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Clinicopathological Features, Diagnosis, and Treatment of Adamantinoma of the Long Bones PANAYIOTIS J. PAPAGELOPOULOS, MD, DSC; ANDREAS F. MAVROGENIS, MD; EVANTHIA C. GALANIS, MD; OLGA D. SAVVIDOU, MD; CARRIE Y. INWARDS; FRANKLIN H. SIM, MD

educational objectives As a result of reading this article, physicians should be able to:

EPIDEMIOLOGICAL DATA

1. Describe the radiographic characteristics of adamantinoma of the long bones.

Adamantinoma of the long bones is a rare tumor that accounts for 0.3%-1% of all

2. List the histological patterns and radiographic characteristics commonly associated with adamantinoma. 3. Discuss treatment options for adamantinoma of the long bones. 4. Discuss the prognosis of patients with adamantinoma.

A

damantinoma of the long bones is a low-grade, slow-growing, primary malignant bone tumor composed of epithelial cells in a fibrous or osteofibrous stroma.1,2 It has a wide range of histological patterns.3-18 Despite the considerable range of each histologic pattern, most current studies have shown that long bone adamantinoma appears to be of an epithelial nature. The tibial lesion’s name comes from its somewhat similar histologic appearance to the more common odontogenic adamantinoma of the jaw bones.18,19 Despite the histological similarities there is no proof that these tumors have a similar histiogenetic origin.19 Long bone adamantinomas can be divided in two main groups with distinct histological and radiographic fea-

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tures. The first–classic adamantinoma–is characterized by an abundance of tumor cells and a destructive growth pattern. The second, differentiated adamantinoma, is characterized histologically by a predominance of an osteofibrous dysplasia-like pattern with a small, inconspicuous component of epithelial tumor elements.12,18,19 Radiographically, classic adamantinomas are either intracortical or associated with complete cortical disruption, intramedullary involvement, and expansion beyond the periosteum into adjacent soft tissues, while differentiated adamantinomas are intracortical and often multicentric lesions.12,19,20 This article discusses the epidemiology, clinicopathological and imaging features, diagnosis, treatment, and prognosis of adamantinoma of the long bones.

Drs Papagelopoulos and Mavrogenis are from the First Department of Orthopedic Surgery, Athens University Medical School, Athens, Greece, Dr Galanis is from the Division of Medical Oncology, Mayo College of Medicine, Rochester, Minn, Dr Savvidou is from the Department of Orthopedics, Thriasion General Hospital, Elefsis, Hellas-Greece, Ms Inwards is from the Division of Anatomic Pathology, Mayo College of Medicine, Rochester, Minn, and Dr Sim is from the Department of Orthopedic Surgery, Mayo College of Medicine, Rochester, Minn. Drs Papagelopoulos, Mavrogenis, Galanis, Savvidou, and Sim and Ms Inwards have no industry relationship to declare. The material presented at or in any Vindico Medical Education continuing education activity does not necessarily reflect the views and opinions of Vindico Medical Education or Orthopedics. Neither Vindico Medical Education or Orthopedics, nor the faculty endorse or recommend any techniques, commercial products, or manufacturers. The faculty/authors may discuss the use of materials and/or products that have not yet been approved by the US Food and Drug Administration. All readers and continuing education participants should verify all information before treating patients or utilizing any product. Correspondence should be addressed to: Panayiotis J. Papagelopoulos, MD, DSc, Athens University Medical School, 4 Christovassili Street, Neo Psychikon, 15451, Athens, Greece.

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2 Figure 2: CT scan of the right leg shows a lytic expansile lesion within the tibia. There is intramedullary involvement with some calcifications and thinning of the cortex, particularly posteriorly.

CLINICAL MANIFESTATIONS

1A

1B

Figure 1: AP (A) and lateral (B) plain radiographs show an expansile mixed lytic and sclerotic lesion of the mid-portion of the right tibia.

