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Metastatic chondroblastoma. Report of a fatal case with a review of the literature on atypical, aggressive, and malignant chondroblastoma.

A boy with metastatic and fatal chondroblastoma is presented. Unlike previously published examples of metastatic chondroblastoma, these metastases developed before any operative manipulation of the primary tumor. The histologic characteristics of the primary, metastatic, and locally recurrent tumors were those of a conventional chondroblastoma. A review of published cases of atypical, aggressive, and malignant chondroblastoma is presented with current follow-up information. Although some metastatic chondroblastomas may result from operative manipulation of the primary tumor and are clinically benign, other histologically benign chondroblastomas exist that are capable of pursuing a malignant course. The authors designate these as malignant chondroblastomas. No histologic criteria exist for the separation of these tumors.

Antineoplastic Combined Chemotherapy Protocols↗

Chondroblastoma and clear cell chondrosarcoma: radiological and MRI characteristics with histopathological correlation.

OBJECTIVE: To analyze and compare the radiological and magnetic resonance imaging (MRI) appearances of chondroblastoma and clear cell chondrosarcoma with histopathological correlation. DESIGN AND PATIENTS: Twelve patients with histologically proven chondroblastoma and of another four patients with clear cell chondrosarcoma were investigated by radiographs and MRI (T1-, T2-weighted sequences, intravenous gadolinium application). Additionally, the clinical and radiologic data of seven cases of clear cell chondrosarcoma without available MRI were considered. The localization, calcification of tumor matrix, periosteal reaction, cortical bone and patterns of bone destruction were analyzed according to the Lodwick radiological grading system (LRGS). The signal intensity on T1- and T2-weighted sequences, characteristics of contrast enhancement, associated bone marrow edema, soft tissue reaction and joint involvement were evaluated. Histopathological specimens were available in all cases. RESULTS: The age of patients with chondroblastoma (range 15-59 years, mean 22.3 years) was lower than that of those with clear cell chondrosarcoma (range 19-61 years, mean 36.6 years), and the lesions were smaller in the chondroblastoma group (range 1-4 cm, mean 2.3 cm) than in patients with clear cell chondrosarcoma (range 3-7.5 cm, mean 5.2 cm). The chondroblastomas were more confined to the epiphysis (10/12) than the clear cell chondrosarcomas. All chondroblastomas and clear cell chondrosarcomas except one were classified as grade 1A or 1B according to the LRGS; one clear cell chondrosarcoma was judged as grade 2. Signal intensity of the tumors on MRI was very heterogeneous in both groups. High signal intensity on T2-weighted MR images in chondroblastoma mostly corresponded to areas with aneurysmal bone cyst components and in clear cell chondrosarcoma to islands of hyaline cartilage. Contrast enhancement occurred in all tumors and tended to be more intense with clear cell chondrosarcoma. Chondroblastoma was more frequently associated with bone marrow edema (11/12), periosteal reaction (10/12), soft tissue reaction (7/12) and synovitis (3/12). CONCLUSION: Chondroblastoma occurs in younger patients, is smaller than clear cell chondrosarcoma and is more confined to the epiphysis. The overlap of signal intensity and contrast enhancement patterns does not allow a reliable differentiation of the two tumors by MRI. Chondroblastomas are typically associated with bone marrow edema, periosteal reaction and soft tissue reaction.

Adolescent↗

The frequency and diagnostic significance of periostitis in chondroblastoma.

A study was performed to determine the frequency of periosteal reaction associated with chondroblastoma, to investigate the underlying pathophysiology of the periosteal reaction, and to postulate the clinical importance of this radiographic observation. Two hundred fourteen histologically proved chondroblastomas were reviewed and observed for the presence or absence of periosteal reaction and for radiographic changes that might explain the cause of the periosteal reaction. A similar review was performed on 30 other epiphyseally centered lesions of various causes. A distinctive thick, solid periosteal reaction distal to the chondroblastoma was present in 47% of all chondroblastomas and 57% of chondroblastomas present in long bones (excluding the greater trochanter). No periosteal reaction was observed in any of the 30 epiphyseally centered lesions of other causes. When available for observation, plain films showed inflammatory changes in the joint surrounding the chondroblastoma, bone scintigraphy showed tracer uptake similar to that observed in inflammatory lesions and aggressive neoplasms, and MR images showed change in the marrow surrounding the chondroblastoma consistent with edema. This suggests an inflammatory reaction to the chondroblastoma, rather than mechanical stress across a weakened epiphysis, as the cause of the periostitis. We conclude that frequently the chondroblastoma produces a distinctive thick solid or layered periosteal response distant from the lesion along the diametaphyseal shaft. Observation of this unique periosteal response may help to distinguish chondroblastoma from other epiphyseally centered lesions.

