Ewing's sarcoma of humerus with epithelial differentiation.
We report an unusual case of Ewing's sarcoma of the humerus with epithelial differentiation. This rare finding has recently been documented in this type of tumor.
Biomedical subjects
Publications and source records attributed to G C Steiner.
We report an unusual case of Ewing's sarcoma of the humerus with epithelial differentiation. This rare finding has recently been documented in this type of tumor.
This article describes 11 cases of myxoid chondrosarcoma (MCS), with 10 arising in soft tissues and one developing in bone. Most of the tumors (six) were located in the lower extremities. Two lesions developed in the fingers, a previously unreported location for MCS. Four cases showed secondary bone destruction, which is a rare feature of this tumor. S100 protein was expressed by tumor cells in all the specimens. Four out of eight tumors studied by electron microscopy contained intracisternal microtubular structures. Two tumors showed areas of spindle cell proliferation that merged with the areas of typical myxoid pattern. The cells in these areas had fibroblastic/myofibroblastic features by electron microscopy and were found to express cytokeratin by immunohistochemistry. The concomitant expression of cytokeratin and S100 protein in the spindle cells suggests that they represent a less differentiated cartilaginous component with unusual features. The clinical significance of the presence of such spindle cell areas presently remains unknown. Although myxoid chondrosarcoma is a slow-growing tumor, it has a high potential for metastases. Four of 11 patients in this series developed metastases.
Osteosarcoma is a malignant, mesenchymal, osteoid, and bone-forming tumor [1-3]. In most cases, typical radiographic features clearly illustrate the aggressive bone-forming nature of the lesion. These features include long-bone metaphyseal location, mixed areas of lysis and sclerosis, cortical destruction, periosteal new bone, and soft-tissue mass. However, numerous factors contribute to misleading radiographic patterns of osteosarcoma. They include histologic low-grade, lytic, or minimally sclerotic lesions, early detection, confinement to the intramedullary canal, benign-appearing periosteal reaction, rare intraosseous locations (subchondral, diaphyseal, intracortical), and rare skeletal sites (e.g., soft tissues, skull, ribs, tarsal bones). Secondary osteosarcomas, such as those arising from infarcts or fibrous dysplasia, may also produce a confusing radiologic picture. The purpose of this pictorial essay is to illustrate cases of osteosarcoma that proved to be difficult diagnostic dilemmas because of their subtle, rare, or misleading plain film features.
Five unusual cases of cystic angiomatosis of bone which presented with the radiologic appearance of osteoblastic lesions are reported. Three patients were female (ages 37, 41, and 65 years) and two were male (ages 24 and 66 years). Although cystic angiomatosis of bone usually produces widespread osteolytic lesions with a honeycombed appearance in the skeletal system, multiple osteoblastic lesions mimicking metastatic osteoblastic carcinoma are sometimes seen. This radiological presentation has not been well emphasized in previous reports. Histologically, in addition to the angiomatous lesions, both mature thickened lamellar bone trabeculae and immature trabeculae of woven bone were found. In one of our patients, increasing density of the osteoblastic lesions was noted over time. One previous study has suggested that the age of the lesions of cystic angiomatosis is related to radiographic density. It is important to recognize this uncommon variant of cystic angiomatosis and to include this entity among the radiologic differential diagnoses when multiple osteoblastic lesions are encountered.
Intracapsular and paraarticular chondromas are rare benign tumors. The authors report four such lesions, three of which occurred about the knee and one at the hip. Radiographs showed calcified soft-tissue masses in the infrapatellar region of the knee in three patients. In the hip, the lesion was not visible on conventional radiographs. Three lesions originated from the joint capsule and one was extracapsular. These lesions should be differentiated from synovial chondromatosis and other calcified lesions occurring about the joints. After local excision, the tumors usually do not recur.
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Pathological examination of the resected femoral heads from approximately 2000 total hip replacement operations carried out at the Hospital for Joint Diseases from 1984 to 1989 identified the presence of osteonecrosis in 345 patients (377 femoral heads). In 232 patients the osteonecrosis, referred to as "idiopathic," had occurred in the absence of a subcapital fracture. The present paper describes the pathology of the necrotic lesions in these 232 patients. The use of undecalcified sections and microradiography provides evidence of bone marrow calcification which, at the margin of the lesion, is sufficient to influence the radiographic features of the lesion significantly. Although a subchondral fracture is an almost constant feature of osteonecrosis when it occurs in a femoral head with a normal articular cartilage, no such fracture was found in cases in which osteonecrosis had occurred in an osteoarthritic joint.
Twenty-seven cases of an unusual, poorly recognized destructive hip arthropathy with radiographic findings of rapid severe joint destruction are presented. Radiographic findings mimicked those of other disorders such as septic arthritis, rheumatoid and seronegative arthritis, primary osteonecrosis with secondary osteoarthritis, or neuropathic osteoarthropathy, but none of the patients had clinical, pathologic, or laboratory evidence of these entities. All patients underwent hip arthroplasty, and osteoarthritis was confirmed at pathologic examination. Rapid progression of hip pain and disability was a consistent clinical feature. The average duration of symptoms was 1.4 years. Radiographs obtained at various intervals before surgery (average, 18 months) in nine patients documented rapid hip destruction. Involvement was unilateral in 89% (24 of 27 cases). Twenty patients (83%) were elderly women. The authors postulate that these cases represent an uncommon, rapidly destructive subset of osteoarthritis.
