Updated: 6/22/2021

Adamantinoma

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Flashcards
4
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Questions
8
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Evidence
6
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Cases
1
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Images
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/Case B - tibia - xray - parsons_moved.png
https://upload.orthobullets.com/topic/8048/images/Case A - tibia - xray a - parsons_moved.jpg
https://upload.orthobullets.com/topic/8048/images/Case A - femur - xray b - Parsons_moved.gif
https://upload.orthobullets.com/topic/8048/images/Case A - femur - xray a - Parsons_moved.gif
https://upload.orthobullets.com/topic/8048/images/Case B - tibia - xray B - Parsons_moved.png
https://upload.orthobullets.com/topic/8048/images/Case A - femur - MRI T1 - Parsons_moved.gif
https://upload.orthobullets.com/topic/8048/images/q22-2b[1]_moved.jpg
https://upload.orthobullets.com/topic/8048/images/Histology A_moved.jpg
https://upload.orthobullets.com/topic/8048/images/adamantinoma histo.jpg
  • summary
    • Adamantinomas are rarelow-grade malignant tumors of unknown etiology that are almost always located in the diaphysis of the mid-tibia.
    • The condition usually presents in patients who are between 20 and 40 years old with regional pain and a palpable mass.
    • Diagnosis is made with a biopsy showing nests of epithelial-like cells arranged in palisading or glandular pattern, in a background of fibrous stroma.
    • Treatment is usually wide-margin surgical resection.
  • Epidemiology
    • Incidence
      • less than 300 cases have been documented
    • Demographics
      • occurs in young adults (20 - 40 years of age)
    • Anatomic location
      • almost always located in mid-tibia 
  • Etiology
    • Pathophysiology
      • unknown
    • 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
  • 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
  • Differential
    • 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
    • Differential diagnosis of Adamantinoma
      Tibial diaphysis lesion
      Treated with wide-resection alone
      Adamantinoma
      Osteofibrous dysplasia
      Chondrosarcoma
      Parosteal osteosarcoma
  • 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
  • Prognosis
    • May metastasize to lungs (25%), therefore long-term followup is recommended
    • Recurrence is uncommon with negative margin excision

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Flashcards (4)
Cards
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Questions (8)
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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?

QID: 4391
FIGURES:
1

Continued observation

5%

(237/4364)

2

Radiation and chemotherapy

6%

(271/4364)

3

Bracing to prevent tibial deformity and pathologic fracture

8%

(357/4364)

4

Immediate above the knee amputation

3%

(148/4364)

5

En bloc resection with wide margins followed by appropriate reconstruction

76%

(3314/4364)

L 2 B

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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?

QID: 3545
FIGURES:
1

Osteofibrous dysplasia

26%

(407/1569)

2

Adamantinoma

56%

(881/1569)

3

Chronic osteomyelitis

1%

(20/1569)

4

Periosteal osteosarcoma

3%

(41/1569)

5

Fibrous dysplasia

14%

(215/1569)

L 3 C

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(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?

QID: 6304
FIGURES:
1

Debridement and IV antibiotics

4%

(13/328)

2

Wide resection of the lesion

62%

(204/328)

3

Chemotherapy alone

10%

(33/328)

4

Observation

16%

(51/328)

5

Amputation

8%

(25/328)

L 4 E

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(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?

QID: 703
FIGURES:
1

Ewing's sarcoma

5%

(34/692)

2

Adamantinoma

80%

(552/692)

3

Osteosarcoma

7%

(50/692)

4

Osteoblastoma

4%

(26/692)

5

PNET

4%

(25/692)

L 1 D

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Evidence (6)
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CASES (1)
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