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Proliferative myositis in a child.

A case of proliferative myositis in the lumbar paraspinal muscles in a 14-year-old boy is presented. Imaging investigations including plain radiograph, ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), bone scan and positron emission tomography (PET) were suggestive of an inflammatory process such as myositis ossificans. The diagnosis was made by incisional biopsy. More pronounced edema, more muscle fiber necrosis and a higher cellularity were found compared to adult cases. The karyotype of the lesion was normal. Clinically, the mass disappeared spontaneously. After 24 months, asymptomatic bridging ossification between the third and fourth lumbar vertebrae was noted.

Adolescent↗

Effects of extremely low frequency electromagnetic field (EMF) on collagen type I mRNA expression and extracellular matrix synthesis of human osteoblastic cells.

Human osteoblastic cells were grown in a three-dimensional (3-D) cell culture model and used to test the effects of a 20 Hz sinusoidal electromagnetic field (EMF; 6 mT and 113 mV/cm max) on collagen type I mRNA expression and extracellular matrix formation in comparison with the effects of growth factors. The cells were isolated from trabecular bone of a healthy individual (HO-197) and from a patient presenting with myositis ossificans (MO-192) and grown in a collagenous sponge-like substrate. Maximal enhancement of collagen type I expression after EMF treatment was 3.7-fold in HO-197 cells and 5.4-fold in MO-192 cells. Similar enhancement was found after transforming growth factor-beta (TGF-beta) and insulin-like growth factor-I (IGF-I) treatment. Combined treatment of the cells with EMF and the two growth factors TGF-beta and IGF-I did not act synergistically. MO-192 cells produced an osteoblast-characteristic extracellular matrix containing collagen type I, alkaline phosphatase, and osteocalcin, together with collagen type III, TP-1, and TP-3, two epitopes of an osteoblastic differentiation marker. The data suggest that the effects of EMFs on osteoblastic differentiation are comparable to those of TGF-beta and IGF-I. We conclude that EMF effects in the treatment of skeletal disorders and in orthopedic adjuvant therapy are mediated via enhancement of collagen type I mRNA expression, which may lead to extensive extracellular matrix synthesis.

Cell Line↗

The significance of bone and cartilage formation in malignant fibrous histiocytoma of soft tissue.

Malignant fibrous histiocytoma of soft tissues (MFH), a mesenchymal tumor of varied morphologic patterns and cell types, sometimes contains bone and cartilage. Such bony and cartilaginous elements in a pleomorphic MFH may pose a difficult diagnostic challenge. An MFH with bone and cartilage can be distinguished from extraosseous osteogenic sarcoma and chondrosarcoma on the basis of qualitative and quantitative features of the osseous and chondroid elements. Five cases of soft tissue MFH containing bone and cartilage reported here showed: 1) that the bony and cartilaginous elements are mostly metaplastic and tend to be in the pseudo-capsule or fibrous septa of the tumor; and 2) that the bony elements may show a zoning pattern with peripheral maturation similar to myositis ossificans. In a single case, there was a small amount of "tumor osteoid." However, this was focal and lacked the diffuse and finely divided or ribbon-like pattern generally associated with osteogenic sarcoma. The probability that MFH with bone and cartilage is less aggressive than soft tissue osteogenic sarcoma or chondrosarcoma underscores the importance of accurate histologic diagnosis of these tumors.

Adult↗

Extraskeletal osteosarcoma arising in an ectopic hamartomatous thymus. Report of a case and review of the literature.

A unique case of an osteosarcoma arising within a hamartoma of the thymic gland located in the left pleural cavity of an 11-year-old girl is described. The presence of abundant mature lamellar bone within this thymic hamartoma provides an explanation for the osseous histogenesis of the sarcoma. The development of an osteosarcoma from extraskeletal osseous tissue is a rare occurrence and has been documented in association with myositis ossificans and most recently in a case of dermatomyositis with metaplastic ossification.

