A slowly growing malignant neoplasm derived from cartilage cells, occurring most frequently in pelvic bones or near the ends of long bones, in middle-aged and old people. Most chondrosarcomas arise de novo, but some may develop in a preexisting benign cartilaginous lesion or in patients with ENCHONDROMATOSIS. (Stedman, 25th ed)
A rare aggressive variant of chondrosarcoma, characterized by a biphasic histologic pattern of small compact cells intermixed with islands of cartilaginous matrix. Mesenchymal chondrosarcomas have a predilection for flat bones; long tubular bones are rarely affected. They tend to occur in the younger age group and are highly metastatic. (DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1456)
A benign neoplasm derived from mesodermal cells that form cartilage. It may remain within the substance of a cartilage or bone (true chondroma or enchondroma) or may develop on the surface of a cartilage (ecchondroma or ecchondrosis). (Dorland, 27th ed; Stedman, 25th ed)
Tumors or cancer located in bone tissue or specific BONES.
Benign growths of cartilage in the metaphyses of several bones.
A malignant tumor arising from the embryonic remains of the notochord. It is also called chordocarcinoma, chordoepithelioma, and notochordoma. (Dorland, 27th ed)
A sarcoma, usually a liposarcoma or malignant fibrous histiocytoma, with an abundant component of myxoid tissue resembling primitive mesenchyme containing connective tissue mucin. (Stedman, 25th ed)
A non-vascular form of connective tissue composed of CHONDROCYTES embedded in a matrix that includes CHONDROITIN SULFATE and various types of FIBRILLAR COLLAGEN. There are three major types: HYALINE CARTILAGE; FIBROCARTILAGE; and ELASTIC CARTILAGE.
Femoral neoplasms refer to abnormal growths or tumors, benign or malignant, located in the femur bone or its surrounding soft tissues within the thigh region.
Neoplasms of the base of the skull specifically, differentiated from neoplasms of unspecified sites or bones of the skull (SKULL NEOPLASMS).
Hereditary disorder transmitted by an autosomal dominant gene and characterized by multiple exostoses (multiple osteochondromas) near the ends of long bones. The genetic abnormality results in a defect in the osteoclastic activity at the metaphyseal ends of the bone during the remodeling process in childhood or early adolescence. The metaphyses develop benign, bony outgrowths often capped by cartilage. A small number undergo neoplastic transformation.
Neoplasms developing from some structure of the connective and subcutaneous tissue. The concept does not refer to neoplasms located in connective or soft tissue.
A sarcoma originating in bone-forming cells, affecting the ends of long bones. It is the most common and most malignant of sarcomas of the bones, and occurs chiefly among 10- to 25-year-old youths. (From Stedman, 25th ed)
A set of twelve curved bones which connect to the vertebral column posteriorly, and terminate anteriorly as costal cartilage. Together, they form a protective cage around the internal thoracic organs.
Bones that constitute each half of the pelvic girdle in VERTEBRATES, formed by fusion of the ILIUM; ISCHIUM; and PUBIC BONE.
Enzymes which catalyze the elimination of glucuronate residues from chondroitin A,B, and C or which catalyze the hydrolysis of sulfate groups of the 2-acetamido-2-deoxy-D-galactose 6-sulfate units of chondroitin sulfate. EC 4.2.2.-.
Rare, benign, chronic, progressive metaplasia in which cartilage is formed in the synovial membranes of joints, tendon sheaths, or bursae. Some of the metaplastic foci can become detached producing loose bodies. When the loose bodies undergo secondary calcification, the condition is called synovial osteochondromatosis.
Glycoproteins which have a very high polysaccharide content.
A cartilage-capped benign tumor that often appears as a stalk on the surface of bone. It is probably a developmental malformation rather than a true neoplasm and is usually found in the metaphysis of the distal femur, proximal tibia, or proximal humerus. Osteochondroma is the most common of benign bone tumors.
Cancer or tumors of the MAXILLA or upper jaw.
A large group of diseases which are characterized by a low prevalence in the population. They frequently are associated with problems in diagnosis and treatment.
Binary classification measures to assess test results. Sensitivity or recall rate is the proportion of true positives. Specificity is the probability of correctly determining the absence of a condition. (From Last, Dictionary of Epidemiology, 2d ed)
Process that is gone through in order for a drug to receive approval by a government regulatory agency. This includes any required pre-clinical or clinical testing, review, submission, and evaluation of the applications and test results, and post-marketing surveillance of the drug.

Analysis of acute vascular damage after photodynamic therapy using benzoporphyrin derivative (BPD). (1/542)

Benzoporphyrin derivative monoacid ring A (BPD-MA, verteporfin) is currently under investigation as a photosensitizer for photodynamic therapy (PDT). Since BPD exhibits rapid pharmacokinetics in plasma and tissues, we assessed damage to tumour and muscle microvasculature when light treatment for PDT was given at short times after injection of photosensitizer. Groups of rats with chondrosarcoma were given 2 mg kg(-1) of BPD intravenously 5 min to 180 min before light treatment of 150 J cm(-2) 690 nm. Vascular response was monitored using intravital microscopy and tumour cure was monitored by following regrowth over 42 days. For treatment at 5 or 30 min after BPD injection, blood flow stasis was limited to tumour microvasculature with lesser response in the surrounding normal microvasculature, indicating selective targeting for damage. No acute changes were observed in vessels when light was given 180 min after BPD injection. Tumour regression after light treatment occurred in all animals given PDT with BPD. Long-term tumour regression was greater in animals treated 5 min after BPD injection and least in animals given treatment 180 min after drug injection. The correlation between the timing for vascular damage and cure implies that blood flow stasis plays a significant role in PDT-induced tumour destruction.  (+info)

