Case report 741: Chondromyxoid fibroma of T2.
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Adult↗
Biomedical subjects
A Roessner
Publications and source records attributed to A Roessner.
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↗
(Sub)periosteal Ewing's sarcoma of bone.
Ewing's sarcoma is a small malignant round-cell tumour that arises from mesenchymal cells, predominantly in the medullary cavity of bone. In exceptional cases it originates in the soft tissues and subsequently invades the underlying bone. A (sub) periosteal origin of Ewing's sarcoma is a very rare condition: only a few cases have been published so far. Three cases of (sub)periosteal Ewing's sarcoma, having received neoadjuvant chemotherapy and radiation therapy as well as wide excision, are reported.
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↗
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↗
Osteonectin in osteosarcomas: a marker for differential diagnosis and/or prognosis?
Seventy-five osteosarcomas at various grades of histologic differentiation were investigated for evidence of osteonectin. According to the results of the study, osteonectin was present in all osteosarcomas. An association between the intensity of the osteonectin antibody reaction and prognosis could not be established. Evidence of osteonectin was also found in other bone tumors. Osteonectin is therefore unsuitable for differential diagnosis, cannot be regarded as a bone specific protein and has not prognostic value.
Biomarkers, Tumor↗
[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↗
Distribution patterns of apolipoproteins A1, A2, and B in the wall of atherosclerotic vessels.
Atherosclerotic vessels were analysed histochemically for distribution, quantity, and composition of apolipoprotein (Apo) types in the vascular wall. The specimens comprised all stages of atherosclerosis, from very discrete intimal changes to complicated lesions. The vessel specimens were marked with antibodies against human Apo A1, A2, and B. Apo A1 can be demonstrated in even the earliest stage of atherosclerosis, and increases with the progression of the disease. In the initial stage, Apo A1 is found first in lumen-adjacent layers of the intima, and is evident in deeper layers of the wall as the disease progresses. Arteries of muscular type show accumulation of Apo in an earlier stage (or in greater quantity at the same stage) than arteries of elastic type. At all stages, the amount of Apo A1 always exceeds that of A2 and B. In the intima, Apo B is higher than Apo A2, the media contains hardly any Apo B, and the adventitia has less B than A2. Within the intimal layer, Apo A1 and A2 are found in an intracellular (mainly in foam cells) or in an extracellular location, according to the stage of atherosclerosis. Apo B is almost exclusively extracellular; only cases of advanced atherosclerosis show some intracellular localization (mostly in foam cells), visualized as electron dense lamellar organelles, probably of lysosomal origin. In the media, Apo A1 and A2 are accumulated in intracellular deposits, whereas the extracellular storage of Apo A1, A2 and B is observed only in cases with the most severe damage. Our investigations suggest that the accumulation of apolipoproteins in the vascular wall is affected not only by insudation from the plasma, but also by neosynthesis and/or metabolism by locally derived cells or cells immigrating in the process of atherosclerosis. The presence of Apo A1 and A2 in the vessel wall is now documented, and their role at this site apparently differs from that in the plasma.
Aorta↗
Histogenesis of clear cell chondrosarcoma. An immunohistochemical study with osteonectin, a non-collagenous structure protein.
The histogenesis of clear cell chondrosarcoma is still unclear: Apart from typical clear cell tumor areas, extensive production of woven bone formation suggests within the clear cell cartilagenous stroma is an intriguing phenomenon. Three cases of clear cell chondrosarcoma documented in the Bone Tumor Registry of Westphalia were examined for their patterns of osteonectin expression, and compared with other bone tumors of either osseous or cartilaginous origin, and with normal cartilage tissue. Found predominantly in osseous structures, the protein osteonectin takes part in the formation of new bone. The three clear cell chondrosarcomas showed a strong immunoexpression of osteonectin in clear cell, chondroid and in osseous tumor areas. Similarly, evidence of osteonectin was also found in osteoblastic and in chondroblastic osteosarcomas as well as in osteoblastomas. In contrast, osteonectin could not be demonstrated in the chondrosarcomas and mesenchymal chondrosarcomas from our registry that were analysed for comparison, and was found only minimally in the fibroblastic areas of dedifferentiated chondrosarcomas. The chondroblastic tumor components were always negative. There was no immunoexpression of osteonectin either in fetal or adult intervertebral disc tissue. The present immunohistochemical study of osteonectin has distinctly separated clear cell chondrosarcoma from the other variants of chondrosarcoma, and aptly verified the specificity of this entity. Moreover, the study would call for further histogenetic evaluation of clear cell chondrosarcoma, since the pattern of osteonectin expression in that tumor seems to indicate an osteogenic rather than a chondrogenic origin.
Adenocarcinoma↗
Case report 669: Osteonecrosis of bone associated with intraarterial therapy using cisplatin.
