Soft Tissue (mesenchymal) tumours Flashcards
What is a vascular malformation caused by?
Developmental anomaly/congenital defect
Localised defects in vascular development during vasculogenesis and especially during angiogenesis
What types of vessels can be involved in vascular malformations?
Lymphatic
Capillary
Venous
Arteriovenous
How do vascular malformations happen?
Not completely understood yet.
Sporadic and genetic/familial factors come into play part of a 2 hit hypothesis
Which conditions with vascular malformation are associated with genetic defects?
Haemorrhagic telangiectasia (HHT)
Inherited cutaneomucosal venous malformation (VMCM)
Glomuvenous malformation (GVM)
Capillary malformation - arteriovenous malformation (CM-AVM)
Cerebral cavernous malformation (CCM)
Syndromic forms of primary lymphoedema
When do lymphatic malformations typically present?
Approximately half present at birth and by 2 years of age 80 - 90% rarely occurs after the age of 2.
How are lymphatic malformations different to venous malformations?
Local infections approximating the course of lymphatic drainage will cause LM to swell, protrude, and sometimes become painful
What are the clinical features of lymphatic malformations?
Most are found in the cervicofacial region and extend to the oral cavity and airway.
Symptoms secondary to bulky disease often include pain, dysphagia, odynophagia, impaired speech, or in severe cases, airway obstruction.
When involving the skeletal framework in this area; osseous hypertrophy leading to dental or extremity abnormalities
How are lymphatic malformations diagnosed?
MRI
How are lymphatic malformations managed?
Sclerotherapy (injecting a sclerosing agent -> fibrosis -> regression)
Carbon dioxide laser therapy may also be employed in limited disease
Surgical excision for disease plus correction of secondary deformities caused by LM such as bony overgrowth of the facial skeleton
Overall, treatment aim-> nil functional/aesthetic impairment
Multiple treatments +/- modalities
What do capillary malformations look like?
CMs present at birth as flat, red or purple, cutaneous patches with irregular borders. They are painless and do not spontaneously bleed
CMs tend to progress with time – darker, more raised and nodular
How are capillary malformations diagnosed?
Physical examination alone if findings are inconsistent then an MRI is indicated (indicated for Sturge Weber Syndrome)
Which condition are capillary malformations associated with?
Sturge Weber Syndrome; this syndrome is associated with variable intractable seizures, mental retardation, and glaucoma
How are capillary malformations treated?
Pulsed dye laser (thermolysis)
How do venous malformations grow?
Grow proportionately when young w/ expansion in adulthood
Rapid growth may occur during puberty, pregnancy, or traumatic injury
How do venous malformations look?
Overlying skin may appear normal or possess a bluish discoloration
With more cutaneous involvement, the lesions appear darker blue or purple
Can bleed and cause pain
How are venous malformations diagnosed?
MRI
How are venous malformations treated?
Monitor
Surgery
Laser
Sclerotherapy
Cryotherapy
What are arteriovenous malformations?
Congenital high-flow vascular malformations
Anomalous capillary beds shunt blood from the arterial system to the venous system.
They are infiltrative and can lead to destruction of local tissue
What are the clinical features of arteriovenous malformations?
Palpable warmth, pulse, or thrill due to its high vascular flow, blush colour
Midface, oral cavity, and limbs
Oral lesions gingival involvement, disruption of deciduous teeth, and profuse periodontal bleeding.
How are arteriovenous malformations diagnosed?
Clinical examination + imagine (MRIs)
How are AV malformations treated?
Embolization, surgery, or a combination
Treatment and timing are often individualized to the patient and the extent of disease
Small-vessel AVMs, localised and can be resected with good long-term outcomes.
Diffuse lesions are a lifelong problem. Long-term follow-up with a multidisciplinary team is important for AVM management.
If it is small cryotherapy can help reduce it without needing to use surgery.
When should lymphatic malformations be treated most ideally?
Good idea to treat because they can stay and become worse throughout adulthood compared to when they are young and it is possible to treat easily. This is because they are often small at that age and when removed no longer an issue. 8 is a little bit late of an age.
What are the histological features of a AV malformation?
Increase in the number of vessels that are abnormally tortuous and enlarged.
What causes fibro-epithelial polyps?
Inflammatory-reactive lesion
Fibrous proliferation occurs in response to chronic irritation
