Osteogenic Tumors Research Articles

Peripheral osteoma in a young patient: A marker for precancerous condition?

Osteoma is a benign osteogenic tumor arising from the proliferation of cancellous or compact bone. The osteoma can be central, peripheral, or of an extraskeletal type. Peripheral type of osteoma is most common in the lower jaws, which occurs at the surface of the cortical bone and is sessile or pedicled. The overall incidence of osteoma is low, affecting 0.01-0.04% of the population; osteomas comprise 12.1% of benign bone tumors and 2.9% of all bone tumors. Most of the osteomas occurring in the mandible are dense osteomas, and the cancellous osteoma is comparatively rare. Maxillofacial osteoma associated with cutaneous sebaceous cysts, multiple supernumerary teeth, and colorectal polyposis is known as Gardener's syndrome. However, in some cases, maxillofacial osteomas with multiple impacted and supernumerary teeth are not accompanied by a fixed complex of symptoms. We report one such case in a 15-year-old female patient.

Two Huge Maxillofacial Osteoma Cases Evaluated By Computed Tomography

Osteomas are benign osteogenic neoplasms or hamartomas with a very slow growth rate. Osteoma is the most common mesenchymal neoplasm of the paranasal sinuses. In the jaws, the mandible is more commonly involved than the maxilla. Osteomas may occur at any age, but most frequently are found in individuals older than 40 years. Although most osteomas are small, some may become large enough to cause severe damage, especially those that develop in the frontoethmoid region. Osteomas composed solely of compact bone are uniformly radiopaque and those containing cancellous bone show evidence of internal trabecular structure. To determine and evaluate the exact extension and internal structure of these lesions, computed tomography (CT) is a more useful imaging modality in comparison to conventional radiography. Hereby, we discuss clinical and imaging features of two osteomas (one in the ethmoid sinus and the other in the mandible) along with the main differential diagnoses and pathologic features.

Clinical-morphological peculiarities of osteoblastomata and their differential diagnosis

Osteoblastoma (OB) is a rare benign osteogenic tumour formed by interwoven with one another osteal trabeculae, which are surrounded by a lot of osteoblasts. The purpose of the present work was to elucidate the nomenclature and main clinical-visualizing pathohistologic features of OB as a true osteogenic tumor, as well as some aspects of its differential diagnosis with other osteogenic tumors and tumor-like bone lesions. Forty-three cases of biopsies obtained from 39 patients with OB served as the material for this research. The article contains data about clinical-visualizing features of OB (X-ray and CT), its macropathology, histopathology and differential diagnosis, as well as clinical-morphological gradations of osteogenic tumours. It was found out that OB passed clinically as an active or aggressive form with signs of a local destructive growth and inclined to relapsing in case of its incomplete removal, but very seldom it subjected to malignancy. OB often becomes an object of complicated differential-diagnostic solutions, which are possible only with application of both clinical-visualizing and histological methods with priority of the latter ones. The nosological units for differential diagnosis of OB should include the following ones: osteoid-osteoma, ossifying periostitis, chronic osteomyelitis (corticolitis-periostitis), osteoblastoma-like central osteosarcoma with a low grade of malignancy.

Primary Neoplasms of Bones in Mice

To compare and summarize the mechanisms, frequencies of occurrence, and classification schemes of spontaneous, experimental, and genetically engineered mouse skeletal neoplasms, the literature was reviewed, and archived case material at The Jackson Laboratory was examined. The frequency of occurrence of spontaneous bone neoplasms was less than 1% for most strains, with the exceptions of osteomas in CF-1 (5.5% and 10% in two studies) and OF-1 outbred strains (35%), and osteosarcomas in NOD/ShiLtJ (11.5%) and NOD-derived (7.1%) mice. The frequency was 100% for osteochondromas induced by conditional inactivation of exostoses (multiple) 1 (Ext1) in chondrocytes, osteosarcomas induced by tibial intramedullary inoculation of Moloney murine sarcoma virus, and osteosarcomas induced by conditional inactivation of Trp53-with or without inactivation of Rb1-in osteoblast precursors. Spontaneous osteogenic neoplasms were more frequent than spontaneous cartilaginous and vascular types. Malignant neoplasms were more frequent than benign ones. The age of occurrence for spontaneous neoplasms ranged from 37 to 720 days (M = 316.35) for benign neoplasms and 35 to 990 (M = 299.28) days for malignant. In genetically engineered mice, the average age of occurrence ranged from 28 to 70 days for benign and from 35 to 690 days for malignant. Histologically, nonosteogenic neoplasms were similar across strains and mutant stocks; osteogenic neoplasms exhibited greater diversity. This comparison and summarization of mouse bone neoplasms provides valuable information for the selection of strains to create, compare, and validate models of bone neoplasms.