The EWS/TEC fusion protein encoded by the t(9;22) chromosomal translocation in human chondrosarcomas is a highly potent transcriptional activator. (2/542)

The EWS/TEC gene fusion generated by the t(9;22) chromosomal translocation found in extraskeletal myxoid chondrosarcomas encodes a fusion protein containing the amino-terminal domain of the EWS protein fused to the whole coding sequence of the orphan nuclear receptor TEC. We have compared the DNA-binding and transcriptional activation properties of various TEC isoforms and the corresponding EWS/TEC fusion proteins. Band-shift experiments show that the full-length TEC receptor can efficiently bind the NGFI-B Response Element (NBRE), whereas an isoform lacking the entire carboxyl-terminal domain of the receptor binds much less efficiently the NBRE. Addition of the amino-terminal domain of EWS to either isoforms does not alter significantly their DNA-binding properties to the NBRE. Co-transfection experiments of COS cells and human chondrocytes indicate that whereas TEC moderately activates transcription from a NBRE-containing promoter, the corresponding EWS/TEC fusion protein is a highly potent transcriptional activator of the same promoter, being approximately 270-fold more active than the native receptor. EWS/TEC may thus exert its oncogenic potential in chrondrosarcomas by activating the transcription of target genes involved in cell proliferation.  (+info)

Sulphation heterogeneity in the trisaccharide (GalNAcSbeta1, 4GlcAbeta1,3GalNAcS) isolated from the non-reducing terminal of human aggrecan chondroitin sulphate. (3/542)

We report here the isolation and sulphation isomer analyses of trisaccharides GalNAcS(beta1,4)GlcA(beta1,3)GalNAcS (in which S indicates sulphate) derived from the non-reducing termini of aggrecan chondroitin sulphate. Rat chondrosarcoma and human aggrecans were digested for 1 h at 37 degrees C with 30 micro-units of endo-chondroitinase ABC per microgram of chondroitin sulphate, and trisaccharides were isolated from the digests by ToyoPearl HW40S gel-filtration chromatography. Four trisaccharide species were identified; their sulphation isomer compositions, as determined by digestion with chondroitinase ACII and fluorescence-based ion-exchange HPLC, were GalNAc4Sbeta1,4GlcAbeta1,3GalNAc4S, GalNAc4Sbeta1,4GlcAbeta1,3GalNAc6S, GalNAc4,6Sbeta1,4GlcAbeta1, 3GalNAc4S and GalNAc4,6Sbeta1,4GlcAbeta1,3GalNAc6S. The abundances of such sequences in chondroitin sulphate on aggrecan from normal (foetal to 72 years of age) and from osteoarthritic human knee cartilages were also established. The results showed that non-reducing terminal GalNAc4S or GalNAc4,6S can be linked to either a 4-sulphated or a 6-sulphated disaccharide, suggesting that the sulphation of the last disaccharide might not have a direct effect on the specificity of chondroitin sulphate terminal GalNAc sulphotransferases. Furthermore, for each aggrecan preparation examined, the 4S-to-6S ratio of all chain interior disaccharides was equivalent to that in the last repeating disaccharides at the non-reducing terminus, suggesting that neither chondroitin 4-sulphotransferase nor chondroitin 6-sulphotransferase shows preferential activity near the chain terminus.  (+info)

EXT-mutation analysis and loss of heterozygosity in sporadic and hereditary osteochondromas and secondary chondrosarcomas. (4/542)

Osteochondromas occur as sporadic solitary lesions or as multiple lesions, characterizing the hereditary multiple exostoses syndrome (EXT). Approximately 15% of all chondrosarcomas arise within the cartilaginous cap of an osteochondroma. EXT is genetically heterogeneous, and two genes, EXT1 and EXT2, located on 8q24 and 11p11-p12, respectively, have been cloned. It is still unclear whether osteochondroma is a developmental disorder or a true neoplasm. Furthermore, it is unclear whether inactivation of both alleles of an EXT gene, according to the tumor-suppressor model, is required for osteochondroma development, or whether a single EXT germline mutation acts in a dominant negative way. We therefore studied loss of heterozygosity and DNA ploidy in eight sporadic and six hereditary osteochondromas. EXT1- and EXT2-mutation analysis was performed in a total of 34 sporadic and hereditary osteochondromas and secondary peripheral chondrosarcomas. We demonstrated osteochondroma to be a true neoplasm, since aneuploidy was found in 4 of 10 osteochondromas. Furthermore, LOH was almost exclusively found at the EXT1 locus in 5 of 14 osteochondromas. Four novel constitutional cDNA alterations were detected in exon 1 of EXT1. Two patients with multiple osteochondromas demonstrated a germline mutation combined with loss of the remaining wild-type allele in three osteochondromas, indicating that, in cartilaginous cells of the growth plate, inactivation of both copies of the EXT1 gene is required for osteochondroma formation in hereditary cases. In contrast, no somatic EXT1 cDNA alterations were found in sporadic osteochondromas. No mutations were found in the EXT2 gene.  (+info)

Chemical modification and site-directed mutagenesis of conserved HXXH and PP-loop motif arginines and histidines in the murine bifunctional ATP sulfurylase/adenosine 5'-phosphosulfate kinase. (5/542)

