included anemia, thrombocytopenia, lymphopenia and colitis. One tumor demonstrated local progression after SBRT, and all others remained stable or with response. In conclusion, combining SBRT with PD-1 inhibition appeared to be safe in this patient population. Expected high rates of treated-tumor local control after SBRT were observed. Two of five patients demonstrated either enhanced local tumor regression, or possible abscopal effect.

A 3-year-old, male neutered domestic shorthair cat, presented for acute onset tail paresis. He was diagnosed with a spindle cell tumour at the level of L7-CD1 and treated with course fractionation radiation therapy. Three years following radiation therapy, the cat developed chondroblastic osteosarcoma of the pelvis, suspected to be secondary to radiation therapy. Hemipelvectomy was performed and the cat was treated with radiation therapy for remaining gross disease. The cat was euthanized 127 days post-operatively due to suspected metastatic disease. Development of radiation-induced tumours should be considered as a rare late complication in cats undergoing radiation therapy.

Chondroblastic osteosarcoma is an uncommon subtype of osteosarcoma, constituting about 25% of all osteosarcoma cases. It has shown high propensity for developing tumor thrombi and thromboembolism, with poor overall survival rate. Differentiation between tumor thrombus and bland thrombus is an important factor in making treatment decisions. 18 F fluorodeoxyglucose positron emission tomography/computed tomography plays a very important role in detecting and differentiating bland thrombus from tumor thrombus.

Periosteal osteosarcoma (PO), an intermediate-grade chondroblastic osteosarcoma (OST) arising from the surface of the bones, is a rare histological subtype among primary bone sarcomas, most commonly diagnosed in young patients. It is characterized by distinct specific radiological and pathological features. The current management strategy is based on several case reports and series, without any solid international recommendations. Most sarcoma experts agree on the crucial role of an optimal complete surgical approach. However, with the paucity of available reports, the role of systemic treatment and its timing remains debatable. With this paper, we will review the available data on the actual impact of chemotherapy in PO patients with emphasis on the radiological, pathological, and therapeutic characteristics of this rare entity.

Summary Bone marrow-derived mesenchymal stem cells (BMSCs) are proposed as the cells of origin of several subtypes of osteosarcoma (OS). However, signals that direct BMSCs to form different subtypes of OS are unclear. Here we show that the default tumor type from spontaneously transformed p53 knockout (p53_KO) BMSCs is osteoblastic OS. The development of this default tumor type caused by p53 loss can be overridden by various oncogenic signals: RAS reprograms p53_KO BMSCs into undifferentiated sarcoma, AKT enhances osteoblastic OS, while cFOS promotes chondroblastic OS formation. We focus on studying the mechanism of cFOS-induced chondroblastic OS formation. Integrated genome-wide studies reveal a regulatory mechanism whereby cFOS binds to the promoter of a key chondroblastic transcription factor, Sox9, and induces its transcription in BMSCs. Importantly, SOX9 mediates cFOS-induced cartilage formation in chondroblastic OS. In summary, oncogenes determine tumor types derived from BMSCs, and the cFOS-SOX9 axis is critical for chondroblastic OS formation., Graphical Abstract, Highlights • The default tumor type from p53_KO BMSCs is osteoblastic OS • Oncogenes reprogram p53_KO BMSCs into different sarcomas • cFOS promotes chondroblastic OS from p53_KO BMSCs • SOX9 is a mediator of cFOS in promoting chondroblastic OS, In this article, Huang and colleagues show that the default tumor type arising from p53 knockout mouse bone marrow-derived mesenchymal stem cells is osteoblastic osteosarcoma (OS), and oncogenic signals can override this default tumor type choice. They focus on studying the mechanisms underlying cFOS-driven chondroblastic OS and demonstrate that SOX9 is one of the mediators of cFOS.

Summary A 3 year-old German shepherd bitch was admitted with a severe enlargement of the right thigh. Uncapsulated large mass, 7 cm × 10 cm thickness was found at necropsy surrounding the contour of the thigh muscles along its whole length. Microscopically, the tumor composed of neoplastic cells infiltrating the stroma, with cellular pleomorphism, hyperchromatism, high atypical mitotic figures (2–4 per high power field) and focal areas of osteoid formation. Chondroblastic differentiation was also present where chondroblastic cells consisted of moderately pleomorphic, irregularly arranged polygonal cells with vacuolated cytoplasm, vesiculated nuclei and large nucleoli. Based on these pathological findings, this tumor was diagnosed as chondroblastic osteosarcoma.