malignant bone tumors.4-9,21 In 1986, Moon and Mori2 performed a meta-analysis of 200 cases. In 1996, Unni18 reported 36 cases in an analysis of 5641 primary malignant bone tumors from a single institution. The literature suggests a slight male predominance.1,2,4-9,21 Adamantinomas usually are diagnosed after skeletal maturity. Although 75% of the cases occur between the second and third decades of life,18 adamantinomas have also been reported in older patients, as well as in children aged ⬍14 years.18,22,23 Differentiated adamantinomas tend to occur in younger patients.18,19 More than 90% of adamantinomas appear in the tibia, with fibular involvement coexisting in 50% of the cases.12,18,19 The tumor usually is located in the diaphysis and, less frequently, the tibial metaphysis. Rare cases of adamantinomas of the olecranon, the ribs, the radius, the spine, the metatarsus, and the humerus also have been reported.24-31

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A history of previous trauma or fracture occurring months or years prior to the onset of symptoms is reported in approximately 60% of patients. The most common presentation is of a gradually evolving mass associated with dull, insidious, aching pain. Bowing deformity of the tibia and pathologic fractures may occur. Advanced or recurrent lesions may be associated with soft-tissue involvement.12,18,21 Severe paraneoplastic, humorally mediated hypercalcaemia, hypercalcaemic coma, and pancreatitis have been reported.32-35

tiple lucencies and sclerotic foci characterizes differentiated adamantinoma. These imaging features may be similar to those observed in osteofibrous dysplasia.12,18,20 Technetium pyrophosphate bone scan shows intense increased uptake tracer accumulation, corresponding closely to the extent of the lesion.18,21,36 In addition, bone scan may show a coexisting fibular involvement. Computed tomography reveals an osteolytic lesion with expansion and destruction of the cortex (Figure 2), and probable extension to the surrounding soft tissues.21,37 Magnetic resonance imaging (MRI) is useful in providing information concerning the intramedullary and soft-tissue extent of the tumor.9,20,36-38 On T1-weighted MRI, the lesion has a low intensity signal, whereas on T2-weighted MRI the signal is much brighter and does not diminish with the fat suppression technique.

Two morphologic patterns were distinguished in MRI: a solitary, lobulated focus versus a pattern of multiple small modules in one or more foci.

IMAGING FEATURES Radiographically, adamantinoma usually appears as a mixed lytic and sclerotic central lesion or multiple, sharply circumscribed lucencies with sclerosis of the intervening bone (Figure 1). This slowly growing, expansile tumor eventually causes endosteal scalloping and thinning, interruption, or destruction of the cortex without periosteal reaction. Moderate to severe anterior bowing deformity is common.19,20 Classical adamantinoma usually is intracortical with evidence of complete cortical disruption and intramedullary and soft-tissue involvement. Involvement of the anterolateral cortex of the tibia with mul-

In a study of 22 patients, two morphologic patterns were distinguished in MRI: a solitary, lobulated focus versus a pattern of multiple small modules in one or more foci; however, these characteristics were not related to the histologic subtype.38

HISTOLOGIC FEATURES Histologically, adamantinoma is composed of epithelial islands in a spindle cell stroma. The relative amounts of the two components can vary considerably (Figure 3). Several histologic variants have been

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3A

3B Figure 3: Histological appearance of adamantinoma. Low-power view (A) shows islands of epithelial cells surrounded by fibrous tissue and high-power view (B) of the cluster of epithelial cells within the fibrous stroma.

described corresponding to a variety of different epithelial cell patterns that can be found. Therefore, this range of morphologic patterns can mimic many primary or metastatic bone lesions.21,39 A number of immunohistochemical and electron microscopic studies have shown adamantinoma to be of epithelial derivation.1,9-11,36,40-44 In some tumors the stromal component has a fibrous dysplsia-like appearance with or without scattered spicules of woven bone.18,19

DIFFERENTIAL DIAGNOSIS Because of its rarity and differing histological patterns, adamantinoma histologically may resemble epithelial metastasis, hemangioendothelioma, hemangiosarcoma, fibrous and osteofibrous dysplasia, nonossifying fibromas, and chondromyxoid fibromas.18,19,44-47 Clinical information such as patient age and history, as well as the location in the tibial diaphysis, may aid in diagnosis. It is important to differentiate adamantinoma from osteofibrous dysplasia.20

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Adamantinoma generally is painful, is observed during adulthood, has different radiographic features, is progressive during adult age, and may expand into the soft tissues. However, osteofibrous dysplasia seldom progresses during childhood, and any progression of the lesion stops after puberty. Exceptional cases have been reported of adamantinoma that began during childhood and have radiographic features similar to osteofibrous dysplasia.45-47 Adamantinoma must be suspected if a tibial lesion becomes painful or if it grows after puberty. In such cases, extensive biopsy from the most radiolucent areas is recommended. However, given the heterogeneity of the histology within different areas of adamantinomas, sampling errors are possible with limited biopsy specimens. These errors have led to reclassification of these tumors from osteofibrous dysplasia to adamantinoma after repeat biopsy with adequate tissue.45-47

