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REVIEW ARTICLE Adamantinoma Acta Orthop. Belg., 2007, 73, 425-431 REVIEW ARTICLE Adamantinoma Panagiotis KITSOULIS, Antonia CHARCHANTI, Georgios PARASKEVAS, Aikaterini MARINI, Georgios KARATZIAS From the Laboratory of Anatomy-Histology-Embryology, Medical School, University of Ioannina, Greece Adamantinoma is one of the rarest low-grade malig- appear in several osseous locations but the mid nant bone tumours, representing less than 1% of shaft of the tibia – affecting or not the fibula – is the them. Fisher in 1913 named this tumour adamantin- commonest location. oma because of its similarity to ameloblastoma of the In an attempt to find a specific cytogenetic jaw. It usually arises in the center of long bones , and profile of adamantinoma, the latest molecular and 97% of all reported cases were in long tubular bones cytogenetic techniques were used. The results and mainly in the tibial mid shaft (80-85%). Other long bones not uncommonly affected are the suggested that these tumours are consistent with humerus, ulna, femur, fibula and radius. Ribs, spine, epithelial or endothelial or synovial cells (7, 20, 37, metatarsal and carpal bones are very rarely affected. 48, 49). According to Schajowicz et al (47) adaman- The symptoms are not specific but most frequently tinoma is a malignant or, at least, locally malignant the patient complains about swelling, redness, pain tumour with a variety of histological patterns ; the and sensitivity of the bone where the tumour is most common is the one with circumscribed or located. Young males are more prone to develop tubular masses of epithelial cells in the middle of adamantinoma than females. The tumour usually spindle-celled fibrous tissue. spreads to the lungs, the regional lymph nodes, or other bones. Wide tumour excision and limb salvage HISTORY reconstruction surgery, or an amputation, are the current surgical treatment options. Radiotherapy Although adamantinoma is an uncommon skele- and chemotherapy have not been shown to be effective modalities of treatment. tal tumour, its origin has been a matter of debate for Keywords : tumour ; adamantinoma ; osteofibrous dysplasia. ■ Panagiotis Kitsoulis, MD, Orthopaedic Surgeon, Lecturer. ■ Antonia Charchanti, MD, Lecturer. ■ Georgios Paraskevas, MD, Lecturer. ■ Aikaterini Marini, MD Assistant in Anatomy. INTRODUCTION Department of Anatomy, Medical School, University of Ioannina, Ioannina, Greece. Adamantinoma of long bones is a rare, distinc- ■ Georgios Karatzias, MD, Otorhinolaryngologist. tive, and low-malignancy primary tumour of the General Hospital of Larissa, Thessaly, Greece. Correspondence : Panagiotis Kitsoulis, MD, Lecturer, appendicular skeleton. Its name is a mistake of the Department of Anatomy-Histology-Embryology, Medical past as “adamantinoma” means an ameloblastoma- School, University of Ioannina, 21th October Street 28, 45332, like tumour. Its exact origin is not yet known ; the Ioannina, Greece. classic form contains an epithelial and an osteo- E-mail : [email protected]. © 2007, Acta Orthopædica Belgica. fibrous component (17, 28). Adamantinoma may No benefits or funds were received in support of this study Acta Orthopædica Belgica, Vol. 73 - 4 - 2007 426 P. KITSOULIS, A. CHARCHANTI, G. PARASKEVAS, A. MARINI, G. KARATZIAS more than seven decades. The first case of a prima- the tibial mid-shaft (80-85%) (3, 4). Other long ry bone tumour with epithelial characteristics is bones not uncommonly affected are the humerus, attributed to Maier (34) in 1900. In 1913, a German ulna, femur, fibula and radius but ribs, spine, pathologist, Fischer (13), named this tumour metatarsal and carpal bones are rare exceptions (41). ‘’adamantinoma’’, because he noted that during Adamantinoma of long tubular bones shows a embryonic development adamantine epithelium predilection for diaphyses, and it rarely forms more was found at the tibia as well as in the intraoral than one or two foci. enamel. Many years later, Maier (34) referred to a tumour in the ulnar diaphysis of a young woman CLINICAL FEATURES that had epidermoid characteristics, which raises the question how this could occur in a bone. Some Pain is thought to be the first symptom of a researchers believed that adamantinoma has a developing adamantinoma, sometimes co-existing double nature, being created by epithelial and with local swelling. Swelling is a more specific spindle-cells elements, features suggesting a sign than pain because adamantinomas may be synovial sarcoma. associated with repetitive trauma which is painful The first evidence for the origin of adamantino- on its own (38, 42). Adamantinoma is a malignant ma came from Changus et al (10) in 1957, who sug- tumour and may metastasize ; although it is of low gested that it could derive from vascular tissue. malignancy, metastases