VASCULAR TUMORSPRESENTOR- Dr. Shankila MittalModerator- Dr. Tanvi Dr. K.D. Barman

WHO CLASSIFICATION OF VASCULAR TUMORS

• Haemangiomas : capillary /cavernous/ arteriovenous/venous

• Epithelioid haemangioma • Angiomatosis Lymphangioma

Benign

• Kaposiform haemangioendotheliomaIntermediate (locally aggressive)

• Retiform haemangioendothelioma• Papillary intralymphatic angioendothelioma • Composite haemangioendothelioma • Kaposi sarcoma

Intermediate (rarely metastasizing)

• Epithelioid haemangioendothelioma • AngiosarcomaMalignant

INFANTILE HEMANGIOMA

• Benign

• Onset- 1st months of life

• MC paediatric tumour

• Risk factors▫ Girls (F:M -3 and 5 : 1)▫ Premature infants

TYPES

• Based on Depth :

1. Superficial hemangiomas – bright red lesions

2. Deep hemangiomas (cavernous) – bluish to skin-colored nodules.

3. Mixed hemangiomas – features of both superficial and deep

BASED ON MORPHOLOGY

• Greater prognostic value• More predictive of risk of complications and need for treatment

1. Focal 2. Multifocal 3. Segmental 4. Indeterminate

ISVVA 2014

CLINICAL FEATURES

• Mean period of proliferation - 5 months

• Involution may begin in 1st year

• Involution is completed in - 50% by 5 years, 70% by 7 years and 90% by 9 years

• After involution▫ Complete disappearance ▫ Telangiectasia ▫ fibrofatty residuum

IHVASCULAR

MALFORMATION RICH NICH

At birth Usually absent at birth  or subtle precursor  lesion present

Usually present at birth

Fully developed at birth

Fully developed at birth

Progression Rapid proliferation and Spontaneous involution over years

•Slow expansion,  proportionate with growth•persists into adulthood

Intrauterine proliferation and Rapid involution during 1st year

Intrauterine proliferation   then proportionate  postnatal growth.Does not involute

Sex Girls > boys Equal Equal Boys > girls

COMPLICATIONS• Ulceration

• Heart failure

• Visual impairment

• Airway obstruction

• Auditory canal obstruction

• Disfigurement

Associated abnormalities:• Spinal dysraphism

• PHACE syndrome

P - Posterior Fossa And Other Structural Brain MalformationsH - HemangiomaA - Arterial Anomalies Of Cervical And Cerebral VesselsC - Cardiac Defects (Especially Coarctation Of The Aorta)E - Eye AnomaliesS - Sternal Defects And Supraumbilical Raphe

PHACES SYNDROME

LUMBAR SYNDROME • Suspect if large hemangiomas on lower

body

SACRAL• Spinal dysraphism• Anogenital• Cutaneous• Renal and urological

anomalies• Angioma of

lumbosacral

PELVIS• Perineal hemangioma• External genitalia

malformations• Lipomyelomeningocele• Vesicorenal

abnormalities• Imperforate anus• Skin tag

WORKUP

• ENT evaluation• Opthalmological evaluation• ECG• Echocardiography• DOPPLER USG• CT AND MRI• CRANIAL USG (< 4 months age)• USG ABDOMEN( if multiple hemangiomatosis)• Immunohistochemistry - GLUT 1

