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The Foot 19 (2009) 58–61
Case report
Adamantinoma of the calcaneum—A case report C.R. Chandrasekar a , R. Mohammed b,∗ , A.A. Rafalla c , R.J. Grimer a a
Musculoskeletal Oncology Unit, Royal Orthopaedic Hospital, Birmingham B31 2AP, United Kingdom b University Hospital Birmingham, Birmingham B29 6JD, United Kingdom c Department of Orthopaedic Surgery, Alexandria University, Cairo, Egypt Received 15 May 2008; accepted 23 September 2008
Abstract Adamantinoma is rare, representing less than 1% of all primary malignant bone tumours. It is a slow growing low grade malignant tumour which is often clinically, radiologicaly and histologicaly mistaken for many other tumours like Ewing’s sarcoma, synovial sarcoma, fibrosarcoma, metastatic carcinoma, fibrous cortical defect and osteofibrous dysplasia. Adamantinoma commonly occurs in the tibia. It is also known to occur in the fibula, femur, humerus, radius and ulna. Adamantinoma of the foot is extremely rare. There are few reported cases of metatarsal and cuneiform bone involvement. To our knowledge adamantinoma of the calcaneum has not been previously reported in the literature. We report the case of a male patient, aged 33 years, who was treated for adamantinoma of the calcaneum and followed for 16 years highlighting the difficulty in diagnosis and management of this rare tumor occurring at a rare site. © 2008 Elsevier Ltd. All rights reserved. Keywords: Adamantinoma; Malignant bone tumour; Calcaneus
1. Introduction Adamantinoma is a rare malignant primary bone tumour, representing less than 1% [0.1–0.48%] of all primary malignant bone tumours [1–3]. It is surpassed in rarity only by primary neurogenic and lipogenic bone tumours [4]. Adamantinoma is a slow growing low grade malignant tumour which is often clinically, radiologicaly and histologicaly mistaken for many other tumours like Ewing’s sarcoma, synovial sarcoma, fibrosarcoma, metastatic carcinoma, fibrous cortical defect and osteofibrous dysplasia. The aetiology of adamantinoma is still a matter of debate. One of the proposed hypotheses is that adamantinoma might arise due to displacement of basal epithelium during the embryological development or trauma. Different cell types have been suggested as the origin of adamantinoma. These include epithelial cells [1–3,5], endothelial cells [2,3,6], as well as synovial cells [2,3]. Greater than 80% of adamantinoma of the long bones occur in the tibia [1]. Other reported sites are the fibula, femur, ∗
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humerus, radius and ulna. There are a number of case reports and small series reported in the literature regarding adamantinoma [1,3,7–15]. Adamantinoma of the foot is extremely rare with few reported cases of metatarsal and cuneiform bone involvement [13]. To our knowledge adamantinoma of the calcaneum has not been previously reported in literature [Pubmed listed journals and internet search using Google]. In this paper we report a case of a middle aged male patient with adamantinoma of the calcaneum followed for a period of 16 years. The rarity of the site, difficulty in making the correct diagnosis and the management aspects are also highlighted.
2. Case report A 33-year-old man initially presented to his local hospital in March 1989 with a 3 months history of left heel pain. The clinical and radiological diagnosis was a simple bone cyst and he was treated with curettage of the lesion and bone grafting in July 1989. The histology was reported as a simple bone cyst according to the local pathologist. Unfortunately he had persistent pain and difficulty in weight bearing with his right leg. In November 1990 the histology was revised
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verified by another eminent pathologist 3 years later and the diagnosis of adamantinoma was confirmed. He had 3 monthly clinical examinations with chest X ray for 2 years, 6 monthly assessments for the next 3 years and yearly thereafter by the his general practitioner. He has no evidence of recurrence or lung metastasis so far after a period of 16 years.
3. Discussion
Fig. 1. Plain radiograph of the hind foot showing lytic lesion of calcaneum [local recurrence following curettage and bone graft].
by another pathologist who concluded that the diagnosis was interosseous synovial sarcoma. He was referred to our tertiary referral musculoskeletal oncology unit in February 1991 for a second opinion. He had further investigations including plain radiographs of the hind foot, chest radiograph; CT scan, MRI and bone scan (Figs. 1–4). The calcaneal lesion was found to be solitary with no evidence of metastatic disease. Histopathology of a repeat biopsy showed spindle cell tumor, comprising predominantly fibroblastic tissue. The diagnosis of fibrosarcoma was made and the options of limb salvage versus a below knee amputation were discussed with the patient. He refused to have an amputation. Chemotherapy with Cisplatin and Adriamycin was instituted in liason with the medical oncologist. After two cycles of chemotherapy, the patient opted to have the below knee amputation which was carried out in June 1991. The final histological diagnosis was adamantinoma of the calcaneum, which was confirmed by immunohistochemistry. No further adjuvant therapy was offered. The below knee amputation stump healed well, and he was fitted with a prosthesis. There were no surgical complications. The histology was independently
Fig. 2. Tomogram of the calcaneal lesion [local recurrence following curettage and bone graft].