The sulfurylase domain of the mouse bifunctional enzyme ATP sulfurylase/adenosine 5'-phosphosulfate (APS) kinase contains HXXH and PP-loop motifs. To elucidate the functional importance of these motifs and of conserved arginines and histidines, chemical modification and site-directed mutagenesis studies were performed. Chemical modification of arginines and histidines with phenylglyoxal and diethyl pyrocarbonate, respectively, renders the enzyme inactive in sulfurylase, kinase, and overall assays. Data base searches and sequence comparison of bifunctional ATP sulfurylase/APS kinase and monofunctional ATP sulfurylases shows a limited number of highly conserved arginines and histidines within the sulfurylase domain. Of these conserved residues, His-425, His-428, and Arg-421 are present within or near the HXXH motif whereas His-506, Arg-510, and Arg-522 residues are present in and around the PP-loop. The functional role of these conserved residues was further studied by site-directed mutagenesis. In the HXXH motif, none of the alanine mutants (H425A, H428A, and R421A) had sulfurylase or overall activity, whereas they all exhibited normal kinase activity. A slight improvement in reverse sulfurylase activity (<10% residual activity) and complete restoration of forward sulfurylase was observed with R421K. Mutants designed to probe the PP-loop requirements included H506A, R510A, R522A, R522K, and D523A. Of these, R510A exhibited normal sulfurylase and kinase activity, R522A and R522K showed no sulfurylase activity, and H506A had normal sulfurylase activity but produced an effect on kinase activity (<10% residual activity). The single aspartate, D523A, which is part of the highly conserved GRD sequence of the PP-loop, affected both sulfurylase and kinase activity. This mutational analysis indicates that the HXXH motif plays a role only in the sulfurylase activity, whereas the PP-loop is involved in both sulfurylase and kinase activities. Residues specific for sulfurylase activity have also been distinguished from those involved in kinase activity.  (+info)

Up-regulation of MDC15 (metargidin) messenger RNA in human osteoarthritic cartilage. (6/542)

OBJECTIVE: The aim of the study was to investigate the messenger RNA (mRNA) expression of the disintegrin metalloproteinase MDC15 (metargidin, or ADAM-15) in normal and osteoarthritic (OA) articular cartilage. METHODS: In situ hybridization experiments and reverse transcription-polymerase chain reaction (RT-PCR) were performed on tissue samples of adult normal and OA articular cartilage. RESULTS: MDC15 mRNA could be detected in normal articular cartilage by RT-PCR using tissue-extracted total RNA as a template. However, the mRNA level remained below the sensitivity of in situ hybridization. In contrast, in situ hybridizations of OA cartilage revealed an intense staining with the MDC15-specific riboprobes. The extension of the analysis to chondrosarcomas showed a strong up-regulation of MDC15 mRNA in these malignant transformed cells. CONCLUSION: Our results demonstrate a markedly strong up-regulation of MDC15 in adult OA and neoplastic cartilage compared with adult normal articular cartilage, indicating a potential role of the disintegrin metalloproteinase in cartilage remodeling.  (+info)

Reconstruction and limb salvage after resection for malignant bone tumour of the proximal humerus. A sling procedure using a free vascularised fibular graft. (7/542)

We assessed the intermediate functional results of eight patients after wide resection of the proximal humerus for malignant bone tumour. We used a free vascularised fibular graft as a functional spacer and a sling procedure to preserve passive scapulohumeral movement. Scapulohumeral arthrodesis was not carried out. Five patients had osteosarcoma, two achondrosarcoma and one a malignant fibrous histiocytoma of the bone. The mean duration of follow-up was 70 months (median, 76) for the seven patients who were still alive at the time of the latest follow-up. One patient died from the disease 12 months after surgery. There were no local recurrences. The functional results were described and graded quantitatively according to the rating system of the Musculoskeletal Tumour Society. Our results were satisfactory with regard to pain, emotional acceptance and manual dexterity. Function and lifting ability were unsatisfactory in two patients. One patient had delayed union between host and graft, but this united after six months without further surgery. Radiographs of the shoulder showed absorption or collapse of the head of the fibula in four of the eight patients and a fracture in another. No functional problems related to absorption or fracture of the head of the fibula were noted. There was no infection or subluxation of the head. We conclude that this is a reasonably effective technique of limb salvage after resection of the proximal humerus.  (+info)

Fusion of the EWS-related gene TAF2N to TEC in extraskeletal myxoid chondrosarcoma. (8/542)

Extraskeletal myxoid chondrosarcomas (EMCs) are characterized by a recurrent t(9;22)(q22;q12) translocation, resulting in the fusion of the EWS gene in 22q12 and the TEC gene in 9q22. Here we report that a third member of the EWS, TLS/FUS gene family, TAF2N, can replace EWS as a fusion partner to TEC in EMC. Two tumors, one with a novel t(9;17)(q22;q11) variant translocation and one with an apparently normal karyotype, expressed TAF2N-TEC fusion transcripts. In both cases, the chimeric transcripts were shown to contain exon 6 of TAF2N fused to the entire coding region of TEC. This transcript is structurally and functionally very similar to the EWS-TEC fusions. The exchange of the EWS NH2-terminal part with the TAF2N NH2-terminal part in EMC further underscores the oncogenic potential of these protein domains as partners in fusion genes.  (+info)

Chondrosarcoma is a type of cancer that develops in the cartilaginous tissue, which is the flexible and smooth connective tissue found in various parts of the body such as the bones, ribs, and nose. It is characterized by the production of malignant cartilage cells that can invade surrounding tissues and spread to other parts of the body (metastasis).

Chondrosarcomas are typically slow-growing tumors but can be aggressive in some cases. They usually occur in adults over the age of 40, and men are more commonly affected than women. The most common sites for chondrosarcoma development include the bones of the pelvis, legs, and arms.

Treatment for chondrosarcoma typically involves surgical removal of the tumor, along with radiation therapy or chemotherapy in some cases. The prognosis for chondrosarcoma depends on several factors, including the size and location of the tumor, the grade of malignancy, and whether it has spread to other parts of the body.

