Introduction Overview non-ossifying fibroma (NOF) is a benign fibrogenic lesion that is the most common benign bone tumor in childhood related to dysfunctional ossification other names metaphyseal fibrous defect nonosteogenic fibroma cortical desmoid fibrous cortical defect fibromatosis fibroxanthoma Epidemiology incidence occurs in 30-40% of skeletally immature children demographics more common in males (2:1) common in children 5-15 years old location metaphysis of long bones 80% in lower extremity distal femur > proximal tibia > distal tibia uncommon in proximal femur, proximal humerus Pathophysiology possibly due to abnormal osteoclastic resorption at the subperiosteal level during remodeling of the metaphysis Associated conditions Jaffe-Campanacci syndrome congenital syndrome of multiple non-ossifying fibromas and cafe au lait pigmentation mental retardation heart, eyes, gonads involved neurofibromatosis familial multifocal NOF ABC Prognosis usually spontaneously resolves no malignant or metastatic potential Presentation Symptoms asymptomatic usually found incidentally may present with pathologic fracture Imaging Radiographs diagnostic metaphyseal eccentric "bubbly" lytic lesion surrounded by sclerotic rim cortex may be expanded and thin length > width as bone grows migrates to diaphysis lesions enlarge (1-7cm) as patient approaches skeletal maturity, lesions become sclerotic avulsion of adductor magnus insertion in the posteromedial aspect of the distal femur may produce a similar looking lesion CT quantitative CT shown to be useful in predicting fracture risk Studies Histology classic characteristics are fibroblastic spindle cells in whirled or storiform pattern (helicopter in wheat field) fibroblastic connective tissue background numerous lipophages and giant cells hemosiderin pigmentation occasional ABC component Differential Giant cell tumor painful rare in skeletally immature no mineralization Osteosarcoma painful irregular zone of bony destruction with less defined zone of transition periosteal reaction mineralized soft tissue mass Treatment Nonoperative observation indications first line of treatment most lesions resolve spontaneously and progressively reossify as child enters 2nd and 3rd decade of life technique radiographs at 6 and 12 months, then annually until reossified casting indication pathologic fracture can be treated as per the fracture alone (long leg casting for distal femur pathologic fx) Operative curettage and bone grafting indication symptomatic and large lesion (> 50-75% cortical involvement) increased risk of fracture shown on quantitative CT Complications Pathologic fracture incidence 90% occur in the lower extremity 50% occur in the distal tibia risk factors >50% involvement of transverse diameter >33mm length in weight-bearing bones (femur and tibia) treatment cast immobilization indications nondisplaced fractures Differentials & Groups "Bubbly" lytic lesion on xray Hemosiderin seen on Histology Treatment is Observation alone (1) NOF • • • ABC • UBC • PVNS • Fibrous dysplasia • Enchondroma • Osteochondroma • Eosinophillic granuloma • Paget's • ASSUMPTIONS: (1) assuming aymptomatic and no impending fracture IBank Location Xray Xray CT B. Scan MRI MRI Histo(1) Case A multiple Case B femur Case C tibia Case D tibia Case E femur Case F tibia with fx Case G tibia Case H tibia (1) - histology does not always correspond to case
