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In the WHO Classification of Tumours of Soft Tissue and Bone, myxomas are included in the section headed ‘Tumours of uncertain differentiation’. There are four main sub-types: acral fibromyxoma; intramuscular myxoma; juxta-articular myxoma; deep (aggressive) angiomyxoma. Of note myxoma may also occur in the heart or the eye. The most common sub-type encountered by the orthopaedic surgeon is the intramuscular myxoma. Patients present with a lump which may or may not be painful. Ultrasound scanning followed by MRI is recommended for investigation. Most centres dealing with soft tissue and bone tumours would recommend biopsy to confirm the diagnosis. A discussion can then ensue with the patient regarding excision if symptomatic. The main risks of excision include wound problems, infection, damage to adjacent critical structures and recurrence.
Intramucular myxomas are benign tumours of mesenchymal origin. The incidence is 1 in 1 million with a slight female preponderance of 57%. The typical age range of presentation is 40-70 years. 64% of patients will notice an enlarging mass, with the rest being incidental finding. 55% report that the tumour is painful. The size on presentation ranges from 1-17cm. They can be solitary or multiple. If they are multiple then the patient should be investigated for Mazabraud syndrome which features monostotic or polyostotic fibrous dysplasia.
It is important to note that if there is any doubt about the diagnosis, a biopsy must be performed to rule out a malignant tumour. There are no reports of malignant transformation of intramuscular myxomas. Therefore if the patient has none or minimal symptoms, non-operative management is recommended. If the patient is symptomatic then excision can be performed. Risks include damage to adjacent critical structures such as large nerve or blood vessels, wound problems, infection and recurrence rates of 30% at a median of 8.5 years.

INDICATIONS
Incidence and presenting features
Intramucular myxomas are benign tumours of mesenchymal origin. The incidence is 1 in 1 million with a slight female preponderance of 57%. The typical age range of presentation is 40-70 years. 64% of patients will notice an enlarging mass, with the rest being incidental finding. 55% report that the tumour is painful. The size on presentation ranges from 1-17cm. They can be solitary or multiple. If they are multiple then the patient should be investigated for Mazabraud syndrome which features monostotic or polyostotic fibrous dysplasia.
Location
These tumours can occur in any muscle, but the most common site is the quadriceps (65%), hip adductors (<35%), hip abductors (<20%), gastrocnemius and upper arm musculature. If they are multiple and occur in conjunction with fibrous dysplasia, then the tumours are located in the vicinity of the bone lesions and in more than three quarters of cases affect the pelvis and lower limbs.
Histological features
Intramuscular myxomas tend to be hypocellular and hypovascular although occasionally the pathologist will report areas of increased cellularity and vascularity. The cells have a stellate shape with small hyperchromatic pyknotic nuclei and scant cytoplasm. In particular there are no cells with evidence of nuclear atypia, mitotic figures or necrosis. The extracellular matrix consists of loose reticulin fibres within an abundant myxoid stroma.
Management
It is important to note that if there is any doubt about the diagnosis, a biopsy must be performed to rule out a malignant tumour. There are no reports of malignant transformation of intramuscular myxomas. Therefore if the patient has none or minimal symptoms, non-operative management is recommended. If the patient is symptomatic then excision can be performed. Risks include damage to adjacent critical structures such as large nerve or blood vessels, wound problems, infection and recurrence rates of 30% at a median of 8.5 years.
Myxoma subtypes
There are four main subtypes of myxoma that may present to the orthopaedic surgeon. These are the intramuscular myxoma, superficial acral fibromyxoma, juxta-articular myxoma and deep (aggressive) angiomyxoma. Intramuscular myxoma is the most common subtype with the others all having an incidence of less than 1 in 1 million.
Superficial acral myxoma
This is an extremely rare tumour with less than 100 cases reported in the literature. It is most commonly located in the subungual and periungual regions of the fingers and toes in adults with a mean age of presentation of 43 years. It is more common in males with a ratio of 2:1. Imaging features are indeterminate due to the small size and rarity of the tumour and therefore diagnosis relies on pathology, usually following an excisional biopsy. Further management depends on patient symptoms although most are cured with an excisional biopsy.
Juxta-articular myxoma
These are benign tumours that tend occur in patients aged 20 to 60 years of age. 88% occur adjacent to the knee joint. These tumour arise in the subcutaneous tissues but may infiltrate deeper structures of the adjacent joint. Typical imaging features on the MRI scan include a thickened wall and multiple septae with a hypointense signal to muscle on the T1 sequences. On T2 sequences, the signal is high and hyperintense to surrounding fat (Daluiski A, Seeger LL, Doberneck SA, Finerman GA, Eckardt JJ. A case of juxta-articular myxoma of the knee. Skeletal Radiol. 1995 Jul;24(5):389-91.). As per intramuscular myxoma, a biopsy is required to exclude malignancy and surgery is offered if the patient is symptomatic.
Deep (aggressive) angiomyxoma
These tumours are very rare with only about 250 cases reported in the literature. It most commonly presents in women of reproductive age with the pelvis being the most affected location. These tumours are usually slow growing but can be infiltrative making excision difficult. Prior to consideration of surgery, a biopsy is mandatory to exclude a malinancy.
SYMPTOMS & EXAMINATION
64% of patients will present having noticed a lump or diffuse swelling. 55% will report pain in the lump which may be exertional or present at rest.
Depending on the location of the myxoma, the tumour may be visible very obvious and easily palpable e.g. if arising in rectus femoris, or there may be a more subtle swelling, with the tumour not palpable e.g. if arising in the hip adductors. Tenderness will vary and there are rarely any neurovascular deficits.
IMAGING
Ultrasound scanning will typically be reported as showing an indeterminate solid mass with internal vascularity.
MRI often features hyperintense signal on the T2 sequence. MRI is also essential to assess proximity to critical structures. The differential diagnoses include chronic haematoma, peripheral nerve sheath tumours, myxofibrosarcoma, lymphangioma, myxoid liposarcoma, myxoid malignant fibrous histiocytoma, extraskeletal myxoid chondrosarcoma, botyroid rhabdomyosarcoma.
ALTERNATIVE OPERATIVE TREATMENT
If the myxoma is symptomatic, intra-lesional excision should be discussed with the patient. Symptomatic lesions are often large, painful and tend to gradually increase in size increasing the risks of surgical complications if excision is not undertaken for a prolonged period of time.
NON-OPERATIVE MANAGEMENT
There are no reports of malignant transformation, so if the myxoma is asymptomatic, then the patient can be advised to monitor it and request review if they are concerned in the future.
It is important to reiterate that because malignant tumours are in the differential diagnosis on imaging, a biopsy must be undertaken to ensure that the tumour is definitely a myxoma.
CONTRAINDICATIONS
Relative contraindications to excision include myxomas adjacent to critical structures which would be at high risk of damage during excision.