Chondrosarcoma, mesenchymal is a type of chondrosarcoma, which is a malignant (cancerous) tumor that arises from cartilaginous tissue. It is a rare and aggressive subtype of chondrosarcoma, accounting for less than 10% of all cases.

Mesenchymal chondrosarcomas are characterized by their undifferentiated small round blue cells intermixed with well-differentiated cartilaginous areas. They can occur in any age group but are more common in children and young adults. These tumors can arise in any bone, but they most commonly involve the long bones of the extremities, pelvis, and spine.

Mesenchymal chondrosarcomas tend to be aggressive with a high risk of local recurrence and metastasis (spread) to other parts of the body, such as the lungs, lymph nodes, or other bones. Treatment typically involves surgical resection of the tumor, often followed by radiation therapy and/or chemotherapy. The prognosis for mesenchymal chondrosarcoma is generally poorer than for other subtypes of chondrosarcoma due to its aggressive behavior and higher likelihood of metastasis.

A chondroma is a benign, slow-growing tumor that develops in the cartilage. Cartilage is a type of connective tissue found in various parts of the body, including the joints, ribcage, and nose. Chondromas are most commonly found in the hands and feet.

Chondromas are typically small, measuring less than 2 centimeters in diameter, and they usually do not cause any symptoms. However, if a chondroma grows large enough to press on nearby nerves or blood vessels, it may cause pain, numbness, or weakness in the affected area.

Chondromas are usually diagnosed through imaging tests such as X-rays, CT scans, or MRI scans. If a chondroma is suspected based on these tests, a biopsy may be performed to confirm the diagnosis and rule out other types of tumors.

Treatment for chondromas typically involves surgical removal of the tumor. In most cases, this can be done using minimally invasive techniques that allow for quicker recovery times. After surgery, patients will need to follow up with their healthcare provider to ensure that the tumor has been completely removed and to monitor for any signs of recurrence.

Bone neoplasms are abnormal growths or tumors that develop in the bone. They can be benign (non-cancerous) or malignant (cancerous). Benign bone neoplasms do not spread to other parts of the body and are rarely a threat to life, although they may cause problems if they grow large enough to press on surrounding tissues or cause fractures. Malignant bone neoplasms, on the other hand, can invade and destroy nearby tissue and may spread (metastasize) to other parts of the body.

There are many different types of bone neoplasms, including:

1. Osteochondroma - a benign tumor that develops from cartilage and bone
2. Enchondroma - a benign tumor that forms in the cartilage that lines the inside of the bones
3. Chondrosarcoma - a malignant tumor that develops from cartilage
4. Osteosarcoma - a malignant tumor that develops from bone cells
5. Ewing sarcoma - a malignant tumor that develops in the bones or soft tissues around the bones
6. Giant cell tumor of bone - a benign or occasionally malignant tumor that develops from bone tissue
7. Fibrosarcoma - a malignant tumor that develops from fibrous tissue in the bone

The symptoms of bone neoplasms vary depending on the type, size, and location of the tumor. They may include pain, swelling, stiffness, fractures, or limited mobility. Treatment options depend on the type and stage of the tumor but may include surgery, radiation therapy, chemotherapy, or a combination of these treatments.

Enchondromatosis is a rare skeletal disorder characterized by the development of multiple enchondromas, which are benign tumors made up of cartilage that form within the bone. These growths can occur in any bone but are most commonly found in the hands and feet. Enchondromatosis can be inherited or may develop sporadically.

There are two main types of enchondromatosis: Ollier disease and Maffucci syndrome. Ollier disease is characterized by multiple enchondromas, typically affecting one side of the body more than the other. Maffucci syndrome is a more severe form of enchondromatosis that includes both enchondromas and benign soft tissue tumors called hemangiomas.

The symptoms of enchondromatosis can vary widely, depending on the size and location of the enchondromas. Some individuals may experience bone pain, fractures, or deformities, while others may have no noticeable symptoms. In rare cases, enchondromas can become malignant, leading to the development of chondrosarcoma, a type of cancer that arises from cartilage.

Treatment for enchondromatosis typically involves monitoring for changes in the size or shape of the tumors and addressing any symptoms as they arise. Surgery may be necessary in some cases to remove large or symptomatic enchondromas, particularly if there is a risk of malignant transformation. Regular follow-up with an orthopedic specialist is recommended to monitor for potential complications.

A chordoma is a rare, slow-growing tumor that typically develops in the bones of the spine or skull. These tumors originate from remnants of the notochord, a structure that forms during embryonic development and eventually becomes part of the spinal cord. Chordomas are usually low-grade malignancies but can be aggressive and locally invasive, potentially causing pain, neurological symptoms, or structural damage to the spine or skull. Treatment typically involves surgical resection, often combined with radiation therapy.

Myxosarcoma is a very rare type of soft tissue sarcoma, a cancer that develops in the soft tissues of the body, such as fat, muscle, nerves, blood vessels, and fibrous tissues. Myxosarcomas are characterized by the presence of mucoid or gelatinous material in the tumor, which is composed of an abnormal accumulation of acid mucopolysaccharides. These tumors typically affect adults, with a peak incidence in the sixth to seventh decade of life. They usually occur in the extremities, particularly the lower limbs, and can also arise in the retroperitoneum or other deep soft tissues. Myxosarcomas are classified into several subtypes based on their histological features, with the most common being the myxofibrosarcoma. Treatment typically involves surgical resection with wide margins, often followed by radiation therapy and/or chemotherapy. The prognosis for patients with myxosarcoma depends on several factors, including the size and location of the tumor, the histological grade, and the patient's age and overall health.

Cartilage is a type of connective tissue that is found throughout the body in various forms. It is made up of specialized cells called chondrocytes, which are embedded in a firm, flexible matrix composed of collagen fibers and proteoglycans. This unique structure gives cartilage its characteristic properties of being both strong and flexible.