This study aims to report of two variants of gnathic osteosarcoma with highlights on the varied histopathological presentation of osteosarcomas (OS). OS present with diverse histological appearances. Despite significant advances in molecular pathogenesis and biomarkers, clinicopathologic correlation is still considered as the important criteria in diagnosis. Chondroblastic osteosarcoma in a 52-year-old female and fibroblastic osteosarcoma in a 35-year-old female. Osteosarcoma is a relatively rare disease of the oral and maxillofacial region. Regular screening and follow-up is highly recommended, as recurrence rates are higher. Thorough understanding of the histologic spectrum of osteosarcoma reduces the diagnostic difficulties in categorizing the OS and separating these neoplasms from benign bone diseases.

Lesions of non-endodontic origin may mimic periapical abscess. Osteosarcoma is a rare malignant lesion. Case report The present report describes a case of chondroblastic osteosarcoma in the periapical region of teeth #29, #30, and #31 of an 18-year-old male. Clinical history showed self-reported discomfort in the right posterior gingiva for over a month. Physical examination showed a small expansion and redness of the right mandibular buccal and lingual cortical plates, but no signs of pain or inflammation were observed. All the teeth responded positively to pulp sensibility. Periapical and panoramic radiographs showed slight periapical radiolucency in the roots of teeth #29 and #30, clear periodontal ligament space widening, and evident loss of lamina dura. Incisional biopsy was performed, and based on microscopic findings the diagnosis of chondroblastic osteosarcoma was confirmed. Conclusions Non-endodontic diseases associated with tooth root apex, such as chondroblastic osteosarcoma, should be included in differential diagnosis of jaw lesions that resemble periapical abscess.

Osteosarcoma of the skull is a rare primary malignant bone tumour in children, representing 1–2% of all cranial tumours. We describe a case of a 17-year-old adolescent with chondroblastic osteosarcoma of the parietal-occipital bone and no distant metastases at presentation treated with neoadjuvant chemotherapy, surgery and, later, concurrent chemoradiotherapy. The patient suffered progressive disease and died 15 months after diagnosis. There are at least 15 paediatric cases of osteosarcoma of the skull described in the literature. Due to its rarity, there are no broad prospective studies on this entity, which has distinctive features when compared to other craniofacial tumours, carrying a worse prognosis. Complete surgical resection is needed for long-term survival, whereas chemotherapy and radiotherapy have still questionable indications.

Aims The aim of this study was to identify factors that determine outcomes of treatment for patients with chondroblastic osteosarcomas (COS) of the limbs and pelvis. Patients and Methods The authors carried out a retrospective review of prospectively collected data from 256 patients diagnosed between 1979 and 2015. Of the 256 patients diagnosed with COS of the pelvis and the limbs, 147 patients (57%) were male and 109 patients (43%) were female. The mean age at presentation was 20 years (0 to 90). Results In all, 82% of the patients had a poor response to chemotherapy, which was associated with the presence of a predominantly chondroblastic component (more than 50% of tumour volume). The incidence of local recurrence was 15%. Synchronous or metachronous metastasis was diagnosed in 60% of patients. Overall survival was 51% and 42% after five and ten years, respectively. Limb localization and wide surgical margins were associated with a lower risk of local recurrence after multivariable analysis, while the response to chemotherapy was not. Local recurrence, advanced patient age, pelvic tumours, and large volume negatively influenced survival. Resection of pulmonary metastases was associated with a survival benefit in the limited number of patients in whom this was undertaken. Conclusion COS demonstrates a poor response to chemotherapy and a high incidence of metastases. Wide resection is associated with improved local control and overall survival, while excision of pulmonary metastases is associated with improved survival in selected patients. Cite this article: Bone Joint J 2019;101-B:739–744.

