RSNA EJ: a supplement to RadioGraphics and a new venture in radiologic publishing.
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Biomedical subjects
Publications and source records attributed to L V Ackerman.
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PURPOSE: To evaluate the imaging and histopathologic findings and clinical course of patients with periosteal Ewing sarcoma (PES). MATERIALS AND METHODS: Conventional radiographs, computed tomographic (CT) scans, and magnetic resonance (MR) images in 10 adolescents and one adult were evaluated for the extent and character of PES. RESULTS: Ten of 11 masses were in the proximal extremities and one in the fibula; nine were diaphyseal and two, metadiaphyseal. Radiographs and CT scans showed a subperiosteal mass that did not invade the medullary cavity, was contiguous with and elevated the periosteum, and produced a Codman triangle and periosteal reaction. No PES exhibited osseous or cartilaginous matrix calcifications. MR imaging and histopathologic examination helped confirm the sparing of cancellous bone and the subperiosteal location. CONCLUSION: PES differs from the more common medullary and soft-tissue Ewing sarcomas in location, marked male predominance, and lack of presenting metastases. Except for the absence of matrix calcifications, PES resembles other periosteal sarcomas in imaging characteristics and a less aggressive clinical course.
Fibrous dysplasia is usually a slowly progressive, benign disease that develops over several years and presents with deformity or mild symptomatology. Five of 34 patients (ages 4-21 years), who were subsequently diagnosed histologically as having fibrous dysplasia of the maxillary sinus, rapidly developed soft tissue masses of the malar region over a period of less than 4 months with accompanying pain (2 patients) and nasal obstruction and exophthalmos (2 patients). Each was clinically suspected of having a sarcoma; two had been thought to have an "osteofibrosarcoma" on initial biopsy at outside hospitals. After resection, all lesions developed regrowth. At histopathologic examination, both initial and recurrent masses proved to be typical fibrous dysplasia with spicules of woven bone in cellular, sometimes vascular, fibrous tissue. No malignant degeneration was found. On conventional radiography, aggressive fibrous dysplasia produced opacification and expansion of the maxillary sinus and apparent disruption of its wall with an associated soft tissue mass. Computed tomography (CT) demonstrated voluminous heterogeneous masses with "ground glass appearance", calcifications, areas of enhancement, low attenuation, cystic areas, and a thinned, sometimes interrupted, maxillary wall. Despite the aggressive clinical course for both initial and recurrent lesions, the CT findings of a "ground glass" mass with calcifications surrounded by a maxillary sinus wall, even if incomplete, can suggest the diagnosis of aggressive fibrous dysplasia.
Rarely, rhabdomyosarcoma can present with bone pain and bone lesions on radiographs without evidence of a primary tumor. Of 428 children with biopsy-proven rhabdomyosarcoma, four presented with radiographic evidence of bone metastases, but no primary tumor was found on subsequent evaluation. On radiographs, these metastases, located most commonly in the metaphyses of the extremities and in the spine, displayed a destructive or diffusely permeative pattern without sclerotic margins and mimicked the more common neuroblastoma. One patient also had diaphyseal cortical lytic metastases of the tibia. Radiographs defined metastases of the extremities better than the correlative bone scans. In the spine, on T2-weighted magnetic resonance (MR) images, metastases displayed high signal intensity which contrasted with the low-signal-intensity marrow in these pediatric patients. On histopathologic examination, metastatic rhabdomyosarcoma was composed of small cells of variable size, shape, and growth pattern similar to other round cell tumors. A positive desmin immunohistochemical test helped to establish the diagnosis. The radiologist, pathologist, and clinician should be aware of this unusual presentation of rhabdomyosarcoma so that suitable immunohistochemical tests are performed and appropriate chemotherapy given.
Among seven patients with extraskeletal mesenchymal chondrosarcoma (EMC), three children (aged 3-6 years) developed EMC in a central location and four adults (aged 38-54 years) developed EMC in both central and peripheral sites. Conventional radiography and tomography and computed tomography (CT) depicted EMC as a soft-tissue mass with ring, arc, stippled, and highly opaque calcifications in four patients. Contrast-enhanced CT showed lobulation and peripheral tumoral enhancement, sometimes with central low-attenuation areas. On magnetic resonance (MR) images, EMC was a lobulated mass with high signal intensity on T2-weighted images and enhancement with low-signal-intensity focal areas on contrast-enhanced T1-weighted images. All adults developed recurrences and/or metastases and died. Of the three children, two were living and free of disease at the end of the study and the third child died of chemotherapeutic-induced leukemia. Although imaging features of EMC are nonspecific, its chondroid-type calcifications and foci of low signal intensity within enhancing lobules may reflect its dual histopathologic morphologic characteristics of differentiated cartilage islands interspersed within vascular undifferentiated mesenchyme.
