Osteogenic Tumors Research Articles

Juvenile trabecular ossifying fibroma of the mandible

Fibro-osseous lesions are bony lesions where the bone is replaced by fibrous connective tissue and include fibrous dysplasia (FD), cemento-osseous dysplasia, and ossifying fibroma (OF). OF is a benign osteogenic mesenchymal tumor and clinicopathologically divided into juvenile and conventional subtypes. Juvenile OF (JOF) is a rare rapidly growing tumor of the craniofacial bones having aggressive clinical behavior with high recurrence rate. Pathologically, JOF is classified into juvenile trabecular OF (JTOF) and juvenile psammomatoid OF. JTOF microscopically shows proliferation of fibrocellular connective tissue stroma along with immature and woven bony trabeculae with varied histologic features. We report a case of JTOF of the mandible in a 12-year-old male with the initial presentation of painless swelling on the right side lower jaw. Clinicoradiologically, the case was suspected as central giant cell granuloma and odontogenic tumors. He was successfully treated with wide local excision and reconstruction and was on follow-up for 8 months without any recurrence of the disease.

A Sizeable Solitary Pedunculated Peripheral Osteoma of the Hard Palate: A Case Report.

Osteomas are benign, slow growing, usually sessile osteogenic tumors of unknown etiology. They arise from proliferation of either cancellous or compact bone. They can be central, peripheral or extra-skeletal based on their origin from endosteum, periosteum or extra-skeletal soft tissue respectively. They are commonly found in the skull and facial bones, the most common site in the maxillofacial region being paranasal sinuses. Among the jaw bones the most common site is the mandible. The hard palate is a rare site for such osteomas with a very few cases reported in literature. We report a case of large pedunculated peripheral osteoma of the hard palate in a 38year old male. The tumor was excised with no recurrence after 3years followup.

Giant Frontal Sinus Osteomas: Demographic, Clinical Presentation, and Management of 10 Cases

Osteomas are rare benign and slow-growing osteogenic tumors mainly involving frontal and ethmoid sinuses. The primary objective of our study is to present the management of cases of giant frontal sinus osteomas. Secondarily, we describe our modified unilateral osteoplastic flap approach without obliteration to remove these osteomas. Retrospective chart review at a tertiary academic center ("Hôpital de l'Enfant-Jésus") from July 2006 to October 2016. Demographics characteristics, tumor characteristics, presenting symptoms, frontal sinus surgery technique (osteoplastic flap, endoscopic surgery, or a combination of both), and outcomes of giant frontal sinus osteomas (≥30 mm) were recorded. For laterally placed osteomas, tumors with posterior wall involvement, orbital roof involvement, or intracranial extension, the modified unilateral osteoplastic flap approach was used. A decision-making algorithm is proposed for the choice of surgical approach. Ten giant frontal osteomas were analyzed (7 men and 3 women). The mean age at diagnosis was 38 years old (range, 24-55 years; median, 39 years; standard deviation, 11 years). The most common presenting symptom was headache (43% of symptomatic patients). Five patients had complications preoperatively due to tumoral extension (sinusitis, cellulitis, mucocele, optic nerve compression, and convulsions). One patient was treated endoscopically, 3 patients had an open approach and 6 patients had a combined technique. One patient experienced a postoperative complication (local infection treated with oral antibiotics). Six patients had minimal residual tumor with one patient needing reoperation. Osteomas are rare paranasal sinus tumors. Due to the proximity to noble structures, a giant frontal osteoma should be managed surgically. The modified unilateral osteoplastic flap without obliteration offers good long-term surgical and aesthetic results. Osteomas are not known for malignant transformation and recurrences are rare; thus, subtotal resection is warranted and safe when a cleavage plan is not found.

Characteristics of Benign and Malignant Bone Tumors Registered in the Hiroshima Tumor Tissue Registry, 1973-2012.