Adolescent↗

Fine needle aspiration cytology of chondroblastoma of bone.

Between 1979 and 1987 12 patients with chondroblastoma underwent fine needle aspiration (FNA). There were eight female and four male patients (age range, 11-35 years) with lesions of the proximal humerus (three cases), distal femur (two cases), proximal tibia (two cases), proximal femur, distal tibia, talus, navicular bone, and fifth metacarpal (one case each). The radiologic features of the tumors were not entirely typical of chondroblastoma in the majority of patients. The aspirate was diagnosed as chondroblastoma in seven cases, was considered strongly suggestive of chondroblastoma in one case, was found to be diagnosable as chondroblastoma on review in one case, and was nondiagnostic in two cases. The remaining case, which showed giant cell tumor-like areas in addition to typical chondroblastoma on histologic sections from the curettage, was interpreted as giant cell tumor on FNA. There was no case in which an aspirate was erroneously diagnosed as chondroblastoma. On FNA, chondroblastoma had three dominant cytologic components: neoplastic mononuclear cells (chondroblasts), multinucleated osteoclast-like giant cells, and chondroid matrix fragments. The chondroblasts tended to lie individually in smears creating a pebbled appearance. They most commonly had round to oval nuclei with fine, evenly distributed chromatin and distinct longitudinal grooves, but indented, lobulated, and pyknotic nuclei were also observed. Their cytoplasm was dense and opaque with rounded well-defined borders. Multinucleated osteoclast-like giant cells were randomly admixed and were indistinguishable from those seen in other bone neoplasms. Chondroid matrix stained magenta with the Diff-Quik stain and green to violet with Papanicolaou. The cytologic features of the chondroblasts are the diagnostic hallmark of chondroblastoma and may allow FNA to become a valuable preoperative technique in the management of these patients.

Adolescent↗

Chondroblastoma is an osteoid-forming, but not cartilage-forming neoplasm.

Chondroblastoma is defined as a 'benign tumour, characterized by highly cellular and relatively undifferentiated tissue composed of rounded or polygonal chondroblast-like cells' and the 'presence of cartilaginous intercellular matrix' (WHO). An extensive analysis of the extracellular matrix composition and gene expression pattern of a large series of chondroblastoma cases shows, however, that type II collagen, which is the main component of any cartilage matrix, is not expressed by the neoplastic cells of this tumour entity and is not deposited into the extracellular tumour matrix. Instead, osteoid and fibrous matrix is formed, with its typical biochemical composition. The multifocal expression of aggrecan proteoglycan in most chondroblastomas explains the bluish, pseudo-chondroid appearance of some of the matrix-rich areas of chondroblastomas. This study did not show chondroid matrix formation or chondroblastic cell differentiation in chondroblastomas, suggesting that chondroblastoma should be classified as a specific bone-forming, rather than cartilage-forming neoplasm.

Aggrecans↗

Chondroblastoma arising from a nonepiphyseal site.