TREATMENT AND PROGNOSIS Owing to the rarity of adamantinomas, data is insufficient concerning the safest and most effective treatment modality. These tumors are of low-grade malignancy and highly radioresistant.48,49 Thus surgery is the treatment of choice. Although limited experience with them has been reported, chemotherapy and radiation therapy have not been shown to be effective.8 Operative treatment includes either surgical resection with wide margins and reconstruction of the segmental defect,4,5,8,9,12,18,21,50-52 or amputation.1,2,5,9 Limb reconstruction options include the use of allografts (intercalary, osteoarticular, or morselized), vascularized and nonvascularized fibular autografts, metallic segmental implants, and distraction osteogenesis.9,21,51,53 Intercalary reconstruction appears to be the most successful method (Figure 4). Qureshi et al9 reported a limb salvage rate of 84%. They used allografts in 61% of the reconstructions after resec-

4A

4B

Figure 4: AP (A) and lateral (B) plain radiographs after wide resection of the tumor and reconstruction using a 13 cm intercalary tibial allograft fixed using a DCP plate and screws and autologous iliac bone grafting at the host bone allograft junction.

tion of adamantinomas of long bones. The majority were intercalary reconstructions. No differences in the efficacy of different fixation techniques were noted. Complications related to the reconstruction were unacceptably high (48% of patients), including nonunion (24%) and fracture of the allograft (23%). Vascularized fibular grafts have been considered the best type of graft for large segmental bone defects after bone tumor resections.54,55 Local recurrence is common in inadequately treated patients.12,21,56,57 Wide operative margins are associated with a lower rate of local recurrence than marginal or intralesional margins.8,9,24 However, a 18.6% rate of local recurrence at

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10 years has been reported after widemargin surgical excision of adamantinomas.9 Local recurrences usually occur 5 to 15 years after primary excision of the tumor. Late local recurrences have been reported at 24 and 36 years after diagnosis.21,56 No statistically significant difference between local recurrence rate and tumor stage, duration of symptoms, patient gender and age, type of biopsy, and type of grafting has been documented.9,12 Wide resection is also indicated for recurrent tumors.56 The near-benign biology and slowgrowing nature of adamantinoma are reflected in the good survival rates even after local recurrences and lung or regional lymph node metastases.21,39,51 Late metastases may occur in 10%-30% of patients with adamantinomas, most frequently in the lungs, the regional lymph nodes, or the bones.19,21,24,32-35 After wide margin surgical excision of the tumor or amputation, overall 10-year survival rates vary from 82% to 87%.1,2,8,12

CONCLUSION Adamantinoma of the long bones is a rare, primary, low-grade, slow-growing malignant bone tumor. It is expressed with a wide range of histological patterns. Histologically, the tumor is composed of an epithelial component surrounded by a fibrous stroma that may or may not contain spicules of woven bone. In the majority of cases, adamantinomas are located in the tibial diaphysis. Limb salvage with wide surgical resection and reconstruction or amputation is the most effective treatment. Local recurrence is associated with incomplete resection. Survival rates range from 82% to 87%. Late metastases, mainly to the lungs, may occur.

REFERENCES 1. Hauben E, van den Broek LC, Van Marck E, Hogendoorn PC. Adamantinoma-like Ewing’s sarcoma and Ewing’s-like adamantinoma. The t(11; 22), t(21; 22) status. J Pathol. 2001; 195:218-221.

