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Imaging findings of extrapulmonary metastases of osteosarcoma.

PURPOSE: To review imaging findings of extrapulmonary metastasis from osteosarcoma and to evaluate them for any consistent pattern and correlation between imaging findings. MATERIALS AND METHODS: This study was retrospectively conducted in 13 patients with extrapulmonary metastasis of pathologically confirmed osteosarcoma. We evaluated the radioisotope (RI) scans (n=16), ultrasonography (USG) (n=4), computed tomography (CT) scans (n=10), MRIs (n=6), clinical records, and pathological reports for assessment of imaging findings and correlation between radiologic findings and RI uptake of the lesions. Points evaluated were the following: uptake on RI scans, presence of mineralization on CT, and MRI, size, enhancement pattern, attenuation on CT, signal intensity (SI) on MRI, and echogenicity on USG. RESULTS: Extrapulmonary metastatic sites were diverse, including another bone other than the primary site (n=6), lymph node (n=4), pleura (n=2), liver (n=2), pancreas (n=1), kidney (n=1), peritoneum (n=1), muscle (n=1), and subcutaneous fat layer (n=1). One patient had tumor growth within the pulmonary artery and jejunum. Among 21 metastatic sites in 13 patients, bone scan was performed in 16 cases and RI uptake was detected in 10 lesions. Calcification was detected in eight lesions on radiologic imaging, including plain radiography, USG, CT, and MRI. Two lesions showed RI uptake without definite calcification or ossification on MRI and plain radiography, respectively. We analyzed the enhancement pattern and mass size in 18 metastatic sites and these factors had poor correlation with uptake on RI scans. CONCLUSION: The sites and imaging findings of extrapulmonary metastasis of osteosarcoma were variable. All the lesions with mineralization were detectable on RI scans prior to radiologic imaging. RI scan has a limited role in the evaluation of metastatic lesions without mineralization.

Adipose Tissue↗

Radiographic and scintigraphic features of modeling and remodeling in the heterotopic skeleton of patients who have fibrodysplasia ossificans progressiva.

To characterize the radiographic and scintigraphic features of modeling and remodeling in the heterotopic skeleton of patients who have fibrodysplasia ossificans progressiva, radiographs from 47 patients and radionuclide bone scans from 12 of those patients, all of whom had a confirmed diagnosis of the disease, were reviewed. A wide range of normal bone modeling and remodeling features was seen in the heterotopic skeleton of all but the youngest two (age, 1 year) of the 47 patients. Characteristic features of normal bone modeling identified on radiographs of the heterotopic skeleton included: (a) the development of tubular and flat bones with mature cortical and trabecular organization; (b) the presence of well defined cortical-endosteal borders enclosing medullary canals; and (c) the presence of metaphyseal funnelization in isolated ossicles or at sites of synostoses. Characteristic features of normal bone remodeling identified on radiographs of the heterotopic skeleton included: (a) the response of heterotopic bone to weight bearing stress with osteosclerosis of use and osteopenia of disuse, and (b) the resistance of heterotopic bone to fatigue failure with the absence of pathologic fractures and stress fractures. Radionuclide bone scans in 12 patients showed that remodeling of mature heterotopic bone occurred at a rate consistent with that of mature normotopic bone. This study documents the radiographic and scintigraphic features of a heterotopic skeletal system in 47 patients who have fibrodysplasia ossificans progressiva. These data provide additional support for the hypothesis that the genetic defect leading to the formation of a heterotopic skeleton involves normal skeletal morphogenesis at heterotopic sites.

Adolescent↗

[Moderate hypobaric hypoxia used as an inducer of congenital vertebral malformation in mouse embryo (author's transl)].

In 1976, we decided to review the files of the scoliosis clinic at Ste-Justine Hospital in Montreal. We found 2,237 patients with scoliosis, 212 of them are classified as congenital. At this time, congenital scoliosis were the major unresolved problems. We decided to look at an experimental model, to study the physio-pathological mechanism of the induction of these malformations. We chose the mouse as the experimental animal, and "hypobaric hypoxia" as the teratogenic agent. With this model, we can produce, malformations at different levels of the spine, if we treat at different days of pregnancy. For example, if we treat the mothers in the 10th day of her pregnancy, we get 90% of malformed embryos, at the lumbar level. All the malformations reproduced in the mice, are similar to those found in humans. We have also demonstrated no difference in the morphology of these malformations from birth to maturity, in contradiction with Epstein, who said that the majority of cleft vertebrae will disappear with bone maturation. We have also demonstrated that these malformations are present in the cartilaginous stage of development of the vertebral column. Our findings are in contradiction with Nassim's who stated, that congenital vertebral malformations are produced by an abnormal ossification process on a normal cartilage primum. Finally, our findings are in agreements with those of Dr. Uhthoff, who showed that congenital vertebral malformations are present in cartilaginous stage of development in human embryos.

