Telangiectatic Osteosarcoma Research Articles

Imaging of Osteosarcoma: Presenting Findings, Metastatic Patterns, and Features Related to Prognosis

Background: Osteosarcomas are rare malignancies (<1% of all cancers) that produce an osteoid matrix. Osteosarcomas are the second most frequent type of primary bone tumor after multiple myeloma and the most prevalent primary bone tumor in children. The spectrum of imaging findings of these malignancies varies significantly, reflecting different histological subtypes. For instance, conventional osteosarcoma typically presents with a mixed radiological pattern (lytic and bone mineralization) or with a completely eburneous one; aggressive periosteal reactions such as sunburst, Codman triangle, and soft-tissue components are frequently displayed. On the other hand, telangiectatic osteosarcoma usually presents as a purely lytic lesion with multiple fluid–fluid levels on MRI fluid-sensitive sequences. Other typical and atypical radiological patterns of presentation in other subtypes of osteosarcomas are described in this review. In addition to the characteristics associated with osteosarcoma subtyping, this review article also focuses on imaging features that have been associated with patient outcomes, namely response to chemotherapy and event-free and overall survivals. This includes simple semantic radiological features (such as tumor dimensions, anatomical location with difficulty of radical surgery, occurrence of pathological fractures, and presence of distant metastases), but also quantitative imaging parameters from diffusion-weighted imaging, dynamic contrast-enhanced MRI, and 18F-FDG positron emission tomography and radiomics approaches. Other particular features are described in the text. Overall, this comprehensive literature review aims to be a practical tool for oncologists, pathologists, surgeons, and radiologists involved in these patients’ care.

Intra-abdominal telangiectatic osteosarcoma: a case report

BackgroundTelangiectatic osteosarcoma is rare and it rarely affects flat bones, especially the bones of the pelvis. It is uncommon for telangiectatic osteosarcoma to be considered as a differential diagnosis when assessing a large intrabdominal mass.Case reportWe present our case of a 33-year-old African female who presented with a sizeable telangiectatic osteosarcoma of the left iliac bone. She reported a 3-year duration of a painless, slow-growing mass arising from the left flank. At examination, a large bony hard mass extending from the left ilium to the umbilicus was noted, almost mimicking an intra-abdominal pregnancy. All laboratory tests were within normal limits and an unconventional surgical approach was used for a one-stage excision of the tumor without complications. The definitive histopathological diagnosis postexcision was that of a telangiectatic osteosarcoma only on the second review of the histological specimen.ConclusionsPelvic telangiectatic osteosarcoma is rare, and the ilium is the commonly affected pelvic bone. These tumors can be sizeable at presentation with intra-abdominal or pelvic extension with a high chance of misdiagnosis. Fortunately surrounding soft tissue involvement seems to be a rare and late finding when present.

Diagnosis and Current Treatment of Aneurysmal Bone Cysts.

The purpose of this review is to increase awareness about the evolution and development of current trends in the diagnosis and treatment of aneurysmal bone cysts (ABCs). ABCs are benign, but locally aggressive bone tumors that mainly affect children. ABCs comprise 1% of all primary bone tumors and occur most frequently during the first two decades of life. The diagnosis is made using a variety of imaging modalities and has the characteristic features of an expansile, radiolucent lesion that is often seen in the metaphyseal region of the bone and has fluid-fluid levels that are apparent on MRI. In the pediatric population, telangiectatic osteosarcoma and unicameral bone cyst (UBC) are the main differential diagnoses of an ABC. Giant cell tumors (GCTs) also include in differential diagnosis, which often manifest in patients older than 15 and do not penetrate the open physis although they develop after the physeal closure. Imaging alone cannot rule out telangiectatic osteosarcoma; therefore, a biopsy is recommended. A variety of treatment options have been described; traditionally, most patients are treated with curettage and bone grafting. Curettage alone, however, usually results in tumor recurrence following excision. A variety of adjuvants have been utilized with varying degrees of effectiveness to reduce the risk of local recurrence. When a cyst is in the pelvis, its location and size are such that surgery is a very risky option. Selective arterial embolization has significantly contributed to the development of effective treatments for these situations. Embolization or radiation, as well as denosumab therapy, are widely used as therapies for ABCs in anatomic locations where surgery would significantly increase morbidity.

