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At least 19 recordsLinked to original sources
PubMed2006

A classic adamantinoma arising from osteofibrous dysplasia-like adamantinoma in the lower leg: a case report and review of the literature.

Adamantinoma is known as a low-grade malignant biphasic tumor. Classic adamantinoma is in general characterized by admixture of both epithelial and osteofibrous components that are associated with various proportions and differentiation patterns. Osteofibrous dysplasia (OFD) is a self-limited benign fibro-osseous lesion of bone during infancy and childhood. OFD-like adamantinoma is characterized by predominance of osteofibrous tissues, in which small groups of epithelial cells are only detected by careful search or immunohistochemistry. There have been controversies as to the potential correlation among OFD, OFD-like adamantinoma and classic adamantinoma. We report an unusual case of adamantinoma arising in the tibia, with an extensive review of the literature. The present findings suggest a direct correlation between OFD-like adamantinoma and classic adamantinoma. At the age of 12 years, the tibial biopsy lesion was diagnosed as OFD. At the age of 23, the lesion became larger and more destructive on x-ray films. The biopsy lesion was diagnosed as classic adamantinoma. Wide excision was performed. The primary lesion was retrospectively diagnosed as OFD-like adamantinoma because of presence of keratin-positive epithelial cells within the stroma. At five years after surgery, the patient was free from recurrence or metastasis. The retrospective histological findings of OFD-like adamantinoma in the original biopsy and of a classic adamantinoma in all sections of the later resection specimen raised the possibility of an unusual progression of OFD-like adamantinoma to a classic adamantinoma. The present case and the literature review suggest that an OFD-like adamantinoma may be a precursor lesion of classic adamantinoma. Therefore, the possibility of progression of OFD-like adamantinoma to a classic adamantinoma should be kept in mind, particularly when the destructive changes are seen radiologically.

Adamantinoma↗
PubMed2004

[Classic adamantinoma, differentiated adamantinoma and osteofibrous dysplasia of long bones].

BACKGROUND: Just for over 100 years, adamantinoma has been recognized as a primary enigmatic bone tumour with epithelial characteristics and predominantly involving the tibia. Several similarities between osteofibrous dysplasia has been recognised, differentiated adamantinoma and classic adamantinoma. The purpose of this study was to compare these lesions and to define their histogenesis and mutual relations. METHODS AND RESULTS: Comparison of the clinical, radiologic, histological, immunohistochemical and electron microscopic findings was carried out on 6 cases of classic adamantinoma, 2 cases of differentiated adamantinoma and 2 cases of osteofibrous dysplasia. This study confirmed the epithelial nature of long bone classical and differentiated adamantinomas irrespective of their wide-ranging morphologic pattern that can mimic tumours of various origins. Both types of adamantinoma were positive for cytokeratins in coexpression with vimentin. The epithelial component of the differentiated adamantinoma was much smaller than in classic adamantinoma and was present in scattered islands or single cells distributed within fibrovascular stoma. In one case the scattered epithelial cells had abundant eosinophilic cytoplasm and they resembled rhabdoid elements. On the basis of distinct histological pattern, a new variant of differentiated adamantinoma was described--a rhabdoid variant. In our cases of osteofibrous dysplasia occurring in two children with deformity of the tibia no epithelial cells were identified by the immunohistochemical methods. The lesions were composed of variably shaped spicules of woven and lamellar bone separated by a fibrovascular stroma. The woven bone spicules were surrounded by a uniform rim of plump osteoblasts. The lesion exhibited a zonal phenomenon with maturation of woven bone to bone with a lamellar configuration at the periphery of the lesion. The similar zonal phenomenon was also observed in the cases of differentiated adamantinoma. CONCLUSIONS: On the basis of the clinical, histological, immunohistochemical and electron-microscopical studies, a common histogenesis for classic adamantinoma, differentiated adamantinoma and osteofibrous dysplasia seems likely. The epithelial cell components of these lesions exhibit similar cytokeratin immunoprofiles and ultrastructural features. However, the prognosis for classic adamantinoma is much less favorable than that for cases of osteofibrous dysplasia and differentiated adamantinoma.

Adolescent↗
PubMed2026

Genomic Characterization of Classic Adamantinoma, Osteofibrous Dysplasia, and Osteofibrous Dysplasia-like Adamantinoma.

