Benign Soft Tissue Tumours
Lipoma
Epidemiology
- Adults
- Men > women
Aetiology
- Tumour of adipose tissue (white fat)
- Tumours of brown fat are called Hibernomas – occur in hibernating animals or relatively inactive young children
- Multiple lipomatosis – Decrum’s disease
Clinical Features
- Subcutaneous usually
- Painless
- Usually <5 cm but can be very large
- Subtypes:
- Intramuscular – must be sure this is not a low-grade liposarcoma
- Pleomorphic
- Spindle cell lipoma
- Both are benign but may be confused with malignant lesions
- Angiolipoma – the only painful lipoma – still benign
Imaging Features
- Lipoma should look the same as fat on every MRI sequence
Management/Outcomes
- Marginal excision
- Recurrence rate <5%
Haemangioma/Intramuscular Haemangioma
Epidemiology
- <30 years old and more common in children
Clinical Features
- If superficial, bluish compressible mass that fills with gravity
- May have a bruit but not usually pulsatile
- Is a venous problem normally
- May be deep in muscle (intramuscular haemangioma)
- Painless normally
Imaging Features
- High signal on T1 and T2
Management/Outcomes
- NSAIDs and compression bandages first line
- Surgical excision has a high recurrence rate
- Sclerotherapy or embolization are often useful alone or with surgery
Neurilemoma (Schwannoma)
Epidemiology
- Young adults
- More common than neurofibroma
Aetiology
- Benign tumour of Schwann cells
- Associated with the NF2 gene
Clinical Features
- Slow-growing painless mass
- May cause neurologic symptoms or pain on stretch but uncommon
Imaging Features
- Low signal on T1, High signal on T2
- Lesion is eccentric to the nerve but in continuity with it
- May have a positive Tinel’s sign
Pathology
- Different cellular areas split into Antoni A and B regions
- Verocay bodies are pathognomonic
- Stains positive for S100 antibody
Management
- Marginal excision if symptomatic
- Shells out like a pea from the nerve
- Observe otherwise
- Preserve nerve fibres if excising
Neurofibroma & Neurofibromatosis
Epidemiology
- Young adults or children
Aetiology
- A benign nerve tumour
- May arise from any nerve
- May be superficial or plexiform (infiltrative)
- Occurs de novo or as part of neurofibromatosis 1 or 2
NF1
- 1/4000 births
- Von Recklinghausen’s disease
- Chromosome 17 translocation
- Autosomal dominant
NF2
- 1/40,000 births
- Chromosome 22 translocation (Remember NF2 – Ch 22)
Diagnostic Criteria for Neurofibromatosis Type 1
At least two of the following criteria (ROLANCO): 1. Relative with NF1 2. Osseus abnormality - Cortical scalloping, pseudoarthrosis, NOF, scoliosis 3. Lisch nodule (hamartoma of melanocytes in the iris) 4. Axillary freckling 5. Neurofibromas x2 or one plexiform neurofibroma 6. Café au lait spots – 6 or more 7. Optic glioma
Clinical Features
- Usually superficial but may be deep and very large
- Generally asymptomatic
- Slow-growing (beware rapid growth)
- Tinel positive rarely
- <5% risk of malignant change to neurofibrosarcoma
- Almost always in NF1 rather than solitary lesions
- Usually in plexiform (infiltrative) neurofibroma
Imaging Features
- Low signal T1, high signal T2
- Fusiform rather than eccentric (schwannoma) on the nerve
- May be dumbbell-shaped
- Look for skeletal signs:
- Pseudoarthrosis, NOF, cortical scalloping, scoliosis
- Scoliosis is most common
Pathology
- Interlacing bundles of elongated cells with dark staining nuclei
Management
- Observe if asymptomatic
- Excise if symptomatic – nerve defect possible
- Treat osseous problems accordingly
Nodular Fasciitis
Epidemiology
- Most common fibrous tumour
Aetiology
- Completely benign but mistaken for malignant
Clinical Features
- Usually on volar aspect of upper limb, neck, back
- Usually superficial
- Rapidly grows over 2 weeks then stabilizes
- Quite small in size
- 50% painful
- Solitary
- No risk of malignant transformation
Imaging Features
- Low signal T1 but enhances highly with gadolinium
- Superficial, deep or along fascia planes
Pathology
- Normal fibroblasts in clumps
Management
- Excision has a very low recurrence rate
Intramuscular Myxoma
Epidemiology
- Older adults
Aetiology
- Mucoid lesion within muscles
Associated Conditions
- Mazabraud Syndrome:
- Multiple myxomas and fibrous dysplasia (Remember M for Mazabrauds and Multiple Myxomas)
Clinical Features
- Slow-growing mass
- Painful in 20%
- May cause mass effect on nerves
- Solitary unless part of a syndrome - Mazabrauds
- Buttocks and thighs usually
Imaging Features
- Lie within muscle compartments
Management
- Marginal excision – low recurrence rate
Desmoid Tumour
Epidemiology
- Young adults
Aetiology
- Part of the fibromatosis family of tumours (e.g., Dupuytren’s, Lederhosen’s)
Clinical Features
- The most common locally aggressive benign soft tissue tumour
- Infiltrates surrounding tissues
- 50% are intra-abdominal, 50% extra-abdominal
- Shoulder, chest wall, back, thigh
- Grow slowly, are usually pain-free and feel ‘rock hard’ and fixed
- Unpredictable natural history
Imaging Features
- Low T1 and T2 signal - Enhance with gadolinium
Pathology
- Fibroblasts, spindle cells, and collagen
Management/Outcomes
- Treat like a sarcoma – Wide excision
- Consider adjuvant radiotherapy or post-operative radiotherapy
- Recurrence rate is high
Elastofibroma
- Rare reactive benign lesion
- Occurs exclusively between scapula and chest wall
- Causes snapping scapula syndrome which may cause pain
- MRI has heterogeneous signal on T1 and T2
- No chance of malignancy
- Treatment is by excision which is successful
- Histology stains for Elastin
Synovial Chondromatosis
Epidemiology
- Young adults
Aetiology
- Metaplastic proliferation of hyaline cartilage in the synovium
- Initially not visible on XR
- Cartilage fragments undergo endochondral ossification to become calcified and visible
Clinical Features
- Affects hip, knee, shoulder, elbow, ankle in that order
- Hundreds of intra-articular loose bodies
- Cause pain and erode articular surfaces
Management
- Synovectomy and removal of all loose bodies
- High recurrence if synovectomy is not complete
Myositis Ossificans
Epidemiology
- Young adults
Aetiology
- Post-traumatic almost exclusively
- Almost always within muscle
Clinical Features
- Usually in quads, gluteals, or brachialis
- Grows in size over several months
- Then stabilizes but becomes hard
- Usually mature by one year
- Often regresses after this
- Causes pain with muscle activity or restricted joint motion
Pathology
- Zonal pattern:
- Periphery: Mature lamellar bone
- Middle: Osteoblasts, fibroblasts, and calcification
- Centre: Fibrous tissue – more lucent on XR
Differential Diagnosis
- Soft tissue sarcoma – histology is completely different
- CT scan and MRI will usually differentiate
Management
- Observe
- NSAIDs
- Do not excise until mature and only if symptomatic