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[Clinicopathological analysis, immunohistochemical study and electron microscopic observation of chondroblastoma of bone].

A series of 73 cases of chondroblastoma of bone, a rare benign tumor, was subjected to clinicopathological analysis, 50 cases of it were studied by immunohistochemistry and affinity histochemistry and 3 cases were observed by electron microscopy. In this series, chondroblastomas were found nearly in patients of all age groups, with the peak incidence occurring in the second decade of life. The lesions were more commonly located in long bone metaphyseal portion, especially in the upper end of femur and the head and neck of femur. It was easily misdiagnosed as giant cell tumor of bone, chondroma, and osteochondroma by clinic and radiography. There was gradual transition from a few chondroblasts to a small focus of clear cells in 3 cases, therefore, chondroblastoma may transform into clear cell chondrosarcoma by anaplastic change of chondroblast, which is one of malignant chondroblastomas. Our immunohistochemistry and affinity histochemistry results supported the point of view of chondroblast originated from epiphyseal chondrocytes. Immunohistochemical assessment of S-100 protein may be one useful parameter for differentiating chondroblastoma from giant cell tumor of bone and aneurysmal bone cyst ultrastructurally, chondroblasts had characteristic lobulated nuclei, compact zones under nuclear membrane, and microvilli on cells surface. In most cells, the Golgi apparatus and RER were inconspicuous without.

Adolescent↗

[Diagnosis and treatment of secondary bone cysts].

The article reported 17 cases of confirmed or suspected secondary bone cyst originating from cystic degeneration of pre-existed tumor or tumor-like lesions. Twelve cases were diagnosed as solitary bone cysts pathologically after operation, but their clinical or radiological manifestations were not in keeping with the pathological findings. Among them aggressive or malignant tumors appeared in 6 cases during follow-up and no primary lesions could be found in other 6 cases. The macroscopic features of the lesion of other 5 cases were very similar to a solitary bone cyst, nevertheless, primary tumors were found histologically. The authors held that when dealing with a cystic lesion of bone, it was important, but sometimes difficult to differentiate a solitary bone cyst from a secondary one, and suggested that if the latter was suspected, injection therapy should not be used, but more radical procedures should be taken for treatment instead and the patient should be observed closely for a longer period.

Adolescent↗

[Diagnosis and treatment of primary malignant fibrous histiocytoma of bone].

Eleven cases of primary malignant fibrous histiocytoma of bone treated surgically were reported. There were 7 males and 4 females, with age ranged from 29 to 50 years (mean age 35.3 Yrs). The sites of involvement were femur (3 cases), tibia (3), humerus (2), radius (1) temporal bone, (1) and maxilla (1). The prominent findings in these cases were pain and swelling associated with regional mass. Differential diagnosis should be made from giant cell tumor, fibrosarcoma, osteosarcoma reticulum cell sarcoma and osteomyelitis. We in order to improve survival rate, think the ablative surgery should be the therapy of choice with post operational adjuvant chemotherapy. The five-year survival was 44% in these cases.

Adult↗

[Capsular retraction of the hip].

The diagnosis of capsular retraction of the hip is based on measurement of the capacity of the joint cavity on arthrography: it is reduced by at least 25% (normally 15 ml +/- 2 ml). The opaque area is only visible reduced in the more severe forms with a capacity of 5 ml or less. The arthrographic image is therefore not the key to the diagnosis. The major clinical sign is restriction of joint movement, especially in abduction and rotation. Secondary, "surgical" capsular retraction of the hip is the most common form. It is associated with synovial chondromatosis in more than one half of cases. The mean capacity is 6.8 ml (range: 0 to 12 ml). Irreducible flexion deformity and limitation of movement are of variable severity. Capsulectomy must be combined with joint debridement (systematically including the depth of the socket). "Medical" capsular retraction of the hip is the rarest form. It may be either idiopathic or secondary to diabetes or chronic barbiturate abuse. It is subacute and resolves within several months to two years. Fluoroscopic intra-articular injection of corticosteroids, repeated as required by pain, constitutes the best treatment.

Acetabulum↗

Angiography in giant cell tumors.