Osteosarcoma is an uncommon primary malignant tumor of bone. Craniofacial osteosarcomas constitute only about 6.5–7% of all osteosarcomas. The most common histopathologic type is chondroblastic type in head and neck group and osteoblastic in extremity group. We present a case report of 21 year old male patient with chondroblastic osteosarcoma with two episodes of recurrence. The tumour was dealt with left hemimandibulectomy followed by radiochemotherapy. After first recurrence he underwent right hemimandibulectomy. During second recurrence excision of growth followed by radiochemotherapy was done. Thus an aggressive multi-modality approach was adopted for treatment. Osteosarcoma is an uncommon primary malignant tumor of bone. Craniofacial Osteosarcomas are considered a separate category in view of their low histologic grade, less frequent metastases and better prognosis. The most common presentation is local swelling with or without pain. Aggressive surgical approach with post-surgical radiochemotherapy can be an effective tool.

SATB2 is commonly expressed in osteosarcomas. Although apparently being a valuable diagnostic marker for differentiating between small cell osteosarcoma (SCO) and other small round cell tumors of bone, for instance Ewing sarcoma family of tumors (ESFT), it has not been tested in a large series of ESFT and chondrosarcomas so far. We studied the immunohistochemical expression of SATB2 in 42 osteosarcomas, 31 chondrosarcomas, and 371 genetically confirmed ESFT. SATB2 positivity was detected in 90.4% of osteosarcomas, 87.5% of SCO, 91.3% of osteoblastic osteosarcomas, and in all chondroblastic and parosteal osteosarcomas. The osteoblastic and SCO subtypes expressed SATB2 more intensely than other histological types. SATB2 was expressed in 46.6% of chondrosarcomas, and in 1.3% of ESFT. Sensitivity and specificity of SATB2 immunoexpression were 90.4% and 95.3%, respectively. The positive and negative predictive values in osteosarcoma diagnosis were 66.6% and 98.9%, respectively. In chondrosarcoma, SATB2 immunoexpression was more frequent and intense in high-grade chondrosarcoma (Grade III) and uncommon in chondrosarcoma grade I. SATB2 positivity was detected in 55.6% of chondrosarcomas grade II. SATB2 apparently cannot distinguish between chondroblastic osteosarcoma and high-grade chondrosarcoma. Nevertheless, SATB2 is frequently expressed in osteogenic tumors, but is rarely positive in ESFT, and with the support of CD99 expression and specific molecular studies, it is very useful for distinguishing between these two lesions. Although SATB2 immunoexpression helps to distinguish osteosarcoma from their mimickers, the identification of malignant osteoid matrix formation and the integration of clinical and radiological data remain the corner stone of osteosarcoma diagnosis and as yet no antibody has equalled the diagnostic value of this important morphologic hallmark.

BACKGROUND Chondroblastic osteosarcoma (COS) is a uniformly fatal bone malignancy if not diagnosed and treated appropriately in a timely manner. Fine-needle aspiration (FNA) of osseous lesions is routinely performed in major medical centers. Appropriate characterization of the tumor will significantly influence patient management and outcomes. METHODS A retrospective review of the cytopathology archives of 2 large tertiary care centers for a 15-year period (2001-2015) revealed 17 cases of COS (9 primary, 6 recurrent, and 2 metastatic cases) in 16 patients. Clinical outcome and histopathologic follow-up were reviewed and correlated. RESULTS There were 9 male and 7 female patients (male-to-female ratio of approximately 1:1), ranging in age from 12 to 70 years (mean age, 29.2 years). The classic locations for osteosarcoma were commonly involved, such as the long bones around the region of the knee in 4 cases and the proximal humerus in 1 case. However, other “nonclassic” sites also were noted to be commonly involved, including the distal tibia or fibula (4 cases), sacroiliac region (4 cases), mandible (3 cases), and skull (1 case). Cytomorphologic characteristics were high cellularity; predominantly discohesive, single cells; small tissue fragments; background osteoid and chondroid matrix; spindled cells and plasmacytoid cells with moderate to abundant basophilic vacuolated cytoplasm and occasional multiple cytoplasmic processes; round to oval nuclei with anisonucleosis; and small to prominent nucleoli. There were scattered binucleated and multinucleated osteoclast-like giant cells. Cases with high-grade morphology demonstrated marked pleomorphism and abundant mitoses. CONCLUSIONS The differential diagnosis of COS includes chondroblastoma, chondrosarcoma, and chondroid chordoma. A definitive diagnosis can be made with clinical and radiological correlation. Cancer (Cancer Cytopathol) 2016. © 2016 American Cancer Society.

