Chir Organi Mov (2008) 92:149–154DOI 10.1007/s12306-008-0060-5

O R I G I N A L A R T I C L E

Adamantinoma: an unusual bone tumour

Pedro Roque · Henry J. Mankin · Andrew Rosenberg

Received: 11 June 2008 / Accepted: 17 September 2008 / Published online: 15 November 2008© Springer-Verlag 2008

P. Roque · H.J. Mankin (�) · A. Rosenberg1122A Jackson BuildingMassachusetts General HospitalHarvard Medical SchoolBoston, MA 02114, USAe-mail: hmankin@partners.org

Introduction

Adamantinoma of long bones is a very rare primarymalignant bone tumour of disputed histogenesis [1–4].The tumour was first described by Maier in 1900 [5] andsubsequently by Fischer in 1913 [6] and both authorsnoted the histologic similarities to an odontogenictumour known as ameloblastoma or adamantinoma.Additional cases were reported by Ryrie in 1932 [7] andHebbel in 1940 [8], Dockerty and Myerding in 1942 [9]and Baker et al. in 1954 [10], but it was MarioCampanacci who subsequently named the tumour,described it in detail and reviewed its biology [1, 2, 11].In addition he distinguished it from a similar but benigndisease, principally occurring in children, which henamed osteofibrous dysplasia [1, 2, 12].

Adamantinoma is a rare condition accounting for lessthan 1% of the connective tissue tumours seen annuallyin the United States [2, 3, 9, 13–22]. It is now thoughtthat there are two types of adamantinoma: the “classical”more aggressive type and the “differentiated” morebenign form, which does not metastasise [2, 3, 21,23–26] and more closely resembles the benign osteofi-brous dysplasia [3, 4, 12, 23–25, 27–29]. Osteofibrousdysplasia, also known as ossifying fibroma, althoughsimilar in structure to adamantinoma, has some distinc-tive differences [1–4, 12, 23–25, 27–29]. The frequencyis less common and the disorder occurs most often inchildren under the age of 10. The tumours are often locat-ed more distally in the tibia and most frequently in theanterior face of the bone, and often cause sometimesmarked bowing [3, 4, 12, 24, 25, 27]. Histologically theyare less aggressive than the classical form of adamantin-oma but closely resemble the differentiated form [1, 3, 4,12, 23, 24, 28, 29].

Abstract Adamantinoma is a rare tumour, which mostoften affects the tibia and produces lytic and sometimesdestructive lesions, which can cause fractures. Thelesions occur principally in adults and are more commonin males. A small percentage of the patients developmetastases, sometimes quite late in the course. Our insti-tution has treated 42 patients with adamantinomas since1972 and has evaluated them by imaging studies and his-tology. The majority of the patients were treated by resec-tion of the lesion and insertion of an intercalary allograft.Only three of the patients died of disease with the timeuntil death ranging from 10 to 17 years. Recurrenceoccurred in only three patients and the allograft successrate in terms of function was 71% at a mean time of 10years.

Keywords Adamantinoma · Tibia · Malignancy · MarioCampanacci

Treatment protocols for adamantinoma have variedover the years but in view of the concern regarding latemetastases, the most logical approach has been one ofmarginal or wide resection of the lesion [2, 3, 11, 13,30–33]. In many cases, intercalary allografts have provedto be successful in decreasing the likelihood of localrecurrence or metastasis [31, 32, 34].

Materials and methods

Between 1972 and 2002, our oncology service has treated42 patients with adamantinoma of the tibia. Patient infor-mation was gathered retrospectively from office chartsand from a computer database [35]. The study was re -viewed and approved by the Hospital Institutional ReviewBoard, and patient confidentiality was not violated.

The entire group of patients was followed for an aver-age of 10.2 years (range 2–26 years). Twenty-six of thepatients were males and 18 were females. The averageage of the patients was 22±13 years (range 4–59 years).The anatomic sites of the tumours included 6 in the prox-imal tibia, 32 in the middle and 4 in the distal portion ofthe bone. As noted in Table 1, the MSTS classification[36] included 7 cases at Stage 0 (these were believed tobe the differentiated types), 16 as Stage IA, 13 as StageIB and 2 as Stage IIA. For five patients the MSTS stageswere not defined.

All 42 patients were studied prior to surgery by avail-able techniques including radiographic imaging, comput-erised tomography, technetium bone scan and, most

recently, MRI. Similar to other reports [2, 3, 11, 15, 18,22, 25, 37], the imaging studies of the affected tibia mostoften showed it to be expanded, but usually not bowed,and the cortex adjacent to the lesion was often thin butrarely destroyed, unless a fracture had occurred (Figs. 1and 2). The typical appearance for osteofibrous dysplasiais similar but the cortex is often thinner and bowingmight be quite severe (Fig. 3).

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Table 1 Description and results for 42 patients with adamantinoma

Total number of cases 42Gender Males 26 (62%) Females 16 (38%)MSTS stage Stage 0 7

Stage IA 15Stage IB 13Stage IIA 2Stage IIB 0Stage III 0

Tumour failuresRecurrence 3 (7%) (only one died)Metastasis 3 (7%) (all died)Death 3 (7%), time to death 13±4 years (10–17 years)

Primary treatmentResection and allograft replacement 34Curettage and allograft chips 4Resection and autograft insertions 3Amputation 1

Success rate for 34 allograft procedures:Good or excellent 24 (71%) 10±8 years (range 1–26 years)Fair or failure 10 (29%) 5±5 years (range 1–13 years)

Allograft complications none 25; non-union 3; fracture 2; infection 4; local recurrence 3.Success rate for 7 patients with local 5 successful procedures, 2 failures (71%)curettage and bone implantationCurettage complications Fracture 1, local recurrence 1