Chondroblastoma is a rare, benign primary bone tumor usually involving secondary centers of enchondral ossification. The consistent epiphyseal location in the great majority of chondroblastomas signifies that the tumor may arise from an aberrant germ cell of the physeal plate. This case report describes a chondroblastoma located in a atypical nonphyseal location, namely in the right fourth metatarsal base. Cases such as these imply that the cell of origin may not exclusively be derived form the physeal plate. The clinical-radiographic significance is that historically chondroblastoma is considered an epiphyseal lesion, but rare cases such as the one reported here and those of the skull demonstrate that this is not always a characteristic of these tumors. The implication from a histogenetic standpoint is that chondroblastoma may, on rare occasions, occur in an area of an enchondrally formed bone other than adjacent to the physeal plate. We are presently investigating the histogenetic relationship of the chondroblastoma (CB) of bone to CB of soft tissue, giant-cell tumor of tendon sheath (GCT-TS), pigmented villonodular synovitis (PVNS) and chondroma of tendon sheath (chondroma of soft parts). We now have collected about 15 cases of GCT-TS and PVNS with extensive areas of chondroid and/or cartilage differentiation that cannot be distinguished from CB of bone by histologic or electron microscopic features alone. From these interesting observations we are developing the histogenetic concept that all of these lesions are interrelated to multipotential mesenchymal and/or synovial cells of the tendon sheath.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Chondroblastoma of the foot.

A total of 322 cases of chondroblastoma were referred to the Armed Forces Institute of Pathology between 1960 and 1990. Ten additional cases of chondroblastoma were treated at Walter Reed Army Medical Center between 1985 and 1993. Forty-two of these involved the foot, two of which were treated at Walter Reed Army Medical Center. Patients with chondroblastoma of the foot were male in 35 (81%) cases, with a mean age of 25.5 years, which was significantly different from the mean age of 17.3 years in patients with chondroblastoma of the long bones (P < 0.0001). Chondroblastoma of the foot is most commonly found in the posterior subchondral areas of the talus and calcaneus as well as in the calcaneal apophysis. Radiographically, the lesion was associated with an articular surface or apophyseal area in all cases and appeared radiolucent, with little to no matrix production. The margins were generally well defined. Cystic features were noted grossly and histologically in 24 (57%) specimens, a feature seen in only 21% of all chondroblastomas overall. Treatment consists of thorough curetting and bone grafting with good oncologic and functional results.

Adolescent↗

Chondroblastoma of the temporal bone: a clinicopathologic study of five cases.

Chondroblastoma is a rare benign bone tumor. It commonly affects the epiphysis of long bones during the second and third decades of life. Chondroblastoma of the temporal bone is extremely rare. We reviewed five cases of chondroblastoma arising in the temporal bone. Four cases were female and one was male. The ages ranged from 41 to 60 years (mean, 53.6 years). All cases involved the temporal bone. Three involved the left side and two the right. Chief complaints were long-standing localized pain and hearing difficulty. A sharply demarcated lobulated mass was the main radiological finding. Microscopic findings were those of chondroblastoma of usual locations. Two cases showed aneurysmal bone cyst-like areas. Immunohistochemical studies for CD34, CD99, S-100 protein and cytokeratin were performed. Tumor cells were diffusely positive for S-100 protein in three cases and weakly positive for cytokeratin in one case. CD34 and CD99 were negative in all cases. In summary, chondroblastoma of the temporal bone is rare and occurs in older age group than reported cases of chondroblastoma of the usual location in the literature.

Adult↗

[Morphologic characteristics of chondroblastoma. A retrospective study of 56 cases of the Hamburg bone tumor register].

Representing only about 1% of all primary bone tumors, chondroblastoma constitutes a very rare bone tumor entity. 56 cases of chondroblastoma, that had been collected by the Hamburg Bone Tumor Registry from 1972 to 1995, were examined histologically together with the radiological and clinical findings. In addition immunohistochemistry with antibodies against S 100, PGM1, LCA and the proliferationmarker MIB 1 was performed. The mean age was 20.4 years and male patients being the majority with a gender ratio of 2.7:1. Predominant localisation was the epiphyses of the long bones, although almost 40% of the tumors were located at untypical sites. Usually a well-circumscribed lysis could be seen on plain X-Ray examination, however partial cortical destruction could be observed in one third of the cases. Histologically characteristic was a polygonal cell component with a weblike chonroid matrix, sometimes with a plane-like appearance. 5 cases showed a distinct nuclear polymorphism making a distinction from osteosarcoma difficult. Using immunohistochemistry all tumors except for one showed positive reaction for S 100 protein. Although the histogenesis of chondroblastoma is not completely understood, morphological findings as well as the observed reactivity with the S 100 protein indicate the chondroid origin. No reactivity for PGM 1 (CD 68) or LCA could be detected. All chondroblastoma showed a low rate of proliferation, thereby being distinguishable from high malignant bone tumors. In general chondroblastoma show a benign biological behavior. Different behavior was observed in 2 cases. One relapse located in the pelvis revealed local aggressive growth while in another case in the humerus a malignant transformation had taken place.