Angiography in 9 cases of giant cell tumors of bone is described. All cases had a greatly increased vascularity of the lesion, with concomitant increase in the diameter of the supplying arteries and draining veins. The arterial branching on the surface as well as within the tumor seemed specific of giant cell tumors. Arteriovenous shunting was marked. There was a marked, long-lasting, non-homogeneous tumor stain. Examination with angiotensin enhanced the pathologic findings. The angiographic appearance, often simulating malignancy, combined with the appearance of the skeletal films, make the diagnosis of giant cell tumor almost certain. Difficulties will be expereinced in differentiating giant cell tumors from metastatic deposits, especially in the spine.

Adolescent↗

[A clinico-pathologic study of primary malignant fibrous histiocytoma of bone].

Primary malignant fibrous histiocytoma of bone (PBMFH) is a rare tumor. In this series 11 cases of PBMFH (7 men, 4 women, mean age 39.6 years, range 15-62) were reported. They were studied by using morphology, immuno-histochemistry combined with the relevant clinical materials. It showed that most of the patients complained of local pain as the first symptom, and it generally arose in proximal or distal end of extremities. Roentgenographically, it mainly displayed a lytic destruction of bone. Like soft tissue malignant fibrous histiocytoma, it was composed of a mixture of plump fibroblast-like cells and large round or polymorphic histiocyte-like cells and usually showed a storiform pattern. But there were no myxoid and inflammatory subtypes encountered in these cases, except the storiform-pleomorphic type (9 cases) and giant cell type (2 cases). So it appears to have some difference between the subtypes of MFH of bone and soft tissue. Since the histomorphology and immunohistochemical reaction are less characteristic, especially with the morphological pleomorphism and marked cellular heterogeneity, diagnosis of PBMFH must depend on multifacet observation and comprehensive assessment.

Adolescent↗

Genuine giant-cell tumour of bone; a combined cytological, histopathological and ultrastructural study.

Three patients with a giant-cell tumour of bone were studied clinically, roentgenologically and morphologically, using cytological, histopathological and electron microscopic methods. The tumours were composed of giant cells possessing a great number of mitochondria and stromal cells exhibiting prominent endoplasmic reticulum and Golgi complex. Mitotic activity was only found among the stromal cells. Autophagic vacuoles, lysosomal bodies and degenerative changes of varying severity were found mainly or exclusively among the giant cells, which may indicate that these cells represent an involutionary form of tumour cell. The giant-cell tumours of bone are believed to arise from undifferentiated cells of the bone marrow. Fine-needle aspiration biopsy represents a valuable complement to histopathological examination in the identification of giant cell lesions and diagnosis of genuine cell tumours of bone.

Adult↗

Bone neoplasm.

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Animals↗

Cortical metastatic lesions of the appendicular skeleton from tumors of known primary origin.

BACKGROUND AND OBJECTIVES: Metastatic disease represents the most common neoplastic process involving bone. Recently, a small subset of cortical based metastatic lesions has been identified. We attempted to delineate the incidence, origin, location, and possible significance of these lesions within an orthopaedic patient population. METHODS: A chart and radiographic review of patients treated for metastatic disease to bone over a 17-year period was performed. Inclusion criteria for lesions were as follows: 1) an appendicular skeletal site, 2) histopathologic confirmation of origin, and 3) presence within a patient diagnosed with a single, known neoplastic process. The lesions were classified as either cortical or medullary based. RESULTS: Eighty-three lesions (70 patients) satisfied inclusion criteria. Most lesions were of pulmonary (26), breast (22), renal (16), or prostatic (8) tumor origin. Eighteen lesions (22%) from 15 patients were identified as cortical and represented initial presentation in 7 patients. These lesions were of pulmonary (11), renal (5), and breast (2) tumor origin. CONCLUSIONS: Cortical based metastases within the appendicular skeleton may occur more frequently than previously expected. While tumors of pulmonary and renal origin accounted for 42 of the 83 (51%) appendicular lesions, they were responsible for 16 of the 18 (89%) cortical metastases. This preponderance of pulmonary and renal metastases to the cortex is consistent with previously published reports. Our findings may be of value when diagnosing and treating patients whose initial presentation is a cortically based lesion.

Adenocarcinoma↗

Rothmund-Thomson syndrome and osteosarcoma.

The Rothmund-Thomson syndrome (RTS), also called poikiloderma congenitale is a rare autosomal recessive disease first described in 1868. This syndrome includes most frequently seen skin lesions (atrophy, telangiectases, pigmentation), cataracts and bone defects (dysostosis, dysplasia). Some authors describe an association with malignancy. We report three cases of Rothmund-Thomson syndrome associated with osteosarcoma. After cutaneous epithelioma, osteosarcoma is the most frequent malignancy. Thus, patients with RTS need a careful survey. The treatment did not differ from sporadic osteosarcoma. Chemosensitivity and toxicity are also not different.

