summary Chondromyxoid Fibromas are rare, benign, chondrogenic lesions characterized by variable amounts of chondroid, fibromatoid, and myxoid elements that are most commonly found in the metaphysis of long bones. Patients typically present between the ages of 10 and 30 with regional pain and swelling. Diagnosis is made with biopsy showing hypercellular area with lobules of fibromyxoid tissue and myxoid stroma with stellate cells. Treatment is usually intralesional curettage and bone grafting (or PMMA). Epidemiology Demographics more common in males most common in second and third decades of life may affect patients up to 75 years old Anatomic location long bones (ie. tibia, distal femur) often affects metaphyseal (proximal tibia) regions pelvis feet or hands Etiology Pathophysiology may arise from physeal remnants Genetics mutations a genetic rearrangement may affect chromosome 6 (postion q13) Presentation History long standing pain (months to years) may be incidentally identified Symptoms pain and mild swelling Imaging Radiographs findings lytic, eccentric metaphyseal lesion sharply demarcated from adjacent bone scalloped and sclerotic rim calcifications are rare cortical expansion may be seen lesion size may range from two to ten centimeters MRI findings low signal on T1-weighted images high signal on T2-weighted images Bone scan findings increased signal uptake will be seen Studies Histology findings low-power biphasic appearance hypercellular area with lobules of fibromyxoid tissue spindle-shaped cells or stellate-shaped cells the cells contain hyperchromatic nuclei multinucleated giant cells and fibrovascular tissue are located between lobules hypocellular area with chondroid material high power myxoid stroma with stellate cells regions of pleiomorphic cells with bizarre nuclei may be seen Diagnostic criteria histopathologic examination is mandatory for confirmation of the diagnosis Differential Radiographic aneurysmal bone cyst (ABC) chondroblastoma non-ossifying fibroma Histologic chondroblastoma enchondroma chondrosarcoma Treatment Operative intralesional curretage and bone grafting (or PMMA) indications mainstay of treatment Complications Recurrence occurs in 25% of cases Prognosis Natural history recurrence in CMF is not uncommon may occur in 20-30% of cases Negative prognostic variables children tumor is more lobulated with abundant myxoid material Metastasis has not been reported
QUESTIONS 1 of 4 1 2 3 4 Previous Next (OBQ12.133) A 12-year-old female presents for evaluation of worsening right wrist pain. She denies any trauma and is otherwise healthy. A current radiograph is shown in Figures A and B. A biopsy is obtained and shown in Figure C. What is the most likely diagnosis? QID: 4493 FIGURES: A B C Type & Select Correct Answer 1 Chondrosarcoma 5% (221/4437) 2 Osteosarcoma 2% (91/4437) 3 Chondromyxoid fibroma 49% (2181/4437) 4 Nonossifying fibroma 33% (1457/4437) 5 Enchondroma 10% (459/4437) L 4 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic 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
All Videos (0) Podcasts (1) Pathology⎜Chondromyxoid Fibroma Team Orthobullets 4 Pathology - Chondromyxoid Fibroma Listen Now 5:36 min 10/21/2019 142 plays 5.0 (2)