Hemangioma

Presented by: Sami Al-Malki Mohammad Al-Saleh

Objectives 
       

Introduction Definition Classification Epidemiology Patho-physiology Clinical Presentation Investigations and Diagnosis Course and Prognosis Management

Introduction
Hemangiomas are tumors identified by rapid endothelial cell proliferation in infancy, followed by involution over time.  The malformations have a normal endothelial cell growth cycle that affects the veins, the capillaries, or the lymphatics, and they do not involute. 

Definition   

Hemangiomas are lesions that are not present at birth, they manifest within the first month of life. They grow rapidly during the first year (proliferating phase), undergo slow spontaneous regression during childhood (involution phase), and remain stable thereafter or to near complete resolution. Vascular malformations are more stable and fail to regress.

Classification   

The present binary biologic classification distinguishes between vascular tumors and vascular malformations. Vascular malformations are subclassified according to the structural components into capillary, venous, lymphatic, arterial, or combined forms. Both vascular tumors and malformations can be separated into slow-flow or fastflow types.

Vasoformative Tumor
Vascular tumors ( i.e. Hemangiomas)

New Nomenclature

Old Nomenclature

Capillary hemangioma

`

Strawberry hemangioma Juvenile hemangioma

Cavernous hemangioma Vascular malformations Venous malformation Cavernous hemangioma Hemangiomatosis Intramuscular venous malformation Capillary malformation Intramuscular hemangioma Capillary hemangioma Port-wine stain Arteriovenous malformation Arteriovenous hemangioma Arterial angioma Arteriovenous aneurysm Cirsoid angioma Red angioma Serpentine aneurysm Capillary lymphangioma Cavernous lymphangioma Lymphangioma Cystic hygroma 

Mixed hemangioma

Parotid hemangioma

Lymphatic malformation

Hemangiomas
³vascular tumors´

Hemangioma of infancy

Definition   

HEMANGIOMA OF INFANCY (HI) ; (Formerly strawberry, cherry, capillary hemangioma): Benign vascular neoplasms that have a characteristic clinical course marked by early proliferation and followed by spontaneous involution. Most common tumors of infancy, and medically insignificant. Rarely, may be associated with one or more underlying congenital anomalies.

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 white infants. Of which 30% present at birth and 70% at the first several weeks of life. Females are affected more often than males by a ratio of 3:1.

Pathophysiology 


HEMANGIOMA OF INFANCY (HI) : Infantile hemangiomas are composed of proliferating, plump endothelial cells. Early in proliferation, the cells are in disarray. But, with time, they form vascular spaces and channels abundant with blood cells.    

These benign-appearing endothelial cells produce limited basement membrane structures. Hemangiomas assume a lobular architecture as proliferation slows and ends. Mast cells appear to affect this process.They also have been found in high concentrations during involution.

Takahashi said:  That during the third trimester of fetal development, immature endothelial cells coexist with immature pericytes, which maintain their proliferative capacity for a limited period during postnatal life. 

Histopathology of a proliferating infantile hemangioma with plump endothelial cells in the dermis.

Clinical feature 
 

80% are focal and solitary. 60% of cutaneous 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.

Contd. 


Early proliferating infantile hemangiomas include: Blanching of the involved skin, followed by fine telangiectasias, and then a red or crimson (purplish-red) macule or papule that often is surrounded by a faint halo of vascular blanching.

Shape:  Depending on (size, depth, morphology, and texture) may be dome shaped, bosselated, plaquelike, tumoral, or any combination of these morphologies.  

Size: Most reach a maximum size of 0.5-5 cm, to greater than 20 cm in diameter. Most remain well circumscribed and focal. A minority may be segmental in nature. 

Investigations and Diagnosis 

Dermatopathology shows various amount of proliferation of endothelial cells in dermis and subcutaneous tissue. Increased GLUT-I immunoreactivity, but not in vascular malformation.  

Diagnosis made on findings on MRI; Doppler and arteriography to demonstrate fast flow. Level of GLUT-I immunoreactivity to rule out vascular malformation. 

Course and prognosis 
    

CHI usually develops at birth or soon after birth (first four weeks). They may proliferates until 9 to 12 months of age. Most are spontaneously resolved: Age 5 years: 50% resolution Age 7 years: 70% resolution Age 9 years: 90% resolution

Contd. 
 

80% involutes without any residual skin change at site of lesion. Residual skin changes include atrophy, depigmentation, and scarring may occur. Deeper lesions of mucous membranes may not involute completely.

Contd. 
   

CHI may associated with: Platelet entrapment Thrombocytopenia DIC Rarely, death secondary to severe hemorrhage or heart failure.

Management  

The vast majority of infantile hemangiomas do not require any medical or surgical intervention because spontaneous resolution gives less scaring and the best cosmetic results. Few cases may require medical or surgical treatment.

Medical treatment
Glucocorticosteroids (topical, intralesional, and oral)  Interferon-alfa  Beta-blockers (propranolol); have recently been shown to induce involution of infantile hemangiomas. 

Surgical treatment  

Laser surgery is beneficial in treating both proliferating and residual vessels from hemangiomas. The flash lamp-pumped pulsed-dye laser has become the most widely used laser for selective ablation of vascular tissue in childhood.

Surgical treatment
Surgical excision of involuted hemangiomas may be used to decrease cutaneous defects resulting from them. 

Cavernous hemangioma

Overview 

Less common and less circumscribed than the capillary variety and more frequently involve deep structures. Rarely, giant forms occur that affect large subcutaneous areas of the face, extremities, or other regions of the body. 

Clinical feature  

Large, irregular, deep dermal and subcutaneous blood-filled channels that impart a purplish discoloration to the overlying skin. They are typically soft, poorly defined, and readily blanch with compression, giving them a characteristic "bag of worms" feel.  



The lesion may expand and darken with crying, when agitated, or when placed in a dependent position . They are readily compressible and fill slowly when released . They lack a prominent pulsation; if they represent an arteriovenous malformation, a thrill may be present .

Course and prognosis 

They are not present at birth but develop during childhood. Usually asymptomatic and demonstrate the same patterns of proliferation as those of capillary lesions. However, involution is often incomplete.  

    

Cavernous hemangiomas may complicated with: Ulceration ( the most common ) Bleeding, thrombocytopenia High-output heart failure Secondary infection Function problems (eg, airway, vision, hearing).

Management
There is no satisfactory treatment except compression.  Some cases ( those with compromised organ function ) may require medical or surgical intervention. 

Medical treatment 

Steroids; high doses of systemic or intralesional steroids are the first-line treatment, and a dramatic response is observed in 30% of patients. Interferon-alfa. 

Surgical treatment
Complete surgical excision of these lesions offers the best chance of cure, but followed by severe functional impairment to vital functions.  Embolotherapy.  Cryosurgery.  Lasers; pulsed dye, argon and CO2. 

References 

 

http://emedicine.medscape.com/article/1084479-overview http://emedicine.medscape.com/article/1083849-overview FITZPATRICK S COLOR ATLAS AND SYNOPSIS OF CLINICAL DERMATOLOGY,SIXTH EDITION

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