Prevalence of bone and soft tissue tumors

Multidisciplinary approach is a necessity for the appropriate diagnosis and treatment of bone and soft tissue tumors. The Ege University Musculoskeletal Tumor Council offers consultation services to other hospitals in the Aegean region. Since 1988 the Council has met weekly and spent approximately 1,500 hours evaluating almost 6,000 patients with suspected skeletal system tumors. Our objective was to present the data obtained from this patient group. A total of 5,658 patients, suspected to have a musculoskeletal tumor, were evaluated retrospectively. Multiple records of the patients due to multiple attendance to the Council were excluded. The prevalance of the bone and soft tissue tumors in these patients were analysed. Malignant mesenchymal tumors accounted for 39.7% of the total patients, benign tumors for 17%, tumor-like lesions for 17.8% and metastatic carsinomas for 8.6%. Malignant bone tumors were 50.2% and malignant soft tissue tumors were 49.8% of all the sarcomas. Among the malignant bone tumors the most common was osteosarcomas at a rate of 33.6%, followed by Ewing-PNET at 25.5%, chondrosarcomas at 19.4% and haematopoietic tumors at 17.6%. Pleomorphic sarcomas (24.5%), liposarcoma (16.4%), synovial sarcoma (13%) and undifferential sarcomas (8.8%) were the most common types of malignant sof tissue tumors. Benign soft tissue tumors (48%), benign cartilage tumors (28%), giant cell tumor (15%) and osteogenic tumors (9%) were found among the benign tumors. Hemangioma, lipoma, agressive fibromatosis, enchondroma, solitary chondroma and osteoid osteoma were the most common tumors in their groups. Lung (27%), breast (24%), gastrointestinal system (10.5%) and kidney (8.2%) carcinomas were the most common primary sites of the bone metastasis. Turkey still lacks a comprehensive series indicating the incidence and diagnostic distribution of bone and soft tissue tumors. The presented data would add to our knowledge on the specific rates of the bone and soft tissue tumors in Aegean region.

The mastoid osteoma, an incidental feature?

Osteoma in the mastoid is a rare benign osteogenic tumour that has been described in literature in only 137 cases. It usually appears in asymptomatic patients, although a few cases are described associated with clinical manifestations. We report three cases of mastoid osteoma: a pedunculated osteoma in the aditus ad antrum (associated with a cholesteatoma), a superficial osteoma of the mastoid surface and a sessile osteoma that progressed to the temporal lobe (associated with vertigo). A brief review of this rare entity is presented and a possible association between mastoid osteoma, cholesteatoma otitis and vertigo is posed.

El osteoma en la mastoides, ¿un hallazgo incidental?

Osteoma in the mastoid is a rare benign osteogenic tumour that has been described in literature in only 137 cases. It usually appears in asymptomatic patients, although a few cases are described associated with clinical manifestations. We report three cases of mastoid osteoma: a pedunculated osteoma in the aditus ad antrum (associated with a cholesteatoma), a superficial osteoma of the mastoid surface and a sessile osteoma that progressed to the temporal lobe (associated with vertigo). A brief review of this rare entity is presented and a possible association between mastoid osteoma, cholesteatoma otitis and vertigo is posed.

Abstract 4836: RB/E2F1 in the crossroad of autophagy and apoptosis

Abstract Autophagy is a protective mechanism that renders cells viable in stressful conditions. Mounting evidence suggests that this cellular process is also a tumor suppressor pathway. We hypothesized that retinoblastoma protein (RB), a key tumor suppressor, induces autophagy. To test this hypothesis, we first transduced RB into RB-defective human cancer cells: sarcoma osteogenic (Saos-2) cells, hepatoma (Hep3B) cells, and brain tumor stem cells (MDNSC23). The ectopic RB induced autophagy as demonstrated by significantly increased percentage of cells with acidic vesicular organelles (from less than 10% to more than 30%, P < 0.05), and the lipidation of LC3-I leading to the formation of autophagosomes/autolysosomes that were visualized by EGFP-LC3 punctation (from less than 20% to more than 80%, P = 0.003). These double-membraned vesicles were clearly shown by ultrastructural study via transmission electron microscopy. However, when Beclin 1 was silenced with siRNA in Saos-2 cells, the RB-mediated autophagy was blocked. In addition, study of autophagy flux with Saos-2 cells expressing double-tagged mRFP-EGFP-LC3 fusion protein revealed that RB stimulated the formation and maturation of autophagosomes. Consistently, RB activators p16INK4a and p27/kip1 caused autophagy in an RB-dependent manner. Importantly, we found RB mutants Delta-22 and R661W deficient for binding E2F failed to induce autophagy. Moreover, E2F1 overexpression antagonized RB-mediated autophagy, leading to apoptosis in Saos-2 cells. In agreement with the above observations, silencing E2F1 with siRNA resulted in autophagy in U-87 MG cells; and autophagy levels increased from 4% in wild-type MEFs to 40% in E2F1 knockout MEFs (P = 0.002). Collectively, our data reveal that RB/E2F1 plays a key role in the decision of a cell to undergo autophagy or apoptosis. Citation Format: {Authors}. {Abstract title} [abstract]. In: Proceedings of the 101st Annual Meeting of the American Association for Cancer Research; 2010 Apr 17-21; Washington, DC. Philadelphia (PA): AACR; Cancer Res 2010;70(8 Suppl):Abstract nr 4836.

