The Turkish Journal of Pediatrics
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Intestinal intussusception due to a pyogenic granuloma
Zorica Stojsic1, Dimitrije Brasanac1, George Kokai2, Dragana Vujovic3, Dragana Zivanovic4, Ivan Boricic1, Dragoljub Bacetic1
1Institute of Pathology, University of Belgrade, Serbia
2Department of Histopathology, Royal Liverpool Children’s NHS Trust – Alder Hey Hospital, Liverpool, United Kingdom
3Departments of Pediatric Surgery, University Children’s Hospital, Belgrade, Serbia
4Departments of Pediatric Gastroenterology, University Children’s Hospital, Belgrade, Serbia
|Stojsic Z, Brasanac D, Kokai G, Vujovic D, Zivanovic D, Boricic
I, Bacetic D. Intestinal intussusception due to a pyogenic granuloma. Turk
J Pediatr 2008; 50: 600-603.|
Pyogenic granuloma (PG), also known as lobular capillary hemangioma, is
a benign vascular tumor, most commonly arising on the skin and the oral
mucosa. Gastrointestinal localization of PG, except for the oral cavity, is
exceptionally rare. We describe a case of ileal PG occurring in a 13-year-old
girl, presenting with intestinal obstruction.
Histological examination revealed proliferation of capillary-sized vessels, with
prominent intravascular component, involving the entire thickness of the
intestinal wall. Immunohistochemistry showed positivity for CD31, CD34
and von Willebrand factor, whereas immunostaining for glucose transporter-
1 protein (GLUT1) and for human herpes virus 8 (HHV-8) was negative.
We suggest that PG should be considered in the differential diagnosis of
childhood gastrointestinal polypoid lesions.
child, intestinal obstruction, pyogenic granuloma, small bowel
|Pyogenic granuloma (PG) is a common benign
vascular tumor that typically arises on the
skin and mucosa of the oral cavity. The basic
lesion is a lobular proliferation of capillary-sized
vessels in a fibromyxoid stroma. Accordingly,
PG has been designated as lobular capillary
hemangioma[1,2]. The lesion may develop at any
age, and both genders are affected equally.
Granuloma gravidarum and subcutaneous and
intravenous PG are rare variants of the tumor[1,3].
PGs are extremely rare in the gastrointestinal
tract, except for the oral cavity. To the best
of our knowledge, only 16 cases have been
reported in the alimentary tract, all of them
occurring in adults[4-7]. Here, we describe a new
case of PG of the ileum in a 13-year-old girl,
causing intestinal obstruction.|
|Case Presentation |
|A 13-year-old girl presented with a three-day
history of vomiting, colicky abdominal pain
and constipation. On clinical examination, the
abdomen was diffusely distended and tender.
Bowel sounds were absent. Laboratory findings showed white cell count of 14.7 x 109/L
with 67.2% neutrophils. Biochemical analysis
results were unremarkable and the patient
was afebrile. Ultrasonographic examination
of the abdomen revealed dilated loops of
small intestine with fluid accumulation. Plain
abdominal radiography showed multiple airfluid
levels (Fig. 1). The patient underwent an
emergency laparotomy with the presumptive
diagnosis of intestinal obstruction. Operative
findings revealed ileo-ileal intussusception,
80 cm proximal to the ileocecal valve, and a
large amount of brown-colored intraperitoneal
fluid. The affected gangrenous loop of the small
bowel was resected and a temporary terminal
ileostomy was performed. The postoperative
period was uneventful. After closure of the
stoma, the patient remained in good general
health, free of any symptoms.|
| ||Fig 1: Abdominal radiography shows multiple air-fluid
levels in small bowel obstruction.|
The resected specimen consisted of a segment
of small bowel measuring 50 cm in length, with
a 4x3x3 cm polypoid lesion in the lumen, which acted as a leading point for the intussusception
(Fig. 2). The bowel wall displayed intensive
congestion with a thin layer of fibrin on
the serosal surface. On opening, the lumen
contained blood-stained fluid.
| ||Fig 2: Pyogenic granuloma (arrow) of the ileum as a
leading point for intestinal intussusception.
The intestinal wall is hemorrhagic and
covered with fibrinous exudate.|
Hematoxylin-eosin (HE) and immunohistochemical
staining were performed on
formalin-fixed, paraffin-embedded tissue. For immunohistochemical examination, sections
were pretreated in a microwave oven and
incubated with primary antibodies against CD31
(Clone JC/70A; dilution 1:25; DAKO, Glostrup,
Denmark), CD34 (QBEnd/10; 1:50; DAKO), von
Willebrand factor (F8/86; 1:25; DAKO), glucose
transporter-1 protein (GLUT1) (1:25; Labvision
Neo Markers, Fremont, CA) and human herpes
virus 8 (HHV-8) (13B10; 1:50; Cell Marque,
Hot Springs, AZ). Immunohistochemistry
was performed using DAKO EnVision kit,
with diaminobenzidine as the chromogen and
Mayer’s hematoxylin for the counterstain.
Microscopic examination revealed a protuberant
growth of capillary-sized vessels, lined by a
single layer of bland endothelial cells, in a
distinctive lobular arrangement. The vascular
channels were intensely congested with massive
and extensive sludging of erythrocytes.