Paget's disease of bone is a disease of unknown etiology. The demonstration of viral-like particles on ultrastructural examination and the putative detection of viral antibodies and nucleic acids in the tissues suggest a possible viral association. The purpose of this study was to search for nucleic acid sequences homologous to measles virus using the recently described reverse transcriptase (RT) polymerase chain reaction (PCR) in situ hybridization (ISH) technique. After performing RT PCR ISH utilizing primers specific for the nucleocapsid region of the measles virus, an intense signal was evident in most measles-infected HeLa cells compared with a weak signal in few of these cells using standard cDNA-RNA ISH analysis. Amplified measles nucleic acid was detected in tissue from a patient who died of measles infection and was not detected in any of the 11 cases of Paget's disease of bone studied or in a giant cell tumor of bone that had tubuloreticular inclusions on electron microscopy. Therefore, these data suggest that infection by the measles virus is not associated with Paget's disease of bone.
The Swarm rat chondrosarcoma has been the subject of extensive biochemical studies. However, to our knowledge, there are no previous reports in the literature on transplantation of this tumor into bone. This article describes the natural history of the tumor when implanted into the bone of the rat, and correlates its histological growth pattern with its radiological appearance. Our results showed that the tumor grows slowly in the bone. The rate of intramedullary growth, however, was variable and was not the same in all the animals. Its growth pattern resembles human chondrosarcoma, with extensive invasion of the marrow and cortex. In the first few weeks after implantation, the only radiological changes noted were mild medullary radiopacities. At a later stage, 12-14 weeks postimplantation, as the tumor infiltrated the bone, significant radiological abnormalities were observed in the medullary cavity and cortex. Periosteal reaction was seen after the tumor invaded the cortex with the production of a soft-tissue mass. Distant dissemination was rare; only 1 of 24 rats developed pulmonary metastases. The Swarm rat chondrosarcoma is a well-differentiated malignant tumor that histologically resembles well-differentiated human chondrosarcoma. Transplanted into bone, it may be useful as an experimental model for comparative studies with human chondrosarcoma.
Macrodactyly is an unusual congenital anomaly in which there is hamartomatous proliferation of the soft tissue of the affected digit. It is usually present at birth or recognized during adolescence. Eleven cases are presented. All showed abnormal accumulation of adipose tissue in the affected digits. In five cases, prominent endoneural and perineural fibrosis was noted. Two cases also demonstrated focal neural and vascular proliferation. This is an unusual finding in macrodactyly and may be a part of the abnormal hamartomatous proliferation that is observed in this condition. None of the patients had clinical evidence of neurofibromatosis. One patient had the Proteus syndrome.
A case of a giant cell tumor of the right proximal femur that developed malignant change following surgical treatment ("secondary" malignant giant cell tumor) is presented. The patient had a cystic lesion that was initially interpreted as an aneurysmal bone cyst. A local recurrence six months later demonstrated the histological features of typical giant cell tumor. A further recurrence 22 months after the original treatment showed histological malignancy. Death was due to massive local recurrence and to extension of the malignancy to the pleura.
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A report of two patients in which a soft tissue mass, initially regarded as a malignant tumor, was shown to be the result of calcium pyrophosphate deposition disease. The first case, a woman aged 71 years, presented with a mass involving the right fifth finger. In the second case, also a woman aged 71 years, the lesion involved the tissues adjacent to the right hip. Each lesion consisted of a mass of highly cellular tissue containing deposits of calcium pyrophosphate dihydrate crystals. The clinical, radiological, and pathological features of the two cases are compared with those of seven similar cases reported in the literature.
The presence and distribution of type II collagen was studied in 36 cartilage and cartilage-related tumors, including five osteosarcomas and one chordoma. A monoclonal antibody prepared from chicken type II collagen was used with paraffin sections, employing the ABC (avidin biotinylated horseradish peroxidase complex) peroxidase technique. Fetal cartilage and fracture callus were used as control materials. Type II collagen was present in the matrix of all the cartilage tumors. The reaction was strongest in areas of well-differentiated cartilage and weakest in the poorly differentiated tissue of high-grade chondrosarcomas. Areas of mineralization or ossification, and areas of eosinophilic, fibrous, or degenerated cartilage gave a negative reaction.
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An immunohistochemical study of six giant cell tumors of bone and eight related lesions (aneurysmal bone cyst, fibrous histiocytoma, and giant cell tumor of tendon sheath) was performed using a panel of monoclonal antibodies directed to the Ia and monocyte-macrophage lineage antigens. In all types of lesion, osteoclastlike multinucleate giant cells were negative for both types of antigen, but a proportion of mononuclear cells gave positive reactions. While the possibility that these cells are reactive cannot be excluded, in giant cell tumor and malignant fibrous histiocytoma, their frequency and their morphologic similarity to the rest of the tissue suggest that they may be an intrinsic part of the neoplasm. This finding is consistent with the presumed fibrohistiocytic nature of these tumors.