Cytodiagnosis of Telangiectatic Osteosarcoma in a Patient with Previous Fracture Humerus Surgery with Plating: A Case Report

Telangiectatic Osteosarcoma (TO) is a rare variant of osteosarcoma, accounting for 0.4-12% of cases. It is a high-grade malignancy occurring in adolescence and the adult population. It commonly occurs in the bones of the limbs but rarely in the humerus. It is assumed to originate in stem cells of the mesenchyme or from transformed osteoblasts. The initial X-ray of TO would show an osteolytic lesion over the metaphysis of the long bone. However, the radiographic imaging techniques may not correlate with the histological variant of osteosarcoma. It has been rarely diagnosed and reported in the literature in terms of diagnostic modality using Fine Needle Aspiration Cytology (FNAC). Its cytological as well as histological diagnosis is challenging. However, the diagnosis of TO has numerous implications such as prognosis, differential diagnosis, and treatment options. The present clinical and pathological case images depict the unusual features of TO involving the upper humerus by an osteolytic lesion. Its clinical and diagnostic chronology is interesting due to the occurrence of TO in a case that underwent surgery for a fractured humerus with plating. The plate fixation in this particular case was surrounded by osteolytic tumour tissue extending into the soft tissue. The FNAC under Ultrasonography (USG) guidance was carried out. The smears of the aspirated material typically showed malignant spindle cell fragments with a few gaps of remnant sinusoids entrapping sparse red blood cells. The smears also showed osteoid material which was lacy in character. This case has several noteworthy features in the form of cytomorphological description of the telangiectatic variant of osteosarcoma, its occurrence following plating, and subsequent confirmation on histology and Immunohistochemistry (IHC).

Imaging findings of primary breast telangiectatic osteosarcoma: a case description.

Imaging findings of primary breast telangiectatic osteosarcoma: a case description.

Telangiectatic Osteosarcoma—A Single-Center Experience

Telangiectatic osteosarcoma is an uncommon subtype of osteosarcoma accounting for less than 4% of all cases of osteosarcoma. It is characterized by distinctive radiographic, pathologic features, and prognostic implications. Radiologically and microscopically, it mimics aneurysmal bone cyst. The objective of this study was to study the clinical profile and treatment outcome of patients with telangiectatic osteosarcoma. Thirteen patients were diagnosed with telangiectatic osteosarcoma in the department of medical oncology at a tertiary cancer center in India during a 12-year period. All patients were above 15 years of age. The median age at presentation was 20 years, males were predominant, and the commonest sites of involvement were lower end of femur and upper end of humerus. Ten patients underwent treatment that consisted of neoadjuvant and/or adjuvant chemotherapy with ifosfamide, doxorubicin, cisplatin regimen and limb sparing surgery or amputation. Currently, eight out of ten patients are alive in remission at a median follow-up of 50 months with survival ranging from 18 to 138 months.

Rare case of pediatric calcaneal osteosarcoma masquerading as a cystic lesion

BackgroundAlthough osteosarcoma remains the most common primary malignant neoplasm in the bone in the pediatric age group, involvement of the foot is extremely rare.Case presentationOur case demonstrates what appeared to be a bone cyst of the calcaneus at an outside institution after injury to the ankle. The patient was sent to our institution for further workup where a Computed Tomography scan revealed a permeative lytic lesion with faint calcifications. Based on our patient’s radiograph, an aneurysmal bone cyst might be included in the differential diagnosis. A telangiectatic osteosarcoma can also demonstrate a cystic and lytic appearance on radiographs. Interestingly biopsy confirmed our patient had an osteoblastic osteosarcoma.ConclusionsA high degree of suspicion is often necessary for diagnosis of calcaneal osteosarcoma, especially in the setting of injury, and should not be overlooked.

