PubMed HealthSearch

Biomedical subjects

G Edel

Publications and source records attributed to G Edel.

At least 19 recordsLinked to original sources

Case report 699. Primary localized skeletal cryptococcosis (torulosis) of the right tibia at its proximal end.

The case of a 58-year-old man with a normal immune status with primary localized skeletal cryptococcosis of the right tibia was presented. Radiologically it appeared as an osteolytic lesion with marginally indistinct borders in the epimetaphyseal region of the proximal tibia. Histologically, necrotizing granulomatous inflammation was associated with a prominent fibrohistiocytic reaction. The presence of cryptococci was confirmed by special stains. Clinicopathological features and the therapeutic aspects of this rare infective bone lesion were discussed. It is uncommon today, particularly in developed countries, to encounter a localized skeletal fungal infection. Therefore, radiologists and pathologists generally may be unfamiliar with the appearance of the lesion and tend not to consider the possibility of fungal osseous disease in the differential diagnosis. Unless it is remembered that fungal infection may cause a localized bone lesion with varied histological responses, the diagnosis may be overlooked, and bacteriologic proof may not be sought.

Bone Diseases

Osteofibrous dysplasia of long bones--a reactive process to adamantinomatous tissue.

The most controversial aspect of osteofibrous dysplasia (OFD) is its possible histogenetic relationship to adamantinoma of long bone. Evidence is recently beginning to accumulate that OFD may be a reactive process to regressive adamantinoma. To verify the concept, 13 lesions of OFD were studied again by immunohistochemistry for cytokeratins of different molecular masses, as well as by conventional stainings. In addition, 2 adamantinomas and 6 fibrous dysplasias of the tibia were studied for reference. A small number of spindle- or ovoid-shaped cells scattered individually in the fibro-osseous stroma showed positive reactions for cytokeratins of 55-57 kDa in 2 lesions, and for those of 45-56.5 kDa in 8 lesions of 13 OFDs, although no definite epithelial island could be detected even by immunohistochemistry. Adamantinomas also showed single cytokeratin-positive cells dispersed in fibroblastic stroma, in addition to epithelial islands positive for cytokeratins of both 55-57 kDa and 45-56.5 kDa. All cases of fibrous dysplasia were negative for cytokeratins. During the observation, no case of OFDs progressed to classic adamantinoma. The present study, demonstrating the existence of an intermediate stage between "differentiated adamantinoma" and total elimination of adamantinomatous components, gives further support for the concept that OFD is a secondary reactive process to adamantinomatous tissue. In practice, the existence of single scattered cytokeratin-immunoreactive cells in otherwise typical OFDs may not indicate the truly malignant behaviour of classic adamantinoma, unless discrete epithelioid cell nests are also found.

Adolescent

Morphological heterogeneity and phenotypical instability versus metastatic stability in the murine tumor model ER 15-P.

