Surgical Science, 2011, 2, 379-384
doi:10.4236/ss.2011.27083 Published Online September 2011 (http://www.SciRP.org/journal/ss)
Infantile Hemangiopericytoma: Two Cases Report
and Literature Review
Gamal Al-Saied1, Amin Abokifa1, Mohamed Al-Saeed1, Mohamed Abdul Aziz1, Medhat Ibrahim1,
Mohamed Shahin1, Tharwat Hussain1, Moemen Hafez2, Dalal Nemenqani2, Naglaa Fawzy3
1Departme n ts of Pediatric S u r g er y, King Abdul Aziz Specialist Hospital, Taif, KSA
2Departments of Pat h ol o gy, King Abdul Aziz Specialist Hospital, Taif, KSA
3Departments of Radiology, King Abdul Aziz Specialist Hospital, Taif, KS A
Received February 14, 2011; revised April 15, 2011; accepted April 23, 2011
Abstract
Hemangiopericytoma is a rare vascular tumour of infants. Although generally considered to be benign, local
recurrence and metastases can occur. Herein, we report on two full term girls, delivered with lumbosacral
swelling and left thigh swelling respectively. Complete surgical excision with safety margins was performed
for each lesion. Histologic examination of both lesions showed picture of infantile hemangiopericytoma.
There is no evidence of local recurrence or distant metastasis during last 20 and 17 months for 1st case and
2nd case respectively. In conclusion; most infantile hemangiopericytoma follow a benign course. Rarely these
tumours behave aggressively with local infiltration, recurrences and even distant metastases. Careful follow
up is therefore essential.
Keywords: Infantile Hemangiopericytoma, Vascular Tumour
1. Introduction
Hemangiopericytoma is an uncommon tumour of infants
which originates from the vascular pericytes, which are
contractile cells that spiral around capillary walls.
Therefore, hemangiopericytoma have a wide distribution
in both sof t tiss u e and skel et al sy st em [1 ].
Although generally considered to be benign, distant
metastases can occur [2].Histologic examination is the
only way to establish the diagnosis of hemangiopericy-
toma. Hemangiopericytoma is unresponsive to steroid
therapy, unlike other vascular malformations, resection
is the treatment of choice [2]. The prognosis of the tu-
mour behaviour is still not feasible and it has the poten-
tial to demonstrate a highly malignant course [1].
Long-term postoperative follow-up is therefore essential
for the early detection of local recurrence and distant
metastases [2]. Herein, we report on the outcome of the
initial treatment of two full term girls, delivered on
January and March 2009 at King Abdul Aziz Specialist
Hospitl, Taif, Saudi Arabia; with lumbosacral swelling
and left thigh swelling respectively finally diagnosed as
infantile hemangiopericytoma by histologic examina-
tion.
2. Case I
3 day-old, full term girl, 3.5 kg, delivered with lum-
bosacral swelling. The baby was generally doing fine,
pinkish on room air, active and crying. Moving her legs
actively. The swelling was measuring 7 × 6 cm, hetero-
geneous consistency, firm in some areas and cystic in
others, non tender, non reducible, non compressible, non
fluctuant. Immovable, fixed to the overlying skin and
under lying structures. On auscultation there is no bruit.
The skin over it was n ormal. No l ymphade nopathy and no
viceromegaly. No other associated congenital anomalies
(Figure 1(a)).
CBC and chemistry were within normal limit. Alpha
fetoprotein was (47,000 ng/ml) and b-hCG (3.05) . Chest
X-rays was normal. CT scan with IV contrast of lum-
bosacral region showed a well defined oval mass with
normal density and sign of calcification of fat or cystic
components but there was no sign of bone erosion or
defect (Figure 1(b)). MRI of lumbosacral region re-
vealed a well defi ned oval m ass appears nor mointense on
T1w and slight hyperintense on T2w images in the right
side of lum bo-sacral canal continued wit h fibrotic ban d at
sacral region without bone erosions or tethering of the