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2. Moon NF, Mori H. Adamantinoma of the appendicular skeleton–updated. Clin Orthop Relat Res. 1986; 204:215-237. 3. Schajowicz F, Ackerman LV, Sissons HA. Histological Typing of Bone Tumors. International Histological Classification of Tumors, No. 6. Geneva, Switzerland: World Health Organization; 1972. 4. Unni KK, Dahlin DC, Beabout JW, Ivins JC. Adamantinomas of long bones. Cancer. 1974; 34:1796-1805. 5. Huvos AG, Marcove RC. Adamantinoma of long bones. A clinicopathological study of fourteen cases with vascular origin suggested. J Bone Joint Surg Am. 1975; 57:148-154. 6. Czerniak B, Rojas-Corona RR, Dorfman HD. Morphologic diversity of long bone adamantinoma. The concept of differentiated (regressing) adamantinoma and its relationship to osteofibrous dysplasia. Cancer. 1989; 64:2319-2334. 7. Campanacci M. Adamantinoma of the long bones. In: Bone and Soft Tissue Tumors. New York, NY: Springer; 1990:629-638. 8. Hazelbag HM, Taminiau AHM, Fleuren GJ, Hogendoorn PC. Adamantinoma of the long bones. A clinicopathological study of thirty-two patients with emphasis on histological subtype, precursor lesion, and biological behavior. J Bone Joint Surg Am. 1994; 76:1482-1499. 9. 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; 82:1122-1131 10. Rosai J, Pinkus GS. Immunohistochemical demonstration of epithelial differentiation in adamantinoma of the tibia. Am J Surg Pathol. 1982; 6:427-434. 11. Hazelbag HM, Fleuren GJ, van den Broek LJ, Taminiau AH, Hogendoorn PC. Adamantinoma of the long bones: keratin subclass immunoreactivity pattern with reference to its histogenesis. Am J Surg Pathol. 1993; 17:1225-1233. 12. Keeney GL, Unni KK, Beabout JW, Pritchard DJ. Adamantinoma of long bones. A clinicopathologic study of 85 cases. Cancer. 1989; 64:730-737. 13. Knapp RH, Wick MR, Scheithauer BW, Unni KK. Adamantinoma of bone. An electron microscopic and immunohistochemical study. Virchows Archive. A, Pathological Anatomy and Histopathology. 1982; 398:75-86. 14. Mori H, Yamamoto S, Hiramatsu K, Miura T, Moon NF. Adamantinoma of the tibia. Ultrastructural and immunohistochemic study with reference to histogenesis. Clin Orthop Relat Res. 1984; 190:299-310. 15. Elliott GB. Malignant angioblastoma of long bone. So-called “tibial adamantinoma.” J Bone Joint Surg Br. 1962; 44:25-33. 16. Llombart-Bosch A, Ortuno-Pacheco G. Ultrastructural findings supporting the angioblastic

nature of the so-called adamantinoma of the tibia. Histopathology. 1978; 2:189-200. 17. Lederer H, Sinclair AJ. Malignant synovioma simulating “adamantinoma of the tibia.” J Pathol Bacteriol. 1954; 67:163-168. 18. Unni KK. Dahlin’s Bone Tumors: General Aspects and Data on 11,087 Cases. 5th ed. Philadelphia, Pa: Lippincott-Raven; 1996:333-342. 19. Dorfman HD, Czerniak B. Bone Tumors. St Louis, Mo: Mosby; 1998:949-973. 20. Kahn LB. Adamantinoma, osteofibrous dysplasia, and differentiated adamantinoma. Skeletal Radiol. 2003; 32:245-258. 21. Filippou DK, Papadopoulos V, Kiparidou E, Demertzis NT. Adamantinoma of tibia: a case of late local recurrence along with lung metastases. J Postgrad Med. 2003; 49:75-77. 22. 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; 36:1068-1074. 23. Kumar D, Mulligan ME, Levine AM, Dorfman HD. Classic adamantinoma in a 3-yearold. Skeletal Radiol. 1998; 27:406-409. 24. Soucacos PN, Hartofilakidis GK, Touliatos AS, Theodorou V. Adamantinoma of the olecranon. A report of a case with serial metastasizing lesions. Clin Orthop Relat Res. 1995; 310:194-199. 25. van Haelst UJ, de Haas van Dorsser AH. A perplexing malignant bone tumor. Highly malignant so-called adamantinoma or nontypical Ewing’s sarcoma. Virchows Archive. A, Pathological Anatomy and Histopathology. 1975; 365:63-74. 26. Beppu H, Yamaguchi H, Yoshimura N, Atarashi K, Tsukimoto K, Nagashima Y. Adamantinoma of the rib metastasizing to the liver. Intern Med. 1994; 33:441-445. 27. Bourne MH, Wood MB, Shives TC: Adamantinoma of the radius: a case report. Orthopedics. 1988; 11:1565-1566. 28. Dimi LI, Mendonca R, Adamy CA, Saraiva GA. Adamantinoma of the spine: case report. Neurosurgery. 2006; S9:E426. 29. Clarke RP, Leonard JR, von Kuster L, Wesseler TA. Adamantinoma of the humerus with early metastases and death: a case report with autopsy findings. Orthopedics. 1989; 12:1121-1125. 30. 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; 18:746-751. 31. Henneking K, Rehm KE, Schulz A. Adamantinoma of the long tubular bones. Case report of a fibular tumor. Chirurg. 1984 ; 55:407-410. 32. Van Schoor JX, Vallaeys JH, Joos GF, Roels HJ, Pauwels RA, Van Der Straeten ME. Adamantinoma of the tibia with pulmonary metastases and hypercalcemia. Chest. 1991; 100:279-281.