There are three main types of cartilage in the human body: hyaline cartilage, elastic cartilage, and fibrocartilage.

1. Hyaline cartilage is the most common type and is found in areas such as the articular surfaces of bones (where they meet to form joints), the nose, trachea, and larynx. It has a smooth, glassy appearance and provides a smooth, lubricated surface for joint movement.
2. Elastic cartilage contains more elastin fibers than hyaline cartilage, which gives it greater flexibility and resilience. It is found in structures such as the external ear and parts of the larynx and epiglottis.
3. Fibrocartilage has a higher proportion of collagen fibers and fewer chondrocytes than hyaline or elastic cartilage. It is found in areas that require high tensile strength, such as the intervertebral discs, menisci (found in joints like the knee), and the pubic symphysis.

Cartilage plays a crucial role in supporting and protecting various structures within the body, allowing for smooth movement and providing a cushion between bones to absorb shock and prevent wear and tear. However, cartilage has limited capacity for self-repair and regeneration, making damage or degeneration of cartilage tissue a significant concern in conditions such as osteoarthritis.

Femoral neoplasms refer to abnormal growths or tumors that develop in the femur, which is the long thigh bone in the human body. These neoplasms can be benign (non-cancerous) or malignant (cancerous). Benign femoral neoplasms are slow-growing and rarely spread to other parts of the body, while malignant neoplasms are aggressive and can invade nearby tissues and organs, as well as metastasize (spread) to distant sites.

There are various types of femoral neoplasms, including osteochondromas, enchondromas, chondrosarcomas, osteosarcomas, and Ewing sarcomas, among others. The specific type of neoplasm is determined by the cell type from which it arises and its behavior.

Symptoms of femoral neoplasms may include pain, swelling, stiffness, or weakness in the thigh, as well as a palpable mass or limited mobility. Diagnosis typically involves imaging studies such as X-rays, CT scans, or MRI, as well as biopsy to determine the type and grade of the tumor. Treatment options may include surgery, radiation therapy, chemotherapy, or a combination of these approaches, depending on the type, size, location, and stage of the neoplasm.

Skull base neoplasms refer to abnormal growths or tumors located in the skull base, which is the region where the skull meets the spine and where the brain connects with the blood vessels and nerves that supply the head and neck. These neoplasms can be benign (non-cancerous) or malignant (cancerous), and they can arise from various types of cells in this area, including bone, nerve, glandular, and vascular tissue.

Skull base neoplasms can cause a range of symptoms depending on their size, location, and growth rate. Some common symptoms include headaches, vision changes, hearing loss, facial numbness or weakness, difficulty swallowing, and balance problems. Treatment options for skull base neoplasms may include surgery, radiation therapy, chemotherapy, or a combination of these approaches. The specific treatment plan will depend on the type, size, location, and stage of the tumor, as well as the patient's overall health and medical history.

Multiple hereditary exostoses (MHE) is a genetic condition characterized by the growth of multiple benign tumors known as osteochondromas. These tumors typically develop at the ends of long bones near the growth plates and can cause various skeletal deformities, limitations in mobility, and other health issues.

MHE is usually inherited in an autosomal dominant pattern, meaning that a child has a 50% chance of inheriting the condition if one parent has it. However, some cases may result from spontaneous mutations. The condition typically becomes apparent during childhood or adolescence and can affect both sexes equally.

The primary diagnostic feature of MHE is the presence of multiple osteochondromas, which are made up of bone and cartilage. These growths can cause a range of symptoms, including pain, swelling, decreased mobility, and an increased risk of fractures. In some cases, they may also lead to complications such as nerve compression or vascular damage.

Treatment for MHE typically involves surgical removal of the osteochondromas, particularly if they are causing significant symptoms or complications. Regular monitoring is also important to detect any new growths and assess their potential impact on health. In addition, physical therapy and other supportive measures may be recommended to help manage symptoms and maintain mobility.

Neoplasms of connective and soft tissue are abnormal growths or tumors that develop in the body's supportive tissues, such as cartilage, tendons, ligaments, fascia, and fat. These neoplasms can be benign (non-cancerous) or malignant (cancerous).

Benign connective and soft tissue neoplasms include:
- Lipomas: slow-growing, fatty tumors that develop under the skin.
- Fibromas: firm, benign tumors that develop in connective tissue such as tendons or ligaments.
- Nevi (plural of nevus): benign growths made up of cells called melanocytes, which produce pigment.

Malignant connective and soft tissue neoplasms include:
- Sarcomas: a type of cancer that develops in the body's supportive tissues such as muscle, bone, fat, cartilage, or blood vessels. There are many different types of sarcomas, including liposarcoma (fatty tissue), rhabdomyosarcoma (muscle), and osteosarcoma (bone).
- Desmoid tumors: a rare type of benign tumor that can become aggressive and invade surrounding tissues. While not considered cancerous, desmoid tumors can cause significant morbidity due to their tendency to grow and infiltrate nearby structures.

Connective and soft tissue neoplasms can present with various symptoms depending on their location and size. Treatment options include surgery, radiation therapy, chemotherapy, or a combination of these modalities. Regular follow-up care is essential to monitor for recurrence or metastasis (spread) of the tumor.

Osteosarcoma is defined as a type of cancerous tumor that arises from the cells that form bones (osteoblasts). It's the most common primary bone cancer, and it typically develops in the long bones of the body, such as the arms or legs, near the growth plates. Osteosarcoma can metastasize (spread) to other parts of the body, including the lungs, making it a highly malignant form of cancer. Symptoms may include bone pain, swelling, and fractures. Treatment usually involves a combination of surgery, chemotherapy, and/or radiation therapy.