Different Subtypes of Osteosarcoma: Histopathological Patterns and Clinical Behaviour

Osteosarcoma (OS) is a primary malignant bone tumour that usually occurs in children and adolescents. OS is a highly aggressive tumour type with a propensity for local invasion and systemic early metastasis to the lungs or other bones. According to the World Health Organization, there are different subtypes of OS, including conventional OS (osteoblastic, chondroblastic, fibroblastic), telangiectatic OS, low-grade OS, small-cell OS, parosteal OS, periosteal OS, and high-grade surface OS. In this mini review, we will discuss the background of OS and histopathological patterns and clinical behaviour of the disease. Understanding the subtypes of OS and their pathogenesis is crucial for developing more precise and effective therapies for OS patients.

Mutation characteristics of osteosarcoma: a single center study of 64 cases using next-generation sequencing

Objective: To investigate the distribution and characteristics of gene mutations in osteosarcoma, and to analyze the frequency and types of detectable mutations, and to identify potential targets for individualized treatment of osteosarcoma. Methods: The fresh tissue or paraffin-embedded tissue samples of 64 cases of osteosarcoma that were surgically resected or biopsied and then subject to next generation sequencing, were collected from Beijing Jishuitan Hospital, China from November 2018 to December 2021. The tumor DNA was extracted to detect the somatic and germline mutations using targeted sequencing technology. Results: Among the 64 patients, 41 were males and 23 were females. The patient age ranged from 6 to 65 years with a median age of 17 years, including 36 children (under 18 years old) and 28 adults. There were 52 cases of conventional osteosarcoma, 3 cases of telangiectatic osteosarcoma, 7 cases of secondary osteosarcoma, and 2 cases of parosteosarcoma. The detection rate of gene mutations was overall 84.4% (54/64). There were 324 variations in 180 mutated genes, including 125 genes with copy number variations, 109 single nucleotide variants, 83 insertions or deletions, and 7 gene fusions. The most common mutated genes were TP53, VEGFA, CCND3, ATRX, MYC, RB1, PTEN, GLI1, CDK4 and PTPRD. Among them, TP53 had the highest mutation rate (21/64, 32.8%), single nucleotide variant was the main mutation type (14/23, 60.9%), and 2 cases carried the TP53 germline mutation. VEGFA and CCND3 showed copy number amplification simultaneously in 7 cases. Conclusions: The high-frequency mutation of TP53 suggests that it plays an important role in the pathogenesis and development of osteosarcoma. VEGFA, CCND3 and ATRX are mutated genes in osteosarcoma and worthy of further studies. Combination of pathologic diagnosis and next generation sequencing with clinical practice can guide individualized treatment for patients with refractory, recurrent and metastatic osteosarcoma.

Prognostic analysis of telangiectatic osteosarcoma of the extremities.

Telangiectatic osteosarcoma (TOS) is a rare but highly malignant subtype of osteosarcoma. Although surgical treatment is the primary treatment modality for osteosarcoma, evidence on the benefits of different surgical methods in patients with TOS is lacking. This study aimed to compare the effects of different surgical and adjuvant treatments on overall survival of TOS, and the association of patient demographics, oncological characteristics, and socioeconomic status on treatment outcomes. This retrospective study selected the most common TOS cases of the extremities registered in the Surveillance, Epidemiology, and End Results (SEER) database from 1989 to 2019. Univariate and multivariate Cox regression models were used to analyze all prognostic factors, and Kaplan-Meier analyses were performed for disease-specific treatment factors of survival. A total of 127 patients were included in the analysis. The average age at initial diagnosis was 20.09 years. In univariate analyses, the absence of metastasis at initial diagnosis, limb-salvage surgery, adjuvant chemotherapy, and no regional lymph node dissection were associated with a lower risk of death. Multivariate analysis further showed that the presence or absence of distant metastasis and regional lymph node dissection, implementation of adjuvant chemotherapy, and choice of surgical method were independent predictors of prognosis. Distant metastasis and regional lymph node dissection are associated with poorer outcomes in TOS, and amputation has no better prognosis than limb salvage surgery. Compared with conventional chemotherapy, neoadjuvant chemotherapy did not significantly improve the prognosis of TOS.