Background:Reported information on the characteristics of benign bone tumors is disjointed, and the long-term trends in the occurrence of malignant bone tumors by histological type have not been reported in Japan. Our aim was to describe the characteristics of both benign and malignant bone tumors as described in cases registered in the Hiroshima Tumor Tissue Registry from 1973 to 2012.Methods:Cases were identified with the International Classification of Diseases for Oncology (ICD-O-3) topography code C40-C41 (bones, joints, and articular cartilage), and histological types were classified according to the World Health Organization 2013 system. We described the distribution of the cases by behavior, sex, skeletal site of tumor occurrence, histological type, period at diagnosis (in 10-year groups), and age at diagnosis (in 10-year groups).Results:We observed 2,542 benign bone tumors, 272 intermediate bone tumors, and 506 malignant bone tumors. We confirmed that 81.6% of benign bone tumors were chondrogenic, consisting primarily of osteochondromas and enchondromas. Giant cell tumor of bone was the most dominant type of intermediate tumor, whereas osteogenic tumors and chondrogenic tumors were the most dominant types of malignant tumors. Among malignant bone tumors, 41.7% of tumors occurred in the long bones of the lower limb, and there were different peaks of age at the time of diagnosis for osteogenic tumors and chondrogenic tumors. A similar distribution of histological types was seen throughout the 40-year observation period.Conclusions:Osteochondroma and enchondroma differed in terms of the age of the patient at the time of diagnosis and the skeletal sites where the tumors most frequently occurred. Giant cell tumor had a large impact on occurrence as a common type of intermediate bone tumor.Clinical Relevance:The results of the present study, based on pathological tissue registry data, provide knowledge about the epidemiological and pathological features of bone tumors in Japan.

Peripheral Osteoma of Mandibular Angulus: Analysis of the Literature and Report of a New Case

Osteoma is a benign osteogenic neoplasm originating from the continuous proliferation of compact and/or cancellous mature bone. The tumor can be classified as peripheral, central or extra-skeletal regarding location and it commonly seen in the cranio-facial region especially at the skull and paranasal sinuses. The exact etiology of the tumor is still controversial; however, it is considered that infection, trauma, muscle activity contributes the occurrence of the tumor. Due to the slow growing nature of osteoma, it is coincidentally detected on radiographs or when the tumor reaches a large size enough to trigger symptoms and cause facial disfigurement. Although mainly detected in the craniofacial bones, osteomas are rarely located in the jaw bones. The purpose of this review, is to present the diagnosis and treatment plan of a peripheral osteoma in the mandibular angulus region of an 8-year-old boy together with a review of published cases of peripheral osteomas of mandibular angulus.

Surgical Treatment of Osteoma in the Basilar Region of the Mandible.

Osteoma is an uncommon benign osteogenic tumor characterized by the proliferation of compact and cancellous bone. The objective of this study was to report a case of osteoma in the basilar region of the mandible. The patient reported no pain, but the affected area was tender to touch and there was esthetic concern because of the apparent swelling. The mass measured approximately 5 cm in diameter and extended to the lingual area of the mandible. A vestibular approach was used for surgical treatment. After infiltration of a local anesthetic and vasoconstrictor, to expose the mandibular ramus, an incision was made with an electric scalpel along the external oblique ridge through the mucosa, submucosa and muscle, fascia and periosteum until the lower second molar, 3 to 5 mm inferior to the mucogingival junction. A mucoperiosteal flap was raised until the base of the mandible to access the tumor. Osteotomy was performed and the tumor was removed. The patient shows no recurrence of the tumor after 1 year and 6 months of follow-up. Periodical clinical and radiographic follow-up are satisfactory since recurrence is rare.

Osteoma: A Rare Case of Painless Postauricular Swelling.

Osteomas are osteogenic tumors that commonly affects frontal and ethmoidal sinuses. Temporal osteomas are extremely rare with less than 200 cases have been reported in the literature; within the temporal bone, osteoma rarely affects the mastoid but commonly involves external auditory meatus, middle ear, and styloid process. We present here a case of osteoma of mastoid region of temporal bone presenting as a single painless postauricular mass.