Patient C.B.S, 30 years old, female, with melanoderma, presented as main complaint increased volume in hemiface on the right side. In the extraoral physical examination, facial asymmetry was observed. In the intraoral examination, a lobed exophytic nodule of firm consistency was palpated, sessile base, covered by an integral mucosa of erythematous color, measuring approximately 3 cm. In the panoramic and periapical radiographs, bone spicules and thickening of the periodontal ligament were observed in the mesial of the tooth 16 in all its extension. Computed tomography was requested, which presented multiple exophytic projections with a classic "sunbeam" appearance caused by the excessive production of osteophytic bone in the form of irregularly shaped hyperdense spikes and imprecise limits, which ruptured and expanded the vestibular and lingual cortical bones. The patient underwent incisional biopsy with fragment removal on the right maxillary alveolar ridge. The diagnosis was of chondroblastic osteosarcoma.

Osteosarcoma of the jaws is uncommon and mainly occurs as a fast-growing painful swelling in the posterior mandible. The knowledge of clinicopathologic aspects of osteosarcoma is essential for early diagnosis and adequate treatment. The aim of this study is to report a clinical case of chondroblastic osteosarcoma in the posterior left mandible in a 53-year-old male patient with complaint of paresthesia in the left lower lip and a fast-growing swelling in the gingival mucosa. Radiographically, the classical sunray appearance can be observed. Microscopically, we identified atypical polygonal cells with large and hyperchromatic nuclei associated with osteoid material and chondroblastic differentiation. The diagnosis of chondroblastic osteosarcoma was made. The patient was referred to an oncologist for treatment with surgery and chemotherapy.

This study was aimed to reveal the changes in survival rates and prognostic factors to survival of chondroblastic osteosarcoma (COS). Patients from the Surveillance, Epidemiology, and End Results (SEER) database were retrieved. Kaplan–Meier survival analysis and Cox proportional hazard model were used during analysis. There were significant differences on overall survival between subtypes of osteosarcoma (P

Osteosarcoma is the most common primary malignant tumor of the long bones. However, primary osteosarcoma of the chest wall, particularly the sternum, is an extremely rare occurrence. We report a 36-year-old male presenting with a hard, immobile, palpable, anterior chest wall mass. A computed tomographic (CT) scan showed a large destructive anterior mediastinal mass involving the manubrium and sternum with multiple bilateral calcified lung masses, pleural effusions and partially calcified aortopulmonary, right hilar and subcarinal lymphadenopathy. Incisional biopsy of the mass revealed grade 2 chondroblastic osteosarcoma. The patient underwent one cycle of chemotherapy with ifosfamide and palliative radiation. Unfortunately, the patient was unable to tolerate ifosfamide and developed severe nausea and vomiting requiring the discontinuation of chemotherapy. Given his metastatic disease and inability to tolerate standard chemotherapy, he was referred to a comprehensive cancer center for advanced clinical trials.

Teratoma Osteosarcoma is the most common malignancy of mesenchymal cells mostly originating within long bones, but rarely in the jaws. The World Health Organization (WHO) assorts several variants that differ in locale, clinical behavior and cellular atypia. This report illustrates a case of one of the histological variants of osteosarcoma i.e., chondroblastic osteosarcoma in the region of anterior maxilla in a 58 year old male patient previously treated for ossifying fibroma of the same site

We report the case of a 32-year-old male patient, who was subjected to cone-beam tomography for planning the removal of an unerupted tooth. Cone-beam CT scans revealed the presence of a mixed lesion with poorly defined limits in the left posterior mandibular region, with "sun-ray" periosteal reaction. An incisional biopsy was performed and the diagnosis was chondroblastic osteosarcoma. In this paper, a discussion of the differential diagnosis criteria and treatment of this unusual malignant neoplasm is performed. Thus, it is intended to provide information that will help in the study of management protocols for the increasingly effective treatment of gnathic osteosarcomas.