GA. No prophylactic antibiotics required.
Double alcoholic skin preparation of the whole leg including the foot. Draping to exclude the lower leg leaving the thigh exposed.
VTE prohpylaxis: Flowtron on the contra-lateral lower leg intra-operatively. Early mobilisation post-operatively. 6 weeks TED stockings. LMWH during the hospital admission and for 10 days post-operatively.

The patient is mobilised as soon as they have recovered from the GA under supervision of a physiotherapist. VTE prophylaxis: 6 weeks TED stockings. LMWH during the hospital admission and for 10 days post-operatively.
Maintain the pressure dressing until the wound review at 10-14 days.
Outpatient follow up clinic in 10-14 days for the wound check and to confirm the pathology result is the same as the biopsy.

Nielsen G et al. Intramuscular myxoma: a clinicopathologic study of 51 cases with emphasis on hypercellular and hypervascular variants. Am J Surg Pathol. 1998 Oct;22(10):1222-7. doi: 10.1097/00000478-199810000-00007.
This is an important paper that describes the histological features of intramuscular myxomas. Occasionally there are hypercellular areas without and it is important that an erroneous diagnosis of sarcoma is not made. In this series of 51 cases, there were no recurrences or evidence of metastatic behaviour although the follow-up ranged from 3-108 months.
Luna et al. Magnetic resonance imaging of intramuscular myxoma with histological comparison and a review of the literature. Skeletal Radiol. 2005 Jan;34(1):19-28. doi: 10.1007/s00256-004-0848-9. Epub 2004 Oct 22.
This paper describes the MRI findings of 17 cases of intramuscular myxomas in detail. All were hyperintense on T2 weighted sequences. Most tumours has a pseudocapsule that could be identified on the MRI and a few had additional intratumoural cysts.
Allen. Myxoma is not a single entity: a review of the concept of myxoma. Ann Diagn Pathol. 2000 Apr;4(2):99-123. doi: 10.1016/s1092-9134(00)90019-4.
This paper comprehensively covers the different types of myxoma focussing on the five entities regarded as mainstream soft tissue myxomas (namely, intramuscular myxoma, juxta-articular myxoma, superficial angiomyxoma, aggressive angiomyxoma, and myxoid neurothekeoma [myxoma of nerve sheath]). It is these five myxoma types that the orthopaedic surgeon needs to be aware of.
Reference
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