Peripheral osteoma of the mandibular crest: a short case study

Introduction: Osteoma is a benign slow-growing osteogenic neoplasm characterized by the proliferation of cancellous and/or cortical bone. Jaw bones are seldom affected. Observation: We observed a rare case of a patient with a peripheral mandibular osteoma, which was surgically removed. Comments: Frequently asymptomatic, a peripheral osteoma looks like a bony swelling that may be sessile or pedunculated. Imaging examinations show a well-circumscribed radio-opaque mass. Symptomatic osteomas must be surgically excised and submitted for histological evaluation. Conclusion: Excessive osseous healing following a tooth extraction may explain this rare form of osteoma.

Peripheral osteoma of the mandible: case report and review of the literature

Osteomas are benign, slow-growing osteogenic tumors commonly occurring in the craniofacial bones. Osteomas are characterized by the proliferation of compact and/or cancellous bone. It can be of a central, peripheral, or extraskeletal type. The peripheral type arises from the periosteum and is rarely seen in the mandible, if involved, the lingual surface and lower border of the body are the most common locations of these lesions. They are usually asymptomatic and can be discovered in routine clinical and radiographic examination. This paper presents a large solitary peripheral osteoma located in the lingual surface of the right posterior mandible. The osteoma was removed surgically, and no recurrence has been observed.Journal of Kathmandu Medical College, Vol. 5, No. 4, Issue 18, Oct.-Dec., 2016, page: 128-130

Efficient generation of P53 biallelic knockout Diannan miniature pigs via TALENs and somatic cell nuclear transfer

BackgroundPigs have many features that make them attractive as biomedical models for various diseases, including cancer. P53 is an important tumor suppressor gene that exerts a central role in protecting cells from oncogenic transformation and is mutated in a large number of human cancers. P53 mutations occur in almost every type of tumor and in over 50% of all tumors. In a recent publication, pigs with a mutated P53 gene were generated that resulted in lymphoma and renal and osteogenic tumors. However, approximately 80% of human tumors have dysfunctional P53. A P53-deficient pig model is still required to elucidate.MethodsTranscription activator-like effector nucleases (TALENs) were designed to target porcine P53 exon 4. The targeting activity was evaluated using a luciferase SSA recombination assay. P53 biallelic knockout (KO) cell lines were established from single-cell colonies of fetal fibroblasts derived from Diannan miniature pigs followed by electroporation with TALENs plasmids. One cell line was selected as the donor cell line for somatic cell nuclear transfer (SCNT) for the generation of P53 KO pigs. P53 KO stillborn fetuses and living piglets were obtained. Gene typing of the collected cloned individuals was performed by T7EI assay and sequencing. Fibroblast cells from Diannan miniature piglets with a P53 biallelic knockout or wild type were analyzed for the P53 response to doxorubicin treatment by confocal microscopy and western blotting.ResultsThe luciferase SSA recombination assay revealed that the targeting activities of the designed TALENs were 55.35-fold higher than those of the control. Eight cell lines (8/19) were mutated for P53, and five of them were biallelic knockouts. One of the biallelic knockout cell lines was selected as nuclear donor cells for SCNT. The cloned embryos were transferred into five recipient gilts, three of them becoming pregnant. Five live fetuses were obtained from one surrogate by caesarean section after 38 days of gestation for genotyping. Finally, six live piglets and one stillborn piglet were collected from two recipients by caesarean section. Sequencing analyses of the target site confirmed the P53 biallelic knockout in all fetuses and piglets, consistent with the genotype of the donor cells. The qPCR analysis showed that the expression of the P53 mRNA had significant reduction in various tissues of the knockout piglets. Furthermore, confocal microscopy and western blotting analyses demonstrated that the fibroblast cells of Diannan miniature piglets with a P53 biallelic knockout were defective in mediating DNA damage when incubated with doxorubicin.ConclusionTALENs combined with SCNT was successfully used to generate P53 KO Diannan miniature pigs. Although these genetically engineered Diannan miniature pigs had no tumorigenic signs, the P53 gene was dysfunctional. We believe that these pigs will provide powerful new resources for preclinical oncology and basic cancer research.

