hemangioma sam 3_2
TRANSCRIPT
8/9/2019 Hemangioma Sam 3_2
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Objectives
Introduction Definition Classification Epidemiology Patho-physiology Clinical Presentation Investigations and Diagnosis Course and Prognosis Management
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Introduction
Hemangiomas are tumors identifiedby rapid endothelial cell proliferation
in inf ancy, followed by involution over time.
The malformations have a normalendothelial cell growth cycle that affects the veins, the capillaries, or the lymphatics, and they do not involute.
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Definition
Hemangiomas are lesions that are not present at birth, they manifest within thefirst month of life.
They grow rapidly during the first year (proliferating phase), undergo slowspont aneous regression during childhood(involution phase), and remain st ablethereafter or to near complete resolution.
Vascular malformations are more st ableand f ail to regress.
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Classification
The present binary biologic classificationdistinguishes between vascular tumors and vascular malformations.
Vascular malformations are sub-classified according to the st ructuralcomponents into capillary, venous,lymphatic, arterial, or combined forms.
Both vascular tumors and malformationscan be separated into slow-flow or f ast-flow types.
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`
Vasoformative Tumor New Nomenclature Old Nomenclature
Vascular tumors( i.e. Hemangiomas)
Capillary hemangioma St rawberry hemangioma
Juvenile hemangioma
Cavernous hemangioma
Mixed hemangioma Parotid hemangioma
Vascular malformations
Venous malformation Cavernous hemangioma
Hemangiomatosis
Int ramuscular venous malformation Int ramuscular hemangioma
Capillary malformation Capillary hemangioma
Port-wine st ain
Arteriovenous malformation Arteriovenous hemangioma Arterial angioma Arteriovenous aneurysm
Cirsoid angiomaRed angiomaSerpentine aneurysm
Lymphatic malformation Capillary lymphangiomaCavernous lymphangiomaLymphangiomaCystic hygroma
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Hemangiomas³vascular tumors´
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Hemangioma of infancy
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Definition
HEMANGIOMA OF INFANCY (HI) ;(Formerly st rawberry, cherry, capillaryhemangioma):
Benign vascular neoplasms that have a characteristic clinical course marked by earlyproliferation and followed by spont aneousinvolution.
Most common tumors of inf ancy, andmedically insignificant. Rarely, may beassociated with one or more underlyingcongenit al anomalies.
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Epidemiology
HEMANGIOMA OF INFANCY (HI) :
Is the most common tumor of infancy.The incidence in newborns is between 1 and 2.5%; most commonly in whiteinf ants. Of which 30% present at birth and70% at the first several weeks of life.Females are affected more often thanmales by a ratio of 3:1.
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Pathophysiology
HEMANGIOMA OF INFANCY (HI) :
Inf antile hemangiomas are composed of proliferating, plump endothelial cells.
Early in proliferation, the cells are indisarray. But, with time, they form vascular
spaces and channels abundant with bloodcells.
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These benign-appearing endothelial cellsproduce limited basementmembrane structures.
Hemangiomas assume a lobulararchitecture as proliferation slows andends.
Mast cells appear to affect thisprocess.They also have been found inhigh concent rations during involution.
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Takahashi said:
That during the third t rimester of
fet al development, immatureendothelial cells coexist withimmature pericytes, which maint aintheir proliferative capacity for a
limited period during postnat al life.
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Histopathology of a proliferating infantile hemangioma with plump
endothelial cells in the dermis.
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Clinical feature
80% are focal and solit ary.
60% of cut aneous heamangiomas occur
on the head and neck. Other sites: liver, GIT, larynx, CNS,
pancreas, gall bladder, thymus, spleen,lymph nodes, lung, urinary bladder, and
adrenal glands.
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Contd.
Early proliferating inf antilehemangiomas include:
Blanching of the involved skin,followed by fine telangiect asias, and
then a red or crimson (purplish-red) macule or papule that often is
surrounded by a f aint halo of vascular blanching.