Large Peripheral Osteoma of the Mandible: A Case Report

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. 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. In this paper, we presented a large solitary peripheral osteoma located in the buccal surface of the left posterior mandible and causing facial deformity in a 37-year-old woman. Radiographic examination by computed tomography revealed radiopacity with a well-circumscribed, pedunculated mass approximately 3 cm in size. The osteoma was removed surgically, and no recurrence has been observed.

A Simple Look-Up On Soft Tissue Osteoma: Report Of A Case.

Soft tissue tumors of the body represent wide variety. Osteoma is a benign, slow-growing osteogenic tumor that is primarily located in the maxillofacial region. However, soft tissue osteoma or osteoma cutis or extraskeletal osteoma is observed extremely rare. Here, we report a soft-tissue osteoma located on the dorsum of the body that had started and grown silently. The main complaint was the mass. Excisional biopsy was successfully carried out. After all, the pathological analysis consisted of osteoma within the cutis.

Gastrointestinal Hyperplasia with Altered Expression of DNA Polymerase β

BackgroundAltered expression of DNA polymerase β (Pol β) has been documented in a large percentage of human tumors. However, tumor prevalence or predisposition resulting from Pol β over-expression has not yet been evaluated in a mouse model.Methodology/Principal FindingsWe have recently developed a novel transgenic mouse model that over-expresses Pol β. These mice present with an elevated incidence of spontaneous histologic lesions, including cataracts, hyperplasia of Brunner's gland and mucosal hyperplasia in the duodenum. In addition, osteogenic tumors in mice tails, such as osteoma and osteosarcoma were detected. This is the first report of elevated tumor incidence in a mouse model of Pol β over-expression. These findings prompted an evaluation of human gastrointestinal tumors with regard to Pol β expression. We observed elevated expression of Pol β in stomach adenomas and thyroid follicular carcinomas, but reduced Pol β expression in esophageal adenocarcinomas and squamous carcinomas.Conclusions/SignificanceThese data support the hypothesis that balanced and proficient base excision repair protein expression and base excision repair capacity is required for genome stability and protection from hyperplasia and tumor formation.

Juvenile aggressive ossifying fibroma of the maxilla

Juvenile aggressive ossifying fibroma is an uncommon and controversial, benign but aggressive osteogenic neoplasm of the jaws commonly occurring in children and young adults. Complete removal of the tumor at the earliest possible stage is recommended owing to its high recurrence rate of 30-58%. Reported here is a case of recurrent juvenile aggressive ossifying fibroma of the maxilla in a 15-year-old male treated with enucleation and curettage. A 10-month follow-up showed no signs of recurrence.

P.004 Endoscopic frontal osteoma removal

Osteomas are uncommon, slow-growing, benign osteogenic neoplasms that arise most frequently in the craniofacial skeleton.1, 2 Osteoma is the most common benign tumor of the nose and paranasal sinuses and the most common neoplasm of the frontal sinus.3, 4, 5 Paranasal sinus osteomas originate in the sinus wall, fill the lumen with well-defined mature osseous tissue, and occasionally extend into the orbit where they give rise to orbital signs and symptoms. Osteomas most commonly become symptomatic in the second to fifth decade in life, but orbital involvement has rarely been reported in patients aged 18 years and younger.2, 6, 7, 8, 9, 10 We report a case of a frontal sinus osteoma with orbital extension in a 13-year-old girl.