MANAGEMENT

Major goals of management of infantile haemangiomas -

• Prevention or reversal of life-threatening complications

• Prevention of permanent disfigurement following involution

• Reduction of psychological distress

• Reduction of toxicity and resultant sequelae from systemic therapy

• Avoidance of excessive scarring from aggressive surgery

• Treatment of ulceration to minimize scarring

INDICATIONS FOR ACTIVE EARLY TREATMENT

1. Life threatening complications

2. Functional impairement

3. Hemangiomas likely to result in disfigurement

4. Ulcerated hemangiomas

5. Large facial hemangiomas, with large dermal component

6. Exophytic hemangiomas likely to leave significant fibrofatty residue

7. Visceral hemangioma

MEDICAL MANAGEMENTINTERVENTION LEVEL OF EVIDENCE

Propranolol 1B

Oral steroids 2A

Topical timolol 1B

Oral propranolol vs steroids 1B

PDL 1B

IMIQUIMOD VS TOPICAL TIMOLOL 3B

INTRALESIONAL AND TOPICAL CORTICOSTEROIDS

• For small and localised hemangiomas

• Total max. dose of triamcinolone - 3–5 mg/kg /treatment session

• Efficacy of topical steroid limited by depth of its penetration

PROPANOLOLMechanism Of Action

EARLY• Change in colour

and softening• Β2 inhibitory effect• Vasoconstriction

INTERMEDIATE• Downregulation of

both VEGF and bfgf• Inhibition of

proangiogenic cascade and angiogenesis

LONG-TERM EFFECTS• Apoptosis• Regression of

haemangiomas

Management of Infantile Hemangiomas: Current Trends. Journal of Cutaneous and Aesthetic Surgery 2014

Inpatient initiation of propranolol:<8 weeks infant or co-morbid conditions

Outpatient initiation of propranolol:>8weeks infant and adequate social condition

Pretreatment assessment

• Assess for contraindications-• Bronchial asthma,HF,sinus bradycardia,

hypoglycemia, hypotension, heart block, allergry to propranolol

Pretreatment evaluation • ECG, CXR, ECHO, Blood sugar

Duration of treatment• 6 months or flattening of lesion(whichever is earlier)• May be longer• End point- complete/near complete resolution

Monitoring • Blood sugar,BP, HR(1-3 hrs after initial dose)vand after dose hike

ASSESSMENT AND MONITORING

Management of Infantile Hemangiomas: Current Trends. Journal of Cutaneous and Aesthetic Surgery 2014

Propranolol vs Corticosteroids for Infantile Hemangiomas

• Multicenter retrospective chart review of 110 patients

• Propranolol therapy v/s oral corticosteroids: ▫ more clinically effective▫ more cost-effective ▫ fewer surgical interventions ▫ better tolerance▫ minimal adverse effects

Propranolol vs Corticosteroids for Infantile Hemangiomas, JAMA Derm, December 2011, Vol 147

Propranolol Oral corticosteroids

Results 56/68 (82%) 12 of 42 (29%)

PROPANOLOL IN PHACES SYNDROME• High risk for both medical morbidities and permanent facial scarring.

CAUTION WITH PROPANOLOL : Increased risk of acute ischaemic stroke if there is Aplasia, hypoplasia, or occlusion of major cerebral artery esp. when >1 vessel is involved or if there is coarctation of the aorta

TOPICAL TIMOLOL

• More effective for plaque than for nodular lesions, and for proliferating than for involuting lesions.

• A RCT of 41 infants with IH topical timolol maleate 0.5% gel to be safe and more effective than placebo.

• Small superficial IH that had not ulcerated and that were not on mucosal surfaces chosen.

• Formulation of timolol 0.5% eye drops

RCT of timolol maleate gel for superficial infantile hemangiomas in 5- to 24-week-olds. Pediatrics. 2013

VINCRISTINE• Chemotherapeutic agent • Administration requires a central venous catheter Side effects include

1. Peripheral neuropathy2. Constipation3. Jaw pain4. Anemia5. Thrombocytopenia

INTERFERON

•Recombinant IFN alpha (2a and 2b) inhibits angiogenesis

•Early involution of large hemangiomas

•Blocks migration and proliferation of endothelial cells, smooth-muscle cells, and fibroblasts by decreasing basic FGF

•Used to treat dangerous hemangiomas not responsive to steroid therapy.

•Daily dose of 1–3 million U/m2/day given s.c.

•Complete involution may take long time

•Side effects - fever, malaise, leucopenia, interstitial nephritis, and hemolytic anemia.