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33. Enneking WF. The staging system for benign and malignant tumors of muskoloskeletal system (Appendix A). In: Clinical Muskoloskeletal Pathology. Gainesville, Fla: University Press of Florida; 1990:451-466. 34. Plump D, Haponik EF, Katz RS, TiptonDonovan A. Primary adamantinoma of rib: thoracic manifestations of a rare bone tumor. South Med J. 1986; 79:352-355. 35. Altmannsberger M, Poppe H, Schauer A. An unusual case of adamantinoma of long bones. J Cancer Res Clin Oncol. 1982; 104:315-320. 36. Brain EC, Raymond E, Goldwasser F, Extra JM, Marty M. Adamantinoma of the proximal end of the tibia. A case. Presse Med. 1994; 23:1522-1526. 37. Garces P, Romano CC, Vellet AD, Alakija P, Schachar NS. Adamantinoma of the tibia: plain-film, computed tomography and magnetic resonance imaging appearance. Can Assoc Radiol J. 1994; 45:314-317. 38. Van der Woude HJ, Hazelbag HM, Bloem JL, Taminiau AH, Hogendoorn PC. MRI of adamantinoma of long bones in correlation with histopathology. AJR Am J Roentgenol. 2004; 183:1737-1744. 39. Johnson LC. Congenital pseudoarthrosis, adamantinoma of long bone, and intracortical fibrous dysplasia of the tibia. J Bone Joint Surg Am. 1972; 54:1355. 40. Hazelbag HM, Fleuren GJ, Cornelisse CJ, van den Broek LJ, Taminiau AH, Hogendoorn PC. DNA aberrations in the epithelial cell component of adamantinoma of long bones. Am J Pathol. 1995; 147:1770-1779. 41. Jundt G, Remberger K, Roessner A, Schulz A,

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Bohndorf K. Adamantinoma of long bones. A histopathological and immunohistochemical study of 23 cases. Pathol Res Pract. 1995; 191:112-120.

long bones. An analysis of nine new cases with emphasis on metastasizing lesions and fibrous dysplasia-like changes. Human Pathol. 1977; 8:141-153.

42. Bovee JV, van den Broek LJ, de Boer WI, Hogendoorn PC. Expression of growth factors and their receptors in adamantinoma of long bones and the implication for its histogenesis. J Pathol. 1998; 184:24-30.

50. Campanacci M, Giunti A, Bertoni F, Laus M, Gitelis S. Adamantinoma of the long bones: The experience at the Instituto Ortopedico Rizzoli. Am J Surg Pathol. 1981; 5:533-542.

43. Kanamori M, Antonescu CR, Scott M, et al. Extra copies of chromosomes 7, 8, 12, 19, and 21 are recurrent in adamantinoma. J Mol Diagn. 2001; 3:16-21.

51. Gebhardt MC, Lord FC, Rosenberg AE, Mankin HJ. The treatment of adamantinoma of the tibia by wide resection and allograft bone transplantation. J Bone Joint Surg Am. 1987; 69:1177-1188.

44. Hazelbag HM, Wessels JW, Mollevangers P, van den Berg E, Molenaar WM, Hogendoorn PC. Cytogenetic analysis of adamantinoma of long bones: further indications for a common histogenesis with osteofibrous dysplasia. Cancer Genet Cytogenet. 1997; 97:5-11.

53. Wang JW, Shih CH, Hsu WW, Chen WJ. Treatment of recurrent adamantinoma of the tibia by wide resection: report of three cases. J Formos Med Assoc. 1993; 92:274-277.