G. AL-SAIED ET AL.
380
Figure 1. (a) 3 day-old, f ull ter m girl , 3.5 kg., deliv ered w ith l umbosacral swelling. 7 × 6 × 7 cm, heterogeneou s consistency, non
tender, non reducible, non compressible and non fluctuant; (b) CT with IV contrast of 1st case revealed solid mass in the
lumbosacral region without extension to spinal canal (white arrow); (c) Complete surgical excision was done for the lum-
bosacral mass with safety margins; (d) 1month after surgery, the w ound is nicely healed (arrow ); (e) Hematoxylin and Eosin
stained section showe d plump spindle sha ped ce lls with pr ominent vascular pattern consisting of thin walled branching vessel
(antler configuration). (H&Ex200); (f) Reticulin stained section showed dense reticulin network surround blood vessels and
individual tumour cells. (Strept.A.B.te ch. × 400); (g) Immunhistochemistry revealed, P ositive  Vimentin, Positive  CD34
Positive  S100, Focal positive  Actin (myopericytes), Negative  Desmin, Negative  Cytokeratin. Branching vascular
spaces some of them showed stag horn configuration (arrow).

G. AL-SAIED ET AL.
381
cord. Conus medullaris was normal. Initially diagnosed
as teratoma versus myelomeningocele.
After induction of general endotracheal anaesthesia,
the girl is placed in prone position with the pelvis ele-
vated. The skin of lumbosacral region down to mid -th igh
is prepared by cleaning with prewarmed povidone io-
dine. A single dose of broad spectrum antibiotics is
given on induction of anaesthesia. A horizontal skin
incision is made over the lumbosacral mass. Then, the
upper and lower skin flaps are dissected off the mass
using bipolar cautery and optical magnification. The
mass was dark red in colour with heterogeneous consis-
tency, firm in some areas and cystic in other areas. Then,
the mass is dissected off the fascia lumbodorsalis and the
fibrotic band tethering the mass to sacral region. There
was no bony defect or connection with dura of lumbo-
sacral canal or tethering of the cord. The subcutaneous
tissue is re-approximated with Vicryl 3/0 and the skin
closed with subcuticular sutures of Vicryl 4/0 (Figure
1(c) and (d)). The excised mass is sent to histopa-
thological examination. The postoperative course was
uneventful. The girl is discharged on the 3rd postopera-
tive day and followed up in outpatient clinic.
The histological and immunhistochemistry features
were consisting with infantile hemangiopericytoma
(Figure 1(e)-(g)). There was no eviden ce of local recur-
rence or distant metastasis during last 20 months.
3. Case II
7 day-old full term girl, 4.140 kg, the baby was pinkish
on room air, active and crying. Moving her legs actively.
Systemic examination was unremarkable. Local exami-
nation revealed a firm, violet-coloured swelling on the
anterior surface of left thigh, and measuring 5 × 6 cm.
There was marked surface telangiectasia and focal
crusting. The surface was easily indented. Non tender to
palpa tion. Non compr essible, non fluctuant. A ttached to
the overlying skin and partially movable over the under
lying muscle and fascia. The lesion was non-pulsatile.
On auscultation there was no bruit. No lymphadenopa-
thy and no hepa tom egal y. No ot her as soci at ed c onge nit al
anomalies (Figure 2(a)).
CBC and chemistry were within normal limit. CT an-
giogram of the pelvis and thigh revealed; a well defined
multilocular cystic lesion is seen subcutaneous in the
anterior aspect of the left upper thigh. It shows faint
enhancement of its wall and septae with no dominant
feeding arteries. There are two main draining veins for
this lesion, one on its upper medial aspect draining into
the left femoral vein, and the other one extends from the
lower medial aspect and drains into the left great
saphenous vein (Figure 1(b)). Initially diagnosed as a
hemangiomatous malformation.