Animals↗

Morphogenesis and dysmorphogenesis of the appendicular skeleton.

Cartilage patterning and differentiation are prerequisites for skeletal development through endochondral ossification (EO). Multipotential mesenchymal cells undergo a complex process of cell fate determination to become chondroprogenitors and eventually differentiate into chondrocytes. These developmental processes require the orchestration of cell-cell and cell-matrix interactions. In this review, we present limb bud development as a model for cartilage patterning and differentiation. We summarize the molecular and cellular events and signaling pathways for axis patterning, cell condensation, cell fate determination, digit formation, interdigital apoptosis, EO, and joint formation. The interconnected nature of these pathways underscores the effects of genetic and teratogenic perturbations that result in skeletal birth defects. The topics reviewed also include limb dysmorphogenesis as a result of genetic disorders and environmental factors, including FGFR, GLI3, GDF5/CDMP1, Sox9, and Cbfa1 mutations, as well as thalidomide- and alcohol-induced malformations. Understanding the complex interactions involved in cartilage development and EO provides insight into mechanisms underlying the biology of normal cartilage, congenital disorders, and pathologic adult cartilage.

Animals↗

Cemento-ossifying fibroma of the orbit.

Cemento-ossifying fibroma presents with ophthalmic symptoms and signs rarely. We report the clinical and pathological findings in a case of cemento-ossifying fibroma of the right maxilla with extension into the orbital floor causing intermittent vertical diplopia, proptosis, and upward displacement of the globe. Compression of the nasolacrimal duct produced epiphora early in the course of the disease. Fibrous dysplasia has often been diagnosed in other cases of benign monostotic fibro-osseous conditions. Ossifying fibroma is easily confused with fibrous dysplasia. The histopathological difference between the two lesions is described.

Adult↗

Transarticular invasion of bone tumours across the sacroiliac joint.

PURPOSE: The purpose of this study was to evaluate the pattern of tumour spread across the SI articulation, correlating with cadaveric anatomic observations, in order to better understand the local spread of tumour and to assist in the assessment of local staging. MATERIAL AND METHODS: Twenty-four consecutive patients (14 male, 10 female; age range 22-89 years, mean 52 years) with primary bone tumours of the iliac bone or sacrum abutting the SI joint, in whom surgical resection of the SI joint was performed, were studied following institutional ethics approval. In all patients, preoperative magnetic resonance (MR) imaging studies of the pelvis and SI joint were reviewed for imaging evidence of transarticular extension across the SI joint. Gross pathologic and histologic assessment of possible transarticular SI joint tumour extension was performed in all patients. Nine cadaveric pelvic specimens without pelvic neoplastic disease (4 male, 5 female; age range 20-84 years, mean 59 years, median 58 years) were anatomically dissected and the articular anatomy of the SI joint examined macroscopically. RESULTS: Twelve of the twenty-four patients demonstrated imaging and histological evidence of transarticular SI joint invasion. Eight tumours infiltrated only the interosseous ligamentous aspect of the SI joint. In the remaining four cases, extensive tumour infiltrated both the cartilaginous and ligamentous aspects of the joint. No case showed tumour involvement isolated to the cartilaginous aspect of the joint. Among the cadaveric specimens studied, degenerative changes were found involving the majority of cases (6/9), with cartilage thinning and fibrillation and antero-superior marginal osteophytes seen involving the cartilaginous portion of the SI joint articulation. Four of the nine specimens demonstrated central ossification bridging the iliac and sacral aspects of the ligamentous (interosseous) SI joint. CONCLUSION: Tumour invasion across the SI articulation favours its interosseous ligamentous portion. Factors influencing transarticular tumour extension and its preferential course across the interosseous component of the articulation have been discussed. It is particularly important to assess the interosseous ligamentous portion of the SI articulation when locally staging primary pelvic bone tumours subjacent to the SI articulation, as this will have a significant impact on planning surgical management.

Adult↗

Bone metaplasia in the urinary tract: a new radiological sign.