The lesion involved the entire thickness
of the bowel wall. The superficial portion
of the tumor and the overlying intestinal
mucosa showed hemorrhagic infarction with
ulceration. In addition, foci of intravascular
proliferation of angiomatous tissue were
present. One large vein, running from the
submucosa to the mesentery and a few
smaller veins in the submucosa contained
proliferation of minute capillaries and scarce
larger vessels with muscular wall, set in the
cellular stroma (Fig. 3). Immunohistochemistry
showed positive reaction for CD31, CD34
and von Willebrand factor in endothelial cells
of the proliferating vessels. Intense GLUT1
immunoreactivity was observed in erythrocytes as well as in the endothelium of all types of
the lesional and native vessels: capillaries,
veins, arteries and arterioles (Fig. 4). HHV-8
immunostaining was negative.
| ||Fig 3: Intravenous component of pyogenic granuloma
within bowel wall (hematoxylin and eosin,
original magnification x40).|
| ||Fig 4: False GLUT1 immunopositivity in the
endothelium of the native vein containing PG,
which normally should not be stained with GLUT1
(streptavidin-biotin, original magnification x100).|
The small bowel, outside the polypoid lesion,
showed hemorrhage affecting the entire thickness
of the wall with foci of mucosal necrosis.
|In the past, PG was regarded as a reactive
lesion secondary to trauma with superimposed
infection. Weiss and Goldblum consider the
lesion a granulation tissue-type hemangioma.
Nowadays, it has been widely accepted that PG
is a true angiomatous neoplasm, not simply a
florid proliferation of granulation tissue[1,2].|
According to the distinctive lobular arrangement
of the lesional capillaries, the lobular capillary
hemangioma has been introduced as the
designation for PG[1,2].
Pyogenic granuloma usually occurs on the skin
and the mucosa of the oral cavity. When present
on the skin, the most frequently involved sites
are the lips, face and fingers. In the oral
cavity, PG commonly involves gingiva, tongue
and cheeks. Sporadic cases of PG affecting the
genital area have been observed as well[8,9].
Only a few cases of PG in the alimentary tract,
other than those in the oral cavity, have been
reported. A gastrointestinal counterpart of the
skin PG has been proposed because of the
same macroscopic and histological features.
According to Kusakabe, only 13 cases of PG arising in the alimentary tract were published
until 2005. Since then, three additional cases
of PG, located in the esophagus, ileum and
colon, respectively, have been reported[5,6].
Gonzales-Vela identified 25 cases of intestinal
PG in the English literature, but the authors
disputed the majority of them, yielding only
one well-documented case of PG in the colon.
In total, 16 cases of PG in the gastrointestinal
tract, other than the oral cavity, have been
reported: four occurred in the esophagus, six
in the ileum, three in the colon, and one each
in the stomach, duodenum and jejunum[4-7].
The present case is the seventh reported in
All the gastrointestinal PG cases reported thus
far occurred in adult patients. We believe our
case to be the first pediatric PG arising in the
gastrointestinal tract, apart from those in the
Gastrointestinal bleeding was the most common
clinical symptom in cases with gastrointestinal
PG[4-7] and none of the reported intestinal PGs
caused intestinal obstruction. Our case was also
the largest PG among the reported alimentary
tract PGs, measuring 40 mm in diameter. The
size of the previously reported gastrointestinal
PGs was usually less than 20 mm, and only a
few of them measured 30 mm[4-7].
Intravenous PG is a rare variant of the tumor,
presenting predominantly on the neck and the
upper extremity[3,10,11]. The tumor arises from
the wall of the vein and protrudes deeply into
the lumen. In the alimentary tract, only one
case of intravenous PG, located in the jejunum,
was published, in a small series of Yao et al..
To the best of our knowledge, the current case
is the second reported gastrointestinal PG with
the intravenous PG component.
Major differential diagnoses of PG include inflammatory
polyp, well-differentiated angiosarcoma,
Kaposi’s sarcoma, and juvenile capillary hemangioma[1-3,6]. Inflammatory polyps are usually
multiple, occurring in association with chronic
intestinal diseases. Histologically, they lack the
lobular pattern. Angiosarcoma is differentiated
from PG by infiltrative growth, irregular vascular
spaces and cellular atypia. Similarly, Kaposi’s
sarcoma is not well circumscribed and it contains,
at least focally, cellular zones of spindle cells
and slit-like vascular spaces. This tumor was
additionally excluded by negative HHV-8 immunostaining. Although never reported in the
alimentary tract, juvenile capillary hemangioma
and congenital hemangiomas should be included
in differential diagnosis due to their lobular
growth. The diagnosis of juvenile capillary
hemangioma is based on the immunopositivity
for GLUT1, the erythrocyte-type glucose
transporter protein. GLUT1 is exclusively
expressed in juvenile capillary hemangiomas,
but not in congenital hemangiomas, vascular
malformations, pyogenic granulomas, and
granulation tissue[12-14]. In extracutaneous infantile
hemangiomas, GLUT1 immunoexpression
is variable, being also positive, or absent/
reduced. GLUT1 is specifically expressed in
the endothelium of capillaries of the lesion, but
not in the arteries and arterioles or native blood
vessels. Accordingly, we regard the GLUT1
immunoreaction as false-positive since we
found the positive reaction in endothelial cells
of both the arteries and veins within the lesion
itself, and of those outside of the lesion. We
assume that this false immunopositivity could be
caused by very marked congestion and sludging
of erythrocytes developed during intestinal
intussusception. Noninvoluting congenital
hemangioma and a similar lesion, congenital
nonprogressive hemangioma, have the lobular
and occasional intravascular growth pattern
and GLUT1 immunonegativity in common with
PG[13,17,18]. These tumors differ from PG in that
they exhibit intra- and extralobular fibrosis,
stromal hemosiderin deposits, focal thrombosis
and sclerosis of capillary lobules[13,18], features
that were absent in our presented case.
In conclusion, we report herein the first documented
case of pediatric PG of the gastrointestinal tract
causing small intestinal intussusception. We
suggest that PG should be considered in the
differential diagnosis of gastrointestinal polypoid
lesions in children.
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