At clinical presentation, the majority of malignant tumors are composed of multiple clonal subpopulations of tumor cells with different phenotypic characteristics. Using the experimental tumor model ER 15-P, a methylcholanthrene-induced pleomorphic sarcoma of the C57 Bl6J mouse, we studied a system of long-term in vivo passages of this primary tumor for cell morphological changes, and alterations in the potential for spontaneous lung metastases. Transplants from the primary after the 4th, 20th, 40th and 80th i.m. passage (referred to as T4, T20, T40, and T80 respectively) together with their lung metastases were investigated by light microscopy, immunohistochemistry, and electron microscopy. In addition, the potential for metastasis to the lungs in each group was determined and compared with that of the parent T4 tumors. T4 tumors were mainly composed of spindle-shaped tumor cells with the ultrastructural features of fibroblasts and myofibroblasts, often arranged in a storiform or fasciculated growth pattern, and intermingled with tumor giant cells. Some small areas contained polygonal or rounded tumor cells, ultrastructurally undifferentiated, and sometimes arranged in a hemangiopericytoma-like growth pattern. Although electron-microscopical findings clearly demonstrated the mesenchymal origin of these tumor cells, immunostaining with a polyclonal antibody to vimentin was unspecific in all tumor cells and normal mouse tissue. Monoclonal antibodies to vimentin from different sources were completely negative in tumor cells and murine stromal components. In contrast, myofibroblast-like tumor cells showed immunohistochemically, a moderate to strong co-expression with monoclonal antibodies to desmin, muscle actin and alpha-smooth muscle actin. On the basis of these morphological findings, the primary ER 15-P was classified as a pleomorphic myofibrosarcoma. The lung metastases of T4 tumors were mainly composed of undifferentiated round to polygonal tumor cells, while the number of desmin-positive, muscle- and alpha-smooth muscle-actin-positive cells was reduced. The morphological features of T20 tumors and their lung metastases were the same as in T4, indicating a relative stability of the phenotype up to that stage. In contrast, T40 and T80 tumors and their lung metastases were found to contain almost exclusively undifferentiated tumor cells and many tumor giant cells. While fibroblast-like tumor cells were seen only occasionally, myofibroblast-like tumor cells had almost completely disappeared. The potential for lung metastases was nearly constant in all groups, suggesting metastatic stability. Obviously, the undifferentiated tumor cells of this model are associated with a higher metastatic potential.

Actins

An immunohistochemical study of the breast using antibodies to basal and luminal keratins, alpha-smooth muscle actin, vimentin, collagen IV and laminin. Part I: Normal breast and benign proliferative lesions.

The distribution of simple epithelial (K8/18/19) and basal (myoepithelial) (K5/14) keratins, alpha-smooth-muscle actin, vimentin, collagen IV and laminin in normal mammary glands and in benign proliferative lesions was studied using monoclonal antibodies (mAbs). These antibodies (Abs) identified myoepithelial cells and luminal cells specifically. In lesions with adenosis and papillomas, the two-layered formation resembled that of normal glands with a purely myoepithelial-epithelial differentiation. In scleradenotic lesions, the main cell was of myoepithelial immunophenotype with intermixed trabecular-tubular proliferations of simple-type epithelium. The sclerosis seems to be the result of an irregular basal lamina synthesis by the myoepithelial cells. In contrast to these lesions, epitheliosis represents a purely intraluminal cell proliferation of clearly simple epithelial immunophenotype and of cells with a basal keratin phenotype, lacking myoepithelial differentiation antigen actin. The basal keratin type epithelium may represent post-stem or intermediate cells developing into luminal epithelium. Epitheliosis appears to be a purely epithelial hyperplasia with striking similarity to the regeneration of normal breast epithelium. The different proliferative patterns may give an explanation for differences in potential cancer risks of patients with these lesions.

Actins

An immunohistochemical study of the breast using antibodies to basal and luminal keratins, alpha-smooth muscle actin, vimentin, collagen IV and laminin. Part II: Epitheliosis and ductal carcinoma in situ.

A detailed immunohistochemical study has been carried out on 63 breast lesions with epitheliosis, ductal carcinoma in situ and clinging carcinoma (lobular cancerization), using antibodies directed against keratins 5/14 and 14, 15, 16, 18, 19, vimentin, smooth muscle actin, collagen IV and laminin. The results have shown that epitheliosis on the one hand and ductal in situ and clinging carcinoma on the other are immunohistochemically different epithelial lesions. Epitheliosis appears to be epithelial hyperplasia with keratin 5/14 and keratin 14, 15, 16, 18, 19-positive cells. Compared to epitheliotic cells tumor cells of clinging carcinoma, lobular cancerization and ductal carcinoma in situ expressed only luminal keratins 14, 15, 16, 18, 19 in 85% of the cases studied; whereas in 15% there was a basal keratin expression. From our results we conclude that the clinging carcinoma (lobular cancerization) represents the initial morphological step in the development of ductal carcinoma in situ and thus may be interpreted as a minimal ductal neoplasia. With the immunohistochemical demonstration of basal and luminal keratins it may be possible in individual cases to differentiate between benign and malignant in situ lesions of the breast.