Bone Neoplasms↗

Imaging assessment of the responses of osteosarcoma patients to preoperative chemotherapy: angiography compared with thallium-201 scintigraphy.

BACKGROUND: Assessment of the responses of osteosarcoma patients to preoperative chemotherapy is of clinical importance. The purpose of this study was to estimate the accuracy of angiography and thallium-201 scintigraphy, compared with histology, in assessing the responses of patients with osteosarcoma to preoperative chemotherapy. METHODS: Nineteen patients with osteosarcoma who were diagnosed between 1992 and 1997 were studied. The findings of angiography and thallium-201 scintigraphy before and after preoperative chemotherapy were compared with the percentage of necrosis of tumor cells and the response grade as determined histologically. Quantitative analysis of the isotopic uptake by thallium-201 scintigraphy before and after chemotherapy, defined as the alteration ratio, was correlated with the percentage of tumor necrosis. RESULTS: Angiography yielded a sensitivity of 88%, a specificity of 73%, and a predictive accuracy of positive test of 70%, whereas thallium-201 scintigraphy achieved 88%, 100%, and 100%, respectively. Both angiographic and scintigraphic assessment showed a significant correlation with response grade as determined histologically (P < 0.05 and P < 0.0003, respectively). The alteration ratio of thallium-201 scintigraphy showed a strong, highly significant correlation with the percentage of tumor necrosis (P < 0.0001). CONCLUSIONS: A change in the tumor uptake of thallium-201 scintigraphy after preoperative chemotherapy can predict the tumor necrosis in osteosarcoma precisely. Thallium scintigraphy is a noninvasive technique and seems to be more useful than angiography in assessing the response of osteosarcoma to preoperative chemotherapy.

Adolescent↗

A solitary functioning osseous metastasis from a malignant pheochromocytoma of the organ of Zuckerkandl.

A case of a malignant phenochromocytoma of the organ of Zuckerkandl is reported. There was a solitary functional metastasis in the left femur, which was treated by hip disarticulation after removal of the primary. The patient is asymptomatic and free of further metastases after 3 years. Other reported cases of solitary metastases from malignant pheochromocytomas are cited. These occurred most frequently in the skeleton system, which is the most common site of distant metastases in malignant pheochromocytoma. The reported case and the review of the literature indicate that solitary or limited metastatic lesions should be treated surgically by partial or, when possible, complete resection.

Adolescent↗

Primary malignant fibrous histiocytoma of bone: report of six cases with ultrastructural study and analysis of the literature.

Six cases of malignant fibrous histiocytoma (MFH) primary in bone were studied clinicopathologically with ultrastructural study of three. The histologic differentiation of MFH from osteosarcoma and from fibrosarcoma is discussed. Two of the six cases had highly malignant tumors histologically designated as Grade III and IV; one died with diffuse metastatic disease eight months after diagnosis and the second developed pulmonary metastases within ten months. Review of the literature revealed that 45 of the total 119 reported cases had been followed for less than five years; these cases were considered unevaluable for assessing biologic behavior. The remaining 74 cases, followed for more than five years or until death, showed a 36.5% five-year survival rate. All but one of the cases which developed metastasis within five years did so within the first two years. It is therefore concluded that MFH of bone is a rapidly metastasizing tumor with a relatively low five-year survival rate. Paradoxically, 13 of these 74 cases (17.6%) survived between five and 38 years with no evidence of disease. This is comparable to the long-term survival rate in osteosarcoma. The five-year survival rate is also comparable to that of fibrosarcoma of bone.

Adult↗

The fibroma-like variant of epithelioid sarcoma. A fibrohistiocytic/myoid cell lesion often confused with benign and malignant spindle cell tumors.

Five cases of a previously undescribed variant of epithelioid sarcoma are presented. This variant differs from the usual lesion in its absence of the typical necrobiotic nodular epithelioid pattern. It is instead composed of deceptively bland fibrohistiocytic and myoid cells arranged in a fibroma-like or dermatofibroma-like pattern with an affinity for osseous involvement. The clinical presentation, ultrastructural features, and presence of vimentin and low molecular weight keratin within the tumor cells justifies their designation as an epithelioid sarcoma variant.

Adolescent↗