We report 7 cases of bone tissue in the upper urinary tract. Routine excretory urography was suspicious for renal lithiasis. However, pathological evaluation of the kidney tissue revealed bone metaplasia in all 7 cases. Retrospective examination of the first 6 cases showed a typical radiological sign, consisting of a radiopaque eccentric halo surrounding an area of lesser radiological density that, in turn, connected with the urothelium. These findings permitted a correct preoperative diagnosis of bone metaplasia in case 7. Accurate preoperative differentiation of bone metaplasia (false calculi) and true renal lithiasis is important owing to the different treatments required.

Adult↗

Pathology of orbital bones. The XXXII Edward Jackson Memorial Lecture.

The orbital bones may show nearly all the pathologic changes observed in the skull and in the face. The congenital anomalies in this area are numerous and involve various forms of craniostenoses. Among the benign osseous tumors the osteoma is most frequently encountered in the orbit. Fibrous dysplasia is a tumefaction of indeterminate behavior that often involves the orbit. Osteosarcoma or other malignant neoplasms are rarely seen in this area. Eosinophilic granuloma and Hand-Schüller-Christian disease are tumor-like lesions that may involve the orbit.

Bone Cysts↗

Heterotopic bone formation in the gastrointestinal tract.

Heterotopic bone is found rarely in the gastrointestinal tract. Here we report four cases, one of which occurred in Barrett's esophagus in an area of metaplasia of specialized cell type without evidence of neoplasia. In the remaining cases, bone was associated with mucin-producing tumors of the appendix, transverse colon, and rectum. The clinical and pathologic findings are discussed in detail. Heterotopic bone formation in the digestive tract usually occurs in the colorectum in association with benign or malignant epithelial tumors, and most often with those that produce abundant mucin. The pathogenesis of the osseous metaplasia in the gastrointestinal tract is not understood, although mucin extravasation is an almost constant feature. Fragments of metaplastic bone in a biopsy of a mucinous neoplasm of the digestive tract should not be misinterpreted as osseous invasion.

Adenocarcinoma, Mucinous↗

[Loose bodies of the temporomandibular joint. A rare pathology].

Loose bodies of the temporomandibular joint (TMJ) are an uncommon condition which can be caused by various complaints that can now be diagnosed with high resolution CT. The authors report on 10 cases observed from 1983 to 1992 which were studied with both conventional radiography and CT. The most common conditions were synovial chondromatosis and osteochondrosis dissecans. In the two cases of synovial chondromatosis, the demonstration of ossified loose bodies made the diagnosis easier. The cases of osteochondrosis dissecans presented with more complex diagnostic problems: in two instances the characteristic subchondral bone fragment in the condylar head was clearly visible, but in the third case this small fragment was difficult to identify as it had migrated backwards, making the disorder troublesome to diagnose. An osteophyte fracture and the presence of a bone fragment following condylar head fracture were easily diagnosed by correctly assessing the associated articular changes resulting from an arthrosic and a traumatic condition, respectively. The only case of loose body due to previous TMJ remodeling was easily diagnosed as the totally ossified formation was not seen on the preoperative X-ray film. Finally, two very similar cases, characterized by the presence of an elongated radiopaque formation in the site of the posterior meniscal ligament, were difficult to interpret as no such case is reported in the literature. In both cases an anatomical variant was presumed, characterized by posterior meniscal ligament ossification.

Adult↗

Osteoarthritis, angiogenesis and inflammation.

Angiogenesis and inflammation are closely integrated processes in osteoarthritis (OA) and may affect disease progression and pain. Inflammation can stimulate angiogenesis, and angiogenesis can facilitate inflammation. Angiogenesis can also promote chondrocyte hypertrophy and endochondral ossification, contributing to radiographic changes in the joint. Inflammation sensitizes nerves, leading to increased pain. Innervation can also accompany vascularization of the articular cartilage, where compressive forces and hypoxia may stimulate these new nerves, causing pain even after inflammation has subsided. Inhibition of inflammation and angiogenesis may provide effective therapeutics for the treatment of OA by improving symptoms and retarding joint damage. This review aims to summarize (i) the evidence that angiogenesis and inflammation play an important role in the pathophysiology of OA and (ii) possible directions for future research into therapeutics that could effectively treat this disease.

Chronic Disease↗

[Bone structure in aseptic necrosis of the femoral head by light microscopy, contact microradiography, and fluorescent microscopy --comparing with osteoarthritis and rapidly destructive coxarthrosis].