45. Campanacci M, Laus M. Osteofibrous dysplasia of the tibia and fibula. J Bone Joint Surg Am. 1981; 63:367-75.

54. Taylor GI. The current status of free vascularized bone grafts. Clin Plast Surg. 1983; 10:185-209.

46. Benassi MS, Campanacci L, Gamberi G, et al. Cytokeratin expression and distribution in adamantinoma of the long bones and osteofibrous dysplasia of tibia and fibula. An immunohistochemical study correlated to histogenesis. Histopathology. 1994; 25:71-76.

55. Weiland AJ, Moore JR, Daniel RK. Vascularized bone autografts. Experience with 41 cases. Clin Orthop Relat Res. 1983; 174:87-95.

47. Campanacci M. Bone and Soft Tissue Tumors: Clinical Features, Imaging, Pathology and Treatment. Wien, Austria: Springer; 1999. 48. Lokich J. Metastatic adamantinoma of bone to lung? A case report of the natural history and the use of chemotherapy and radiation therapy. Am J Clin Oncol. 1994; 17:157-159. 49. Weiss SW, Dorfman HD. Adamantinoma of

52. Rock MG, Beabout JW, Unni KK, Sim FH. Adamantinoma. Orthopedics. 1983; 6:472.

56. Szendroi M, Renyi-Vamos A, Marschalko P, Minik K, Kiss AL. Behavior of adamantinoma of the long bones based on long-term follow up studies. Magyar Traumatologia Ortopedia Kezsebeszet Plasztikai Sebeszet. 1994; 37:37-44. 57. De Keyser F, Vansteenkiste J, Van Den Brande P, Demedts M, Van de Woestijne KP. Pulmonary metastases of a tibia adamantinoma. Case report and review of the literature. Acta Clin Belg. 1990; 45:31-33.

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cme QUIZ

Adamantinoma of the Long Bones 1.

Instructions

A. Adamantinomas of the long bones are rare tumors accounting for 0.3%-1.0% of all malignant bone tumors. B. Differentiated adamantinomas tend to occur in younger patients. C. The tibial diaphysis is the most common location, with fibular involvement coexisting in 50% of the cases. D. All of the above.

1. Review the stated learning objectives at the beginning of the CME article and determine if these objectives match your individual learning needs. 2. Read the article carefully. Do not neglect the tables and other illustrative materials, as they have been selected to enhance your knowledge and understanding. 3. The following quiz questions have been designed to provide a useful link between the CME article in the issue and your everyday practice. Read each question, choose the correct answer, and record your answer on the CME REGISTRATION FORM at the end of the quiz. 4. Type or print your full name and address and your date of birth in the space provided on the CME Registration Form.

2.

3.

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Which of the following is false regarding imaging of adamantinomas of the long bones? A. Classical adamantinomas usually are intracortical, with evidence of complete cortical destruction and intramedullary and soft-tissue involvement. B. A mixed lytic and sclerotic lesion, periosteal reaction, and the Codman’s triangle usually are observed. C. Moderate to severe anterior bowing deformity is common. D. Differentiated adamantinomas usually involve the anterolateral tibial cortex, with imaging features similar to osteofibrous dysplasia.

7. Your answers will be graded, and you will be advised whether you have passed or failed. Unanswered questions will be considered incorrect. A score of at least 80% is required to pass. If a passing score is achieved, Vindico Medical Education will issue an AMA PRA Category 1™ certificate within 4-6 weeks.

CME ACCREDITATION This activity has been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education through the joint sponsorship of Vindico Medical Education and ORTHOPEDICS. Vindico Medical Education is accredited by the ACCME to provide continuing medical education for physicians. Vindico Medical Education designates this educational activity for a maximum of 1 AMA PRA Category 1 Credit™. Physicians should only claim credit commensurate with the extent of their participation in the activity. This CME activity is primarily targeted to orthopedic surgeons, hand surgeons, head and neck surgeons, trauma surgeons, physical medicine specialists, and rheumatologists. There is no specific background requirement for participants taking this activity.

Clinical manifestations of adamantinomas of the long bones at presentation include: A. A history of trauma or fracture few months or years prior to the onset of symptoms in approximately 60% of patients. B. Dull, aching pain associated with a gradually evolving mass. C. Regional lymph node involvement or distant metastases. D. A and B.