The prognostic value of PCNA expression in patients with osteosarcoma: A meta-analysis of 16 studies.

Background:Numerous studies have attempted to determine the prognostic role of proliferating cell nuclear antigen (PCNA) expression in patients with osteosarcoma with no consistent conclusion. We performed this meta-analysis to systematically elucidate the association in a more precise manner.The purpose of this meta-analysis is to determine the prognostic role of PCNA in patients with osteosarcoma.Methods:A systematic search of relevant studies was performed in 6 electronic databases including PubMed, Embase, Web of Science, Wanfang database, China National Knowledge Internet (CNKI) database, and Chinese Biological Medical (CBM) Database (up to March 1, 2016) with the following keywords: (PCNA OR proliferating cell nuclear antigen) AND (osteosarcoma OR osteogenic tumor). A manual search of references on relevant articles was also conducted by 2 investigators independently. We performed a comprehensive evaluation of the correlation between PCNA expression and overall survival (OS) or disease-free survival (DFS) by calculating relative ratios (RR) and their corresponding 95% confidence intervals (CI) using STATA software. A fixed- or random-effect model was chosen based on the between-study heterogeneity.Results:In total, 16 studies with 691 osteosarcoma patients were included in this meta-analysis. PCNA overexpression was found in approximately 57.31% of the patients with osteosarcoma. The meta-analysis suggested that PCNA overexpression in osteosarcoma patients is associated with low OS, but not significantly with DFS (RR = 1.82, 95% CI 1.53–2.18, P = .000; RR = 1.15, 95% CI 0.91–1.44, P = 0.234). Sensitivity analysis for OS and DFS showed no significant difference and the pooled RRs were stable when the included studies were removed one by one. Similar results were also obtained for subgroup analysis based on different follow-ups and cutoffs to determine PCNA expression.Conclusion:The findings from this meta-analysis indicate that PCNA overexpression is an effective biomarker for poor prognosis in patients with osteosarcoma for OS. Hence, more large-scale studies are still needed to further warrant this conclusion.

Peripheral Solitary Osteoma of the Zygomatic Arch: A Case Report and Literature Review.

Osteoma is a benign slow-growing osteogenic neoplasm commonly occurring in the craniofacial skeleton, characterized by the proliferation of compact and/or cancellous bone. Osteomas may be peripheral, central, or extraskeletal. Peripheral osteomas arise from the periosteum and are quite uncommon in the jaw bones. The exact aetiology and pathogenesis of peripheral osteoma are unknown. Clinically, peripheral osteomas are usually asymptomatic, but depending on the location and size of the lesion, it may cause swelling, pain, esthetic disfigurement and functional impairment. On radiological imaging, a peripheral osteoma appears often as well-circumscribed, round to oval, pedunculated radiopaque mass attached to the cortex by a broad base or a pedicle. Asymptomatic osteomas are treated conservatively, while surgical excision is indicated when the lesion is symptomatic, actively growing, or for cosmetic reasons. Histologically, osteomas are composed of a normal-appearing, dense mass of lamellar bone. Recurrence of peripheral osteoma after surgical removal is extremely rare and there are no reports of malignant transformation. A review of the literature disclosed only 7 well-documented cases of peripheral osteoma located at the zygomatic bone. The purpose of this article is to present the clinical, radiographic, surgical and histological features of a solitary peripheral osteoma of the left zygomatic arch in a 55-year-old woman and to review the literature about this uncommon pathologic entity.

Solitary peripheral osteoma of the body of the mandible

Osteomas are benign osteogenic tumors that are often slow growing and frequently arising in the craniofacial bones and rarely originating from the mandible. Central, peripheral, and extraskeletal are the three varieties of osteomas of which the central and the peripheral types are commonly seen in the facial bones. Peripheral osteomas most commonly were seen in the frontal, ethmoidal, and maxillary sinus and very rarely seen in the jaws. Typically asymptomatic in nature, osteoma presents itself as a welldefined radiopaque mass. Herein, we report a rare case of peripheral osteoma of the body of the mandible of a 45-year-old female patient.

