tumors arising from nerve tissue & fat tissue in bones
TRANSCRIPT
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NEUROFIBROMAS NEUROFIBROMATOSIS
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Benign peripheral nerve sheath tumours. Peak age......... 20-30yrs No sex predilection
3 types Localized Diffuse Plexiform
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Most common, 90% Solitary, not associated with NF 1 Affects superficial cutaneous nerves Slow growing <5cm in size at presentation
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Children, young adults. Solitary, not associated with NF 1. Located within s/c tissue of head & neck. Cause plaque like elevation of skin with
thickening of entire sub cutis.
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Diffuse involvement of long nerve segment and its branches, extending beyond epineurium into surrounding tissue
Pathognomic of NF 1 Early childhood Malignant transformation
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Neurofibroma arising in the obturator ring has caused considerable pressure erosion of both right pubic rami, particularly the superior one. The margins of the pressure defect are sharply defined.
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Two large ovoid neurofibromas (arrows) are shown as filling defects within the thecal sac at the L4/5 level
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Majority solitary Neurofibromatosis Multiple neurofibromas...diagnostic of NF 1
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CT Well defined, hypodense mass Little or no contrast enhancement
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Multiple paravertebral neurofibromas which are hypodense, well defined and enlarge neural foramina
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ct shows contrast enhancing soft tissue mass parietal and occipital on the left side. Bone window images show destruction and deformity of the occipital bone due to biopsy proven neurofibroma
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MRI T1: hypointense T2: hyperintense
▪ Target sign
T1 post contrast: heterogeneous enhancementPLEXIFORM: large lobulated and conglumerated
masses extending along nerves and its branches
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T1w axial through cervical spine shows a low signal intensity mass extruding through right foraminal canal at c5-c6 level, eroding the vertebral body.
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T2w hyperintense neurofibroma
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Multisystem neurocutaneous disorder Most common phakomatosis Most common inherited CNS disorder,
autosomal dominated disorder 1:3000, M=F 50% inherited, 50% spontaneous
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>6 cafe au lait spots Neurofibromas
2 cutaneous or 2 subcutaeous or 2 nodular or 1 plexiform
Optic nerve glioma Distinctive osseous lesions Sphenoid wing dysplasia Two or more iris hamartomas (Lisch nodules) Axillary or inguinal freckling Primary relative with NF1
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Cutaneous neurofibromata
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Subcutaneous neurofibroma
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Optic nerve glioma Soft tissue density lobulated lesion encasing the left optic nerve
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Plain radiograph of forearm of 15 yr old boy shows hypoplasia and deformity of radius and ulna. intramedullary sclerotic streaks in radius and periosteal deformity
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sphenoid wing dysplasia ,t2
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NF1 gene locus is on 17q11.2 and the gene product is neurofibromin, act as a tumour suppressor.
Tumour is primarily a hamartomatus disorder involving ectoderm and mesoderm
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Kyphoscoliosis Posterior vertebral scalloping Posterior hypoplastic elements Enlarged neural foramina Ribbon rib deformity, rib notching, dysplasia Dural ectasia Tibial pseudoarthrosis, bony dysplasia affecting
tibia Severe bowing Multiple NOF Limb hemihypertrophy
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Kyphoscoliosis Combination of outward curvature and lateral curvature
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Posterior vertebra scalloping
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Ribbon ribs
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Sagittal T1WI unenhanced scan in a28 yr old women shows dural ectasia and a large lobulated anterior sacral meningocele.
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Pseudarthroses of the tibia and fibula shown in an infant. Bowing of bone and absence of any evidence of bone repair are typical.
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Axial sonogram through right iliac fossa in 28 YR OLD man with nf1 shows a dumbbell shape neurofibroma
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solitary neurofibroma displacing the femoral vessels there is clear mesenchymal dysplasia, with extensive abnormalities of subcutaneous tissue and hemihypertrophy.
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CNS: dysplasias,hamartomas,neoplasms OCCULAR: buphthalmos GI: carcinoid tumor ENDOCRINE: pheocromocytoma GU: wilms tumor PULMONARY: interstitial fibrosis
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Rare autosomal dominant neurocutaneous disorder
Not associated with neurofibromas
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MISME Multiple inherited schwannomas Meningioma Ependymoma
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LIPOMA INTRAOSSEOUS PARAOSTEAL
LIPOSARCOMA
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Rare bone lesion Most common lipogenic tumor in bone 4-5th decade of life Slight male predilection
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PAIN Anywhere in the skeleton Long bones..........metaphysis, medullary
cavity
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o Lower limb 71%o Calcaneum 32%o Femur 20%o Tibia 13%o Fibula 6%
o Upper limb 7%o Skull, mandible 7%o Spine and pelvis 12%o Ribs 2.5%
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PLAIN FILM Osteolytic Well defined margins
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intraosseous lipoma within the calcaneum
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3 CATEGORIES• TYPE 1: sharply delineated, viable lipomas,
homogenous fat content
Homogenous hyperintense fat signal on T1 and T2WI, suppressed by STIR
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• TYPE 2: predominantly fatty lesions with central necrosis, calcifications or ossifications
Hypointense inclusions on T1WI which may appear hyperintense on STIR(granulation tissue) or hypointese( calcifications or ossifications)
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• TYPE 3: heterogeneous, fat containing lesions with necrosis, cystic transformation, wall sclerosis, extensive calcifications or ossifications.
Fluid equivalent cavities, signal void bony septae, surrounded by signal void rim of sclerotic bone.
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Intraosseous lipoma with cystic transformation
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CALCANEAL CYST
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Rare benign tumour associated with periosteum
0.3% of lipomas
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Slow growing, painless, immobile mass, not fixed to skin
Nerve compression with forearm lesions Firmly adherent to underlying cortex Femur, proximal radius
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PLAIN FILM Bowing of bone or cortical erosion
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Parosteal lipoma .A discrete tumour arises from the interosseous membrane of the forearm
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CT Septations Prominent osseous protuberance Mild enhancement in fibrous tissue
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MRI Juxtacortical heterogeneous lobulated sepated
mass with signal intensity similar to subcutaneous fat.
Adjacent muscle atrophy is better seen on T2
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Axial t1wi showing a high signal intensity mass arising from surface of humerus and bulging from beneath the deltoid muscle with low signal exophytic mass arising from humerus
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BONE SCINTIGRAPHY Increased uptake
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PARAOSTEAL LIPOSRACOMA Soft tissue fails to contain fatty lucencies
INTRAOSSEOUS LIPOSARCOMA Ill-defined lytic lesion Femur, tibia Vascular Rapid extension into soft tissue Early pulmonary metastasis
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