Fig. 1 A classic radiographic appearance of an adamantinoma of themid tibia, showing the lytic area in the anterior aspect of the bone.The cortex is thinned and irregular in structure but no soft tissue massoutside the bone is evident

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Fig. 2 A more destructive and extensive adamantinoma with a patho-logic fracture through the proximal portion of the lesion. The corticesare thinned and irregular in structure but without great distortion

Fig. 4 Different patterns for the cellular components of an adamantinoma of the tibia can be noted by examination of several haematoxylin and eosinstained sections. a shows somewhat variably structured epitheliod appearing cells surrounded by a fibrous stroma. Occasional vascular channels arenoted (H and Ex100). b shows irregular bony structure surrounding the typical cells. Both osteoblasts and osteoclasts are present (H and Ex200)

Fig. 5 Comparison of histologicappearances of adamantinoma andosteofibrous dysplasia. a An ada -mantinoma with cor ds in islands ofepitheliod cells surrounded by spin-dle cell stroma. Some of the islandsof tumour cells are adjacent to trabec-ulae of woven bone. b An osteofi-brous dysplasia with trabeculae ofwoven bone surrounded by osteo -blasts. Benign appearing proliferatingspindle cells are arranged in a stori-form pattern in the background

Fig. 3 The classic appearance of an osteofibrous dysplasia of the tibiain a 6-year-old child. Although the lesion resembles the adamantino-ma, the tibia shows bowing

a b

a b

As reported in the literature, the histologic appear-ance of the tumours was principally epithelial and gener-ally benign, with little atypism and only rare mitotic fig-ures [2, 14, 16, 18–20, 23, 28, 38–41]. The cells in thisseries of patients were sometimes basaloid, tubular orsquamous in character and often presented in nests with-in fibrous stroma or bony spicules (Fig. 4). The appear-ances for the classical and differentiated forms were sim-ilar [2, 3, 41]. The histologic images for osteofibrousdysplasia differed from those of classical adamantinomaand appeared quite benign (Fig. 5).

Results

None of the patients in this series were thought to haveosteofibrous dysplasia. Thirty-four of the patients withadamantinoma were treated by resection and intercalaryallograft replacement (Fig. 6). Four were treated bycurettage and insertion of allograft chips, 3 with curet-tage and insertion of autograft bone and one by amputa-tion (Table 1). Three of the patients developed a localrecurrence but only one of these died. Three of thepatients with MSTS Stage IIA tumour developed metas-tases and all of them died (7%). The duration of illnessfor those that died was 13±4 years (range 10–17 years).

In terms of function, the results for the patients whoreceived allografts were evaluated by review of the datafrom the patient’s last visit, which included physical and

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radiographic examinations. A grading system based onfunctional status originally introduced in 1983 was utilised[32]. A score of excellent meant that the patient had notumour recurrence, was pain free and maintained normalfunction. A score of good indicated no tumour recurrence,mild to moderate pain and moderate activity restrictions. Ascore of fair was defined as no tumour recurrence, mild tomoderate pain and marked functional limitations. A scoreof failure indicated a complication necessitating graftremoval or amputation. Successful procedures were grad-ed as excellent or good; failures as fair or failure.

Outcome studies showed that 71% of the patients wereclassified as excellent or good at 10±8 years (range 1–26years) and 10 were classified as fair or failure at 5±5 years(range 1–13 years). Only one patient required an amputa-tion, as treatment for a local recurrence (Table 1).

Kaplan–Meier [42] and Cox [43] regression analysesfailed to identify any factors including age, gender, stage,date, site, surgical procedure or presence of a local recur-rence, which was statistically related to either patient sur-vival or allograft success.

Conclusions

Adamantinomas are infrequent in occurrence and diffi-cult to assess and manage. Our experience suggests thatdiagnosis is relatively straightforward and that allograftreplacement appears to be a successful treatment [31].

Fig. 6 A 14-year-old female presented with tibialpain in 1988. The classical X-ray imaging is shownin a. b The intercalary allograft inserted into thespace and maintained in alignment with a plate andscrews. c discloses the histologic pattern whichappears benign and with less cellularity and limitedatypism. Desmoid tissue is present in large quanti-ties. It is likely that this is the differentiated form ofthe disease (H and Ex100). d An X-ray of the tibia20 years after the surgery. The patient has not had arecurrence, metastases or allograft complicationsand currently has no physical limitations or pain atthe site

a

c d

b

Although the complications, particularly infection, frac-ture and non-union, may result in failures of the graft,patients rarely require amputation [31, 34].

There are, however, some major issues. The first is dis-tinguishing the malignant classical form from the differenti-ated tumour, both of which have very similar histologic andimaging characteristics The treatment may be the same butpatients with the classical form must be carefully watched.Metastasis and death can occur as late as 16 years after suc-cessful removal of the primary lesion [2, 3, 11, 26, 30].

Another important problem, which is sometimes verydifficult, is distinguishing the adamantinoma from osteofi-brous dysplasia [1, 2, 4, 12, 25, 28]. Both the differentiatedand the osteofibrous dysplasia are similar histologically,but patient age and imaging of bone structure may help,since the osteofibrous dysplasias occur in younger childrenand most often cause significant bowing [2, 4, 12, 25].

As a special comment, the authors wish to expressappreciation for the contributions of Mario Companacci,who not only named and described adamantinoma, butalso distinguished it from the more benign forms. In thepast, several persons have suggested that the disorder beknown as “Campanacci Disease of the Tibia” and theauthors of this publication believe that may be an appro-priate nomenclature.

Conflict of Interest statement The Authors certify that there is noactual or potential conflict of interest in relation to this article.

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