QUESTIONS 1 of 17 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 Previous Next Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ07.43) A 6-year-old boy falls off the monkey bar and presents to the emergency room with an abrasion on his knee and mild knee pain. He is able to bear weight without discomfort and has full range of knee motion. A plain radiograph is shown in Figure A. What is the most appropriate next step in management? Review Topic QID: 704 FIGURES: A 1 CBC, ESR, CRP with bone aspiration for gram stain and culture 2% (37/1891) 2 Biopsy with neoadjuvant chemotherapy followed by limb salvage surgical resection and adjuvant chemotherapy 2% (37/1891) 3 Repeat radiographs in 3 months 90% (1696/1891) 4 Biopsy with external beam irradiation followed by limb salvage surgical resection 0% (8/1891) 5 MRI and CT scan of the chest 5% (101/1891) ML 1 Select Answer to see Preferred Response PREFERRED RESPONSE 3 (OBQ12.273) An 18-year-old male presents with leg pain after tripping during a soccer game. He has no history of leg pain or trauma. Which of the following is the most likely diagnosis and recommended treatment for the finding seen in his radiograph in figure A? Review Topic QID: 4633 FIGURES: A 1 Enchondroma, observation 4% (187/4238) 2 Enchondroma, surgical biopsy 1% (50/4238) 3 Nonossifying fibroma, observation 90% (3800/4238) 4 Nonossifying fibroma, surgical biopsy 1% (49/4238) 5 Aneurysmal bone cyst, curettage and bone grafting 3% (134/4238) ML 1 Select Answer to see Preferred Response PREFERRED RESPONSE 3 (OBQ06.96) A 20-year-old man falls while skiing and complains of knee pain. When he presents to the office 2 weeks later his physical exam is normal and his pain has resolved. Radiographs are shown in Figures A & B. What is the next most appropriate step in management? Review Topic QID: 207 FIGURES: A B 1 Reassurance and weightbearing as tolerated 86% (980/1137) 2 Needle biopsy 6% (70/1137) 3 Incisional biopsy 6% (65/1137) 4 Excisional biopsy 1% (14/1137) 5 Wide resection and reconstruction 0% (3/1137) ML 1 Select Answer to see Preferred Response PREFERRED RESPONSE 1 (OBQ05.252) A 14-year-old child is referred to your office for evaluation of a tibia lesion found incidentally after a minor ankle injury. A radiograph of the child's ankle is shown in Figure A. What treatment do you suggest? Review Topic QID: 1138 FIGURES: A 1 Endocrine consultation secondary to associated endocrine abnormalities 1% (6/418) 2 Surgical consultation secondary to associated gastrointestional cancers 1% (3/418) 3 Short leg cast and non-weight bearing for a minimum of 6 weeks 2% (7/418) 4 Open biopsy and tumor staging 8% (34/418) 5 Routine followup of tibial lesion 87% (363/418) ML 1 Select Answer to see Preferred Response PREFERRED RESPONSE 5 (OBQ10.112) A 9-year-old boy injures his ankle while jumping on a trampoline and cannot bear weight on the extremity. A radiograph taken in the emergency room is displayed in Figure A. A biopsy of this lesion would most likely be consistent with which of the following histology slides? Review Topic QID: 3206 FIGURES: A B C D E F 1 Figure B 3% (48/1757) 2 Figure C 7% (122/1757) 3 Figure D 5% (87/1757) 4 Figure E 34% (590/1757) 5 Figure F 51% (896/1757) ML 4 Select Answer to see Preferred Response PREFERRED RESPONSE 5 (OBQ08.143) You are asked to see an 16-year-old patient by his pediatrician after a lesion is found in the child's distal fibula by radiographs taken for a sprained ankle (Figure A). The child is otherwise healthy, active, and has no pain or limitation of motion. Your management should consist of: Review Topic QID: 529 FIGURES: A 1 Non-weight bearing short leg cast 1% (18/1445) 2 Tumor staging including chest CT, bone scan, MRI of entire bone 1% (17/1445) 3 Contacting local child protective services 0% (3/1445) 4 Activities as tolerated, repeat radiographs in 3 to 6 months 94% (1360/1445) 5 Curettage and allograft bone packing to lesion. 3% (37/1445) ML 1 Select Answer to see Preferred Response PREFERRED RESPONSE 4 Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK
Upgrade to View Premium Videos Upgrade to View Premium Videos Pediatric Benign Bone Tumors (The Importance & Challenges Of Diagnosis) - Dr. Chappie Conrad Derek Moore Pathology - Non-Ossifying Fibroma 4/4/2015 937 views