Approximately 3% of osseous tumors occur in the foot and ankle. Adamantinoma is an extremely rare primary bone tumor. It is believed to represent less than 1% of all malignant bone tumors. Because of its low incidence, reports of large series from single centres are rare [6,16–19]. A meta analysis of 200 cases of Adamantinoma by Moon and Mori [13] and an international study by Qureshi et al. [2] involving 70 patients with adamantinoma are two of the large series reported in the literature. According to their data, the tumour showed a slight preference for males and was most commonly seen in the second and third decades. Adamantinoma has been found in all the long bones of the body, with the most common presentation being in the tibia [80%]. The foot was one of the extremely rare sites for occurrence of an adamantinoma. Involvement of metatarsal bones and cuneiform bone has been reported [13,20]. Adamantinoma of the foot usually presents with chronic pain with or without swelling, and radiographs show an eccentric lytic and expansile lesion, often involving the cortex as well as the medullary cavity. Solitary bone cyst, chondroblastoma, intraosseous lipoma, osteoid osteoma, chondrosarcoma, osteosarcoma, giant cell tumor, metastatic carcinoma, Ewing’s sarcoma and osteomyelitis seem to have a predilection for the calcaneum. They must be considered in the differential diagnosis of a calcaneal lesion. Adamantinoma has not previously been reported as an isolated lesion of the calcaneum and this was one of the reasons for the difficulty in diagnosing this condition. A MRI scan can show the soft tissue extent of the tumour. Biopsy must be carefully planned, as some areas may not be representative, composed of necrotic tissue or benign looking fibrous tissue and the islands of tumour tissue could be missed [1]. Biopsy of a rare tumour occurring at a rare site can be difficult to diagnose even to the experienced pathologists who specialise in orthopaedic oncology and have the benefit of better clinical and radiological input in addition to the availability of advanced histological facilities. Hence a wrong initial histological diagnosis was not uncommon as demonstrated in this case report. The definitive diagnosis of adamantinoma was eventually confirmed after 4 attempts. Previously described treatment options for adamantinoma include curettage, en-bloc excision, and amputation. Chemotherapy and radiation have had extremely low success rates and were not recommended for primary treatment of adamantinoma [13]. Curettage has been found to be
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Fig. 3. Bone scan.
inadequate for treatment of adamantinoma as there was very high risk of local recurrence. Moreover such an inadequate intervention may change the indolent nature of the lesion to behave in a more aggressive way [6,13]. Local recurrence
Fig. 4. T2 weighted MRI scan showing the calcaneal lesion.
often occured in those patients whose tumours were treated by intralesional procedures [6,13]. Local recurrence occurred in our case following curettage and bone grafting of the calcaneal lesion presumed to be a simple bone cyst. The treatment of choice in resectable adamantinoma especially in the tibia, was wide excision, followed by reconstruction using allografts, fibular auto graft, bone transport using external fixator, endoprosthetic replacement, or reimplantation of the bone tumour segment after being sterilised by irradiation in combination with a pedicled fibular graft. This is not always technically feasible especially when calcaneum is involved, as achieving wide margin and subsequent limb salvage can result in complications and poor function. Primary amputation can provide early recovery and useful function if there are no stump related problems. Primary amputation of the affected limb had been recommended by some authors as the most definitive and complete treatment, possibly leading to lower rates of distant metastasis and reduced the risk of local recurrence [14]. In spite of being a slowly growing tumour, rarely metastatic at presentation,
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adamantinoma can eventually metastasize in about 15–20% of patients [6,13,14]. The most common site for metastasis is the lungs; lymph nodes, other long bones and the abdominal viscera are occasionally involved [13]. Definitive below knee amputation was the preferred treatment for the calcaneal lesion in our case. This resulted in good functional results and oncological outcome after nearly 16 years. 4. Conclusion In conclusion the case report illustrated the difficulty in the diagnosis and management of a rare tumour presenting at a rare site. References [1] Mohler DG, Cunningham DC. Adamantinoma arising in the distal fibula treated with distal fibulectomy: a case report and review of the literature. Foot Ankle Int 1997;18(11):746–51. [2] Qureshi AA, Shott S, Mallin BA, Gitelis S. Current trends in the management of adamantinoma of long bones. An international study. J Bone Joint Surg Am 2000;82A(8):1122–31. [3] Van Rijn R, Bras J, Schaap G, van den Berg H, Maas M. Adamantinoma in childhood: report of six cases and review of the literature. Pediatr Radiol 2006;36(10):1068–74. [4] Mirra JM. Adamantinoma and fibrous dysplasia. In: Mirra JM, editor. Bone tumours. 1st edition Philadelphia: Lea and Febiger; 1989. p. 1203–31. [5] Czerniak B, Rojas-Corona R, Doforman HD. Morphologic diversity of long bone adamantinoma. The concept of differentiated (regressing) adamantinoma and its relationship to fibrous dysplasia. Cancer 1989;64:2319–34. [6] Huvos AG, Marcove RC. Adamantinoma of long bones. A clinicopathological study of fourteen cases with vascular origin suggested. J Bone Joint Surg Am 1975;57-A:148–54.
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