Mastoid osteoma: Report of a case and literature review

Mastoid osteoma is a benign osteogenic tumor with few reported cases in the literature. The exact cause is unknown; however, developmental anomaly, trauma, and infection have been implicated. Majority are asymptomatic, and diagnosis can be made with computed tomography scan. Surgical excision is curative and offers acceptable cosmetic results. We aimed to report a case of mastoid osteoma coexisting with subgaleal lipoma and outline its management. A 35-year-old woman presented with left mastoid and frontal swellings because of unacceptable disfigurement. Clinical and radiological evaluation was suggestive of mastoid osteoma and sublingual lipoma. The diagnosis was confirmed by histology, after total surgical excision. The patient made uneventful recovery and had acceptable curative and esthetic results. Mastoid osteoma can occur alone or in association with other lesions such as lipoma, especially in syndromic type. In general, asymptomatic and cranial computed tomography scan remained an imaging of choice and surgical resection confers good outcome.

Le Fort I Disassembly for the Management of Juvenile Ossifying Fibroma Extending To the Skull Base.

Ossifying fibroma is a rare benign osteogenic neoplasm arising from undifferentiated cells of the periodontal ligament. Ossifying fibroma have a well-defined border that differentiates it from fibrous dysplasia clinically; these tumors manifest as a round or ovoid, expansile, painless, slow-growing mass may displace the roots of adjacent teeth and also cause root resorption. A variety of approaches for resection of the maxilla have been described. Most involve the use of facial and lip-splitting incisions to gain wide access. Surgical approach specifically to the ossifying fibroma located in the midface includes the Le Fort I approach, Caldwell-Luc access, lateral rhinotomy with medial or total maxillectomy, external ethmoidectomy, and endoscopic surgery. The access through Le Fort I disassembly is a versatile approach not only because of the aesthetic potential in using intraoral incision but also due to its minimal invasiveness, lesser complications and gives the possibility of reconstruction in a single operation. Le Fort I disassembly followed by an excision appears to be a versatile, secure, and satisfactory option.

Osteogenic tumour in Australopithecus sediba: Earliest hominin evidence for neoplastic disease

We describe the earliest evidence for neoplastic disease in the hominin lineage. This is reported from the type specimen of the extinct hominin Australopithecus sediba from Malapa, South Africa, dated to 1.98 million years ago. The affected individual was male and developmentally equivalent to a human child of 12 to 13 years of age. A penetrating lytic lesion affected the sixth thoracic vertebra. The lesion was macroscopically evaluated and internally imaged through phase-contrast X-ray synchrotron microtomography. A comprehensive differential diagnosis was undertaken based on gross- and micro-morphology of the lesion, leading to a probable diagnosis of osteoid osteoma. These neoplasms are solitary, benign, osteoid and bone-forming tumours, formed from well-vascularised connective tissue within which there is active production of osteoid and woven bone. Tumours of any kind are rare in archaeological populations, and are all but unknown in the hominin record, highlighting the importance of this discovery. The presence of this disease at Malapa predates the earliest evidence of malignant neoplasia in the hominin fossil record by perhaps 200 000 years.

The utility of SATB2 immunohistochemical expression in distinguishing between osteosarcomas and their malignant bone tumor mimickers, such as Ewing sarcomas and chondrosarcomas

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.