Osteogenic tumours in Lkb1-deficient mice

Germline mutation in LKB1 is the cause of Peutz–Jeghers Syndrome in humans, a rare disorder predisposing to cancer and multiple gastrointestinal hamartomous polyps. Mice harboring a germline inactivating Lkb1 mutation develop similar gastrointestinal polyps and liver neoplasia. We observed paralysis in ∼ 2% of Lkb1 +/− mice on two genetic backgrounds, C57BL/6J and 129/sv, at around 300 days of age. Stepped serial sectioning of the whole spinal column found multiple osteogenic tumours that were lobulated, showed osteoid formation and had an infiltrative growth pattern, which extended into the surrounding muscle. Osteogenic tumours were also present in asymptomatic Lkb1 +/− mice ( n = 12) in the lateral spinous processes, spinous vertebral bodies and the bodies of sacral tail vertebrae. Although asymptomatic, the proliferation in several mice caused a narrowing and compression of the spinal canal. The long bones of Lkb1 +/− mice had osteoblastosis within the femur and tibia indicating that the process is multi-focal; bone remodelling was accompanied by angiogenesis. No wild type Lkb1 +/+ siblings ( n = 12) showed aberrant osteoblastosis or bone remodelling. This is the first report of multifocal osteoblastic tumours in Lkb1 +/− mice and our observations indicate that Lkb1, like Pten, may have a distinct role in controlling osteoblast proliferation in the mouse.

Solitary central osteoma of the jaws: a diagnostic dilemma

Osteomas are relatively rare benign osteogenic neoplasms, characterized by the proliferation of compact and/or cancellous bone. Osteomas can develop as peripheral (periosteal) masses attached to the cortical plates or as central lesions arising from endosteal bone surfaces. Although multiple osteomas of the jaws are a hallmark of Gardner syndrome (familial adenomatous polyposis), nonsyndromic cases are typically solitary. A search of the literature identified 91 well documented cases of solitary osteoma of the jaws published since 1955: 93.4% were peripheral and only 6.6% central, a ratio of 14:1 in favor of peripheral osteomas. Solitary central osteomas of the jaws seem to be very rare, with only 6 such cases reported since 1955. Adding the 4 new cases described here brings the total to 10 central osteomas, 30% occurring in the maxilla and 70% in the mandible. Whereas peripheral osteomas are fairly easy to diagnose, central osteomas pose a more challenging diagnostic problem and need to be differentiated from other similar lesions of the jaws, such as central ossifying fibroma, condensing osteitis, idiopathic osteosclerosis, osteoblastoma, cementoblastoma, and complex odontoma. With such a low number of cases of central osteoma reported, the objectives of the present report are to describe 4 new cases, analyze the clinical and radiographic characteristics, and discuss the features which distinguish these lesions from other similar lesions of the jaws.

Parosteal Osteosarcoma of the Orbit

A 60-year-old woman was referred to Bristol Eye Hospital because of a progressive, painless, right proptosis. CT revealed a well-defined, hyperdense lesion adjacent to the lateral orbital wall. A marginal excision was performed. The mass was stony-hard, lobulated, and encapsulated. Histopathologic findings were consistent with a low-grade parosteal osteosarcoma. Parosteal osteosarcoma is a rare osteogenic tumor that usually affects the long bones. It represents a malignant, though well differentiated, tumor that has a relatively good prognosis after a wide excision. However, local recurrences are not rare and have been associated with dedifferentiation. The latter affects the prognosis adversely and, thus, regular follow-ups are strongly suggested after the initial tumor excision. In the present case, no further treatment was administered and the patient was reported disease free 26 months after surgery.

Role of Wnt Signaling in Bone Remodeling and Repair

The Wnt genes encode a highly conserved class of signaling factors required for the development of several types of tissues including musculoskeletal and neural structures. There is increasing evidence that Wnt signaling is critical for bone mass accrual, bone remodeling, and fracture repair. Wnt proteins bind to cell-surface receptors and activate signaling pathways which control nuclear gene expression; this Wnt-regulated gene expression controls cell growth and differentiation. Many of the components of the Wnt pathway have recently been characterized, and specific loss-of-function or gain-of-function mutations in this pathway in mice and in humans have resulted in disorders of deficient or excess bone formation, respectively. Pharmacologically targeting components of the Wnt signaling pathway will allow for the manipulation of bone formation and remodeling and will have several orthopedic applications including enhancing bone formation in nonunion and osteoporosis and restricting pathologic bone formation in osteogenic tumors and heterotopic ossification.