After induction of general endotracheal anaesthesia,
the girl is placed in supine position. The skin of left
thigh is prepared by cleaning with prewarmed povidone
iodine. A single dose of broad spectrum antibiotics is
given on induction of anaesthesia. An elliptical trans-
verse skin incision is made around the mass. Then, mass
is dissected off the underlying quadriceps femoris muscle
using bipolar cautery and optical magnification. On cut
section the mass was white-grey in colour and soft in
consistency. The upper and lower skin edges are under-
mined with good h emostasis. The subcutaneous tissue is
re-approximated with Vicryl 3/0 and the skin closed
with subcuticular sutures of Vicryl 4/0 (Figure 2(c) and
(d)). The excised mass is sent to histopathological exami-
nation. The gir l is discha rged o n the 3rd postoperative day
and followed up in outpatient clinic.
The histological and immunhistochemistry features
were consisting with infantile hemangiopericytoma (Fi-
gure 2(e)-(g)). There was no evidence of local recur-
rence or distant metastasis during last 17 months.
Microscopic Description of Both Lesions, Hema-
toxylin and eosin stained sections revealed plump spin-
dle shaped cells with prominent vascular pattern con-
sisting of thin walled vessels lined by single layer of
flattened endothel ial c el ls w i th gapping v as cu l ar spa c es,
some of the m show ed s tag ho rn c onfi gu rati on . I ncrea sed
mitotic activity was noted (Figure 1(e) and Figure
2(e)).
Reticulin stained section showed dense reticulin net-
work surrounding blood vessels and individual tumour
cells. Focal myxoid area was noted. Isolated tumour
nodules within the fibro pseudocapsule of the main mass
were seen (Figure 1(f) and Figure 2(f)).
Immunhistochemistry of Both Lesions revealed,
Positive  Vimentin, Positive  CD34 (endothelial
lini n g o f inters p ersed v a s cular sp a c e s), Po s i tive  S100,
Focal positive  Actin (myopericytes), Negative 
Desmin, Negative  Cytokeratin. (Figure 1(g) and
Figure 2(g)).
The morphological criteria suggested by Enzinger and
Smith were used to distinguish a benign from a malig-
nant tumour. The criteria were: large size (>5 cms), in-
creased mitotic rate (4 or more per 10 HPF), high degree
of cellularity, immature and pleomophic tumour cells,
foci of hemorrhage an d ne crosis [3 ].
These histological and immunhistochemistry features
of both lesions were consisting with infantile heman-
giopericytoma. In accordance with Enzinger and Smith’s
criteria both lesions were histologically benign. No evi-
ence of malignancy at the time of examination. d

G. AL-SAIED ET AL.
382
Figure 2. (a) 7 day-old full term girl, delivered with a firm, violet-coloured swelling on the anterior surface of left thigh,
measuring 5 × 6 cm, with marked surface telangiectasia and focal crusting; (b) CT angiogram of pelvis and thigh showing a
well defined multilocula r c ystic lesion. It shows faint enhancement of its wall and septae with no dominant feeding arteries; (c)
Complete elliptical surgical excision was performed with safety margins; (d) After complete surgical excision with safety
margins, the biopsy was sent for histopathological examination; (e) Hematoxylin and Eosin stained section showed plump
spindle shaped cells with prominent vascular pattern consisting of thin walled branching vessel (antler configuration). In-
creased mitotic activity was noted. (H & E × 400); (f) Reticulin stained section showed dense reticulin network surround blood
vessels and individual tumour cells. (Strept.A.B.tech. × 400); (g) Immunhistochemistry revealed, Positive  Vimentin, Positive
 CD34 (endothelial lining of interspersed vascular spaces), PositiveS100, Focal positiveActin (myopericytes), Negative
Desmin, Negative  Cytokeratin.
