Fibrodysplasia ossificans progressiva (myositis ossificans progressiva) treatment with disodium etidronate.
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By light microscopy the subdermal nodule of a patient with fibrodysplasia ossificans progressiva (FOP) had a fibromatoid histologic appearance. The cytoplasm of the cells stained strongly for mannose-rich glycoprotein with the concanavalin A-horseradish peroxidase (con A-HRP) method. The tumors also exhibited abundant hyaluronidase-digestible mucopolysaccharide in the interstitium with various basic staining reagents. This material appeared to consist principally of hyaluronic acid or chondroitin sulfate with few or mainly masked sulfate esters. At the ultrastructural level, cells interpreted as the tumor cells in the subdermal nodule from the patient displayed extremely hyperplastic granular reticulum and well-developed Golgi elements and appeared very active in synthesis and secretion of protein. The material in the dilated cisternae of the granular reticulum stained for glycoprotein with the con-A-HRP method. Macrophages which comprised the other main cell type in the nodules commonly contacted the tumor cells and occasionally evidenced engulfment of these cells. The intercellular matrix of the nonossified subdermal nodule exhibited greatly increased mucosubstance and, by electron microscopy, showed an unusual network of dialyzed iron-reactive acid muco-substance in the interstitium.
A case of myositis ossificans, following a hemiparesis due to a cerebral haemorrhage and treated with synthetic slamon calcitonin, is described. An improvement in joint range and a cessation of further ectopic calcification was seen but, because of the remitting nature of myositis ossificans itself, the therapeutic role of calcitonin in this case is unproven.
Myositis ossificans circumscripta is the heterotopic formation of nonneoplastic bone and cartilage in soft tissue. These benign lesions can mimic more serious lesions, both radiographically and histopathologically. Recognition of the benign character of myositis ossificans is imperative in order to avoid mutilating surgical procedures. Five cases are presented with emphasis on angiographic signs which differentiate this lesion from histopathologically similar lesions. Three of these are illustrated, along with an example of an osteosarcoma for comparison. The angiographic manifestations of myositis ossificans differ in the various phases of the disease. In the active stage, the lesions have numerous fine vessels causing a diffus stain. Malignant tumors such as osteosarcoma, extraosseous osteosarcoma, and fibrosarcoma, which are included in the differential diagnosis, often present clearly defined pathologic vessels as well as arteriovenous shunts, venous lakes, amputated vessels, invasion of large arteries and veins, and large abnormal draining veins. In the healing stage, the lesions are usually avascular, and there is no difficulty in differentiating this condition from malignant bone lesions with the sole exception of well differentiated parosteal osteosarcomas. In these cases, the plain radiographic features are most important in arriving at the correct diagnosis.
A 28 year old patient complained of sensory disturbances and pain in the right upper arm during pregnancy. During the 32nd week of her pregnancy, a large painful mass developed in the flexor muscles which, radiographically, showed some calcification. A diagnosis of a parosseous sarcoma was made; biopsy, however, indicated a diagnosis of non-traumatic myositis ossificans. Since the histological appearances of active myositis may be vary difficult to distinguish from a juxtacortical sarcoma, a right brachial angiogram and scintiscan were obtained. The angiographic and scintigraphic findings were erroneously considered to suggest malignancy. Following delivery, the tumour was removed. Futher histology confirmed the diagnosis of localised, non-traumatic myositis ossificans. The value of radiology, biopsy, angiography and scintigraphy are discussed with reference to our experience.
Two cases of the so-called fascial analogue of myositis proliferans were investigated by histological and electron microscopic methods. It was found that the structure of the fascial variant corresponds almost completely to the true myositis proliferans localized within the musculature. The electron microscopic observations show a preponderantly histiocytic differentiation of the cells and strongly activated proliferating capillaries, and exclude a myogenic origin of the characteristic ganglion-like giant cells. Ultrastructurally a traumatic genesis appears possibly, the cells of the lesion could derive from multipotent cells of the microvasculature. The relations to myositis ossificans and fascitis nodularis are discussed.
A case of myositis ossificans traumatica has been reported. Clinical, histopathologic, and roentgenographic entities have been referred to. An up-to-date review of the literature on this disorder has been presented.
Two cases of myositis ossificans involving the quadriceps femoris muscle are described following total knee replacement arthroplasty. This condition appears to be a very rare complication of this operation. One case was helped by ultrasound therapy.
The clinical and laboratory features of a case of myositis ossificans progressiva are described, and the recent literature concerning the pathogenesis and treatment of the condition is briefly reviewed.
Localized areas of active myositis ossificans, occurring without a clear history of antecedent trauma, have been referred to as a "pseudo-malignant osseous tumor of soft tissue." This lesion may be mistaken both roentgenographically and pathologically for a malignancy. The roentgenographic signs which favor a diagnosis of non-neoplastic heterotopic bone formation include a lucent zone between the lesion and the adjacent bone, an intact underlying cortex, diaphyseal location, dense calcification in the periphery, and loss of volume on serial films.
Ultrasonic evaluation of a soft tissue mass of the thigh was performed and suggested the diagnosis of myositis ossificans. Correlation with routine radiographic studies is made, as well as with the follow-up radiographic examination.
The clinical, pathological and radiological features and the differential diagnosis of the very rare condition of non-traumatic myositis ossificans circumscripta are illustrated by one patient. The characteristic radiological appearances make a correct diagnosis of this localised, non-malignant process possible.
Seven cases of pseudomalignant ossifying myositis with a typical clinical symptomatology have been reported. None of the cases had experienced an injury. All the lesions were intramuscular and all of them showed a zonal arrangement. Electron microscopy in three cases allowed the demonstration of cells showing morphological features of myofibroblasts and monocytic cells of the macrophage type. These previously unreported features together with the zonal pattern of the lesions indicate their reparative nature.
A 20-year-old woman with a severely crippling myositis ossificans progressiva was treated with a diphosphonate (EHDP), 10-20 mg/kg/day. While being treated with this drug, surgical removal of ectopic bone was performed. Although ectopic calcification recurred postoperatively, considerable functional improvement was achieved. At the highest dosage of EHDP, hypercalcaemia gradually appeared, but was reversible upon cessation of drug treatment. It is probably related to a direct effect of EHDP on the bone.
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