AGGRESSIVE MANAGEMENT OF SURFACE ANEURYSMAL BONE CYST: A CASE REPORT

Aneurysmal bone cyst originating from the surface of the bone is an unusual subtype. Surface ABCs have a predilection for diaphysis of long tubular bones. Radiologically it may mimic telangiectatic osteosarcoma which can be differentiated on the basis of histology. We present here a case of 17 year old male with proximal humerus surface ABC with extensive cortical breach. Clinico-radiographic presentation of the patient was more aggressive than conventional ABC. Plain radiograph demonstrated a permeative type of destructive pattern and the cross-sectional imaging demonstrated extensive cortical breach and intramedullary spread. MR Imaging suggested that intramedullary spread was more extensive in dimension as compared to the subperiosteal component. Biopsy was suggestive of ABC. Preoperatively selective arterial embolisation was done. Management was done by wide en-bloc resection and treatment of resected segment with liquid nitrogen by free freezing method followed by reimplantation and internal xation with a proximal humerus plate along-with bular strut graft. Patient on follow up after 5 months post operatively was able to perform his activities of daily living. Most cases reported in literature were managed by curettage and bone grafting. Literature for aggressive management of surface ABC's is sparse with no literature available regarding the use of liquid nitrogen in its management

Telangiectatic Osteosarcoma of the Tibiotarsus in a North Island Brown Kiwi (Apteryx mantelli).

A North Island brown kiwi (Apteryx mantelli) with lameness and weight loss had a telangiectatic osteosarcoma. The left proximal tibiotarsus had bony lysis with multiple blood-filled spaces separated by thick fibrous septa and neoplastic mesenchymal cells producing osteoid (5-bromo-4-chloro-3'-indolyl phosphate/nitro blue tetrazolium positive on cytology). No metastases were noted on necropsy.

Malignancy of Aneurysmal Bone Cyst in Osteosarcoma and Angiosarcoma: Series of 2 Cases and Literature Review

Aneurysmal bone cyst (ABC) is a rare benign bone tumor characteristic of the first decades of life. Malignization of ABC in the absence of risk factors such as radiotherapy is extremely rare, with few cases published in the literature. There are malignant tumors such as telangiectatic osteosarcoma with an initial clinical, radiological and anatomopathological resemblance that requires special attention in their differential diagnosis. We present two patients with an initial histologic diagnosis of ABC who developed true malignancy, in the absence of risk factors, into osteoblastic osteosarcoma and epithelioid angiosarcoma. In spite of its exceptional nature, in the presence of an aggressive type of ABC malignant degeneration should always be suspected and therefore, an open biopsy and wide resection performed for a correct diagnosis to avoid residual microscopic disease in case of aggressiveness. Epithelioid angiosarcoma from an ABC tumor malignancy has never been described before.

Lung metastases pattern in limb osteosarcoma: A population-based study from 2010 to 2018.