In medical terms, ribs are the long, curved bones that make up the ribcage in the human body. They articulate with the thoracic vertebrae posteriorly and connect to the sternum anteriorly via costal cartilages. There are 12 pairs of ribs in total, and they play a crucial role in protecting the lungs and heart, allowing room for expansion and contraction during breathing. Ribs also provide attachment points for various muscles involved in respiration and posture.

The pelvic bones, also known as the hip bones, are a set of three irregularly shaped bones that connect to form the pelvic girdle in the lower part of the human body. They play a crucial role in supporting the spine and protecting the abdominal and pelvic organs.

The pelvic bones consist of three bones:

1. The ilium: This is the largest and uppermost bone, forming the majority of the hip bone and the broad, flaring part of the pelvis known as the wing of the ilium or the iliac crest, which can be felt on the side of the body.
2. The ischium: This is the lower and back portion of the pelvic bone that forms part of the sitting surface or the "sit bones."
3. The pubis: This is the front part of the pelvic bone, which connects to the other side at the pubic symphysis in the midline of the body.

The pelvic bones are joined together at the acetabulum, a cup-shaped socket that forms the hip joint and articulates with the head of the femur (thigh bone). The pelvic bones also have several openings for the passage of blood vessels, nerves, and reproductive and excretory organs.

The shape and size of the pelvic bones differ between males and females due to their different roles in childbirth and locomotion. Females typically have a wider and shallower pelvis than males to accommodate childbirth, while males usually have a narrower and deeper pelvis that is better suited for weight-bearing and movement.

Chondroitinases and chondroitin lyases are enzymes that break down chondroitin sulfate, a type of glycosaminoglycan (GAG) found in connective tissues such as cartilage. Glycosaminoglycans are long, unbranched polysaccharides made up of repeating disaccharide units. In the case of chondroitin sulfate, the disaccharide unit consists of a glucuronic acid residue and a N-acetylgalactosamine residue that may be sulfated at various positions.

Chondroitinases are enzymes that cleave the linkage between the two sugars in the chondroitin sulfate chain, specifically between the carbon atom in the fourth position of the glucuronic acid and the nitrogen atom in the first position of the N-acetylgalactosamine. This results in the formation of unsaturated disaccharides. Chondroitinases are produced by certain bacteria and are used in research to study the structure and function of chondroitin sulfate and other GAGs.

Chondroitin lyases, on the other hand, are enzymes that cleave the same linkage but in the opposite direction, resulting in the formation of 4,5-unsaturated disaccharides. Chondroitin lyases are also produced by certain bacteria and are used in research to study the structure and function of chondroitin sulfate and other GAGs.

It is important to note that while both chondroitinases and chondroitin lyases break down chondroitin sulfate, they do so through different mechanisms and produce different products.

Synovial chondromatosis is a rare condition that affects the synovial membrane, which is the lining of joints, bursae (fluid-filled sacs that cushion bones), and tendon sheaths. In this condition, nodules made up of cartilage form in the synovial membrane. These nodules can detach from the synovial membrane and float freely in the synovial fluid, which lubricates the joint. If they become numerous, they can cause joint pain, stiffness, and decreased range of motion. In some cases, the loose bodies may also cause locking or catching sensations in the joint. Surgery is typically required to remove the cartilaginous nodules and relieve symptoms. If left untreated, synovial chondromatosis can lead to osteoarthritis and other joint problems.

Proteoglycans are complex, highly negatively charged macromolecules that are composed of a core protein covalently linked to one or more glycosaminoglycan (GAG) chains. They are a major component of the extracellular matrix (ECM) and play crucial roles in various biological processes, including cell signaling, regulation of growth factor activity, and maintenance of tissue structure and function.

The GAG chains, which can vary in length and composition, are long, unbranched polysaccharides that are composed of repeating disaccharide units containing a hexuronic acid (either glucuronic or iduronic acid) and a hexosamine (either N-acetylglucosamine or N-acetylgalactosamine). These GAG chains can be sulfated to varying degrees, which contributes to the negative charge of proteoglycans.

Proteoglycans are classified into four major groups based on their core protein structure and GAG composition: heparan sulfate/heparin proteoglycans, chondroitin/dermatan sulfate proteoglycans, keratan sulfate proteoglycans, and hyaluronan-binding proteoglycans. Each group has distinct functions and is found in specific tissues and cell types.

In summary, proteoglycans are complex macromolecules composed of a core protein and one or more GAG chains that play important roles in the ECM and various biological processes, including cell signaling, growth factor regulation, and tissue structure maintenance.

Osteochondroma is a benign (noncancerous) bone tumor that typically develops during childhood or adolescent growth years. It usually forms near the end of long bones, such as those in the arms and legs, but can also occur in other bones. An osteochondroma may have a cartilage cap covering its surface.

This type of tumor often grows slowly and typically stops growing once the person has stopped growing. In many cases, an osteochondroma doesn't cause any symptoms and doesn't require treatment. However, if it continues to grow or causes problems such as pain, restricted movement, or bone deformity, surgical removal may be necessary.

Most osteochondromas are solitary (occurring singly), but some people can develop multiple tumors, a condition known as multiple hereditary exostoses or diaphyseal aclasis. This genetic disorder is associated with a higher risk of developing sarcoma, a type of cancerous tumor that can arise from osteochondromas.

It's essential to have regular follow-ups with your healthcare provider if you have an osteochondroma to monitor its growth and any potential complications.

Maxillary neoplasms refer to abnormal growths or tumors in the maxilla, which is the upper jaw bone. These growths can be benign (non-cancerous) or malignant (cancerous). Benign neoplasms are slow-growing and do not spread to other parts of the body, while malignant neoplasms can invade surrounding tissues and spread to distant sites.