Nuclear Imaging of Osteoma

Abstract: Osteomas are benign osteogenic tumors that arise fromthe proliferation of cancellous or compact bone. Osteomas areusually found in craniofacial bones. Surgery is indicated only whenthe lesions exhibit cosmetic changes, become symptomatic, or growactively. The usefulness of Tc-99m MDP bone scintigraphy forosteoma has been reported previously. Herein, we report a 32-year-old woman with osteoma of the right temporal bone. Tc-99m MDPbone scintigraphy showed a typical uptake pattern at the tumor site,but no abnormal uptake was observed using F-18 FDG PET. Tc-99mMDP bone scintigraphy is more useful for localizing other sites andmeasuring activity than F-18 FDG PET. Key Words: osteoma, FDG, PET, bone scintigraphy, neoplasm( Clin Nucl Med 2008;33: 135–136) REFERENCES 1. Fidler MW, Hoefnagel CA. Lateral and computerized transverse 99mTc-Mdp bone scintigrams to supplement the anteroposterior bone scintigramfor spinal hot spot localization. Report of a case. Spine . 1984;9:655–657.2. Okuyama T, Umehara I, Kuwabara Y, et al. Bone scintigraphy of themaxillofacial fibro-osseous lesion.

개의 유선에서 발생한 혼합성 골육종 및 샘포 상피세포의 전이성 암종

본 증례는 개의 유선에서 발생한 골형성성의 악성 혼합 유선 종양으로 샘포의 암종성 변화를 동반하고 있다. 종양은 12년령 암캐의 좌측 5번째 유선에서 절제되었으며 직경 <TEX>$2{\sim}2.5cm$</TEX>의 단단한 mass로 절단 시 경도가 높은 골성의 구조를 가지고 있었다. 현미경학적 관찰 시, 골유사 물질이 미네랄 침착되고 있었으며 다수의 골형성 세포와 일부 파골세포가 골생성 종양 기질 전반에 걸쳐서 관찰되었다. 이러한 골유사 병변은 높은 밀집도를 보이는 근상피 세포와 연접하고 있었으며 이러한 세포들은 수 개의 유사분열상을 나타내고 있었다. 이와 더불어, 유선세관과 샘포의 암종성 변화를 보이는 세포들이 인접 기질로 침습하고 있는 모습이 관찰되었고 이것 역시 증가된 근상피 세포들로 둘러싸여 있었다. 본 증례에서 볼 수 있는 이러한 세포들의 출현은 동시 발생된 악성 종양의 형태를 제시할 수 있으며 종양의 기원은 상피 유래의 암종성 조직과 중간엽 유래의 육종성 연골 및 골 조직으로 구별할 수 있겠다. We describe here a case of malignant mixed osteogenic tumor of the mammary gland with alveolar carcinomatous appreance. A firm, 2 to 2.5cm (in diameter) mass under the 5th nipple, showing the structure of extraosseous osteogenic sarcoma, was removed from the left 5th mammary gland of 12-year-old female dog. When investigated under the microscope, the osteoid material undergoing mineralization was surrounded by numerous scattered osteoblasts and a few osteoclastic cells throughout the osteoid tumorous stroma. The osteoid lesions were continuous with hypercellular myoepithelial cells of a very immature character with several mitotic figures. In addition, there were also carcinomatous tubules and alveoli, with invading cells into peripheral stroma, surrounded by myoepithelial cells in the mammary gland. In these lesions, emanating cords of tumor cells appear to be continuous with the myoepithelial cell layer of a duct. The presence of all these cell types suggests the existence of a common malignant origin, the stem cell being differentiated into epithelial carcinomatous and mesenchymal sarcomatous chondral and osteogenic tissues.

Ethmoid osteoma with frontal, nasal and orbital extension: case report and literature review

Introduction: Osteomas are rare slow growing benign osteogenic tumours which are almost exclusively restricted to the craniofacial skeleton. These lesions are often asymptomatic however they may exhibit a variety of symptoms and signs which depend on the location, dimension and extension of the tumour. Case description: We report on a case of an 11-year-old boy with a 3-week history of headaches, facial asymmetry, right orbital proptosis and diplopia on extreme upward gaze. Computed tomography and magnetic resonance imaging demonstrated a calcified mass of the right ethmoid sinus with extension into the frontal sinus, nasal wall and orbit with proptosis of the right globe. The patient underwent a bicoronal flap incision with an osteoplastic frontal sinus flap. The abnormal bone was debulked from the orbit roof and medial wall, ethmoid sinuses and frontal sinus with frontal sinus cranialization. The defects were reconstructed with grafts from the iliac crest. Facial symmetry and resolution of proptosis and diplopia was achieved without complications. Histological examination confirmed the diagnosis of Osteoma. After a 3-year follow-up period, he remained asymptomatic and without recurrence. Conclusion: Surgical treatment is indicated for symptomatic osteomas, although debate persists regarding the optimal approach. Recurrence of these tumours after resection is rare.