Classic adamantinoma, osteofibrous dysplasia (OFD), and OFD-like adamantinoma are rare bone tumors arising primarily in the tibiae. Their distinction can be challenging; data on their molecular pathogenesis remain limited. We searched our pathology files in 2004-2024 for available cases and performed targeted next-generation sequencing along with whole-genome single-nucleotide polymorphism arrays and 3-dimensional genomics/Hi-C sequencing in selected cases. Our cohort included 3 classic adamantinomas (2 females and 1 male; age, 14-56 years), 5 OFDs (3 females and 2 males; age, 9-25 years), and 2 OFD-like adamantinomas (1 female and 1 male; age, 30-41 years). Of the 10 tumors, 9 arose from the tibiae; 1 classic adamantinoma originated from the radius. The 3 classic adamantinomas harbored multiple copy number gains involving chromosome 7, 8, 10, 12, and/or 19. Focal deletion of chromosome 17, intergenic rearrangement involving FGFR1, and NRAS p.G12D were each present in 1 classic adamantinoma. Of the 5 OFDs, KMT2A p.C2441F, KMT2D p.S1040P, PHOX2B p.G213D, and RIF1 deletion were each present in 1 case; no additional copy number/single-nucleotide variants were identified. Of the 2 OFD-like adamantinomas, one case with tumor clusters visible only on cytokeratin immunostain harbored no variants, whereas another case with tumor clusters visible on light microscopy and cytokeratin/p40 immunostains showed gains of chromosome 7, 8, 19, and 20. By Hi-C, 1 classic adamantinoma harbored an approximately 9 Mb tandem duplication on chromosome 12q, 1 OFD harbored a rearrangement with breakpoints near MECOM and HOOK3, and the OFD-like adamantinoma with tumor clusters visible only on cytokeratin immunostain harbored no structural variant. In conclusion, classic adamantinomas and OFD might be genetically distinct. Classic adamantinomas harbored multiple alterations, including chromosome/arm-level copy number gains, the detection of which could aid their distinction from OFDs. Using genomics as the benchmark, OFD-like adamantinomas might be better delineated by light microscopy or p40 than by cytokeratin immunohistochemistry. These data expanded our molecular understanding of these rare bone tumors.

Humans↗
PubMed1989

Morphologic diversity of long bone adamantinoma. The concept of differentiated (regressing) adamantinoma and its relationship to osteofibrous dysplasia.

A review of the clinical, radiologic, and histologic features of 25 cases of long bone adamantinoma is presented. To answer some questions concerning the nature of these tumors, relevant tissue markers were analyzed in seven cases using immunohistochemical assays. This study confirmed the epithelial nature of long bone adamantinomas irrespective of their wide-ranging morphologic patterns that can mimic tumors of various origin. On the basis of distinct radiologic, histologic, and immunohistochemical patterns, a new type of adamantinoma termed "differentiated adamantinoma" could be distinguished from the classic long bone adamantinomas. The diagnostic features characteristic of the differentiated adamantinoma include: patient age (first two decades), intracortical location of the entire lesion, uniform predominance of an osteofibrous dysplasia-like pattern, and scattered positivity of epithelial elements for cytokeratin. We postulate that the predominance of an osteofibrous dysplasia-like pattern in differentiated adamantinoma is the result of a secondary reparative process overgrowing matured and regressing tumor tissue. It is possible that this process may lead to the total elimination of recognizable tumor cells from the lesion. Therefore, osteofibrous dysplasia (ossifying fibroma) of long bones, which has a similar anatomic location, age distribution, and radiologic appearance as differentiated adamantinoma, may, in some cases, represent the evolution of an underlying adamantinoma. Our analysis suggests that long bone adamantinoma could be another member of the unique family of tumors that may regress spontaneously.

Adolescent↗
PubMed1992

A clinicopathological and immunohistochemical study of osteofibrous dysplasia, differentiated adamantinoma, and adamantinoma of long bones.

A clinicopathological and immunohistochemical study of 12 cases of osteofibrous dysplasia (OFD), two cases of differentiated adamantinoma, and five cases of adamantinoma of long bones is presented. Although OFD and differentiated adamantinoma showed similar radiologic findings, differentiated adamantinoma was more likely to be a recurrent lesion than osteofibrous dysplasia and seemed to require a more extensive surgical procedure. Immunohistochemically, cytokeratin- and vimentin-positive cells were seen in both OFD and differentiated adamantinoma. The positive cells were scattered in the former, and were both scattered and nest-like in the latter. Both these lesions, however, were negative for epithelial membrane antigen. Excluding two cases of Ewing-like adamantinoma, the other three cases of adamantinoma were also positive for cytokeratin and vimentin. These results suggest that these three lesions share the same histogenetic origin. The two cases of Ewing-like adamantinoma differ from tibial adamantinoma in their radiological, histological and immunohistochemical aspects, and seem to constitute a distinct variant of adamantinoma with a different histogenesis.

