Introduction Rare form of fibrous dysplasia that primary affects the tibia and is confined to the cortices also known as ossifying fibroma and Campanacci lesion Epidemiology demographics usually found in younger children (< 10 years old) males>females location primarily in anterior tibia Genetics trisomy 7, 8, 12, 22 have been reported does NOT have Gs alpha activating mutation like fibrous dysplasia has Prognosis lesions usually regress and do not cause problems in adulthood 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 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 Differentials & Groups Tibial diaphysis lesion Treatment is Observation alone (2) Osteofibrous dysplasia • • Fibrous dysplasia • Adamantinoma • Eosinophilic granuloma • Enchondroma / Olliers / Marfucci's • Osteochondroma / MHE • NOF / Jaffe-Campanacci syndrome • Paget's • ASSUMPTIONS: (1) Younger patient is > 40 yrs; (2) assuming aymptomatic and no impending fracture IBank Location Age Xray Xray CT B. Scan MRI MRI Histo(1) Case A tibia 12 yrs. (1) histology does not always correspond to clinical case
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? Review Topic QID: 4364 FIGURES: A B C 1 Osteofibrous dysplasia 77% (3566/4659) 2 Nonossifying fibroma 8% (373/4659) 3 Osteoblastoma 5% (238/4659) 4 Chondroblastoma 3% (149/4659) 5 Ewing's sarcoma 6% (292/4659) ML 2 Select Answer to see Preferred Response PREFERRED RESPONSE 1 (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? Review Topic QID: 4629 FIGURES: A B 1 Observation 61% (1326/2164) 2 Bracing 9% (198/2164) 3 Casting and monthly follow up 2% (50/2164) 4 Curettage and bone grafting 24% (521/2164) 5 Osteotomy with plate application 3% (60/2164) ML 3 Select Answer to see Preferred Response PREFERRED RESPONSE 1 (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? Review Topic QID: 3112 FIGURES: A B C 1 Non-ossifying fibroma 13% (183/1395) 2 Aneurysmal bone cyst 3% (48/1395) 3 Low-grade chondrosarcoma 2% (33/1395) 4 Osteofibrous dysplasia 67% (929/1395) 5 Adamantinoma 14% (198/1395) ML 3 Select Answer to see Preferred Response PREFERRED RESPONSE 4 (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? Review Topic QID: 333 FIGURES: A 1 Fibrous dysplasia 19% (241/1290) 2 Unicameral bone cyst 3% (36/1290) 3 Aneurysmal bone cyst 6% (83/1290) 4 Osteofibrous dysplasia 64% (830/1290) 5 Non-ossifying fibroma 7% (92/1290) ML 3 Select Answer to see Preferred Response PREFERRED RESPONSE 4 Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK
Osteofibrous Dysplasia Pathology - Osteofibrous Dysplasia - Radiology Rounds Rare form of fibrous dysplasia that primary affects the tibia and is confined to... 9/8/2018 71 views