eMedicine Specialties > Radiology > Musculoskeletal

Adamantinoma

Christopher D Smelser, DO, Staff Physician, Department of Radiology, William Beaumont Army Medical Center
Robert D Stoffey, DO, Director of Women's Imaging, Department of Radiology, Chief of Mammography Section, William Beaumont Army Medical Center; B Wade Mahaney, MD, Staff Physician, Department of Radiology, William Beaumont Army Medical Center; Sean C Keenan, MD, Staff Physician, Department of Radiology, William Beaumont Army Medical Center; Gary E Simmons, MD, Chief, Department of Radiology, William Beaumont Army Medical Center
Contributor Information and Disclosures

Updated: Mar 19, 2008

Introduction

Background

Adamantinoma is a rare tumor, and its origin remains controversial. Fischer first described the tumor in 1913,7 and since then, only approximately 200 cases have been reported. The tumor occurs almost exclusively in the long bones; tumors in the tibia account for more than 80% of cases. The diaphyseal region is the area most commonly affected.

Adamantinomas are classified into 2 distinct types: classic and differentiated. Classic adamantinomas usually occur in patients older than 20 years, whereas differentiated adamantinomas occur almost exclusively in patients younger than 20 years. In addition, the 2 classifications of adamantinomas have distinct radiographic and histologic differences.

Patients with adamantinomas present with variable signs and symptoms; most commonly, they report pain and swelling. The tumor is slow growing, and patients may describe discomfort lasting months to years. Although ascertaining accurate mortality statistics is difficult because of the extremely rare nature of this tumor, the 10-year survival rate is believed to be 10%. Treatment options for adamantinoma are surgical and include either marginal or en bloc resection. Unfortunately, neither radiation therapy nor chemotherapy has been proven effective in the treatment of this insidious tumor.1, 2

Pathophysiology

Although adamantinomas can cause a variety of pathologic and histologic abnormalities, the tumors are usually lobular and well defined. Generally, adamantinomas remain intracortical; however, when prior surgery has been performed or when the tumor is of the classic type, the lesions may extend beyond the periosteum.

The histologic features of adamantinomas have a multitude of variations that are present not only among patients but also among different areas of the same tumor. The histologic features of differentiated adamantinomas are predominantly characterized by a pattern of osteofibrous dysplasia. Conversely, in classic adamantinomas, the cells form a tubular, basaloid, squamoid, or spindled pattern. As a result of the variety of manifestations of the tumor, multiple biopsies must often be performed to obtain a representative sample for study.

Adamantinomas typically range from 3-15 cm in size, and metastases occur in approximately 15-20% of patients. Metastases generally appear in the lungs and local lymph nodes. Adamantinomas can be found in many other bones in addition to their usual location in the tibia. The humerus, ulna, femur, fibula, and radius are other possible locations in the long bones. Occasionally, the tumor occurs in the spine. Adamantinomas are locally aggressive and extremely slow growing.

Related eMedicine topics:
Osteofibrous Dysplasia

Related Medscape topics:
CME Surgery More Effective Than Nonsurgical Treatment for Spinal Stenosis
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Frequency

United States

Because only approximately 200 well-documented cases of adamantinoma have been identified in the almost 100 years since the tumor has been classified, it can be described only as extremely rare.

International

Adamantinomas are extremely rare.

Mortality/Morbidity

Despite their slow-growing nature, adamantinomas can cause significant morbidity and mortality. The 10-year survival rate is estimated to be approximately 10%. In addition, painful bowing deformity of the tibia is not an infrequent occurrence in this condition.

Race

To the authors' knowledge, no racial comparative studies have been performed because of the infrequent occurrence of the tumor and because of the difficult and technologically sophisticated diagnosis. Knowing how many cases have been missed in the less developed regions of the world is impossible, just as knowing how many cases remain undiagnosed in industrialized Western nations is difficult.

Sex

Adamantinomas have a male predominance, with an approximate male-to-female ratio of 1.25:1.

Age

The more common classic adamantinoma usually occurs in individuals older than 20 years, whereas the differentiated form of the tumor almost exclusively occurs in those younger than 20 years. However, the tumors have occurred in individuals aged 10-70 years.3

Anatomy

The tibia is by far the most commonly affected bone; tibial tumors account for 80-85% of primary adamantinomas. Other long bones are also commonly affected; occasionally, primary involvement of the spine occurs.