Adolescent↗
PubMed2001

Adamantinoma-like Ewing's sarcoma and Ewing's-like adamantinoma. The t(11; 22), t(21; 22) status.

Adamantinoma of the long bones and Ewing's sarcoma are two malignant tumours between which, at first sight, there seems to be no morphological and clinical relationship. Both tumours, however, are known to express cytokeratins. Adamantinoma is a tumour of true epithelial nature, predominantly expressing cytokeratins 14 and 19. Ewing's sarcoma, believed to be from neuroectodermal origin, like other mesenchymal tumours, can aberrantly express cytokeratin 8 and 18. In the literature there are some reports of tumours showing clinical and/or morphological overlap between adamantinoma and Ewing's sarcoma, suggesting a possible relationship. These studies are mostly based on the epithelioid configuration of these lesions and their cytokeratin expression on immunohistochemistry. This raises the question of whether there is occasionally a morphological similarity between adamantinoma and Ewing's sarcoma, or whether there is a common genetic background. The Ewing's sarcoma/primitive peripheral neuroectodermal tumour (PNET) family is characterized in 90-95% of cases by a t(11; 22) and in 5-10% of cases by t(21; 22). In the few reports in the literature on cytogenetic investigations on adamantinoma, these translocations were never found using classical karyotyping. This study investigated the putative presence of t(11; 22) and t(21; 22) in 14 cases of adamantinoma by RT-PCR. These translocations were not found in any of these cases. The results support the view that these tumours are genetically and clinically distinct, but may eventually show overlapping morphological and immunohistochemical features.

Ameloblastoma↗
PubMed2003

Adamantinoma, osteofibrous dysplasia and differentiated adamantinoma.

For just over 100 years, adamantinoma has been recognized as a primary bone tumor with epithelial characteristics and predominantly involving the tibia. Osteofibrous dysplasia is a fibro-osseous lesion also predominantly confined to the tibia with radiologic features similar to those of adamantinoma. This lesion has been shown by immunohistochemical studies to frequently contain cytokeratin-positive epithelial cells. More recently, a third group of cases with clinical and radiologic features similar to those of osteofibrous dysplasia have demonstrated more overt strands of epithelial cells within a fibro-osseous background and have been categorized as "differentiated", "regressive", "juvenile intracortical" or "osteofibrous dysplasia-like" adamantinoma. Cytokeratin subset immunohistochemical stains and cytogenetic studies performed in recent years suggest a common histogenesis for these three entities. This article reviews the clinical, radiologic and pathologic features of these entities as well as their prognostic significance. It also reviews the results of the immunohistochemical and cytogenetic studies which establish a common histogenetic relationship.

Ameloblastoma↗
PubMed2004

Osteofibrous dysplasia and adamantinoma: correlation of proto-oncogene product and matrix protein expression.

To investigate the relationship between osteofibrous dysplasia (OFD) and adamantinoma, we analyzed the expression of several proto-oncogene products and extracellular matrix proteins by immunohistochemistry and correlated our results with histological and ultrastructural findings. C-fos and c-jun, but not c-Met, were observed in OFD and in the fibrous and epithelial components of differentiated and classical adamantinomas. Staining for collagen IV, laminin and galectin-3, a laminin binding protein was seen in OFD and around cell nests in adamantinoma. E-, P-, and N-cadherin expression was found in all cases of classical adamantinoma, but not in differentiated adamantinoma or OFD. Osteonectin was detected in both the epithelial and fibrous components of adamantinomas, but osteopontin and osteocalcin were not seen in classical adamantinomas. The results show common expression of a number of oncoproteins and bone matrix proteins in adamantinoma and OFD, some of which are associated with mesenchymal-to-epithelial cell transformation. These findings would be in keeping with the hypothesis that OFD represents a precursor lesion of adamantinoma. Differential expression of a number of bone matrix protein in adamantinoma may also be of diagnostic use in distinguishing these 2 lesions immunohistochemically.

Adolescent↗
PubMed1998

Osteofibrous dysplasia-like adamantinoma of bone: a report of five cases with immunohistochemical and ultrastructural studies.

