summary Osteofibrous Dysplasia is a rare benign tumor-like condition that primarily affects the cortices of the tibia. The condition typically presents in patients less than 10 years of age with painless anterior shin swelling. Diagnosis is made by a combination of radiographs showing an anterior eccentric lytic tibial lesion and biopsy showing fibroblast proliferation surrounding islands of woven bone with osteoblastic rimming. Treatment is usually observation alone as lesions usually regress and do not cause problems in adulthood. Epidemiology Demographics usually found in younger children (< 10 years old) males>females Anatomic location primarily in anterior tibia Etiology Genetics trisomy 7, 8, 12, 22 have been reported does NOT have Gs alpha activating mutation like fibrous dysplasia has Presentation Symptoms may be asymptomatic painless swelling anterior or anterolateral bowing of the tibia pseudoarthrosis develops in 10-30% of patients Physical exam may have local tenderness over the tibia Imaging Radiographs recommended views AP and lateral of affected area findings anterior eccentric lytic tibial lesion in child that often leads to tibial bowing usually diaphyseal no periosteal reaction confined to anterior cortex radiographic differential includes adamantinoma Studies Histology histology similar to fibrous dysplasia EXCEPT osteoblastic rimming is present fibroblast proliferation surrounding islands of woven bone with osteoblastic rimming mitotic figures are common may have giant cells Differential Adamantinoma Osteofibrous Dysplasia Differential Tibial diaphysis lesion Treatment is Observation alone Osteofibrous dysplasia • • Fibrous dysplasia • Adamantinoma • Eosinophilic granuloma • Enchondroma / Olliers / Maffucci's • Osteochondroma / MHE • NOF /Jaffe-Campanacci syndrome • Paget's • Treatment Nonoperative observation indication first line of treatment alone is the treatment for most patients bracing indication if deformity significant and interfering with walking Operative deformity correction with osteotomy indication rarely needed significant deformity perform after skeletal maturity Prognosis Lesions usually regress and do not cause problems in adulthood
QUESTIONS 1 of 5 1 2 3 4 5 Previous Next (OBQ12.4) A 9-year-old male presents with a mass on his lower leg. He denies any history of trauma, and is otherwise healthy. A current radiograph of the affected leg is shown in Figure A. A biopsy of the lesion is obtained, and is shown in Figures B and C. What is the most likely diagnosis in this patient? QID: 4364 FIGURES: A B C Type & Select Correct Answer 1 Osteofibrous dysplasia 77% (4676/6057) 2 Nonossifying fibroma 8% (460/6057) 3 Osteoblastoma 5% (295/6057) 4 Chondroblastoma 3% (190/6057) 5 Ewing's sarcoma 6% (383/6057) L 2 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 1 Review Tested Concept Review Full Topic (OBQ12.269) A 10-year-old male presents for evaluation of a painless deformity of his lower leg. Based on the radiograph and histology slide shown in Figures A and B, what is the next best step for this patient? QID: 4629 FIGURES: A B Type & Select Correct Answer 1 Observation 64% (1904/2979) 2 Bracing 8% (240/2979) 3 Casting and monthly follow up 2% (65/2979) 4 Curettage and bone grafting 23% (678/2979) 5 Osteotomy with plate application 3% (78/2979) L 3 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 1 Review Tested Concept Review Full Topic (OBQ10.24) A 12-year-old female complains of a prominence below her left knee. It is tender when bumped, but painless at rest and with everyday activities. She denies fatigue, weight loss, and any other masses. Physical exam is notable for mild tenderness with firm palpation of the bump. She has full painless range of motion of the knee and ankle. A radiograph, CT scan, and histology slide are provided in Figures A, B, and C respectively. Which of the following is the most likely diagnosis? QID: 3112 FIGURES: A B C Type & Select Correct Answer 1 Non-ossifying fibroma 14% (269/1926) 2 Aneurysmal bone cyst 5% (90/1926) 3 Low-grade chondrosarcoma 2% (45/1926) 4 Osteofibrous dysplasia 65% (1253/1926) 5 Adamantinoma 14% (263/1926) L 3 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ06.147) A 3-year-old boy was referred to you for evaluation of a bowing deformity of his leg and a persistent limp. Radiograph is shown. What is the most likely diagnosis? QID: 333 FIGURES: A Type & Select Correct Answer 1 Fibrous dysplasia 18% (350/1894) 2 Unicameral bone cyst 3% (54/1894) 3 Aneurysmal bone cyst 7% (126/1894) 4 Osteofibrous dysplasia 65% (1230/1894) 5 Non-ossifying fibroma 7% (125/1894) L 3 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic
All Videos (0) Podcasts (1) Pathology⎪Osteofibrous Dysplasia Orthobullets Team Pathology - Osteofibrous Dysplasia Listen Now 5:23 min 6/16/2020 100 plays 5.0 (1)