A case is presented of an 18-year-old women who suffered pain and swelling after intraarterial cisplatin chemotherapy for osteosarcoma of the left fibula. Radiological studies showed minimal changes at the metadiaphyseal portion of the left tibia. Bone scans and MR studies were highly suggestive of necrosis of bone and muscle of the proximal end of the tibia. Multiple biopsies of the tibia at the time of excision and 2 years later showed histological features consistent with a slowly healing osteonecrosis of bone. Review of the literature reported transient pain and swelling after intraarterial chemotherapy but did not demonstrate the association with necrosis of bone and muscle. It can be anticipated that as intraarterial chemotherapy of malignant tumors of bone and soft tissues becomes more widely used, these complications will be observed more frequently.
Adolescent↗
Joint occurrence of collagen mRNA containing cells and macrophages in human atherosclerotic vessels.
Cells with enhanced levels of collagen type I and III mRNA were identified and localized in frozen tissue sections from samples of human atherosclerotic renal and common iliac arteries by in situ hybridization using complementary 35S-labeled RNA probes. Serial sections were immunohistochemically stained for smooth muscle cells, monocytes, and differentiated macrophages. In the fibromuscular intima and in the fibrous plaques, cells with enhanced transcriptional activity were located mainly in the vicinity of differentiated macrophages. In three patients, lack of enhanced transcriptional activity in a proliferated intima was connected with complete absence of macrophages, thus indicating a quiescent stage of atherosclerosis. Immunohistochemical staining of serial sections for smooth muscle cells (SMC) revealed the presence of this cell type throughout the proliferated intima in atherosclerotic arteries including those areas in which enhanced collagen mRNAs were detected. The present results support the idea that macrophages play an important role in the activation of collagen synthesis in SMC of atherosclerotic vessel walls.
Adolescent↗
Immunohistochemical double labeling of macrophages, smooth muscle cells, and apolipoprotein E in the atherosclerotic plaque.
To find out which cell types in the atherosclerotic plaque are actively involved in apolipoprotein-secretion, we stained tissue samples from the ascending aorta of patients undergoing coronary bypass surgery. We used monoclonal antibodies against macrophages, smooth muscle cells and apolipoprotein E. The present in situ-study of the atherosclerotic plaque has shown that apolipoprotein synthesis is predominantly secreted by macrophages transformed into foam cells. Besides this, there are obviously some smooth muscle cells equally transformed to foam cells, which also carry apolipoprotein E in their cytoplasm. Our data suggest that both cell types in the atherosclerotic plaque are actively involved in apolipoprotein secretion.
Apolipoproteins E↗
Apolipoproteins and immunohistological differentiation of cells in the arterial wall of kidneys in transplant arteriopathy. Morphological parallels with atherosclerosis.
22 nephrectomy specimens of renal allografts in chronic rejection after periods between 3 and 96 months, were studied immunohistologically. Various cell types in the arterial wall were characterized with antibodies specific against different cells of the mononuclear phagocyte system, against smooth muscle cells, and against differentiating lymphoid cells. In addition, the metabolism of lipoproteins was investigated using appropriate antibodies against several apolipoproteins. Subendothelial plaques of foam cells were found to consist of macrophages in foamy transformation. At the stage of intimal fibrosis the smooth muscle cells are more prominent. Lymphatic infiltration consists almost exclusively of T-lymphocytes. Apolipoprotein analysis reveals deposits of Apo A1, A2 and B1, most of them extracellular. According to these results, it is not only immunologic factors that are involved in arterial wall reactions during chronic transplant arteriopathy, but disorders of the lipoprotein metabolism--probably due to endothelial dysfunction--are also playing an important role like in atherosclerosis.
Adolescent↗
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↗
Parosteal osteosarcoma. 2-23-year follow-up of 33 patients.
In a study of the working group for bone tumors of the German Orthopedic Society, 33 patients with a histologically confirmed parosteal osteosarcoma at reexamination underwent clinical and radiographic follow-up. Local recurrence occurred in all the cases after intralesional surgery and in 4 of 8 cases after marginal excision. The grade of differentiation was decisive for the prognosis. Despite intralesional surgery, the prognosis for Grade 1 tumors was good. Metastases developed in 1 of 23 patients with a Grade 1 tumor and in 4 of 9 patients with a Grade 2 tumor. The single patient with a Grade 3 tumor was treated with adjuvant chemotherapy and was free of disease after 5 years.
Adolescent↗
The impact of osteonectin for differential diagnosis of bone tumors. An immunohistochemical approach.
75 osteosarcoma at various grades of histologic differentiation, including chondroblastic and small cell variants, and 5 fibrosarcomas of bone, 5 Ewing's sarcomas, 5 malignant fibrous histiocytomas of bone, 8 chondrosarcomas, and 2 dedifferentiated chondrosarcomas, were investigated immunohistochemically for evidence of osteonectin. According to the results of our study, osteonectin is present in all osteosarcomas, with special topographic preponderance in the osteoblastic and chondroblastic variants. Evidence of osteonectin was also found in all other bone tumors we had analysed so far. In chondrosarcomas, positive reactions appeared only in the vicinity of trabeculae and in dedifferentiated areas. Thus, osteonectin cannot be regarded as a bone-specific protein. Although a high affinity for the osseous matrix is one of its undoubted features, it is therefore unsuitable for differential diagnostic purposes.
Bone Neoplasms↗