Angiography↗

Immunohistochemical study of childhood rhabdomyosarcomas and related neoplasms. Results of an Intergroup Rhabdomyosarcoma study project.

The authors assessed a panel of immunohistochemical stains against 109 pediatric solid tumors, primarily rhabdomyosarcomas, under the auspices of the Intergroup Rhabdomyosarcoma Study. Fresh tumor tissue received from participating organizations was divided into portions that were either frozen or fixed in formalin, alcohol, or B5. Immunostaining was performed by the avidin-biotin complex method using monoclonal antibodies to desmin, neurofilaments, vimentin, cytokeratin, and leukocyte common antigen on cryostat sections. Tissue was also embedded in paraffin and stained with antimuscle-specific actin (MSA) and polyclonal antibodies to desmin, creatine kinase M subunit (CKM), myoglobin, and neuron-specific enolase (NSE). Antidesmin staining of cryostat sections was the most sensitive indicator of rhabdomyosarcoma (58 of 62 specimens positive). Results with this reagent in alcohol-fixed and formalin-fixed tissue were similar (46 of 56 positive versus 43 of 56 positive, respectively) and comparable with results with anti-MSA in formalin-fixed tissue (43 of 55 positive). However, the proportion of cells stained by antidesmin was higher in alcohol-fixed tissue than in formalin-fixed tissue. Staining with antimyoglobin and anti-CKM was much less satisfactory, with positivity rates of 17 of 37 and 11 of 57, respectively, in formalin-fixed rhabdomyosarcomas. Immunostaining of muscle markers revealed evidence of myogenesis in six undifferentiated sarcomas and in two sarcomas with inadequate histologic study on hematoxylin-eosin-stained sections. However, positivity was also noticed in samples of fibromatosis, Wilms' tumor, ectomesenchyoma, peripheral primitive neuroectodermal tumor, renal rhabdoid tumor, myositis ossificans, malignant fibrous histiocytoma, and embryonal sarcoma of the liver. The authors conclude that combined use of antidesmin and anti-MSA enhances the diagnosis of childhood sarcomas, especially when employed with other techniques such as electron microscopic study.

Actins↗

Brief communication: the Galler Collection: a little-known historic Swiss bone pathology reference series.

The aim of this article is to briefly present the Galler Collection, a little-known historic Swiss bone reference series of high value for paleopathological research. The Galler Collection consists of approximately 600 mostly dry-bone specimens of many major bone diseases dating from the late 19th and early 20th centuries. Clinical information and autopsy reports are available for the majority of specimens. Rarely documented conditions represented in the collection include: severe forms of myositis ossificans progressiva, kyphoscoliosis, endemic cretinism, hypo- and hyperparathyroidism, and phosphorus-induced bone necrosis. At present, the Galler Collection is located at the National History Museum in Basel (Augustinergasse 2, 4001 Basel, Switzerland, Telephone: +41612665500, Fax: +41612665546).

Bone Diseases↗

Creatine kinase brain isoenzyme (BB-CK) presence in serum distinguishes osteopetroses among the sclerosing bone disorders.