4. Discussion
Infantile hemangiopericytoma is a rare vascular tumour
in children below one year of age [4]. In 194 2, Stout and
Murray [5] first described hemangiopericytoma in the
lungs as that arising from the vascular pericytes of
Zimmermann which are contractile cells that spiral
around capillary walls and differing from a glomus tu-
mour or a hemangioma but they did not differentiate
between infantile and adult forms. The infantile type
occur in 10% of cases [6] and in boys more than in girls
[7, 8]. Approximately 30% to 50% are fo und in the limbs

G. AL-SAIED ET AL.
383
with the remainder either in the head and neck or the
trunk. There is seldom discolouration of the lesions in
the limb in contrast to those in the head and neck [6].
Infantile hemangiopericytoma tends to be located more
superficially than that in adults [9].
In our two cases, both masses were lying subcutane-
ously, in lumbosacral region without skin discoloration
in 1st cases. Whereas, the 2nd mass was violet-coloured
on the anterior surface of left thigh.
The diagnosis can be aided by plain radiography
which shows a soft-tissue mass, with or without calcific
stippling. Ultrasound determines the size of the lesion,
vascular loops, the echogenic content and whether or not
a capsule is present, and angiography shows the vascular
pattern and the presence of feeder vessels. CT and MRI
can provide information about the extent of the tumour
and help in preoperative planning [9].
MRI and CT scan of lumbo-sacral canal (pre-and post
IV contrast ) of 1st case incorrectly interpreted the lesion as
teratoma versus myelomeningocele.
CT angiogram of pelvis and thigh of 2nd case, initially
diagnosed the lesion as a hemangiomatous malformation.
The diagnosis is confirmed by excisional biopsy [9].
The gross appearance of this lesion is usually transparent
or white-grey [6]. Feeder vessels may be prominent, and
the infantile form tends to be more multilobular than in
the adult. Microscopically; the pericyte may be round or
oval in shape. The tumour tends to be densely cellular,
with prominent vascular channels. Mitotic figures are
usually easily identified. The tumour cells stain posi-
tively with vimentin [6,9].
In our two cases the final diagnosis was achieved by
excisional biopsy. The histological and Immunhistochem-
istry features were consisting with infantile heman-
giopericytoma. In accordance with Enzinger and Smith's
criteria [3], both lesions were histologically benign.
There was no evidence of malignancy at the time of ex-
amination.
The differential diagnosis of congenital hemangio-
pericytoma includes all tumours which present as a soft
tissue mass, including lipoma, hemangioma, and lym-
phangioma. Some tumours have similar histological fea-
tures, such as infantile myofibromatosis, synovial sar-
coma, fibrosarcoma, malignant fibrous histiocytoma,
mesenchymal chondro sarcoma, and leiomyosarcoma [10-
13]. It is therefore impo rtan t to examine multip le areas of
the specimen to confirm the diagnosis [9].
The management of infantile hemangiopericytoma
differs from that in adults, but there is no consensus as to
the best method [9]. Wide surgical excision with safety
margin is the most accepted method of treatment
[6,14,15]. That is was our preferred method in manage-
ment of both cases. Some centres use adjuvant therapy to
reduce the risk of local recurrence [6-20] but the value is
difficult to assess because of the rarity of these tumours.
Chemotherapeutic regimens have included the use of
vincristine, doxorubicin, actinomycin and cyclophos-
phamide. Jha et al. [21] reported success with radiother-
apy when there was gross or microscopic evidence of
tumour remaining after excision, and adjuvant radio-
therapy was used with success by Borg and Benjamin
[22]. Other authors have reported that radiotherapy is
ineffective [14,15,23]. Metastatic disease has only been
reported in three cases of congenital hemangiopericy-
toma [4,15,24] although the adult form of the tumour has
a rate of distant metastasis of up to 56%, mainly to the
lung and skeleton [8,25]. Careful follow up is therefore
required. When recurrence does occur, adjunctive che-
motherapy has been used successfully [15].
Fortunately, there is no evidence of local recurrence or
distant metastasis during last 20 and 17 months in 1st
case and 2nd case respectively.
5. Conclusions
Most infantile hemangiopericytoma follow a benign
course. Rarely these tumours behave aggressively with
local infiltration, recurrences and even distant metastases.
Careful follow up is therefore essential.
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