Maxillary neoplasms can cause various symptoms such as swelling, pain, numbness, loose teeth, or difficulty in chewing or swallowing. They may also cause nasal congestion, nosebleeds, or visual changes if they affect the eye or orbit. The diagnosis of maxillary neoplasms usually involves a combination of clinical examination, imaging studies such as CT or MRI scans, and biopsy to determine the type and extent of the tumor.

Treatment options for maxillary neoplasms depend on several factors, including the type, size, location, and stage of the tumor, as well as the patient's overall health and preferences. Treatment may include surgery, radiation therapy, chemotherapy, or a combination of these modalities. Regular follow-up care is essential to monitor for recurrence or metastasis and ensure optimal outcomes.

A rare disease, also known as an orphan disease, is a health condition that affects fewer than 200,000 people in the United States or fewer than 1 in 2,000 people in Europe. There are over 7,000 rare diseases identified, and many of them are severe, chronic, and often life-threatening. The causes of rare diseases can be genetic, infectious, environmental, or degenerative. Due to their rarity, research on rare diseases is often underfunded, and treatments may not be available or well-studied. Additionally, the diagnosis of rare diseases can be challenging due to a lack of awareness and understanding among healthcare professionals.

Sensitivity and specificity are statistical measures used to describe the performance of a diagnostic test or screening tool in identifying true positive and true negative results.

* Sensitivity refers to the proportion of people who have a particular condition (true positives) who are correctly identified by the test. It is also known as the "true positive rate" or "recall." A highly sensitive test will identify most or all of the people with the condition, but may also produce more false positives.
* Specificity refers to the proportion of people who do not have a particular condition (true negatives) who are correctly identified by the test. It is also known as the "true negative rate." A highly specific test will identify most or all of the people without the condition, but may also produce more false negatives.

In medical testing, both sensitivity and specificity are important considerations when evaluating a diagnostic test. High sensitivity is desirable for screening tests that aim to identify as many cases of a condition as possible, while high specificity is desirable for confirmatory tests that aim to rule out the condition in people who do not have it.

It's worth noting that sensitivity and specificity are often influenced by factors such as the prevalence of the condition in the population being tested, the threshold used to define a positive result, and the reliability and validity of the test itself. Therefore, it's important to consider these factors when interpreting the results of a diagnostic test.

"Drug approval" is the process by which a regulatory agency, such as the US Food and Drug Administration (FDA), grants formal authorization for a pharmaceutical company to market and sell a drug for a specific medical condition. The approval process is based on rigorous evaluation of clinical trial data to ensure that the drug is safe and effective for its intended use.

The FDA's approval process typically involves several stages, including preclinical testing in the lab and animal studies, followed by three phases of clinical trials in human subjects. The first phase tests the safety of the drug in a small group of healthy volunteers, while the second and third phases test the drug's efficacy and side effects in larger groups of patients with the medical condition for which the drug is intended.

If the results of these studies demonstrate that the drug is safe and effective, the pharmaceutical company can submit a New Drug Application (NDA) or Biologics License Application (BLA) to the FDA for review. The application includes data from the clinical trials, as well as information about the manufacturing process, labeling, and proposed use of the drug.

The FDA reviews the application and may seek input from independent experts before making a decision on whether to approve the drug. If approved, the drug can be marketed and sold to patients with the medical condition for which it was approved. The FDA continues to monitor the safety and efficacy of approved drugs after they reach the market to ensure that they remain safe and effective for their intended use.