Actins

Chondroblastoma of bone. A clinical, radiological, light and immunohistochemical study.

The clinical and morphological findings of 53 chondroblastomas in the files of the Bone Tumour Registry of Westphalia are presented. The mean age of all patients was 19.2 years. The male-to-female ratio was 1.5:1. Forty-two of the tumours (79.8%) were located in the long tubular bones and short tubular bones of the hands and were closely related to the growth plate. Six cases (11.3%) were found in the flat bones, 4 cases (7.5%) in the tarsal bones and 1 case (1.9%) in the craniofacial bones. The characteristic radiological feature of 44 investigated lesions was a mostly eccentric radiolucency with a geographic pattern of bone destruction and matrix calcifications. Periosteal reaction was evident in 9% of the cases. Most tumours demonstrate the typical morphological features of chondroblastoma, but 3 cases resembled a giant cell tumour. In 2 cases a haemangiopericytoma-like growth pattern was observed. Nine of the tumours had an aneurysmal bone cyst-like component. Vascular invasion was seen in 1 case. Immunohistochemically most cells in 30 of the cases and fetal chondroblasts in 3 cases were strongly positive with vimentin and S-100 protein. Collagen type II was positive in the chondroid matrix of the tumours and in fetal cartilage tissue; collagen type VI was present focally around individual tumour cells and was always seen in the chondroid matrix of the lesions and in fetal cartilage. These findings support the cartilaginous nature of these tumours. In paraffin sections, 46.6% of the cases revealed a distinct positive reaction of some tumour cells with the monoclonal cytokeratin antibody KL1 (molecular weight 55-57 kDa). Only 4 of them demonstrated a coexpression with the other monoclonal cytokeratin antibody CK (clone MNF 116, molecular weight 45-56.5 kDa). In paraffin sections all fetal chondroblasts were negative with both cytokeratin antibodies. Frozen sections of 3 tumours showed a strong positive reaction with both cytokeratin antibodies in many chondroblasts, indicating an "aberrant" cytokeratin expression. Osteoclast-like giant cells stained positive with leucocyte-common antigen (LCA) and with the macrophage-associated antibody KP1, but were negative with the other macrophage-associated antibody MAC 387. Recurrence rate was 10.7%. The clinical course of all tumours was benign.

Adolescent

"Solid" variant of aneurysmal bone cyst.

A case of the so-called "solid" variant of aneurysmal bone cyst is reported. A 12-year-old girl with a few weeks' history of backache presented with a tender palpable mass located thoraco-spinal in the back at Th 3. Radiologically, the lesion was consistent with conventional aneurysmal bone cyst. Morphologically, it showed fibroblastic, fibrohistiocytic, fibromyxoid, osteoclastic and osteoblastic components as well as small aneurysmal sinusoids. Based on four other well documented cases, the clinico-pathological features and the differential diagnostical problems are discussed.

Bone Cysts

[Dynamic MR tomography in the diagnosis of inflammatory and tumorous space-occupying lesions of the musculoskeletal system].

In 216 inflammatory and tumorous lesions of bone and soft tissue, dynamic Gd-DTPA enhanced MR imaging was performed. For that purpose, 12 FLASH sequences (TR = 40 ms/TE = 10 ms/NSA = 2/flip angle = 90 degrees) were acquired within 4 minutes; and Gd-DTPA bolus injection was administered following the first sequence. Slopes from the signal intensity curves were calculated. Estimated slopes allowed an assessment of the malignant potential of a lesion with an accuracy of 88%. Further more, a reliable differentiation between vital and necrotic tissue and between tumour and peritumoral edema was reliably possible. With this technique, response of malignant tumours to preoperative chemotherapy was correctly predicted in 83% of the cases. No differentiation of inflammatory from tumorous lesions was possible.

Bone Neoplasms

Pathologic explanation for hypoechoic halo seen on sonograms of malignant liver tumors: an in vitro correlative study.