ANTI ANGIOGENIC AGENT -• Treatment options include batimastat, thrombospondin, angiostatin IL-12• High cost of production

IMIQUIMOD • Limited evidence• Induces antiangiogenic cytokines such as IFN-α, IL 12, Tissue inhibitor of metalloproteinase• Not approved by FDA for use in children. • There are a few case series and one small prospective study of 5% imiquimod cream for the treatment of proliferating hemangiomas.

SURGICAL MANAGEMENT

1. To treat involuted lesions2. To remove fibro-fatty tissue and redundant skin esp in cosmetically

sensitive areas.

LASER THERAPY

Greatest consensus surrounding use of PDL for IH in treatment of residual telangiectases after involution

Epithelioid Haemangioma/Angiolymphoid Hyperplasia With Eosinophilia

• Age: young adults

• sexes – M=F

• Spontaneous regression seen

• Peripheral blood eosinophilia in < 10% patients

HISTOPATHOLOGY

Plump, epithelioid endothelial cells

Lymphocytes and eosinophils around blood vessels

MANAGEMENT

• Spontaneous regression- observe for 3–6 months

• Surgery• Radiotherapy • Nd-yag Laser

Local Recurrences Are Common

LOBULAR CAPILLARY HAEMANGIOMA/ PYOGENIC GRANULOMA

• Reactive lesion

• Develops Rapidly, Often At Site Of Recent Injury

• Sex- M>F( Except In Oral Cavity >F)

• Peak Age- 2nd Decade

• Granuloma Gravidarum - variant of pyogenic granuloma that presents in oral cavity during pregnancy

CLINICAL FEATURES

• Sites - hands, esp. Fingers , feet, lips, head and upper trunk, and mucosal surfaces of mouth and perianal area

• Spontaneous disappearance rare

• Complain of recurrent bleeding

• Reported after▫ HAART( indinavir)▫ gefitinib , capecitabine▫ Systemic 5-fluorouracil▫ Retinoids▫ Cyclosporine

HISTOPATHOLOGYLobular proliferation of small blood vessels

Plump endothelial cells

Treatment • Recurrence common

• Surgical Excision

• Electrocautery

• Nd:yag Laser

• Cryosurgery

• Flash Lamp PDL

Treatment options for cutaneous pyogenic granulomas: a review. J Plast Reconstr Aesthet Surg. 2011

ANGIOSARCOMA

• Malignant vascular tumour from both vascular and lymphatic endothelium▫ Idiopathic angiosarcoma of face, scalp and neck▫ Angiosarcoma associated with chronic lymphoedema (Stewart–Treves

Syndrome) ▫ Postirradiation Angiosarcoma

• Present as area of bruising-dusky blue or red nodules - haemorrhagic blisters, ulcerate

• Multifocality frequent

• Poor prognosis

HISTOPATHOLOGY

• Vascular channels infiltrate normal structures

• Dissection of collagen• Tumour cells plumper• Advancing malignancy- loss of

vascular Pattern and proliferation of cell masses

• Immunohistochemical studies antibodies to-▫ CD31 ▫ von Willebrand factor▫ CD34

TYPE CLINICAL FEATURES HISTOPATHOLOGY TREATMENT SPECIAL FEATURE/ASSOCIATION

Glomeruloid haemangioma

•Adults (M=F)•Multiple vascular papules on trunk and limbs

Similarity torenal glomeruli

•No spontaneous regression•Surgical removal not practical

• POEMS syndrome• Multicentric Castleman’s disease

Hobnail haemangioma

•Adults (M>F)•central, raised, violaceous papule surrounded by paler brown halo (targetoid appearance)

hobnail (‘matchstick’) appearance

•Simple surgical excision •no tendency for recurrence

may vary according to menstrualcycle

Kaposiform haemangioendothelioma

•Locally Aggressive•<2 years•M=F

nodules with haemorrhage and surrounding fibrosisCleft-like spacesbetween spindle-shaped cells

•Spontaneous regression not occur•Complete excision

•Lymphangiomatosis(<20%)•Kasabach–Merritt syndrome

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