Coincidence of Compound Odontoma and Cemento Ossifying Fibroma; a rare case report

Objective: Cemento-ossifying fibroma defines as a relative rare osteogenic neoplasm of the jaw. This tumor includes fibrous and osseous components. Odontoma is the most common odontogenic tumor containing enamel ,dentin ,cementum and pulp tissue. in this paper we report a rare case of ossifying fibroma associated with compound odontoma in the mandible. Case: A 37-years-old woman was referred to Oral Medicine department , Shahid Beheshti Dental School with complaint of swelling in the anterior part of the mandible, over 6 years period. Clinical examination revealed mandibular enlargement in right –anterior region with labial and lingual expansion and canine missing . panoramic view showed a large mixed radiolucent - radiopaque lesion associated with impacted canine.The differential diagnosis include calcifying odontogenic cyst (COC) and cemento-ossifying fibroma(COF). Histopathologic examination established diagnosis of COF with multiple compound odontoma .Conclusion: The relationship between the occurrence of these two lesions is not clear and more studies are needed to establish the relationship between them.

Osteogenic tumors of bone

Osteogenic tumors include malignant and benign tumors that produce tumor osteoid and/or bone tissue. Osteosarcoma is the most common malignant bone tumor, especially in children and young adults. The entities with their characteristic morphological features are described to enable the reader to come to a diagnosis and differential diagnosis on the basis of patient age, history and predominant location of the tumor. For this review we selectively used mainly large published patient cohorts. Our own and externally published data on widely accepted tumor criteria were also compared. Detection is the initial diagnostic step for an osseous lesion, and is determined by the sensitivity of the method applied. Plain X‑ray films in twoplanes and CT are the basics in the radiological toolkit for osteogenic tumors. For evaluation of local tumor extension and biopsy planning MRI or scintigraphy should be combined. MRI as a stand-alone diagnostic tool is insufficient. For malignant bone tumors staging should be performed, applying avariable combination of thoracic CT, MRI, scintigraphy, and positron emission tomography (PET). Osteosarcoma, along with Ewing sarcoma and chondrosarcoma, are the most common malignant bone tumors; all sub-entities are significantly rarer. Among benign bone tumors, osteoid osteomas have the highest incidence, presenting with typical pain, location, and age predilection. Diagnostics and treatment of malignant bone tumors should preferably be performed in specialized centers because of significant therapeutic implications for patients. In uncertain cases, asecond opinion should always be obtained.

HER-2 expression in biopsy and surgical specimen on prognosis of osteosarcoma

Numerous original clinical studies have attempted to investigate the prognostic value of HER-2 overexpression in osteosarcoma, but the results of these studies are not consistent. This meta-analysis and systematic review was performed to further assess the correlation between HER-2 expression and prognosis in patients with osteosarcoma. A detailed search of relevant publications was conducted using 7 electronic databases: PubMed, Embase, the Cochrane library, the Wanfang database, the China National Knowledge Internet (CNKI) database, the Chinese VIP database, and the Chinese Biological Medical (CBM) Database for publications through August 1, 2015, using the following keywords (HER-2 OR ErbB-2 OR C-erbB-2 OR neu) AND (osteosarcoma OR osteogenic tumor). The bibliographies of potentially relevant articles and identified articles were then searched by hand. Eligible studies were those that enrolled participants with osteosarcoma and provided survival outcome in HER-2 positive and negative groups. The hazard ratio (HR) and 95% confidence interval (CI) for each individual study was calculated and pooled to obtain integrated estimates, using random effects modeling. Sixteen studies involving 934 participants with osteosarcoma met our inclusion criteria. HER-2 overexpression was documented in 42.2% of patients with osteosarcoma. Compared with patients without HER-2 overexpression, those overexpressing HER-2 had decreased overall survival (HR = 2.03, 95% CI: 1.36–3.03, P < 0.001). Statistical associations between HER-2 overexpression and unfavorable overall survival (OS) were observed for both biopsy and surgical removal specimens (HR = 2.07, 95%CI: 1.16–3.72, P = 0.014; and HR = 2.02, 95%CI: 1.10–3.71, P = 0.024). Results for disease-free survival (DFS) were similar. Overexpression of HER-2 is significantly associated with poor outcome for patients with osteosarcoma and should be assessed at diagnosis and after surgery as a prognostic factor. However, larger-scale multicenter clinical studies are needed to further support these findings.