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DMD3 > Soft Tissue (mesenchymal) tumours > Flashcards

Soft Tissue (mesenchymal) tumours Flashcards

1
Q

What is a vascular malformation caused by?

A

Developmental anomaly/congenital defect

Localised defects in vascular development during vasculogenesis and especially during angiogenesis

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2
Q

What types of vessels can be involved in vascular malformations?

A

Lymphatic

Capillary

Venous

Arteriovenous

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3
Q

How do vascular malformations happen?

A

Not completely understood yet.

Sporadic and genetic/familial factors come into play part of a 2 hit hypothesis

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4
Q

Which conditions with vascular malformation are associated with genetic defects?

A

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

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5
Q

When do lymphatic malformations typically present?

A

Approximately half present at birth and by 2 years of age 80 - 90% rarely occurs after the age of 2.

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6
Q

How are lymphatic malformations different to venous malformations?

A

Local infections approximating the course of lymphatic drainage will cause LM to swell, protrude, and sometimes become painful

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7
Q

What are the clinical features of lymphatic malformations?

A

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

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8
Q

How are lymphatic malformations diagnosed?

A

MRI

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9
Q

How are lymphatic malformations managed?

A

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

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10
Q

What do capillary malformations look like?

A

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

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11
Q

How are capillary malformations diagnosed?

A

Physical examination alone if findings are inconsistent then an MRI is indicated (indicated for Sturge Weber Syndrome)

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12
Q

Which condition are capillary malformations associated with?

A

Sturge Weber Syndrome; this syndrome is associated with variable intractable seizures, mental retardation, and glaucoma

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13
Q

How are capillary malformations treated?

A

Pulsed dye laser (thermolysis)

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14
Q

How do venous malformations grow?

A

Grow proportionately when young w/ expansion in adulthood

Rapid growth may occur during puberty, pregnancy, or traumatic injury

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15
Q

How do venous malformations look?

A

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

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16
Q

How are venous malformations diagnosed?

A

MRI

17
Q

How are venous malformations treated?

A

Monitor

Surgery

Laser

Sclerotherapy

Cryotherapy

18
Q

What are arteriovenous malformations?

A

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

19
Q

What are the clinical features of arteriovenous malformations?

A

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.

20
Q

How are arteriovenous malformations diagnosed?

A

Clinical examination + imagine (MRIs)

21
Q

How are AV malformations treated?

A

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.

22
Q

When should lymphatic malformations be treated most ideally?

A

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.

23
Q

What are the histological features of a AV malformation?

A

Increase in the number of vessels that are abnormally tortuous and enlarged.

24
Q

What causes fibro-epithelial polyps?

A

Inflammatory-reactive lesion

Fibrous proliferation occurs in response to chronic irritation

25
Q

How are fibro-epithelial polyps treated?

A

Complete surgical excision

26
Q

What is another name for a fibroepithelial polyp?

A

Fibroepithelial polyp is the most common connective tissue mesenchymal soft tissue growth. It is also known as a fibroma (in the US). Only way to get rid of them is to cut them out.

27
Q

What is a fibrous epulis?

A

Fibrous epulis is exactly the same as a fibro-epithelial polyp but it is seen in the gingiva. They are reactive inflammatory fibrous response to chronic irritation.

Fibrous proliferation in response to chronic irritation

Recommended to treat fibrous epulous because it is associated with fibrous symptoms.

28
Q

How is fibrous epulis treated?

A

Complete surgical excision and curettage

29
Q

What causes denture hyperplasia?

A

Fibrous proliferation in response to chronic irritation. it is an inflammatory-reactive lesion

30
Q

How is denture hyperplasia treated?

A

Complete surgical excision

Denture hygiene

New prostheses

31
Q

What is inflammatory papillary hyperplasia?

A

Asymptomatic nodular/papillary mucosal lesion

Palate of denture wearers (most often)

32
Q

What causes inflammatory papillary hyperplasia?

A

Ill fitting denture, poor denture hygiene

Chronic irritation -> fibrous proliferation

Possibly caused by C. albicans

33
Q

How is inflammatory papillary hyperplasia treated?

A

Cryotherapy

Blade

Electrosurgery

CO2 Laser

Antifungal?

Denture hygiene

New Prosthesis

34
Q

What is a pyogenic granuloma correctly named?

A

Vascular epulis (angiogranuloma)

35
Q

What causes pyogenic granuloma?

A

Debatable; reactive lesion

Infection

MO: Ulcerated» Superficial

Hormonal  5% preg
F=M

Always start off with trauma.

Trauma: 1/3. Chronic Irritation

36
Q

What are the clinical features of pyogenic granuloma?

A

Reddish purple swelling

Painless

Soft non fluctuant

Bleeds

GIngiva, tongue, lips, buccal mucosa

37
Q

What treatment is used for pyogenic granuloma?

A

Surgical excision: curettage

Most commonly seen on the gingival or the tongue. Can be removed in pregnant women.

38
Q

What are the histological features of pyogenic granuloma?

A

Pyogenic granuloma is partly covered or completely covered by parakeratotic or non-keratinized stratified squamous epithelium

squamous epithlium.

39
Q

What are denture hyperplastic lesions also called?

A

Leaf fibromas

DMD3 (53 decks)

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