OBJECTIVE: The purpose of this study was to evaluate the morphologic substrate of the hypoechoic halo seen on sonograms of malignant liver tumors. MATERIALS AND METHODS: We used sonograms and pathologic examinations to evaluate 17 cadaveric livers with macroscopic tumors (three primary liver tumors, 14 metastases). During sonography (3.5 and 5.0 MHz), a representative section plane was marked, and the same section was examined histologically. Emphasis was placed on the architecture of the tumor and the morphology of the periphery of the tumor that could account for the hypoechoic halo seen on sonograms. RESULTS: In 13 of 17 hepatic tumors, a hypoechoic halo was detected on sonograms. Histopathologic examination showed an intratumoral rim consisting of proliferating tumor cells in 12 cases and an extratumoral rim of compressed liver parenchyma in all 13 cases. A detailed comparison of sonographic and histopathologic findings showed that the hypoechoic halo corresponded to a greater concentration of tumor cells and areas of less marked fibrosis and necrosis in the periphery of the tumors. This occurred in 11 cases. In one case, histologic studies showed that the hypoechoic rim was caused by compressed liver parenchyma. In another case, the hypoechoic halo was caused by intratumoral (cellular peripheral zone of tumor) and extratumoral (compressed liver parenchyma) components. All four tumors without a halo at sonography were uniform histologically. CONCLUSION: The sonographic halo seen on sonograms of malignant liver tumors seems to be caused predominantly by a zone of proliferating tumor in the periphery of the lesion.

Adenocarcinoma

[Non-Hodgkin's lymphoma with primary osseous manifestation, from the files of the Bone Tumor Registry of Westphalia].

30 cases of non-Hodgkin-lymphoma with primary bone manifestation were selected from the files of the Bone Tumor Registry of Westfalia from 1975 until 1992. Clinical data and radiologic aspects were evaluated. 60% of the lesions were localized within the long bones (femur 10, humerus 6, tibia 2). The lymphomas were classified based on paraffin-embedded material in combination with immunohistochemical findings according to the updated Kiel-classification. 29 B-cell lymphomas and one T-cell lymphoma of medium-sized pleomorphic type were found. 5 B-cell lymphomas were of low and 23 of high malignancy. Almost 50% of the tumors were of centroblastic type. The Kiel-classification was not applicable for one lymphoma, 3 others could not be subclassified because of shrinking artefacts. Prognosis in patients with localized disease (9 cases) was favorable, whereas only 25% of patients with generalized disease (12/cases) survived for 3 years or longer.

Bone Neoplasms

Juvenile intracortical adamantinoma of the tibia with predominant osteofibrous dysplasia-like features.

In view of the still disputed relationship between adult adamantinoma and osteofibrous dysplasia in children, a unique case of adamantinoma, indicating a direct relationship between the two lesions, is presented with a review of the literature. The patient was a six-year-old boy who complained of pain and swelling in the left lower leg. Roentgenographs showed a loculate osteolysis surrounded by sclerosis within the cortex of the tibial shaft that would be typical of osteofibrous dysplasia. Although an osteofibrous-dysplastic component predominated histologically, some small islands of epithelial cells were scattered throughout the lesion. Immunohistochemically, the tumor cells of these epithelial islands gave a constant positive reaction for cytokeratin as well as vimentin, while the stromal cells in the osteofibrous dysplasia-like lesion were positive for vimentin only. This type of lesion is recorded in the Bone Tumor Registry of Westphalia at a rate of 8.3% for osteofibrous dysplasia, and of 25% for adamantinoma. A review of the literature, yielding reports with remarkable uniformity on 14 cases beyond the present one, suggests the existence of a separate clinicopathologic entity to be called juvenile intracortical adamantinoma with predominant osteofibrous dysplasia-like features, and which might be a regressing form of adamantinoma specific in childhood.

Ameloblastoma

[Tuberculosis of the hand].

Skeletal tuberculosis of the hand has become rare. The authors report a 27-year-old female patient with tuberculous osteomyelitis of the middle phalanx of the left ring finger without occupational exposure of the hand and with positive chest roentgenogram. The case shows the diagnostic difficulties of the uncommon manifestation of skeletal tuberculosis in the short tubular bones of the hand. By means of a review of the literature the differential diagnosis, the course and necessary treatment are discussed.