Creatine kinase (CK) isoenzyme BB-CK is predominantly found in brain and is not normally detected in the blood. A few recent reports, however, have described BB-CK in serum from several patients with osteopetrosis (OP). To evaluate the presence and specificity of BB-CK in serum in the osteopetroses among disorders that increase skeletal mass, we quantitated total CK activity and CK isoenzymes in 15 patients representing the five major clinical forms of OP (2 infantile, 3 intermediate, 7 adult [2 type I, 5 type II], and 3 carbonic anhydrase II [CA II] deficiency cases) and in 22 patients representing 14 other types of sclerosing bone disease. All OP patients (except the two adult type I subjects) had BB-CK readily detected in their serum. Conversely, only 1 of the 22 patients with other sclerosing bone disorders had detectable BB-CK in serum (1 of 3 patients with fibrodysplasia [myositis] ossificans progressiva who had barely measurable activity). In three OP patients (one of two with the infantile form and two of five with adult, type II disease), BB-CK values were sufficiently high that serum total CK activity was elevated. In a newborn with malignant OP, both cord blood plasma and peripheral blood serum had substantial amounts of BB-CK. In three subjects (with adult type II OP), who were restudied 2-6 years later, BB-CK was still elevated in their blood. BB-CK in serum appears to distinguish the osteopetroses among the sclerosing bone disorders. Absence of serum BB-CK in adult type I disease suggests that this condition may not be a genuine form of OP. Assay of BB-CK in fetal blood could be studied as a means for prenatal diagnosis of malignant OP. Why the osteoclast failure that characterizes all true forms of OP is associated with BB-CK in the circulation is a new question for skeletal biologists.

Adolescent↗

p53 immunohistochemistry in malignant fibrous histiocytomas and other mesenchymal tumours.

In this study we analysed by immunohistochemistry the expression of p53 protein in 14 malignant fibrous histocytomas (MFHs), 22 other types of sarcoma (eight leiomyosarcomas, four rhabdomyosarcomas, four liposarcomas, two fibrosarcomas, two chondrosarcomas, one malignant schwannoma, and one dermatofibrosarcoma protuberans), and 25 non-malignant mesenchymal lesions (eight dermatofibromas, four cases of nodular fasciitis, three leiomyomas, three fibromatoses, two epithelioid.leiomyomas, two neurofibromas, one schwannoma, one myositis ossificans, and one giant cell tumour of tendon sheath). Four MFHs and nine other types of sarcoma (four leiomyosarcomas, two chondrosarcomas, one liposarcoma, one fibrosarcoma, and one dermatofibrosarcoma protuberans) showed nuclear positivity for p53. Of the benign soft tissue lesions, p53 positivity was observed in two fibromatoses, one nodular fasciitis, and one dermatofibroma. The number of p53-positive cells in these benign lesions was considerably smaller than that in most of the p53-positive sarcomas. The p53 positivity in MFHs and other types of sarcoma indicates that p53 gene alterations may play a part in the neoplastic transformation of these tumours. The occurrence of p53 positivity in benign mesenchymal lesions suggests that sometimes p53 protein may accumulate in cells without an associated malignancy. Because of this, p53 immunoreactivity cannot, by itself, be used as a criterion of malignancy. According to our results, p53 positivity in over 1 per cent of tumour cells in mesenchymal lesions favours malignancy.

Genes, p53↗

[Neurologic-psychiatric follow-up studies of reanimated tetanus patients (author's transl)].

During a study of sequela of intensive care therapy eight patients with longterm respirator treatment for severe tetanus infection were studied by neurologic-psychiatric examination two years or more after discharge from the hospital. Two out of four patients with permanent damage suffered from ankylosis of the hip joint caused by myositis ossificans. One patient showed hyperaesthetic-emotional syndrom and IQ-reduction caused by cardiac arrest. Two further patients were resuscitated from cardiac arrest without persisting psycho-physiological injury. A 70 year old man suffered from cardiopulmonary injury, lack of initiative and fatigability. None of the patients complained of depressing memories and all emphazised their pleasure of being alive. The permanent damage fation and not to the tetanus toxin per se. Improved techniques for tetanus patients on curare and artificial respiration may result in better therapeutical results in the future.

Adolescent↗

Benign bone-forming lesions: osteoma, osteoid osteoma, and osteoblastoma. Clinical, imaging, pathologic, and differential considerations.