Myositis ossificans is a relatively rare, well-defined entity. The 23 cases sent for consultation to two of us (L.V.A. and A.N.) were reviewed. Clinical, radiologic, and microscopic information was reexamined, and special attention was given to features infrequently seen in typical myositis ossificans. Average age at presentation was 23 years, but 1 patient was younger than 2 years, and 2 were younger than 10 years. Due to the uncommon location of 15 lesions and an unusual presentation in 5, the correct diagnosis was not obvious in these cases. A history of trauma was elicited in 8 patients, denied by 7, and was not queried in the remainder. Radiologic studies raised the possibility of a malignant bone-forming tumor in at least three instances; myositis ossificans was originally diagnosed in 6 cases radiologically. In 8 cases, histologic evidence suggested malignancy, including osteosarcoma, either parosteal or extraosseous, in 6. Other diagnoses included epithelioid sarcoma and callus formation. Presentation of these variations from the norm highlights the importance of recognizing the evolution of a nonneoplastic fibro-osseous and cartilaginous entity in which conservative treatment is curative.
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Two cases of neuropathic disease of the shoulder without evidence of a neurological disorder are described. The absence of any neurological disorder led to the cases being initially mistaken for primary neoplasms of bone, lending support to the vascular theory of pathogenesis of this lesion. Careful attention to the radiographic features and knowledge that a neurological disorder need not be present should lead to the correct diagnosis.
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Thirty-eight cases of histologically confirmed chondromyxoid fibroma were reviewed and their radiographic features recorded. These findings, coupled with a review of the English-language medical literature, suggest that this rare, benign bone tumor has a characteristic but not specific radiographic appearance and may often mimic more common tumors. Chondromyxoid fibroma may occur anywhere in the skeleton, but almost half of the cases occur around the knee. The possibility of chondromyxoid fibroma should always be considered when a focal bone lesion is evaluated that has geographic bone destruction, a sclerotic rim, lobulated margins, and septation. The diagnosis of chondromyxoid fibroma is most likely when the patient is in the 2nd decade of life.
The gastrointestinal tract lends itself quite well to digital imaging. Since fluoroscopy is already employed, the images can easily be obtained in digital format and several manufacturers have now developed systems for commercial use. Because of the type of pathology as well as the inherent subject contrast, it would appear the resolution requirements for digital imaging of the gastrointestinal tract are less than in other organ systems. The necessary resolution level is already technically available and feasible without significant cost. Digital imaging also holds promise of at least a modest reduction in radiation dose to the patient, as well as a reduction in costs, although these factors are operator dependent. However, digital imaging provides a latitude in performing the examination that is not available with screen-film systems. Finally, there is promise that by acquiring gastrointestinal images in digital format, manipulation of the images may help increase diagnostic accuracy by improving both technical and perceptive components of diagnosis. Not only will simple image manipulation be helpful but there is even potential for computer-assisted evaluation of gastrointestinal images.
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Five radiographs of double-contrast colon examinations demonstrating subtle mucosal changes of inflammatory bowel disease and five radiographs of healthy colonic mucosa were selected and digitized to four levels of resolution. Pixel sizes of 0.1 mm, 0.2 mm, 0.4 mm, and 0.8 mm were used. Ten radiologists interpreted the images, which were displayed on laser-printed film. Analysis of variance with repeated measures was performed and receiver operator characteristic curves were determined. The results demonstrate that the sensitivity in detecting subtle mucosal abnormalities improved as the resolution improved, with the best sensitivity at the highest resolution; more experienced readers detected details well even at the poorer levels of resolution; the resolution necessary for successfully evaluating the colonic mucosa was lower than expected; and given low noise levels, the matrix size used in conventional television fluoroscopy would be adequate for mucosal evaluation.