CT scan and gross pathology of a chondrosarcoma 2012-12-18 "Chondrosarcoma". The Lecturio Medical Concept Library. Retrieved 8 ... A chondrosarcoma is a member of a category of tumors of bone and soft tissue known as sarcomas. About 30% of bone sarcomas are ... Nearly all chondrosarcoma patients appear to be in good health. Often, patients are not aware of the growing tumor until there ... Chondrosarcoma is a bone sarcoma, a primary cancer composed of cells derived from transformed cells that produce cartilage. ...
... is a type of chondrosarcoma. It has been associated with a t(9;22) (q22;q12) EWS/CHN gene fusion. Goh YW ... November 2001). "Extraskeletal myxoid chondrosarcoma: a light microscopic, immunohistochemical, ultrastructural and immuno- ... "Molecular analysis of the fusion of EWS to an orphan nuclear receptor gene in extraskeletal myxoid chondrosarcoma". Am. J. ...
... (EMC) is a rare low-grade malignant mesenchymal neoplasm of the soft tissues, that differs ... when they discussed the different species of extraskeletal chondrosarcoma, but EMC concept was firstly proposed in 1972 by ... Jacobi, Adam; Khanna, Neha; Gupta, Sushilkumar Satish (2017-03-01). "Curious case of extraskeletal myxoid chondrosarcoma". Lung ... cite book}}: ,journal= ignored (help) "Myxoid Chondrosarcoma - an overview , ScienceDirect Topics". www.sciencedirect.com. ...
"Chondrosarcoma". The Lecturio Medical Concept Library. Retrieved 8 July 2021. "Synovial Chondromatosis". Genetic And Rare ... Synovial chondrosarcoma Other: Osteochondritis dissecans Sequestrum from osteomyelitis Neuropathic (Charcot) joint Intra- ... Malignant transformation to synovial chondrosarcoma. This is a very rare complication occurring in chronic cases. Treatment ...
Chondrosarcoma is a more malignant type of tumor, but most are low grade tumors and often appear in the axial skeletal region. ... S2CID 44703653.[permanent dead link] Aufderheide, A.C.; Rodríguez-Martín, C.; Langsjoen, O. (2011). "Chondrosarcoma". The ...
GDF5 Chondrosarcoma; 215300; EXT1 Chondrosarcoma, extraskeletal myxoid; 612237; TAF15 Chondrosarcoma, extraskeletal myxoid; ... 612237; TFG Chondrosarcoma, extraskeletal myxoid; 612237; CSMF Chorea, hereditary benign; 118700; NKX2-1 Choreoacanthocytosis; ...
Most chondrosarcomas develop in the pelvis, legs or arms. Benign counterparts are known as enchondromas. Chondrosarcomas are ... Clear cell Clear cell chondrosarcomas are rare, slow-growing, and seldom spread. Mesenchymal Mesenchymal chondrosarcomas can ... A chondrosarcoma is the type of bone cancer that starts in the cartilage cells. It is the most common type found in adults. ... There are three main types of bone sarcoma based on tissue type - an osteosarcoma, a Ewing's sarcoma, and a chondrosarcoma. The ...
Depending on the type of chondrosarcoma, it ranges from a slow growth which is able to be removed, to a rapid growth and ... "Chondrosarcoma - Symptoms and causes". Mayo Clinic. Retrieved 2020-04-23. Reference, Genetics Home. "Chordoma". Genetics Home ... There are multiple other bone cancers that are more rare: Chondrosarcoma is identified mainly through the production of ...
... related to chondrosarcoma; CA11, related to stomach cancer; and surfactant protein C (SP-C), related to respiratory distress ...
Coltrera MD, Googe PB, Harrist TJ, Hyams VJ, Schiller AL, Goodman ML (1986). "Chondrosarcoma of the temporal bone. Diagnosis ...
Dawn Coe-Jones, 56, Canadian golfer, chondrosarcoma. Punya Datta, 92, Indian cricketer (Bengal). Louis Devereux, 85, English ...
"Chordoma and chondrosarcoma gene profile: implications for immunotherapy". Cancer Immunology, Immunotherapy. 58 (3): 339-49. ... Chondroid chordomas histologically show features of both chordoma and chondrosarcoma. In most cases, complete surgical ...
A person with HME has an increased risk of developing a rare form of bone cancer called chondrosarcoma as an adult. Problems ... Kivioja A, Ervasti H, Kinnunen J, Kaitila I, Wolf M, Böhling T (March 2000). "Chondrosarcoma in a family with multiple ...
" "Chondrosarcoma Masquerading as Cardiomyopathy" ; Charles R. Mulligan, Jr, Houman Tavaf-Motamen, Robert Stewart, and William ...
The benign tumors are called chondroma, the malignant ones chondrosarcoma. Tumors arising from other tissues may also produce a ...
However, the gene has been found to be highly expressed in chondrosarcoma. Chondrosarcoma is the cancer of the cells that ... Therefore, there seems to be an association between IFFO1's filamentous characteristic and chondrosarcoma. One nuclear export ...
Zhibek Nukeeva, 22, Kyrgyz beauty queen, Miss Kyrgyzstan (2013), chondrosarcoma. George A. Romero, 77, American-Canadian film ...
Kukreti died from a rare form of cancer, undifferentiated chondrosarcoma. "USA Cricket: Former USA wicketkeeper Rahul Kukreti ...
ISBN 978-0-323-52479-7. Wang XL, De Beuckeleer LH, De Schepper AM, Van Marck E (2001). "Low-grade chondrosarcoma vs enchondroma ... When differentiating an enchondroma from a chondrosarcoma, the radiographic image may be equivocal; however, periostitis is not ...
Drummer Randy Miller died from chondrosarcoma on November 5, 2010. The family set up a place for people to donate money to his ...
B16F10 and chondrosarcoma". Life Sciences. 40 (16): 1601-1607. doi:10.1016/0024-3205(87)90126-3. PMID 3561167. Fryklund, Linda ...
... is implicated in a number of different cancers such as osteosarcomas, chondrosarcomas, bladder cancer, and ... "The clinical significance of tenascin-C splice variant expression in chondrosarcoma". Oncology. 61 (4): 306-14. doi:10.1159/ ...
B16F10 and chondrosarcoma". Life Sciences. 40 (16): 1601-7. doi:10.1016/0024-3205(87)90126-3. PMID 3561167. Fryklund, Linda; ...
"Specific loss of chondromodulin-I gene expression in chondrosarcoma and the suppression of tumor angiogenesis and growth by its ... "Expression of the chondromodulin-I gene in chondrosarcomas". Cancer Letters. 204 (1): 61-8. doi:10.1016/j.canlet.2003.09.015. ...
Fibrosarcoma is the most common VAS; other types include rhabdomyosarcoma, myxosarcoma, chondrosarcoma, malignant fibrous ...
Osteosarcoma, chondrosarcoma arise in bone and cartilage, lymphoma in haematological disorders (1). The most common ...
Failure to stop growth can be indicative of transformation to malignant chondrosarcoma. Treatment is not indicated unless ...
These conditions include chondrosarcoma, osteosarcoma, giant cell tumor, and aneurysmal bone cyst. The temporomandibular joints ...
Abnormalities of chromosome 13 have been observed in canine osteoid chondrosarcoma and lymphosarcoma. Trisomy 13 in dogs with ...
While chondrosarcoma is the most common form of a secondary malignant bone neoplasm found in cases of Ollier disease, other ... The average age of patients of Ollier disease for their first surgery to treat their chondrosarcoma is thirty-three. Some ... Ding, Chang; Chen, Wei; Liu, Fujun; Xiong, Moli; Chen, Jing (July 2019). "Skull Base Chondrosarcoma Caused by Ollier Disease: A ... The incidence of a secondary chondrosarcoma in Ollier disease is most commonly approximated at 25-30% with some projections ...

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