5. Complete the Evaluation portion of the CME Registration Form. Forms and quizzes cannot be processed if the Evaluation portion is incomplete. The Evaluation portion of the CME Registration Form will be separated from the quiz upon receipt at ORTHOPEDICS. Your evaluation of this activity will in no way affect the scoring of your quiz. 6. Send the completed form, with your $15 payment (check or money order in US dollars drawn on a US bank, or credit card information) to: ORTHOPEDICS CME Quiz, PO Box 36, Thorofare, NJ 08086, OR take the quiz on-line. Visit www. ORTHOSuperSite.com for details.

Which of the following is true regarding epidemiology of adamantinomas of the long bones?

4.

Which of the following is true regarding histopathology of adamantinomas of the long bones? A. Adamantinomas show a wide range of morphologic patterns, with a biphasic (epithelial and mesenchymal) origin. B. In immunohistochemical studies, epithelial cells express basal cell type cytokeratins, which are probably the malignant element of the tumor. C. Mesenchymal component is composed of a loose stroma of immature spindle cells producing collagen, resembling osteofibrous dysplasia. D. All of the above.

5.

Differential diagnosis of adamantinomas of the long bones includes: A. B. C. D.

Fibrous and osteofibrous dysplasia. Haemangioendothelioma and haemangiosarcoma of bones. Low-grade chondrosarcoma. A and B.

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cme QUIZ

CME Registration Form Note: $15 payment must accompany this form. Questions about CME and ORTHOPEDICS? Call us at 800-257-8290 or write to: ORTHOPEDICS, PO Box 36, Thorofare, NJ 08086

MARCH 2007

6.

7.

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Which of the following is true regarding biopsy of suspected tibia lesions for adamantinomas of the long bones?

Adamantinoma of the Long Bones Black out the correct answers 1.

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A. Extensive biopsy is recommended from the most radiolucent areas. B. Sampling errors are rare, even with limited biopsy specimens. C. The lesion shows histologic homogeneity. D. B and C.

Number of hours you spent on this activity_____

Current treatment for adamantinomas of the long bones includes:

Deadline for mailing: For credit to be received, the envelope must be postmarked no later than March 31, 2008. If paying by credit card, you may fax your form to (856) 848-6091.

A. Limb salvage and neo-adjuvant chemotherapy. B. Limb salvage with wide surgical resection and intercalary reconstruction, or amputation. C. Limb salvage and adjuvant radiation. D. Limb salvage, chemotherapy, and radiation.

PLEASE PRINT

After surgical resection of adamantinomas of the tibial diaphysis, reconstruction of the bone defect can be achieved by:

Mailing Address

A. B. C. D.

Intercalary allograft. Vascularized fibular autograft. Metallic segmental implant. All of the above.

(reading article and completing quiz)

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City

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EVALUATION (must be completed in order for your CME Quiz to be scored)

9.

Which of the following is false regarding local recurrences of adamantinomas of the long bones? A. Local recurrences usually occur 5-15 years after the primary excision of the tumor. B. Local recurrence depends on the duration of the clinical symptoms and the stage of the tumor. C. Very late local recurrences have been reported. D. Wide resection is also indicated for the recurrent tumors.

10.

Which of the following is false regarding prognosis of adamantinomas of the long bones? A. Late metastases may occur in 10%-30% of the patients. B. Metastases usually are to the lungs, the regional lymph nodes, or the bones. C. The overall 10-year survival rates vary from 82%-87%. D. Poor survival rates are associated with the occurrence of local recurrences, regional lymph nodes, and lung metastases.

MARCH 2007 1.

The content of the article was accurately described by the learning objectives.

Yes

a. Describe the radiographic characteristics of adamantinoma of the long bones. _____ b. List the histological patterns and radiographic characteristics commonly associated with adamantinoma.

2.

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No _____ _____

c. Discuss treatment options for adamantinoma of the long bones.

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d. Discuss the prognosis of patients with adamantinoma.

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This activity will influence how I practice orthopedics.

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_____

a. If you answered yes, list one new thing you learned as a result of this activity___________________________________________________________ 3.

The quiz questions were at an appropriate level for assessing my learning.

4.

Please rate the degree to which the content presented in this activity was free from commercial bias. No bias Significant bias 5 4 3 2 1

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MARCH 2007 | Volume 30 • Number 3

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