Adolescent↗

Histology and biology of metastatic chondroblastoma. Report of a case with a review of the literature.

This case report of a metastasizing chondroblastoma with a review of the literature was undertaken to gain a better understanding of the biologic behavior of this exceedingly rare tumor and thus to facilitate its clinical management. The lung was by far the most frequent metastatic site. Thus, all 7 patients with a proven metastatic chondroblastoma recorded up to now including the present case had developed multiple pulmonary metastases. The interval between the initial diagnosis of the primary and manifestation of lung metastases proved to be long, with a mean of 8.4 years. The average survival time was at least 12.3 years. The mean interval between diagnosis of metastatic disease and death amounted to at least 6.3 years. The histomorphologic features of metastatic chondroblastoma, its local recurrences and of the metastatic lesions differed in no way from conventional chondroblastomas. Because of the lack of cellular criteria of malignancy it is impossible to predict the potential biologic behavior of chondroblastomas, in particular with respect to their ability to metastasize. However, the presence of tumor emboli in the primary lesion is highly suggestive of a subsequent development of metastatic disease. The delayed induction of hematogenous metastases is best explained by a limited growth potential of the tumor cells. In case of a large primary tumor--especially in flat bones--with soft tissue invasion or in the presence of tumor emboli an aggressive surgical approach is suggested. When lung metastases have developed their surgical removal is recommended to hopefully prolong live expectancy or even to obtain a curative effect.

Adult↗

Treatment of chondroblastoma of the calcaneus with a secondary aneurysmal bone cyst using endoscopic curettage without bone grafting.

Chondroblastoma is a relatively rare benign bone tumor. Approximately 7% of chondroblastomas occur in the calcaneus, and 17% of chondroblastoma associated with cystic lesions. We report a case of a chondroblastoma in the calcaneus with a secondary aneurysmal bone cyst treated successfully by endoscopic curettage without bone grafting. New bone formation is facilitated by minimal damage to the bone and soft tissue. The cosmetic results of this procedure are good. Two years later, the patient is asymptomatic with no radiographic evidence of recurrence. Endoscopic curettage without bone grafting is a promising new treatment for chondroblastoma.

Adult↗

[Characterisation and differentiation of chondroblastomas and chondromyxoidfibromas - presence and expression of collagen types I and II].

AIM: Chondroblastomas and chondromyxoidfiibromas are rare benign skeletal neoplasms with reported overlapping histology. Aim of this study was to analyse the biochemical composition of the matrix of these tumour entities in order to further characterise the cellular phenotypes of these neoplasms using typical cell biological marker genes. METHODS: The matrix compositions of chondroblastomas and chondromyxoidfibromas were analyzed by HE-histology, histochemistry, and immunolocalization techniques. Cellular gene expression patterns were detected by mRNA in situ hybridization. RESULTS: Chondroblastomas are rich in collagen type I and show foci of an osteoid-like matrix, whereas collagen type II as a typical marker of chondrocytic differentiation was not detected in any of the specimens. Chondromyxoidfiibromas had foci of chondroid appearance with chondroblastic cellular differentiation characterised by collagen type II expression. CONCLUSION: These results characterise chondroblastomas and chondromyxoidfiibromas as skeletal neoplasms that have a different biology and which can be distinguished by matrix protein expression products: collagen type II, the typical marker of chondroblast differentiation, could only be detected in chondromyxoidfibromas, but not in chondroblastomas. Thus, both neoplasms are clearly different on the cell biological level.

Biomarkers, Tumor↗

Chondroblastoma of the patella associated with an aneurysmal bone cyst.