Background Nonepithelial tumors of the breast are extremely rare and have an incidence of less than 1%. The most common nonepithelial breast tumor is phyllodes tumor (PT), which accounts for 61%. Primary osteosarcomas of the breast contribute up to only 12.5% of all the extraosseous osteosarcomas, and among young females, they are extremely rare, especially in those without a previous history of primary bone osteosarcoma. A case of a 16-year-old female with primary chondroblastic osteosarcoma of the left breast is herein presented. Case Description In this report, we describe and report a 16-year-old female with neither a previous history of bone osteosarcoma nor a family history of breast cancer who was diagnosed with a primary chondroblastic osteosarcoma of the left breast. The mass was shining, warm, firm, and slightly fixed. The excisional biopsy showed a large tumor measuring 11 × 9 × 7 cm, encapsulated, grayish-white, and nodular. The H&E-stained sections showed a tumor composed of atypical spindle cells, large pleomorphic epithelioid cells, and multinucleated cells of osteoclastic type with osteoid formation with abundant areas having malignant chondroid matrix. About 40 atypical mitoses/10 HPF were seen. A diagnosis of primary osteosarcoma was made. Immunohistochemically, the tumor was triple negative, vimentin was strongly positive, SMA was focally positive, and AE1/AE3 was negative. The patient was put under observation. Her postoperative course is generally promising without any complaint of pain. We present a case of a very young female with the rarest diagnosis of primary osteosarcoma involving the left breast. Conclusion Primary osteosarcomas of the breast carry poor prognosis by being triple negative, and because of being the rarest tumors, they pose a challenge in managing the patients due to lack of established treatment modalities.

NELL-1 is a novel osteochondral differentiation factor protein with increasing usage in tissue engineering. Previously, we reported the expression patterns of NELL-1 in bone-forming skeletal tumors. With increasing interest in the use of NELL-1 protein, we sought to examine the expression of NELL-1 in cartilage-forming tumors.Immunohistochemical expression was examined in human pathologic specimens.Consistent NELL-1 overexpression across all cartilage-forming tumors was observed. Similar degrees of expression were observed in enchondroma, chondrosarcoma, and chondroblastic osteosarcoma. NELL-1 expression did not significantly vary by tumor grade.In summary, NELL-1 demonstrates reliable and consistent expression across cartilage-forming skeletal tumors.

Highlights • Chondroblastic ostsarcomas of breast is extremely rare. • Histology report of biopsy showed a malignant phyllodes with chondrosarcomatous differentiation. • Mastectomy was followed by Adjuvant chemotherapy. • There was recurrence after 6 months of surgery. • Recurrence was managed by radical mastectomy with LD flap. • Now there is extensive metastasis., Introduction Primary extra osseous osteogenic sarcomas have been reported in many tissues of the body but their occurrence in the breast is extremely rare. It can arise as a result of osseous metaplasia in a pre-existing benign or malignant neoplasm of the breast. Case presentation A 24 year-old young lady was diagnosed to have mass in right breast.Tru cut biopsy showed it was malignant phyllodes tumour. There was no distant metastasis or any axillary lymph nodes palpable. Simple mastectomy was done. The histology report of biopsy showed a malignant phyllodes with chondrosarcomatous differentiation an myxoid changes. She was given 6 cycles of chemotherapy. There was recurrence after 6 months of surgery. Then radical mastectomy with latissimus dorsi musculocutaneous flap was used to cover the anterior chest wall defect. Sections from the mastectomy specimen confirmed the diagnosis of chondroblastic osteosarcoma. Axillary nodes free of tumour cells. Now the patient again came with recurrence after 5 months, and disseminate subcutaneous nodules. Conclusion A diagnosis of chondroblastic osteosarcoma of the breast was made based on histology report and after excluding any osteogenic sarcoma arising from underlying ribs and sternum. It is a very rare disease with only few case reports in literature.

Chondroblastoma is a rare primary bone tumor of young people that typically arises in the ends of the long bones. Radiologic investigations show a small, circumscribed, lytic lesion. The tumor is characterized histologically by the proliferation of chondroblasts along with areas of mature cartilage, giant cells, and occasionally, secondary aneurysmal bone cyst formation. Chondroblastoma, however, may also present with atypical features, such as prominent hemosiderin deposition, numerous giant cells, or the presence of a large aneurysmal bone cyst component. Malignant entities such as clear cell chondrosarcoma and chondroblastic osteosarcoma must also be considered. Recently, immunohistochemical stains such as DOG1 and SOX9 have been described in chondroblastoma, and K36M mutations in either the H3F3A or H3F3B genes have also been identified. While generally regarded as a benign entity, chondroblastoma manifests an intermediate type of behavior, given its ability to recur locally, and rarely, metastasize.