Updated: 3/27/2017

Adamantinoma

Topic
Review Topic
0
0
Questions
8
0
0
Evidence
3
0
0
Cases
1
https://upload.orthobullets.com/topic/8048/images/Case A - tibia - xray b - parsons_moved.jpg
https://upload.orthobullets.com/topic/8048/images/Case A - Tibia - T1 - parsons_moved.jpg
https://upload.orthobullets.com/topic/8048/images/q22-2b[1]_moved.jpg
https://upload.orthobullets.com/topic/8048/images/Case B - tibia - xray - parsons_moved.png
https://upload.orthobullets.com/topic/8048/images/Histology A_moved.jpg
https://upload.orthobullets.com/topic/8048/images/adamantinoma histo.jpg
Introduction
  • Rare low-grade malignant tumor of unknown etiology that is almost always located in the mid-tibia 
  • Epidemiology
    • incidence
      • less than 300 cases have been documented
    • demographics
      • occurs in young adults (20 - 40 years of age)
  • Associated conditions
    • osteofibrous dysplasia
      • historically, it was thought that osteofibrous dysplasia (OFD) was a precursor to this adamantinoma, however current studies have cast doubt on this theory
  • Prognosis
    • may metastasize to lungs (25%), therefore long-term followup is recommended
    • recurrence is uncommon with negative margin excision
Presentation
  • Symptoms
    • pain of months to years duration
  • Physical exam
    • bowing deformity or a palpable mass of tibia is common
Imaging
  • Radiographs
    • multiple sharply circumscribed lucent lesions ("soap bubble" appearance) with interspersed sclerotic bone in mid tibia
      • some lesions may destroy cortex
    • may see bowing of the tibia
    • radiographic evolution of lesions is helpful in the diagnosis as lesions may continue to grow and erode thru the cortex
    • unlike other primary bone tumors, adamantinoma typically shows no periosteal reaction
Studies
  • Histology
    • biphasic 
    • contains both epithelial and fibrous mesenchymal cells
      • nests of epithelial-like cells arranged in palisading or glandular pattern   
        • stain for keratin
      • background of fibrous stroma
Treatment
  • Operative
    • wide-margin surgical resection  
      • indications
        • standard of care in most patients
      • techniques
        • often requires intercallary resection with allograft or intercallary megaprosthesis reconstruction
        • as adamantinoma is a low-grade malignancy, radiotherapy and/or chemotherapy is not typically used for local control of disease
Differentials & Treatment Groups
  • Osteofibrous dysplasia
    • differentiating between osteofibrous dysplasia and adamantinoma is critical
      • osteofibrous dysplasia is benign and  treated with observation
      • adamantinoma is malignant and treat with surgical resection
 
 
Tibial diaphysis lesion
     
Treated with wide-resection alone (2)
   
Adamantinoma
     
   
Osteofibrous dysplasia
           
Chondrosarcoma        
   
Parosteal osteosarcoma        
   
ASSUMPTIONS: (1) Older patient is > 40 yrs; (2) assuming no impending fracture
 
  Location
Xray
Xray
CT
B. Scan
MRI
MRI
Histo(1)

Case A

tibia
   

Case B

tibia
   

Case C

tibia
   

Case D

tibia  
   
(1) - histology does not always correspond to clinical case
 

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Questions (8)

(SAE08PA.43) A 30-year-old woman has had pain in her right leg for the past 6 months. A lytic lesion is noted in the anterior cortex of the midtibia, extending 5 cm in length without a soft-tissue mass. A radiograph and a biopsy specimen are shown in Figures 35a and 35b. What is the preferred treatment? Review Topic

QID: 6304
FIGURES:
1

Debridement and IV antibiotics

5%

(4/76)

2

Wide resection of the lesion

51%

(39/76)

3

Chemotherapy alone

14%

(11/76)

4

Observation

21%

(16/76)

5

Amputation

8%

(6/76)

ML 4

Select Answer to see Preferred Response

PREFERRED RESPONSE 2
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(OBQ12.31) A 28-year-old male presents for evaluation of leg pain. He denies trauma, and is otherwise healthy. A lateral radiograph of the affected leg is shown in Figure A. A biopsy is taken, and the low and high power histology specimens are shown in Figures B and C. Which of the following should be offered as definitive treatment of this lesion? Review Topic

QID: 4391
FIGURES:
1

Continued observation

5%

(203/3777)

2

Radiation and chemotherapy

7%

(248/3777)

3

Bracing to prevent tibial deformity and pathologic fracture

8%

(309/3777)

4

Immediate above the knee amputation

3%

(130/3777)

5

En bloc resection with wide margins followed by appropriate reconstruction

76%

(2854/3777)

ML 2

Select Answer to see Preferred Response

PREFERRED RESPONSE 5
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(OBQ11.122) A 21-year-old female presents with 7 years of leg pain and deformity. Radiographs from when she first noticed the lesion at age 14 are shown in Figures A and B. Current radiographs are shown in Figures C and D. Current MRI and histology section are shown in Figures E & F. What is the most likely diagnosis? Review Topic

QID: 3545
FIGURES:
1

Osteofibrous dysplasia

25%

(331/1318)

2

Adamantinoma

57%

(751/1318)

3

Chronic osteomyelitis

1%

(16/1318)

4

Periosteal osteosarcoma

2%

(29/1318)

5

Fibrous dysplasia

14%

(186/1318)

ML 3

Select Answer to see Preferred Response

PREFERRED RESPONSE 2

(OBQ07.42) A 24-year-old male presents with pain in his tibia. Radiographs are shown in Figure A. Histology is shown in Figure B. What is the most likely diagnosis? Review Topic

QID: 703
FIGURES:
1

Ewing's sarcoma

6%

(21/343)

2

Adamantinoma

78%

(268/343)

3

Osteosarcoma

7%

(25/343)

4

Osteoblastoma

5%

(16/343)

5

PNET

3%

(11/343)

ML 1

Select Answer to see Preferred Response

PREFERRED RESPONSE 2
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ARTICLES (5)
CASES (1)
Topic COMMENTS (9)
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