Presentation

Patients present with complaints such as a dull ache or bone pain that has been present for months to years. Another common complaint is localized swelling. Often, a history of trauma may be elicited. A palpable mass may be firm or soft; its diameter is usually in the range of 3-15 cm.4

Preferred Examination

Plain radiography, CT, and MRI may all be used to help assess suspected adamantinomous tumors. However, CT and MRI are not specific in the differentiation of this tumor from other conditions; findings often overlap with those of other tumors and tumor-like lesions. A variety of tumors and tumor-like lesions can mimic an adamantinoma. Histologic examination is key to the identification of an adamantinoma; the histologic features of these tumors have many variations.5, 6

Limitations of Techniques

Limitations of plain-film radiography include the relatively long list of differential diagnoses for adamantinoma. Many pathologic conditions that are as rare as or more common than adamantinoma demonstrate similar characteristics on plain radiographs, as well as CT scans and MRIs. This fact, coupled with the limited experience that most radiologists (and physicians in general) have in dealing with this tumor, makes the diagnosis and treatment of adamantinomas challenging.

Differential Diagnoses

Aneurysmal Bone Cyst
Chondromyxoid Fibroma
Chondrosarcoma
Fibrous Dysplasia

Other Problems to Be Considered

Eosinophilic granuloma
Hemangioendothelioma
Nonossifying or ossifying fibroma
Osteofibrous dysplasia
Simple bone cyst

Contents

Overview: Adamantinoma
Imaging: Adamantinoma
Follow-up: Adamantinoma
Multimedia: Adamantinoma

References

  1. Dorfman HD, Czerniak Bone Tumors Mosby-Year Book;1998:949-73

  2. Kitsoulis P, Charchanti A, Paraskevas G, Marini A, Karatzias G Adamantinoma Acta Orthop Belg Aug 2007;73(4):425-31 [Medline]

  3. Gleason BC, Liegl-Atzwanger B, Kozakewich HP, Connolly S, Gebhardt MC, Fletcher JA, et al Osteofibrous Dysplasia and Adamantinoma in Children and Adolescents: A Clinicopathologic Reappraisal Am J Surg Pathol Mar 2008;32(3):363-376 [Medline]

  4. Jain D, Jain VK, Vasishta RK, Ranjan P, Kumar Y Adamantinoma: A clinicopathological review and update Diagn Pathol Feb 15 2008;3(1):8 [Medline]

  5. Resnick D Diagnosis of Bone and Joint Disorders Vol 6 Philadelphia, PA: W. B. Saunders Co;1995:3882-4

  6. Taveras JM Radiology, Diagnosis, Imaging Intervention Vol 5 Philadelphia, PA: Lippincott Williams & Wilkins;1993.

  7. Fischer B Uber ein primares Adamantinom der Tibia 12 Frankfurt: Zeitschr. f. Path.; 1913:422-441

Further Reading

Keywords

long bone tumors, classic adamantinomas, differentiated adamantinomas, adamantinomous tumors, primary adamantinomas

Contributor Information and Disclosures

Author

Christopher D Smelser, DO, Staff Physician, Department of Radiology, William Beaumont Army Medical Center
Christopher D Smelser, DO is a member of the following medical societies: American Osteopathic Association
Disclosure: Nothing to disclose

Coauthor

Robert D Stoffey, DO, Director of Women's Imaging, Department of Radiology, Chief of Mammography Section, William Beaumont Army Medical Center
Robert D Stoffey, DO is a member of the following medical societies: American College of Radiology, American Medical Association, American Osteopathic Association, American Roentgen Ray Society, and Radiological Society of North America
Disclosure: Nothing to disclose

B Wade Mahaney, MD, Staff Physician, Department of Radiology, William Beaumont Army Medical Center
Disclosure: Nothing to disclose

Sean C Keenan, MD, Staff Physician, Department of Radiology, William Beaumont Army Medical Center
Sean C Keenan, MD is a member of the following medical societies: American Medical Association
Disclosure: Nothing to disclose

Gary E Simmons, MD, Chief, Department of Radiology, William Beaumont Army Medical Center
Gary E Simmons, MD is a member of the following medical societies: American Roentgen Ray Society, Radiological Society of North America, and Society of Skeletal Radiology
Disclosure: Nothing to disclose

Medical Editor

Michael A Bruno, MD, Associate Professor, Departments of Radiology and Medicine, Pennsylvania State University College of Medicine; Director, Radiology Quality Management Services, Milton S Hershey Medical Center, Pennsylvania State University College of Medicine
Michael A Bruno, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, Association of University Radiologists, Radiological Society of North America, Society of Nuclear Medicine, and Society of Skeletal Radiology
Disclosure: Nothing to disclose

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose

Managing Editor

Murali Sundaram, MBBS, FRCR, FACR, Consulting Staff, Department of Diagnostic Radiology, The Cleveland Clinic Foundation
Disclosure: Nothing to disclose

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose

Chief Editor

Felix S Chew, MD, MBA, EdM, Professor, Department of Radiology, Vice Chairman for Radiology Informatics, Section Head of Musculoskeletal Radiology, University of Washington
Felix S Chew, MD, MBA, EdM is a member of the following medical societies: American Roentgen Ray Society, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose

 
 
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