Infantile (Juvenile) Hemangioma


Clinical Photograph
Photograph shows a medium-sized infantile hemangioma on the face of a female infant. Most of these lesions are located in the head and neck region. (Courtesy J. Hall, MD.)



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Infantile Hemangioma: Lobular Growth
Low-power examination reveals multiple lobules composed of tightly packed capillaries, separated by fibrous septa. Note the scattered, centrally located feeding and draining vessels image.

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Facial Infantile Hemangioma
Facial infantile hemangioma is seen in a patient with PHACES (posterior fossa malformations, hemangiomas, arterial and cardiac anomalies, eye abnormalities, and sternal cleft raphe). (Courtesy S. Yashar, MD.)

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Infantile Hemangioma: Ulcerated
The skin overlying infantile hemangioma may ulcerate, resembling an inflamed granulation tissue or pyogenic granulomas. The sharp separation between the 2 nodules of proliferating capillaries is a useful diagnostic clue.


TERMINOLOGY


Synonyms




• Hemangioma of infancy

• Juvenile hemangioma

• Cellular hemangioma of infancy

• Strawberry nevus/hemangioma


Definitions




• Benign proliferations of endothelial tissue; represent most common tumors arising in neonatal period
• Vascular neoplasm of infancy with characteristic onset, rapid growth, and spontaneous involution

• Does not include congenital hemangiomas, as they are clinically, histologically, and immunohistochemically distinct from infantile hemangiomas

image Both below are negative for GLUT1 and Lewis-Y antigen

– Rapidly involuting congenital hemangioma

– Noninvoluting congenital hemangioma


CLINICAL ISSUES


Epidemiology




• Incidence
image Most common tumor of infancy

image Affects ~ 4% of children

• Sex
image F > M

• Ethnicity
image Caucasians more frequently affected


Site




• Skin and subcutis
image Head and neck (60%)

image Extremities, trunk, and genitals

• Viscera


Presentation




• Appears within 1st few weeks after birth
image Blanched telangiectatic area


Natural History




• Rapidly enlarges over several months
image Maximum size usually achieved by 6-12 months

• Hemangiomas typically achieve 80% of their final size by end of early proliferative phase
image Occurs at mean age of 3.2 months

• Regresses over several years
image 75-90% involute by age 7 years


Treatment




• Options, risks, complications
image Propranolol

image Corticosteroids

image Pulsed dye laser

image Surgical excision

image Watchful waiting

– Small innocuous lesions

image Interferon-α

– Restricted to life-threatening lesions

image Topical imiquimod


Prognosis




• Excellent; all eventually spontaneously regress


MACROSCOPIC


General Features




• Crimson-colored multinodular mass

• Can start out as flat red or purple patch, frequently less than 5 cm in diameter

• Then gradually enlarges and develops raised surface


MICROSCOPIC


Histologic Features




• Multiple lobules composed of tightly packed small- to medium-sized capillaries
• Early lesions

image Plump endothelial cells that line small vascular spaces

image Inconspicuous vascular lumina

image Distinct lobules separated by normal stroma

image Moderate mitotic activity and scattered mast cells

Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Infantile (Juvenile) Hemangioma

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