may be found in the diag- Llombart-Bosch and Ortuno-Pacheco (33) based on nostic setup, usually in the lungs and the neigh- ultrastructural studies supported this theory. boring lymph nodes (50, 56). These studies revealed that tumour cells have basement membranes, microvilli and tonofibrils RADIOLOGIC FEATURES forming the desmosomes which are indispensable for a cell-to-cell attachment. The imaging of an adamantinoma is of impor- Lately, investigators used immunohistochem- tance in diagnosis (fig 1). Using roentgenography, istry to show that the cells of adamantinoma are adamantinoma appears as an eccentric, lobular, keratin-positive, which proves an epithelial ori- lytic lesion with poor or sharp delination in the gin (44). Adamantinoma cells were also positive for middle or distal third of the diaphysis or meta- alkaline phosphatase and had structures which physis of a long bone with some peripheral sclero- were similar to Weber-Palade bodies, thereby sug- sis. New bone formation may be also present. gesting an endothelial cell origin. Furthermore, small radiolucent areas may be pre- It was also noted that adamantinomas show sent around the main focus of the lesion. A immunochemical positivity for factor VIII antigens periosteal reaction may be found but is usual only and vimentin (30). when a fracture exists in the same area. It is impor- tant to remember that lesions tend to appear in the INCIDENCE tibia and not in the fibula. The characteristic ‘’soap- bubble’’ picture is formed by radiolucent areas sur- Adamantinoma is an extremely rare tumour rep- rounded by ring-shaped dense areas (26, 29, 32, 43). resenting 0.33-0.48% of primary malignant bone Computed Tomography (CT) more accurately tumours. There is a prevalence of males over reveals the same osteolytic formation in the cortex females, with a ratio of 5 to 4. Adamantinomas may of the bone, even in the soft tissues lying above. appear at any age but in clinical practice the tumour Magnetic Resonance Imaging (MRI), as a special appears in females between 11 and 30 years old technique for soft tissues, seems to be the gold and in males between 30 and 50 years of age (39, standard examination for the differential diagnosis 53). Elderly individuals and children represent a between adamantinoma, Ewing’s sarcoma, fibrous minority of patients. Most (97%) of all the report- bone dysplasia, osteosarcoma and metastatic carci- ed cases were in long tubular bones and mainly in noma (4, 5, 14, 36, 46, 58). Acta Orthopædica Belgica, Vol. 73 - 4 - 2007 ADAMANTINOMA 427 Fig. 2. — Computed Tomography HISTOPATHOLOGICAL FEATURES Classic adamantinoma is composed of an epithe- lial and an osteofibrous component. These compo- nents may be intermingled in various proportions and following different patterns. The main histo- logical patterns of classic adamantinoma include basaloid, tubular, spindle cell, and squamous pat- terns (40). The most common histological patterns are the basaloid and the tubular but all the main his- tological patterns may be encountered in one lesion. Fig. 1. — Radiologic features of adamantinoma The spindle cell component is more often encountered in recurrences, as well as in metas- tases. The osteofibrous component is composed of spindle cells showing a storiform pattern. Woven bone trabeculae are often observed in or next to the MRI may also reveal the exact location and central area of the lesion, and varying amounts of volume of the tumour and clarify whether or not it transformation to lamellar bone at the periphery of has affected the surrounding soft tissues (53, 58). the tumour. Foam cells or myxoid change may be Other general characteristics are its average encountered. A low number of mitoses is found. length which is about 11 cm, as it has a longitu- Another histological pattern, the so-called osteofi- tional orientation, and the marked destruction of brous dysplasia-like variant, is composed mainly of the bone medulla in contrast to ossifying fibroma osteofibrous tissue, in which small groups of which causes less damage. Additionally there is the epithelial cells are only observed by careful search chance of finding congenital pseudarthrosis of the or by using immunohistochemistry. Most classic tibia which leads the diagnosis to fibrous dysplasia and osteofibrous dysplasia-like adamantinomas rather than adamantinoma (1). In conclusion, it is show a “zonal” architecture (55). In classic adaman- important to use radiographs in order to diagnose tinoma, the central area is usually occupied by the an adamantinoma, and CT and MRI for the differ- epithelial component, with only a few small imma- ential diagnosis, and to assess the aggressiveness ture bone trabeculae present in the fibrous tissue. In and extension of the tumour. osteofibrous
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