Osteosarcoma (OS) is one of the most prevalent malignant bone tumors. The proportion of limb OS is relatively high, and lung metastases (LM) are one of the most prevalent metastatic types. A total of 1694 new cases of limb OS were identified in the surveillance, epidemiology and end results (SEER) database from 2010 to 2018. Cox regression analyze was performed to identify prognostic factors for limb OS with LM, and univariate and multivariate logistic regression analyses were used to assess risk factors for LM. Kaplan-Meier analysis was performed to calculate overall survival for LM, and a log-rank test was used for comparison. A total of 287 patients (16.94%) were diagnosed with limb OS with LM. 25 to 59 years old (odds ratio, OR 0.68; 95% confidence interval, CI: 0.46-0.99), larger than 100 mm tumors (OR 3.65, 95% CI: 1.54-8.64), telangiectatic osteosarcoma type (OR 0.24, 95% CI: 0.07-0.81), central osteosarcoma type (OR 0.44, 95% CI: 0.19-0.99), T2 stage (OR 2.59, 95% CI: 1.18-5.69), N1 stage (OR 7.79, 95% CI: 3.90-15.56), presence of bone metastases (OR 4.58, 95% CI: 2.43-8.63) and surgical treatments of primary site (OR 0.22, 95% CI: 0.14-0.33) were significant correlations with lung metastases. Elderly age, black race and absence of surgery were harmful for survival. Age between 25 and 59 years, telangiectatic osteosarcoma and central osteosarcoma were identified as high-risk factors in limb OS patients with LM, and surgical treatment of the primary site significantly increased the survival rate of LM in these patients.

Large pulmonary metastasis or primary lung cancer? Management particularities of an osteosarcoma case

Abstract Osteosarcoma is the most frequent malignant tumor of the bone which is diagnosed most frequently in children or young adults, with a high possibility of metastasis, especially in the lung. We report a case of 39-year male patient with a history of an above-the-knee left limb amputation after tibial osteosarcoma with no follow-ups in the last two years and with an expansive apical and left paramediastinal mass found on chest X-ray. We performed a bronchoscopy and the histopathological diagnosis of swabbed samples was fibroblastic type of classic osteosarcoma with focal areas of telangiectatic osteosarcoma and areas showing giant cells. The particularity of the case consisted in the challenge of establishing the origin of pulmonary mass. Whilst increased dimensions militated for a primary pulmonary neoplasm, the histological examination ascertained the metastatic etiology of the tumor.

Telangiectatic Osteosarcoma of the Mandible: A Case Report and Review of Literature

Telangiectatic Osteosarcoma of the Mandible: A Case Report and Review of Literature

Multicentric epithelioid hemangioendothelioma of humerus and scapula

Epithelioid hemangioendothelioma (EH) is a rare malignant vascular tumor occurring mainly in the liver and lungs, with bones being a rare site and primarily seen in the adult population. We present a rare case of multicentric EH in the right humerus in an adolescent male, who presented with complaints of pain and swelling in the right shoulder. Radiographs and Computed tomography showed a large expansile lytic lesion in the proximal end of the right humerus with areas of cortical destruction and matrix calcification. On Magnetic resonance imaging, the lesion was predominantly isointense on T1-weighted image, hyperintense on T2-weighted, and Short Tau Inversion Recovery images with prominent flow voids within. On post-contrast sequences, the lesion showed intense heterogenous enhancement with a non-enhancing central necrotic area. Multiple smaller lytic lesions with similar imaging characteristics were also found in the mid and distal shaft of the right humerus and the right coracoid process. Differentials of Telangiectatic osteosarcoma, Giant cell tumor, brown tumors of hyperparathyroidism, and metastasis were considered. Core biopsy revealed that the lesion was an EH. Though the imaging features of EH are non-specific, it may be considered in the differential diagnosis of an expansile lytic bone lesion with no periosteal reaction, showing cortical break and soft tissue component, especially if it is multifocal and multicentric.

Characteristics and prognosis of telangiectatic osteosarcoma: a population-based study using the Surveillance, Epidemiology and End Results (SEER) database.