This report describes five cases of osteofibrous dysplasia-like adamantinoma of the tibia in young patients ranging from ages 4 1/2 to 14 years. Radiologically and histologically, these cases were indistinguishable from osteofibrous dysplasia of bone, and no epithelial cells were recognized on routine staining. However, epithelial differentiation was seen in the form of scattered keratin-positive cells in all five cases, and tonofilaments in four cases. The patients were treated by curettage, and three had recurrences. Follow-up showed no progression to classic adamantinoma. Osteofibrous dysplasia-like adamantinoma is a special histological type of adamantinoma that affects children and adolescents. It differs from classic adamantinoma in that it lacks conspicuous nests and masses of epithelial cells, and the prognosis after conservative treatment is generally good. Recent publications suggest that osteofibrous dysplasia-like adamantinoma is a precursor of classic adamantinoma. In a comparative study of three cases of classic adamantinoma, we found, in the fibroblastic stroma of the tumors, spindle epithelial cells that were indistinguishable from the epithelial cells of osteofibrous dysplasia-like adamantinoma. This finding suggests that there is an overlap between these conditions. Four additional cases of osteofibrous dysplasia of the tibia from our files lacked epithelial differentiation. It is most likely that osteofibrous dysplasia is part of the morphologic spectrum of adamantinoma.

Adolescent↗
PubMed1998

Expression of growth factors and their receptors in adamantinoma of long bones and the implication for its histogenesis.

Adamantinoma of long bones is a rare bone tumour with (immuno-) histological features of epithelial cells, surrounded by various amounts of osteofibrous tissue. Recent studies have indicated that cells with an epithelial phenotype are most probably the malignant element. There is still debate as to whether the fibrous part should be designed as a benign neoplastic element of a biphasic tumour or as a reactive non-neoplastic tissue next to an epithelioid bone tumour. The expression of fibroblast growth factor type 2 (FGF-2), epidermal growth factor (EGF), and their respective receptors FGFR-1 and EGFR, as well as the proliferation marker Ki-67, was studied in both constituents of adamantinoma in serial sections of 25 cases by immunohistochemistry. Expression of FGF-2 and its receptor was present in both constituents of adamantinoma, but predominated in the epithelial component. Expression of EGF and its receptor was restricted to the epithelial component of adamantinoma. Comparing osteofibrous dysplasia (OFD)-like adamantinoma with classic epithelial cell-rich adamantinoma, the expression of FGF-2, EGF, and EGFR was more intense and in a higher percentage of cells in classic adamantinoma. Proliferative activity was found nearly exclusively in the epithelial component. These data further substantiate the hypothesis that epithelial cells constitute the proliferating tumour cell population responsible for the malignant behaviour of adamantinoma. The data indicate that during progression, the epithelial cells acquire expression of FGF-2, EGF, and EGFR, accompanied by a higher proliferative activity. Within the epithelial cell population, there exists an autocrine pathway of growth stimulation. Furthermore, these data point to an interaction between the epithelial and fibrous components, in which the epithelial cells additionally stimulate fibrous cell growth via a paracrine pathway involving FGF-2.

Ameloblastoma↗
PubMed1998

Classic adamantinoma in a 3-year-old.

Classic adamantinoma of the long bones is a rare, low-grade malignant neoplasm arising most often in the tibia and usually in patients during the second to fifth decades. Although adamantinomas have been described in children, the histologic pattern in this age group is different from that seen in adults and resembles osteofibrous dysplasia. The usual pattern of adamantinoma in children has been termed "differentiated adamantinoma" and follows a benign course. We report a case of adamantinoma in the proximal tibia of a 3-year-old patient. The lesion had abundant epithelial component with formation of keratin pearls, a pattern that has been described only in classic adamantinoma occurring in adults. Since differentiated adamantinomas are essentially benign and classic adamantinomas are low-grade malignancies, the finding of a classic variant at this young age raised important therapeutic and prognostic issues.

Ameloblastoma↗
PubMed2003

Dedifferentiated adamantinoma with revertant mesenchymal phenotype.

In adamantinoma of long bones, an osteofibrous dysplasia-like form with scattered epithelial elements and a classic form with abundant epithelium are distinguished. Osteofibrous dysplasia-like adamantinomas occur in children and adolescents and behave relatively benign, whereas classic adamantinomas predominate in adults and have a more aggressive clinical course. Because some osteofibrous dysplasia-like tumors have progressed to classic adamantinomas, it is hypothesized that the former is a potential precursor of the latter, showing mesenchymal-to-epithelial transformation. We report a new morphologic variant of adamantinoma in three patients with sarcomatoid transformation of the epithelial component: one in a primary tumor and two in local recurrences. One patient died of metastatic disease. Histologically, the tumors showed loss of the original characteristic epithelial differentiation with transition to fields of highly pleomorphic cells without epithelial features, high mitotic count, and deposition of osteoid and chondroid matrix. These dedifferentiated areas showed pankeratin positivity as well, although there were some changes in keratin subclass profile compared with other classic adamantinomas. This peculiar variant of long bone adamantinoma shows that in addition to mesenchymal-to-epithelial transformation in the early stage of development, progression to an aggressive subtype may be associated with epithelial-to-mesenchymal transition ("sarcomatoid dedifferentiation"), in which the epithelial immunophenotype is conserved. Thereby it may serve as an example of the plasticity of the mesenchymal phenotype. When confronted with a biopsy of a cortical tumor of the tibia showing sarcomatoid morphology and keratin positivity, adamantinoma should be included in the differential diagnosis, as its distinction has important implications for treatment and prognosis.