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Size: Most reach a maximum size of 0.5-5 cm, to greater than 20 cm in
diameter.
Most remain well circumscribed andfocal. A minority may be segment al innature.
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Investigations and Diagnosis
Dermatopathology shows variousamount of proliferation of endothelial
cells in dermis and subcut aneoustissue.
Increased GLUT-I immunoreactivity,but not in vascular malformation.
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Course and prognosis
CHI usually develops at birth or soonafter birth (first four weeks).
They may proliferates until 9 to 12months of age.
Most are spont aneously resolved:
Age 5 years: 50% resolution
Age 7 years: 70% resolution
Age 9 years: 90% resolution
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Contd.
80% involutes without any residual skinchange at site of lesion.
Residual skin changes include at rophy,depigment ation, and scarring mayoccur.
Deeper lesions of mucous membranes
may not involute completely.
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Contd.
CHI may associated with:
Platelet ent rapment
Thrombocytopenia DIC
Rarely, death secondary to severehemorrhage or heart f ailure.
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Management
The vast ma jority of inf antilehemangiomas do not require anymedical or surgical intervention becausespont aneous resolution gives lessscaring and the best cosmetic results.
Few cases may require medical or
surgical t reatment.
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Medical treatment
Glucocorticosteroids (topical,int ralesional, and oral)
Interferon-alfa Beta-blockers (propranolol); have
recently been shown to induceinvolution of inf antile hemangiomas.
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Surgical treatment
Laser surgery is beneficial in t reatingboth proliferating and residual vesselsf rom hemangiomas.
The flash lamp-pumped pulsed-dyelaser has become the most widely usedlaser for selective ablation of vascular
tissue in childhood.
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Surgical excision of involuted
hemangiomas may be used todecrease cut aneous defects resultingf rom them.
Surgical treatment
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Cavernoushemangioma
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Overview
Less common and lesscircumscribed than the capillaryvariety and more f requently involve
deep st ructures.
Rarely, giant forms occur that affect large subcut aneous areas of
the f ace, ext remities, or other regionsof the body.
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Clinical feature
Large, irregular, deep dermal andsubcut aneous blood-filled channels that impart a purplish discoloration to theoverlying skin.
They are typically soft, poorly defined,and readily blanch with compression,
giving them a characteristic " bag ofworms" feel.
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The lesion may expand and darkenwith crying, when agit ated, or whenplaced in a dependent position .
They are readily compressible and fillslowly when released .
They lack a prominent pulsation; if
they represent an arteriovenousmalformation, a thrill may be present .
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They are not present at birth but develop during childhood.
Usually asymptomatic and demonst ratethe same patterns of proliferation asthose of capillary lesions. However,involution is often incomplete.
Course and prognosis
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Cavernous hemangiomas maycomplicated with:
Ulceration ( the most common )
Bleeding, thrombocytopenia
High-out put heart f ailure
Secondary infection
Function problems (eg, airway, vision,hearing).
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There is no satisf actory t reatment except compression.
Some cases ( those withcompromised organ function ) mayrequire medical or surgicalintervention.
Management
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Steroids; high doses of systemic or int ralesional steroids are the first-line
t reatment, and a dramatic response isobserved in 30% of patients.
Interferon-alfa.
Medical treatment
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Complete surgical excision of theselesions offers the best chance of cure,
but followed by severe functionalimpairment to vit al functions.
Embolotherapy.
Cryosurgery.
Lasers; pulsed dye, argon and CO2.
Surgical treatment
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References
http:// emedicine.medsca p e.com / ar ticl e /108447 9-overvi ew
http:// emedicin e.medsc a p e.com/ ar ticl e /108384 9-ov er vi ew
FITZPATRICKS COLOR ATLAS AND SYN OP SIS OF C LINI CAL DERM ATO LOG Y,SIXTH EDITION
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