Adult

Flow cytometric DNA analysis of bone tumors.

Flow cytometric DNA analysis was performed in a total of 203 bone tumors, benign and malignant. In more than 80% of cases the material studied was paraffin-embedded tumor tissue, mainly from the archives of the Bone Tumor Registry of Westphalia in Münster. Compared with ethanol-fixed fresh tumor samples, the variation coefficient in DNA histograms of the stored material was increased by a factor of 1.2-1.5, which means that resolution was decreased and that, in many cases, accurate cell cycle analysis was not feasible. However, the results of cell cycle analysis in bone tumors, even if performed on optimally fixed specimens, have to be evaluated with caution and full reference to the corresponding histological slides, since these histograms are apt to show various superpositions from the inflammatory infiltrate. The assessment of DNA ploidy is unimpaired if, in agreement with most researchers today, deviations smaller than +/- 10% from the diploid standard are still defined as DNA diploid, peridiploid, or pseudodiploid. The coefficient of variation should be kept as low as possible. If it is between 10% and 15%, the near-diploid stemlines with DNA indices of 0.9 or 1.1 may be hard to delineate. On account of the particularly marked regressive changes, the resolution of DNA histograms was most strongly impaired in chondromatous tumors, whereas it was mostly excellent in highly cellular viable tumor tissue, such as that from Ewing's sarcoma or osteoblastoma. On the whole, there was a distinct correlation between DNA ploidy and the biological behavior of bone tumors (Table 8). The highest rates of DNA aneuploidy were found in highly malignant OSs (18/21) and FSs (14/16), thus reflecting their poor prognosis. Of six juxtacortical OSs, three well-differentiated parosteal OSs and two periosteal OSs were DNA diploid, whereas one highly malignant surface OS and five highly malignant extraskeletal OSs, all DNA aneuploid, corresponded fully to the medullary OSs. Judging by preliminary results, adjuvant preoperative chemotherapy (COSS 80/82: Bösing et al. 1987) may reduce the rate of DNA aneuploidy and, consequently, of stem cell heterogeneity in general. A selective destruction of those stemlines that respond particularly to chemotherapy appears probable. In contrast to their high malignancy, Ewing's sarcomas showed an unexpectedly low proportion of DNA aneuploid stemlines (14/24). The comparatively favorable prognosis of MFH of bone is reflected in a lower rate of aneuploidies (2/10), which is also rather low (probably too low) when compared to our own data from soft tissue MFH (9/19).(ABSTRACT TRUNCATED AT 400 WORDS)

Bone Neoplasms

Immunohistochemical investigation of chordomas: histogenetic and differential diagnostic aspects.

Chordomas are rare tumors of neuroectodermal origin and often show a very heterogeneous histological picture. In a combined histochemical and immunohistochemical study of 32 chordomas collected in the Bone Tumor Registry of Westphalia we were able to show that the immunoreactivity of the cells in both chordoma and notochordal structures are in close relationship with the extracellular matrix and depends more on the metabolic activity of these cells than on the origin of the cells of the neuroectoderm. All tumor cells show a bimodal immunoreaction with cytokeratin and vimentin, as well as a strong immunoreaction with the oncofetal markers CEA and AFP. The differentiation of chordomas from other malignant tumors, mainly the myxoid variant of chondrosarcoma, may cause major difficulties, especially if only a little biopsy material is available. Here we can see that in tumors with bimodal immunoexpression of vimentin and cytokeratin, as can be found in chordomas, the further use of antibodies offers a reliable differential diagnostic tool. The positive reaction of chordomas with all epithelial tumor markers offers a clear differentiation from chondrosarcomas, which, unlike chordomas, do not express cytokeratin. The identification of a marker profile by employing common antisera is of major value in the differentiation of chordoma from other epithelial or mesenchymal tumors.

Adult