Undifferentiated Pleomorphic Sarcoma and the Importance of Considering the Oncogenic and Immune-Suppressant Role of the Human T-Cell Lymphotropic Virus Type 1: A Case Report.

Introduction: Soft-tissue sarcomas account for 0.7% of all malignant tumors, with an incidence rate of 3 per 100,000 persons/year. The undifferentiated pleomorphic sarcoma (UPS) with giant cells, a high grade tumor of soft tissue, is very unusual, especially in young adults before the age of 40.

IDH Mutation Analysis in Ewing Sarcoma Family Tumors.

Background: Isocitrate dehydrogenase (IDH) catalyzes the oxidative decarboxylation of isocitrate to yield α-ketoglutarate (α-KG) with production of reduced nicotinamide adenine dinucleotide (NADH). Dysfunctional IDH leads to reduced production of α-KG and NADH and increased production of 2-hydroxyglutarate, an oncometabolite. This results in increased oxidative damage and stabilization of hypoxia-inducible factor α, causing cells to be prone to tumorigenesis.

Juvenile Paget's disease with heterozygous duplication within TNFRSF11A encoding RANK.

Mendelian disorders of RANKL/OPG/RANK signaling feature the extremes of aberrant osteoclastogenesis and cause either osteopetrosis or rapid turnover skeletal disease. The patients with autosomal dominant accelerated bone remodeling have familial expansile osteolysis, early-onset Paget's disease of bone, expansile skeletal hyperphosphatasia, or panostotic expansile bone disease due to heterozygous 18-, 27-, 15-, and 12-bp insertional duplications, respectively, within exon 1 of TNFRSF11A that encodes the signal peptide of RANK. Juvenile Paget's disease (JPD), an autosomal recessive disorder, manifests extremely fast skeletal remodeling, and is usually caused by loss-of-function mutations within TNFRSF11B that encodes OPG.

Osteosarcoma of the jaw: an analysis of a series of 74 cases.

Aims: To analyse a series of cases of osteosarcoma of the jaw.

Methods And Results: The study included 74 cases of osteosarcoma of the jaw. Their clinical, radiographic and histopathological features were analysed, and their frequency with respect to aggressive and malignant pathologies of the jaw was determined.

Expression of heat shock proteins in osteosarcomas.

Aims: Heat shock proteins (HSPs) protect cells against stress-associated injuries and are overexpressed in several malignant tumours. We investigated the potential roles of HSP27, HSP60, and HSP70 in conventional and low grade central osteosarcoma.

Methods: Expressions of HSP27, HSP60, and HSP70 were analysed using immunohistochemistry on tissue sections from 52 cases of conventional osteosarcoma and 21 cases of low grade central osteosarcoma.

Evaluation of nucleolar organizer regions in maxillary osteosarcoma.

Maxillary osteosarcomas are a relatively frequent malignant tumor of the oral cavity. Similarly to other skeletal osteosarcomas, they exhibit different cellular differentiation patterns, i.e.

Malignant fibrous histiocytoma associated with coxofemoral arthrodesis.

The discovery of biomaterials led to their use in the manufacture of implants for biomedical applications. In vivo, no metal or alloy is completely inert. The potential toxicity of some of the metals most frequently employed in the manufacture of orthopedic implants has been reported.

Upregulation of the oncogenic helix-loop-helix protein Id2 in Ewing sarcoma.

Aims And Background: Id helix-loop-helix proteins function as regulators of cell growth and differentiation. However, they can induce malignant transformation when overexpressed. The EWS/ETS chimeric proteins in Ewing sarcoma act as aberrant transcription factors leading to tumorigenic processes.

Epiphyseal primary location for osteosarcoma and Ewing sarcoma in patients with open physis.

Conventional osteosarcoma and Ewing sarcoma are the most common childhood malignant tumors of bone. These tumors arise in the metaphysis and diaphysis of long bones and may extend secondarily into the epiphysis. Primary epiphyseal location for these tumors is rare and may constitute a diagnostic and therapeutic challenge.

Human leukocyte antigen matching, radiographic score, and histologic findings in massive frozen bone allografts.

Forty-six patients receiving frozen bone allografts, preoperatively tissue typed for human leukocyte antigen and ABO antigens, were radiographically evaluated according to the Musculoskeletal Tumor Society scoring system at a mean followup of 55 months. Patients who matched for 1 or 2 Class I human leukocyte antigens with the donor scored higher than patients totally mismatched, but differences were not significant. Matching for Class II human leukocyte antigen and ABO antigens seemed not to influence radiographic outcome of allografts.

Osteoid osteoma of the hip. Percutaneous resection guided by computed tomography.

Seven patients with a presumptive diagnosis of osteoid osteoma located at the hip were treated with percutaneous resection of the nidus through computed tomography guidance. Histologic confirmation was obtained in 5 of the 7 patients. The average hospital stay was 27 hours.

Adamantinoma of the tibia masked by fibrous dysplasia. Report of three cases.

Three cases of adamantinoma of the tibia associated with and mimicking fibrous dysplasia or osteofibrous dysplasia are reported in children aged three, nine, and 16 years. The roentgenographic features were typical of intracortical fibrous dysplasia (osteofibrous dysplasia). These entities are not two distinct diseases, but rather are different histologic expressions of essentially the same process, which varies depending on location (predominantly intracortical or medullary) and age.

Osteosarcomas arising on the surfaces of long bones.

Malignant bone-forming tumors that arise from the surfaces of long bones are far less common than those that arise from within bone. These surface osteosarcomas are clinically and radiographically similar, yet histologically they are quite distinct. In reviewing the literature, we classified the tumors according to three subgroups: parosteal (juxtacortical) osteosarcoma, periosteal osteosarcoma, and high-grade surface osteosarcoma.

Metastases of carcinoma in the pagetic bone. A report of two cases.

Two men, aged 68 and 77 years, had metastases of carcinoma in pagetic bones. These cases were initially diagnosed clinically as Paget's sarcoma. Roentgenograms, scintigrams, and bone puncture biopsy specimens (BPBs) of the right scapula and the sacrum, respectively, showed only Paget's disease.

Spontaneous fractures of uninvolved bones in patients with Paget's disease during unduly prolonged treatment with disodium etidronate (EHDP).

Three patients with Paget's disease of bone were treated by disodium etidronate (EHDP) without interruption during periods of 18 to 30 months. In one case the daily dose was also unduly high (approximately equal to 18 mg/kg/day). A moderate to conspicuous diminution of the renal function was observed in all cases.

Sarcoma complicating Paget's disease of bone. A clinicopathological study of 62 cases.

Out of 21 900 cases filed at the Latin-American Registry of Bone Pathology between April 1940 and July 1981, there were 987 with Paget's disease (4.51 per cent); 62 of these (6.28 per cent) were complicated by sarcoma and two were associated with giant-cell tumours of bone (osteoclastoma) without signs of malignancy.