Hemangioma

Scrotal Hemangioma in an Infant

A 24-month-old boy presented to the clinic for a routine health examination. He was a singleton, born at term, and he was an appropriate weight. At birth, his physical examination results were unremarkable except for a reddish-purple hemangioma on the right scrotum (Figure). At 14 months of life, he was referred to a dermatologist, and the family was advised to watch and wait for the lesion to spontaneously resolve. However, the lesion has remained unchanged. 

At the clinic, the boy underwent ultrasonography of the scrotum, which revealed a thickened, hypervascular anterior right hemiscrotal wall without extension into surrounding structures. The patient was referred to dermatology and urology for further evaluation.

DISCUSSION

Hemangiomas are proliferations of vascular endothelial cells that typically present at or soon after birth or appear via abnormal vasculogenic stimulation during the first few years of life.1 They are found in up to 10% of infants by 1 year of age, but genital hemangiomas comprise only 2% of the total.2,3 

Hemangiomas arise from primitive blood vessels known as angioblasts and are hypothesized to proliferate due to in-utero hypoxia.4,5 Some propose that hypoxia causes vascular proliferation in hemangiomas in order to improve the oxygen content of hypoxic tissues.6 They are more common in infants who experience placental hypoxia, such as in cases of multiple gestations, prematurity, and low birth weight.7,8 Other factors include overexpression of vascular endothelial growth factor (VEGF), fibroblast growth factor 2, and type IV collagenase that promote angiogenesis and endothelial cell proliferation as well as imbalances in VEGF receptor tyrosine kinases.9-12 Although most hemangiomas occur sporadically, familial transmission has been reported.13 The natural history involves an early proliferation phase followed by involution that typically does not require medical intervention; 30% of hemangiomas resolve by 3 years of life, 50% by 5 years of life, and 70% to 90% by 7 years of life.2,14,15 

Most hemangiomas are clinically diagnosed.16 However, imaging studies can detect underlying structural abnormalities.17 Ultrasonography results can reveal hyperechoic or hypoechoic areas, depending on the contents of the vascular abnormality.17,18 Computed tomography and magnetic resonance imaging help define the relationship of the hemangioma with adjacent anatomical structures before intervention.17 A typical radiographic finding is a soft-tissue mass containing phleboliths, suggestive of a cavernous hemangioma.17 Finally, if the anatomical abnormality is thought to be malignant, a biopsy may be warranted.19 

In contrast to hemangiomas in other locations, treatment for scrotal hemangiomas may help prevent complications.2 Reported complications have included hemorrhage, rectal bleeding, or hematuria through extension of the lesion into the rectum or bladder, ulceration with subsequent infection, possible decreased fertility due to the heat generated by the hemangioma, scrotal hemorrhage after trauma, or decreased testicular size ipsilateral to the hemangioma.20 Propranolol, given orally, is the first-line treatment, with a target dose of 1 to 3 mg/kg/day, divided in 3 daily doses at least 6 hours apart.21 It causes vasoconstriction and inhibition of angiogenesis, which leads to apoptosis of the affected tissue.20 If propranolol fails, surgery with preservation of the scrotum and minimal testicular tissue removal is another option.20 Less invasive procedures such as flash dye pulsed laser, cryotherapy, or sodium chloride 15% infusion can also be useful. Because of the increased risk for complications, referral to dermatology and/or urology should be considered if spontaneous involution does not occur. 

Kelly Wilmas, BS, and Hunter P. Nolen, BS, are with the John P. and Kathrine G. McGovern Medical School at University of Texas Health Science Center in Houston.

Lynnette Mazur, MD, MPH, is a professor of pediatrics at the John P. and Kathrine G. McGovern Medical School at University of Texas Health Science Center in Houston.

References 

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19.     Frieden IJ, Eichenfield LF, Esterly NB, Geronemus R, Mallory SB. Guidelines of care for hemangiomas of infancy. American Academy of Dermatology Guidelines/Outcomes Committee. J Am Acad Dermatol. 1997;37(4):631-637.

20.     Patoulias J, Farmakis K, Kaselas C, Patoulias D. Ulcerated scrotal hemangioma in an 18-month-old male patient: a case report and review of the literature. Case Rep Urol. 2016;2016:9236719.

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