Adolescent↗
PubMed1994

Relationship between osteofibrous dysplasia and adamantinoma.

The records and computer files of 32 patients treated at 1 institution for ossifying fibroma, fibrous dysplasia, osteofibrous dysplasia-like adamantinoma, or adamantinoma of the tibia were reviewed. Nineteen patients had their diagnosis changed, either because of a recurrence or through review of their histology. Six of the 9 patients who had a typical adamantinoma and 6 of the 10 patients who had an osteofibrous dysplasia-like adamantinoma had a diagnosis of 1 of the benign conditions before their adamantinoma was recognized. Only 6 patients actually had osteofibrous dysplasia. Three patients required a resection, but only 1 of the other 3 has been observed > 5 years. This review suggests that many patients with a diagnosis of fibrous dysplasia or osteofibrous dysplasia of the tibia actually have an adamantinoma, and that osteofibrous dysplasia is often a locally aggressive lesion that progresses until it is widely resected. In addition, osteofibrous dysplasia and adamantinoma appear to be related, and osteofibrous dysplasia may be a precursor of adamantinoma.

Bone Diseases, Developmental↗
PubMed2006

Adamantinoma of the spine: case report.

OBJECTIVE: We report a patient with a cervicothoracic spinal and a mandibular adamantinoma. Adamantinoma is a rare malignant neoplasm of bone and, to our knowledge, there have been only five cases of spinal adamantinoma reported. The pathogenesis of the adamantinoma, as well as the management of this extremely rare spinal tumor, is reviewed. CLINICAL PRESENTATION: A 55-year-old man was admitted to our service with cervical pain and signs of C8 and T1 radiculopathy. On physical examination, cervical spine deformity, swelling in the left mandible region, and signs of C8 and T1 radiculopathy were observed. Neuroradiology examinations showed an osteolytic mass of the C6, C7, and T1 vertebral bodies, extending into the lateral masses and transverse processes. After surgical procedures, the patient had clinical improvement. INTERVENTION: Corpectomy of C6, C7, and T1 was performed through a cervicothoracic anterior approach. Anterior stabilization of the spine was obtained using an autologous iliac crest graft and osteosynthesis with an anterior plate. On a second procedure, posterior tumor resection and spinal stabilization were performed. After the 1-year follow-up examination, a new anterior procedure was performed because of tumor recidivity and spine instability. CONCLUSION: Adamantinoma, an extremely rare lesion, is a locally aggressive tumor with slow growth and the potential to metastasize. Although it is an extremely rare occurrence in the spine, adamantinoma should be considered on the diagnosis of tumors of the vertebrae. Neuroradiological examinations are not specific in the differentiation of this tumor from other conditions. This fact, coupled with the limited experience that most physicians in general have in dealing with this tumor, makes the diagnosis and treatment of adamantinoma challenging.

Adamantinoma↗
PubMed2003

Classic adamantinoma with osteofibrous dysplasia-like foci and secondary aneurysmal bone cyst.

Adamantinoma, a rare bone lesion of the tibia and fibula, has two distinct variants, classic adamantinoma and osteofibrous dysplasia-like adamantinoma. Composite lesions have not been described. Aneurysmal bone cyst is a benign cystic lesion which may also occur in the tibia and fibula. We report an unusual case of classic adamantinoma with osteofibrous dysplasia-like areas and foci of secondary aneurysmal bone cyst with prominent giant cells. A lesion was diagnosed in a 17-year-old girl with a 14-year history of a slowly enlarging left tibial mass and increasing deformity. Pathologically, the predominant pattern was classic adamantinoma, with minor foci of osteofibrous dysplasia-like adamantinoma and areas of secondary aneurysmal bone cyst with abundant multinucleated giant cells. We report the clinical, radiologic, and pathologic features of this case, and summarize lesions associated with secondary aneurysmal bone cyst. To our knowledge, the association of adamantinoma with secondary aneurysmal bone cyst has not been previously reported.

Adolescent↗