Penile Hemangioma

17

Abstract
Penile hemangioma is a very rare benign vascular neoplasm, mostly seen
in children and young adults with different features.

Keywords
Haemangiomas • Strawberry nevus • Klippel-Trenaunay syndrome •
Kasabach-Merritt syndrome • Tissue expander • Angiokeratoma of Fordyce

Incidence 17.1 Definition

Generally haemangiomas are the most common Haemangiomas are benign vascular lesions
pediatric neoplasm, occur in about 10 % of all resulting from abnormal proliferation of blood
births, and usually appear between 1 and 4 vessels, capillary hemangioma appears as a
weeks after birth, but penile hemangioma com- raised, red, lumpy area of flesh anywhere on the
prises less than 1 % of all hemangiomas. body, without any known etiology (Fig. 17.1).
Capillary hemangioma is the most common
variant of hemangioma, it occurs 5 times more
often in female infants than in males, and mostly
in Caucasian populations. The incidence of cap-
illary hemangioma in the penis and scrotum is
rare, which presented with scrotal enlargement
and tenderness, only a few number of articles
describing scrotal hemangioma have been
reported [1].

Nomenclatures

A capillary hemangioma also known as an

Infantile hemangioma, Strawberry hemangioma, Fig. 17.1 Capillary haemangioma of the penis, with
and Strawberry nevus. glanular affection

© Springer International Publishing Switzerland 2017 115

M. Fahmy, Congenital Anomalies of the Penis, DOI 10.1007/978-3-319-43310-3_17
116 17 Penile Hemangioma

17.2 Classification basic findings; enlarging haemangioma, throm-

bocytopenia and consumption coagulopathy
Hemangiomas are generally classified into capil- [2]. Klippel-Trenaunay syndrome (KTS) is
lary, cavernous, arteriovenous, venous and mixed another syndrome with genital affection, it was
subtypes and may be composed of vessels whose first described in 1900 by two French physi-
walls are abnormal and cannot be identified as cians, Klippel and Trenaunay. It is a rare con-
arterial or venous (Figs. 17.2 and 17.3). Complex genital syndrome of venous, lymphatic, and
vascular malformation of the penile skin may capillary malformations (port-wine stain and
give a false picture of penile gigantism or mega- varicose veins) with soft tissue and bone hyper-
lopenis (Chap. 10). trophy. Patients can be diagnosed with KTS
Penoscrotal area may be affected along the with only one or more of the above-mentioned
vascular syndromes like; Kasabach-Merritt features since patient might not have all the fea-
syndrome which is a rare type of vascular lesion tures [3]. Scrotum usually affected along the
with peculiar characteristics based upon three course of the diseases, and rarely the lesion
may be extended to the penile shaft or the pre-
puce (Figs. 17.4 and 17.5).

Fig. 17.2 Mixed arteriovenous malformation affecting Fig. 17.3 Mixed vascular malformation of the penis with
the skin of the penis, prepuce and scrotum a superficial capillary heamnagioma

Fig. 17.4 Kasabach-­

Merritt syndrome affecting
the right lower limb
17.4 Management 117

lished, eradication of the lesion is recommended

and the lesion must be complete removed to
avoid recurrence. Genital haemangioma deserve
special attention than other body area, as small
hemangioma is liable to trauma, ulceration, and
disfigurement if untreated at childhood, it will
interfere with normal sexual activities with sub-
sequent psychic trauma and depression, also
minute residual scar in the penis and glans will
not be appreciated by patients or parents.
Fig. 17.5 Penoscrotal lesions of Kasabach-Merritt syn-
Propranolol is thought to inhibit the growth of
drome, “same patient in Fig. 17.4”
blood vessels and constrict existing blood vessels
within the haemangioma, and should be tried in
17.3 Diagnosis all cases with an appropriate dose [5], small
lesions like the one seen in Fig. 17.6 could be
Some haemangiomas are very small and controlled with laser application.
hardly visible while others are large produc- Bulky venous, or mixed genital vascular mal-
ing with a significant disfigurement (Fig. 17.1). formation should be excised surgically with the
Arteriovenous malformation and mixed vascular expected problem of skin deficiency to cover the
anomalies, are rarely diagnosed in the genital penile shaft, which could be achieved by either skin
region, usually obvious since birth, but it may graft or rotation flap. Alternatively, specially in
show progressive increase in size with age, it cases with large extensive lesion, a serial excision
may be subjected to ulceration with severe bleed- with tissue expansion was feasible and applicable
ing from minor trauma, also it may get infection with manageable complications and acceptable
with skin erosion and ulcerations (Figs. 17.2 and by the patients and their parents [6]. Figures 17.7,
17.3). Ultrasonography is useful for diagnosing
scrotal and penile hemangiomas, but rarely defin-
itive. It can determine the extent of the lesion,
delineate its relationship with adjacent structures,
demonstrate the nature of the mass and help plan
therapy. In sonography, hemangiomas vary from
hypoechoic to hyperechoic, or they may be het-
erogeneous. Colour Doppler may demonstrate
blood flow within these lesions but the absence of
flow does not rule out the presence of the lesions.
Magnetic Resonance Imaging (MRI) may pro-
vide more useful information for differentiation
between haemangiomas and other vascular mal-
formations, specially the angio MRI; which will
give a precise idea about the feeding vessels [4].

17.4 Management

Capillary hemangiomas: It may grow rapidly,

before stopping and slowly fading. Some are
Fig. 17.6 Small haemangioma of the glans penis, this
gone by the age of 2, about 60 % by 5 years, and
small lesion is liable to bleeding from minor trauma, and
90–95 % by 9 years. When the diagnosis is estab- could be managed with laser
118 17 Penile Hemangioma

17.8, 17.9, and 17.10 showing a neonate with

huge mixed vascular malformation involving the
penis, prepuce, proximal scrotum and suprapubic
region, I managed him with sequential implanta-
tion of three different sizes tissue expanders in the
normal lower abdominal wall, repeated serial exci-
sion of the malformed skin and rotational flaps to

Fig. 17.7 Huge vascular malformation of the penis and

lower suprapubic region in a neonate

Fig. 17.9 Same patient after three sessions of tissue

expansion

cover the penis and scrotum at our centre, so he

reach to an acceptable look by the age of 12 years
(Fig. 17.10).
Kasabach-Merritt syndrome shows wide vari-
ation in its response to different treatment modal-
ities, as different interventions are recommended
including compression, embolization, use of
interferon, steroids, laser therapy, sclerotherapy,
chemotherapy, radiation and surgery [2].
Mucosal lentigines (also known as “Labial,
penile, and vulvar melanosis,” and “Melanotic
macules”) is a cutaneous condition characterized
by light brown macules on mucosal surfaces, and
very rarely it may affect the penile shaft [7].
Angiokeratoma of Fordyce (also known as
Angiokeratoma of the scrotum and vulva) is a
Fig. 17.8 Same patient in Fig. 17.6 at the age of 3 years skin condition characterized by red to blue pap-
with lower abdominal wall tissue expander ules on the scrotum or vulva.
References 119

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scrotum, perineum, and rectum- A rare tumor. Saudi
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gov/pubmed/23728247.
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2010;81(2):167–74.
Fig. 17.10 Same patient in Fig. 17.6 at the age of 12
years after 8 subsequent operations with almost complete
removal of vascular anomalies and restoration of near nor-
mal penoscrotal look