BackgroundTelangiectatic osteosarcoma (TOS) is a rare type of osteosarcoma for which limited clinical data is available. Furthermore, the clinical characteristics and prognosis of TOS remain unclear.MethodsA large population-based cohort analysis was conducted using the Surveillance, Epidemiology and End Results (SEER) registry. The data of TOS and conventional osteosarcoma (COS) patients from 2000 to 2017 were collected. The categorical variables were assessed by Chi-squared tests. Kaplan-Meier curves and log-rank (Mantel-Cox) tests were used to examine the survival outcomes between the groups. Cox proportional hazard models were used for univariate and multivariate analyses of TOS patient survival-related variables.ResultsA total of 141 TOS patients and 2961 COS patients were included in this analysis, and the mean age at diagnosis was 23.5 and 29.4 years, respectively. Compared to COS patients, TOS patients were more likely to be under 20 years old (61.7% vs. 51.7%, P=0.022), and without a second peak of incidence after 60 years of age. The median overall survival (mOS) of TOS patients was not reached compared to a median survival of 84 months for COS patients (hazard ratio 0.75, 95% confidence interval 0.59 to 0.95, P=0.0175). After adjusting these data for age at diagnosis, stage, and surgery at the primary site, no significant differences in mOS were observed between the two groups. In univariate analyses, being under 20 years of age, having localized or regional stage disease, and having undergone surgery were associated with a decreased risk of death. Subsequent multivariate analysis indicated that age at diagnosis, stage, and surgery at the primary site were all independent predictors of prognosis in TOS patients.ConclusionsPatients with TOS were younger than patients with COS and did not show a second peak after 60 years of age. Age, summary stage at diagnosis, and surgery at the primary site were independent predictors of survival for TOS patients.

Diaphyseal aneurysmal bone cysts (ABCs) of long bones in extremities: Analysis of surgical management and comparison with metaphyseal lesions

IntroductionAneurysmal bone cysts (ABCs) are benign lesions of long bone metaphysis affecting mostly medullary region in younger age below 20 years of life. These may be originated rarely either in the cortex or in the superficial regions of diaphysis. The study highlighted the differences of diaphyseal lesions from the usual metaphyseal ones in view of their clinical, radiological and biological behavior and also discussed their management in brief. Material and methodsWe reviewed a total of 84 aneurysmal bone cysts over the past fourteen years (2004–2017) and evaluated their surgical outcomes retrospectively. Total ten lesions were diaphyseal cortical in location (group I), while 74 lesions were conventional metaphyseal type (group II). ResultsWe observed that cortical ABCs were occurred commonly in diaphysis of femur, humerus, tibia and radius while presentation was at an older age than metaphyseal type. Radiographically these were eccentric lesion and more prone for pathological fractures than conventional type. These are differentiated radiographically from other benign lesions but also mimicking malignant conditions including low grade surface osteosarcoma and telangiectatic osteosarcoma while resemble similar to these on histopathological examination (HPE). ConclusionBiological behavior of cortical lesions does not differ significantly than conventional type but these are more prone for pathological fractures so these eccentric cortical ABC lesions should be treated with adequate internal fixation along with curettage and bone grafting. Although incidence of cortical or surface variety of ABCs is rare but surgeons might evaluate its severity in view of aggressive benign or malignant lesions of diaphysis.

Analysis of a Preliminary microRNA Expression Signature in a Human Telangiectatic Osteogenic Sarcoma Cancer Cell Line.

Telangiectatic osteosarcoma (TOS) is an aggressive variant of osteosarcoma (OS) with distinctive radiographic, gross, microscopic features, and prognostic implications. Despite several studies on OS, we are still far from understanding the molecular mechanisms of TOS. In recent years, many studies have demonstrated not only that microRNAs (miRNAs) are involved in OS tumorigenesis, development, and metastasis, but also that the presence in high-grade types of OS of cancer stem cells (CSCs) plays an important role in tumor progression. Despite these findings, nothing has been described previously about the expression of miRNAs and the presence of CSCs in human TOS. Therefore, we have isolated/characterized a putative CSC cell line from human TOS (TOS-CSCs) and evaluated the expression levels of several miRNAs in TOS-CSCs using real-time quantitative assays. We show, for the first time, the existence of CSCs in human TOS, highlighting the in vitro establishment of this unique stabilized cell line and an identification of a preliminary expression of the miRNA profile, characteristic of TOS-CSCs. These findings represent an important step in the study of the biology of one of the most aggressive variants of OS and the role of miRNAs in TOS-CSC behavior.