In order to obtain further information of the pathological changes and bone structure of aseptic necrosis (AN), the present author carried out histological investigation on 20 femoral heads removed by prosthesis or total hip replacement from 18 patients. Both decalcified and undecalcified sections were investigated by microscopy, contact microradiography (CMR) and fluorescent microscopy. Schemata of individual cases were drawn to know the relationship between femoral head deformity and bone reactivity. Moreover, 19 femoral heads with secondary osteoarthritis (OA) removed from 18 patients and 7 with rapidly destructive coxarthrosis (RDC) from 6 patients were similarly investigated to compare with AN. The following results were obtained; Histologically, there are four different areas in AN. From the top of the femoral head, the lesion consisted of (1) subchondral necrosis, (2) granulation tissue, (3) an area of variable bone formation, and (4) normal bone tissue. As the advancement of the head deformity, fibrous component in the granulation increases and seems to divide the femoral head into necrotic and living areas, which by fibrous as well as enchondral ossifications become more prominent in the edge of the demarcation. As for the bone formation, four different types of bone tissues are discernible by CMR; (1) low calcified appositional bone and its absorption, (2) low calcified, appositional bone on wide trabeculae, (3) low calcified appositional bone on narrow trabeculae, and (4) irregularly calcified woven bone. Although during the comparatively early stage, the bone formation is seen in almost all of the living area, as the advancement of the head deformity, its activity decreases and results in overt head deformity to resemble that of OA which would be caused by mechanical factor. Increase of fibrous component in the granulation along with decrease of the bone formation would reduce the repairing activity of the femoral head. In the femoral heads with OA, a wedge-shaped area of bone formation is formed of which peak is located on the medial thickened cortex. In the femoral heads with RDC, a large amount of woven bone with irregular calcification is found in the bone formation area, in the upper part of a femoral head.

Adult↗

[Posttraumatic ectopic calcification as an additional pathologic factor of brachial plexus palsy].

The authors describe a case of a 54-year-old male, who was attacked with a broken bottle and wounded in left supraclavicular region. No neurologic deficit was observed immediately after injury. Some days later signs of upper trunk brachial plexus palsy were detected. Gradually symptoms of the Erb syndrome have developed. He was operated on 5 months after injury. During surgery no discontinuity of the brachial plexus was found. Unexpectedly there were an inner scar and free bony fragment compressing the upper trunk and the suprascapular nerve. Both the scar and bony fragment were carefully dissected and removed. Result of surgery: pain relief, restoration of normal sensation and partial restoration of biceps function.

Brachial Plexus↗

[Fundamental and clinical studies on CT gas myelography of the cervical spine].

We applied CT gas myelography at the cervical spinal region and investigated the clinical value of CT gas myelography as a supplementary diagnostic method for the spinal cord and vertebral diseases. Fundamental studies of the conditions of window width and window height and the permitted limit of the angle of incidence were made with phantoms of human neck to establish proper conditions of these factors. In clinical studies, 23 adult persons, who had no abnormality in the cervical spine and in the cervical spinal cord were observed to have normal CT gas myelograms of the cervical region, whereas 37, of clinical cases including 18 of cervical spondylosis, 10 of OPLL, 2 of cervical discopathy, 3 of fracture and dislocation of the cervical spine, 2 of cervical vertebral tumor and 2 of CYL, were found to show clear pathologic findings in the cross sections of the vertebral foramen, subarachnoidal space and spinal cord on the CT gas myelograms. The representative cases of these diseases were presented. The gas myelograms were morphologically classified and investigated in relation to the clinical findings. From the result of these investigations, CT gas myelography appears to be highly useful as a supplementary diagnostic method for diseases of the cervical spine and the cervical spinal cord. Furthermore, we compared the effect of this method with that of CT myelography using metrizamide, a recently developed water-soluble contrast medium.

Adult↗

[Lethal osteogenesis imperfecta. Anatomopathologic (optical and structural) study of 8 autopsy cases].

A pathologic review of lethal osteogenesis imperfecta was performed based in nine cases and mainly concerning eight cases with total postmortem study. One of the cases was classified as type III while all other were of type II. Emphasis is made on the high rate of prematurity, small for date and podalic presentation at birth. In those six cases of lower age lung hypoplasia was present. Well known findings in membranous or enchondral bones were confirmed in this series. Long bones showed characteristic bowings related to the nutrient artery point of entry. Among bone collagen fibres some of them were anomalous in shape or thickness but with normal striation. The only anomaly detected at the cartilaginous epiphysis was the increased wideness of the vascular channels because the existence of loose connective tissue and multivacuolated cells of unknown significance. At the growth plates the vascular channels were too much widened and of abnormal distribution. The proliferative and columnar zones showed poor cellular population and some condrocytes were abnormally vacuolated. The perichondrial ossification groove of Ranvier was absent or underdeveloped in all bones studied coexisting with epiphyseal-metaphyseal deformities. In our present cases, ocular, dental, dermal, cardiac valves and hepatic alterations have been detected. The weight increase and haematopoiesis of the liver was correlated to the hyperplasia and fibrosis of the bone marrow.