The benign bone lesions--osteoma, osteoid osteoma, and osteoblastoma--are characterized as bone-forming because tumor cells produce osteoid or mature bone. Osteoma is a slow-growing lesion most commonly seen in the paranasal sinuses and in the calvaria. When it occurs in the long bones, it is invariably juxtacortical and may need to be differentiated from, among others, parosteal osteosarcoma, sessile osteochondroma, and a matured juxtacortical focus of myositis ossificans. Osteoid osteoma and osteoblastoma appear histologically very similar. Their clinical presentations and distribution in the skeleton, however, are distinct: osteoid osteoma is usually accompanied by nocturnal pain promptly relieved by salicylates; osteoblastoma arises predominantly in the axial skeleton, spinal lesions constituting one-third of reported cases. This review focuses on the application of the various imaging modalities in the diagnosis, differential diagnosis, and evaluation of these lesions. Their histopathology also is discussed, and their treatment briefly outlined.

Bone Neoplasms↗

The gene for bone morphogenetic protein 2A (BMP2A) is localized to human chromosome 20p12 by radioactive and nonradioactive in situ hybridization.

Bone morphogenetic protein 2A (BMP2A), a member of the decapentaplegic-Vg-related family, belongs to the transforming growth factor beta superfamily and has a striking sequence similarity to the decapentaplegic locus in Drosophila melanogaster, a major determinant of pattern specification during embryogenesis. BMP2A is thought to be involved in cartilage and bone formation during embryogenesis, but may have additional functions in morphogenesis as implied by its expression in various organs and embryonic tissues of mice. Human BMP2A, assigned to chromosome 20 by the use of human-Chinese hamster ovary cell hybrids, is considered to be a reasonable candidate gene for the autosomal dominant disease of fibrodysplasia (myositis) ossificans progressiva. We have confirmed the localization of BMP2A to chromosome 20 and regionally assigned the locus to 20p12 by radioactive and nonradioactive in situ hybridization.

Bone Morphogenetic Proteins↗

Posttraumatic calcification of the annular ligament of the radius.

Eight cases of calcification following anterior dislocation of the head of the radius are described. It was demonstrated as early as 2 weeks after injury and in 7 cases appeared in the form of a shell on the volar aspect of the head. The other type exhibits a cuff of calcification related to the radial neck. Parallel cases are drawn from a literature review, and the distinction is made between calcification in the disrupted annular ligament/capsule or oblique cord and myositis ossificans. Regression in several cases may be a consequence of open reduction.

Adolescent↗

Osteoma of soft parts.

Osteomas of soft tissue are rare tumors. All previously reported cases occurred in the head, usually in the posterior tongue. We present two patients with soft-tissue osteomas in the thigh. Both masses were densely ossified and consisted of mature trabecular bone on computed tomography and magnetic resonance examination. Increased uptake of bone-seeking radiopharmaceutical was found on scintigraphy. Histologically, mature lamellar bone was seen with small amounts of cartilage seen in the periphery. Symptoms of mass effect which resolved following surgery were noted. We believe that this previously unrecognized soft-tissue appendicular neoplasm lies at the end of a spectrum of post-traumatic ossifying lesions which includes soft-tissue chondromas and osteochondromas, as well as myositis ossificans.

Aged↗

Calcification in the soft tissues of the chest after thoracotomy.

We have observed soft-tissue calcification of the chest in 4 of 54 patients following thoracotomy in the neonatal period for a Blalock-Taussig or a Blalock-Hanlon procedure. The time of appearance ranged from 2 weeks to 11 months. This calcification is similar to myositis ossificans in having a traumatic origin. In one of the patients, it resolved spontaneously.

Calcinosis↗

The "pelvic digit"--an unusual developmental anomaly.

Five cases of a rare developmental anomaly involving extraskeletal bone formation are described--four in the pelvis and one arising from the 12th rib. Three of these bony growths, called "Pelvic digits" had well developed "joints" or pseudoarticulations. The similarity of this anomaly to posttraumatic myositis ossificans and avulsion injuries of the pelvis (rectus femoris) is pointed out and a differential diagnosis offered.

Adult↗

Soft tissue osteochondroma. A report of three cases.