Chondroblastoma is a rare, benign tumor of bone, accounting for about 1% of all bone tumor cases. It tends to affect the epiphyseal ends of long bones, most often in males during the first and second decades of life. It has well-characterized radiographic and histologic features but despite its histologically benign appearance a few cases of metastases have been reported. Local recurrences after curettage and bone grafting occur in 11% to 25% of cases. The features of a patellar chondroblastoma are the same as for other locations. In reviewing the literature we found an unusually high male-to-female ratio. It is interesting that the usual treatment of the patellar chondroblastoma has been patellectomy, whereas curettage and bone grafting has predominated in the other locations. We present a computer tomography and magnetic resonance imaging study of a case of chondroblastoma of the patella associated with an aneurysmal bone cyst. To our knowledge, it is the seventh case reported and the second with computer tomography and magnetic resonance imaging studies. We also review and discuss in detail all the cases of patellar chondroblastoma that we found in the literature.

Adult↗

Expression of collagen type II, S100B, S100A2 and osteocalcin in chondroblastoma and chondromyxoid fibroma.

Chondroblastoma and chondromyxoid fibroma (CMF) has been investigated in numerous histological studies, but its biological nature and histogenetic origin are still a matter of debate. We evaluated the expression of type II collagen, S100A2, S100B, and osteocalcin to study the matrix biochemistry and phenotype of the neoplastic cells in these tumors. Immunohistochemically, the expression of type II collagen was diffuse in 15 cases (75%) among total 20 chondroblastomas and 12 (85.7%) among total 14 CMFs. The expression of S100B was also diffuse in all cases (100%) of chondroblastomas and 12 cases (85.7%) of CMFs. We were able to obtain the expression of S100A2, focally. The expression of osteocalcin was focally noted in 18 cases (90%) of chondroblastomas and 8 cases (57.1%) of CMFs. In conclusion, our data support that chondroblastoma and CMF are a distinct tumor entity with the basic expression of chondroid markers. But these tumors also show histologic diversity with focal co-expression of osteoid markers.

Bone Neoplasms↗

[Electron microscopic and immunohistochemical studies on chondroblastoma].

Six clinically and patho-histologically proven cases of chondroblastoma were studied with electron microscopic and immunohistochemical methods. The tumor tissues of chondroblastoma exhibited biphasic pattern i.e., chondroid and cellular area. In the chondroid area, small and round tumor cells contained many filaments in the cytoplasm and small processes in the cell wall. Many glycogen granules were present in the tumor cells in some cases. Intercellular matrix was immunohistochemically positive for S-100 protein and showed fine collagen fibers were very similar to those of articular and epiphysial cartilage. Many portions of mitochondria, cell wall and matrix of chondroblastoma tissue clearly exhibited calcification but were not definitely ossification. In the cellular area, tumor cells were composed of small immature mesenchymal and clear large degenerated tumor cells, histiocytes with lysosomes and osteoclast-like multinuclear giant cells. Immunohistochemical studies in the cellular area revealed that there were many tumor cells positive for lysozyme, alpha 1-antitrypsin and alpha 1-antichymotrypsin. In the transitional zone between chondroid and cellular area, degenerated tumor cells were found. The chondroblastoma was composed of chondroid and histiocytic areas which were very similar to those of chondromyxoid fibroma. The present study appears to demonstrate that chondroblastoma originates from a mixture of chondrocytic and histiocytic tumor cells but not from articular and epiphysial cartilage.

Adolescent↗

[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↗

Benign metastasizing chondroblastoma: a case report.

BACKGROUND: Metastasis of histologically benign chondroblastoma is a rare event. The authors report a new case, 12th in the literature, wherein multiple lung metastases appeared almost simultaneously with the primary lesion in the right talus bone. METHODS: A histologic evaluation of the primary lesion in the talus and the pulmonary metastasis was performed, and an ultrastructural study of the latter was done. Published literature on metastasizing chondroblastoma was reviewed to identify any consistency in the pattern and the outcome. RESULTS: Metastasis of chondroblastoma is uncommon but well known. Although radiologic and histologic aggressive features have been sought, they do not necessarily correlate with the outcome. CONCLUSIONS: Metastasis in chondroblastoma has been insufficiently stressed in the literature, unlike metastasis in giant cell tumors. The purpose of this case report is not only to document this uncommon event (the 12th case of lung metastasis) but also to emphasize that patients with chondroblastoma may have metastasis at presentation. Hence, all patients need to be evaluated regularly from the onset for possible lung metastasis so that deposits can be detected early for total resection.

Adult↗