Bone and Bones↗

Abl expression in human fetal and adult tissues, tumours, and tumour microvessels.

Abl kinases encoded by the abl oncogenes inhibit apoptosis without affecting cell proliferation. The aim of this study was to examine a wide range of normal fetal and adult human tissues and a variety of tumour types for Abl immunoreactivity. Sections from 193 paraffin blocks of normal fetal and adult tissues and 72 blocks from representative tumours were stained immunohistochemically using a polyclonal antibody to c-Abl/Bcr-Abl oncoprotein. Weak Abl immunoreactivity was observed in many adult tissues. Moderately intense or strong staining (cytoplasmic, nuclear or membranous) was consistently seen in hyaline cartilage, adipocytes, and ciliated epithelium. In fetal tissues, there was a broadly similar staining pattern, but Abl expression was also seen in muscle (all types) and occasionally in endothelial cells. The most intense staining was seen in sites of endochondral ossification and in the umbilical cord stroma. Negatively staining tissues included epidermis and squamous mucosa, lymph nodes, tonsil, spleen, hepatocytes, and adrenals. Most tumours showed focal or weak Abl immunoreactivity. The most intense staining was seen in chondrosarcoma, liposarcoma, and diffuse gastric (signet ring) adenocarcinoma. In the latter two tumour types, Abl expression was also observed in tumour microvessels. These results suggest that Abl not only functions as an apoptosis inhibitor, but also may have a role in connective tissue maturation and differentiation and in tumour growth and angiogenesis.

Adult↗

Classification of bone tumors.

The classification of bone tumors relies on the cytologic features and products of tumor cells. This classification is reproducible and accepted by pathologists, oncologic surgeons and oncologists. Chondrogenic tumors are the second largest group of bone tumors. Their histologic pattern suggests a relationship to hyaline cartilage. Exostoses, or osteochondromas, represent about 1/3 of chondrogenic lesions. Chondromas are hyaline cartilage tumors which can be found centrally or subperiosteally; they may contain some calcifications and/or ossifications. Chondroblastomas are tumors whose cells produce, at least focally, a matrix similar to hyaline cartilage. Histology of chondromyxoid fibromas shows large or small areas where proliferating cells produce a matrix resembling the hyaline cartilage. Chondosarcomas are tumors whose malignant cells produce a cartilaginous matrix. Most of them occur in previously normal bones; they are classified as conventional or primary chondrosarcomas. Secondary chondrosarcomas result from the malignant transformation of a benign cartilaginous lesion less commonly enchondromas and most commonly osteocartilaginous exostoses, or osteochondromas. Less common variants include dedifferentiated, mesenchymal and clear cell chondrosarcomas. Osteogenic tumors are the third largest group of bone tumors, with osteosarcomas being the most frequent type. The most important criterion for a tumor to be considered an osteosarcoma is that the malignant tumor cells must produce a recognizable osteoid matrix, at least focally. Osteosarcomas are divided into three groups: osteoblastic, chondroblastic and fibroblastic, according to the dominant histologic feature. Osteosarcomas can be multifocal, synchronous or metachronous; they are also classified by the histologic grade of malignancy. Pathologically low grade lesions, which are clinically indolent, are generally known as low grade central and parosteal osteosarcomas.

Bone Neoplasms↗

Heterotopic bone formation in two cases of colon carcinoma.

Heterotopic bone formation is rare in the gastrointestinal tract. We here present the clinical and pathologic details of a 56-year-old male patient with mucinous adenocarcinoma of the colon and a 70-year-old male patient with colon metastasis previously operated on for signet-ring-cell carcinoma of the stomach who was treated with radiotherapy postoperatively. Both of them showed diffuse bone metaplasia. Heterotopic bone formation is usually present with mucin-producing benign or malignant tumors. The pathogenesis of osseous metaplasia is not well known; however, it is speculated that the extravasation of mucin may have a stimulatory role.

Adenocarcinoma, Mucinous↗