Three cases of benign soft tissue osteochondroma, a lesion of uncertain pathogenesis, are reported. Two cases were located in the subcutaneous tissues beneath the calcaneus. The other was located in the soft tissues near the left ankle joint. The diagnosis of soft tissue osteochondroma should be considered when a well-defined osseous mass is located in the soft tissues. The differential diagnosis includes myositis ossificans, tumoral calcinosis, synovial chondromatosis, and soft tissue osteosarcoma.

Adolescent↗

Analysis of mutant P53 protein in osteosarcomas and other malignant and benign lesions of bone.

Alterations of tumour suppressor genes are considered crucial steps in the development of human cancers. Expressions of p53 protein, a product of the tumour suppressor gene altered most commonly in human cancers examined so far, were investigated immunohistochemically in 18 osteosarcomas and 40 other malignant and benign lesions of bone. A monoclonal antibody clone PAb240, which recognizes a common conformational epitope of mutant p53 proteins, stained nuclei of tumour cells in 12 of 18 osteosarcomas (67%). Six tumours (33%) particularly showed positive immunoreactions in more than half of the tumour cells. PAb240 also stained tumour cells in a small number of other malignant bone tumours, such as malignant fibrous histiocytoma, chondrosarcoma, and Ewing's sarcomas. Furthermore, a small number of cells of giant-cell tumours were positively stained. In contrast, PAb240 was completely negative in 21 benign bone tumours and reactive lesions examined. Another monoclonal antibody clone PAb1801, which reacts with both wild- and mutant-type p53 protein, reacted in nuclei of tumour cells of 7 osteosarcomas (39%). Most of those also reacted with PAb240. PAb1801 was expressed much more frequently in other malignant bone tumours and giant-cell tumours. In addition, PAb1801 showed intranuclear positive reactions in tumour cells of a benign chondroblastoma, and reactive cells such as actively proliferating preosteoblasts in a myositis ossificans and osteoclast-like giant cells in a giant-cell tumour. The immunoelectron-microscopic observation that p53 protein was localized in euchromatic areas of nuclei of osteosarcoma cells supported the specificity of immunoreaction for p53 protein, indicating an active role of p53 protein in the regulation of DNA synthesis and transcription. These findings suggest that point mutation of the p53 gene is frequently involved in the development of osteosarcomas. PAb240 may be a useful tool not only in screening point mutations of the p53 gene in osteosarcomas but also in the differential diagnosis between osteosarcomas and reactive bone-forming lesions. Expressions of mutant p53 protein were not correlated with any clinical or pathological factors examined, although the results should be confirmed in studies of a large number of osteosarcomas.

Adolescent↗

Bone remodeling in pathologic conditions. A scanning electron microscopic study.

Bone remodeling in pathologic conditions was studied with the scanning electron microscope (SEM). Benign and malignant ossification were examined in cases of myositis ossificans, ossifying fibroma, osteoid osteoma, and osteosarcoma, Resorption of bone due to invasion by non-ossifying tumors was found in cases of squamous cell carcinoma, adenocarcinoma, ameloblastoma, and multiple myeloma. Bone formation due to excessive production of growth hormone was studied in a case of acromegaly. Resorption of bone due to pathologic processes resembled the pattern found in surfaces which were undergoing resorption by osteoclasts. Lamelar-cortical bone formation in acromegally was similar in nature to normal bone. The deformities were rleated to the excessive continuous osteogenesis that occurs in these instances. Neoplastic ossification was characterized by calcifying globules, the diameters of which ranged from 1 to 3 micron. The surfaces of these globules were constructed of minute calcospherites with diameters ranging from 0.1 to 0.3 micron. It is suggested that the pattern of globular calcification is similar to the type that was found with the SEM in fetal bone and cartilage, during healing of fractured bone, and also with the TEM in normal and pathologic calcification.

Acromegaly↗