AJR 2001; 177:1073-1081
© American Roentgen Ray Society
Gastrointestinal Hemangiomas
Imaging Findings with Pathologic Correlation in Pediatric and Adult Patients
Angela D. Levy1,2,
Robert M. Abbott2,3,
Charles A. Rohrmann, Jr.1,4,
Aletta Ann Frazier1,5 and
Amir Kende6
1
Department of Radiologic Pathology, Armed Forces Institute of Pathology, 6825
16th St. N.W., Bldg. 54, Rm. M-121, Washington, DC 20306-6000.
2
Department of Radiology and Nuclear Medicine, Uniformed Services University of
the Health Sciences, 4301 Jones Bridge Rd., Bethesda, MD 20814.
3
Department of Radiology, Wilford Hall Medical Center, Lackland Air Force Base,
San Antonio, TX 78238.
4
Department of Radiology, University of Washington, 1959 NE Pacific, Seattle,
WA 98195-7115.
5
Department of Radiology, University of Maryland Medical System, 29 S. Greene
St., Baltimore, MD 21201-1544.
6
Department of Gastrointestinal Pathology, Armed Forces Institute of Pathology,
Washington, DC 20306-6000.
Received February 14, 2001;
accepted after revision March 8, 2001.
The opinions and assertions contained herein are the private views of the
authors and are not to be construed as official or as representing the views
of the Departments of the Army, Air Force, or Defense.
Address correspondence to A. D. Levy.
Introduction
Gastrointestinal hemangiomas are uncommon benign vascular tumors that may
occur anywhere in the gastrointestinal tract as single or multiple lesions.
Multiple lesions are often associated with similar neoplasms in other organs,
such as the liver and skin, and may also be caused by Osler-Weber-Rendu
disease, Maffucci's syndrome,
Klippel-Trénaunay syndrome, or the congenital
blue rubber bleb nevus syndrome. Gastrointestinal bleeding is the most common
clinical presentation. The bleeding may be slow and insidious or massive and
life-threatening. Patients may also present with abdominal pain, mechanical
bowel obstruction, intussusception, or perforation. Gastrointestinal
hemangiomas may be polypoid or diffusely infiltrating in appearance on gross
pathology, giving rise to a spectrum of radiologic appearances.
Our article is based on the records of 22 patients with gastrointestinal
hemangiomas accessioned into the radiologic pathology archive at the Armed
Forces Institute of Pathology over a 27-year period. The case material
includes two esophageal hemangiomas, one gastric hemangioma, 12 hemangiomas of
the small intestine, and six of the colon. In one patient, the entire abdomen
was involved. Four of the six cases of patients with hemangiomas of the colon
have been included in a review of colorectal hemangioma
[1], and the case of the single
patient with gastric hemangioma has been published as a case report
[2]. The purpose of this
pictorial essay is to review the imaging manifestations of gastrointestinal
hemangiomas with pathologic correlation.
Pathologic Features
Most hemangiomas are seen as pedunculated intraluminal polypoid masses on
gross pathology, but occasionally they may have an infiltrative submucosal
growth pattern (Fig.
1A,1B,1C).
They may be solitary, multifocal, or diffuse. Hemangiomas are typically red,
purple, or bluish (Fig. 2) and
are soft and compressible unless they contain areas of thrombosis or
phleboliths [3].
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Fig. 1A. —Hemangioma growth patterns. Drawings depict solitary polypoid
growth of hemangioma in bowel lumen (A), multifocal polypoid
hemangiomas in bowel lumen (B), and infiltrative and annular growth of
hemangioma with intraluminal and extraserosal extension of tumor
(C).
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Fig. 1B. —Hemangioma growth patterns. Drawings depict solitary polypoid
growth of hemangioma in bowel lumen (A), multifocal polypoid
hemangiomas in bowel lumen (B), and infiltrative and annular growth of
hemangioma with intraluminal and extraserosal extension of tumor
(C).
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Fig. 1C. —Hemangioma growth patterns. Drawings depict solitary polypoid
growth of hemangioma in bowel lumen (A), multifocal polypoid
hemangiomas in bowel lumen (B), and infiltrative and annular growth of
hemangioma with intraluminal and extraserosal extension of tumor
(C).
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Fig. 2. —48-year-old man with melena and multiple cavernous
hemangiomas of small bowel. Photograph of resected cut specimen of small bowel
reveals multiple bluish polyps emanating from small-bowel mucosa.
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Histologically, hemangiomas are classified and named according to their
major components. There are two principal types: capillary and cavernous.
Capillary hemangiomas are a proliferation of small capillaries composed of
thin-walled, blood-filled spaces lined by endothelial cells (Fig.
3A,3B).
Cavernous hemangiomas consist of large blood-filled spaces or sinuses lined by
single or multiple layers of endothelial cells (Fig.
4A,4B).
Focal calcification, thrombi, and hyalinization may be present and represent
degenerative changes. Cavernous hemangiomas may infiltrate large segments of
the intestine and mesentery.
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Fig. 3A. —62-year-old man with capillary hemangioma. Photomicrograph of
resected small intestinal hemangioma shows pedunculated polyp arising from
submucosa. Surface erosion of overlying mucosa covering polyp has occurred,
and focus of hemorrhage within polyp (arrow) is visible. (H and E,
x4)
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Fig. 3B. —62-year-old man with capillary hemangioma. Photomicrograph at
greater magnification than A shows numerous thin-walled capillaries
(arrows) lined with endothelial cells. Capillaries are separated by
stromal edema. (H and E, x80)
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Fig. 4A. —20-year-old man with cavernous hemangioma. Photomicrograph of
colonic resection shows proliferation of large blood-filled spaces in
submucosa and pericolonic soft tissue. (H and E, x4)
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Small Intestine
The small intestine is the most common site of gastrointestinal
hemangiomas, which represent approximately 7-10% of all benign tumors in the
small intestine. Patients of any age may be affected; men are more than 1.5
times more likely to have hemangiomas in the small intestine than are women
[3]. Most patients present with
evidence of acute or chronic gastrointestinal bleeding. Obstruction (Fig.
5A,5B),
intussusception (Fig.
6A,6B,6C),
and perforation may also occur. The jejunum is the most common site of
involvement.
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Fig. 5A. —Capillary hemangioma of duodenum in 7-week-old boy who
presented with vomiting and palpable right upper quadrant mass. Radiograph of
upper abdomen obtained with patient in supine position shows soft-tissue mass
displacing and obstructing duodenum. Artifacts from clothing are also
present.
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Fig. 5B. —Capillary hemangioma of duodenum in 7-week-old boy who
presented with vomiting and palpable right upper quadrant mass. Left posterior
oblique upper gastrointestinal series shows air-contrast image of duodenum
with obstructing intraluminal mass (arrows).
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Fig. 6A. —Cavernous hemangioma of ileum in 27-year-old woman with long
history of anemia and bloody stools. Contrast-enhanced CT scan of pelvis shows
intussusception involving distal small bowel (arrow).
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Fig. 6C. —Cavernous hemangioma of ileum in 27-year-old woman with long
history of anemia and bloody stools. Photograph of resected cut specimen shows
intraluminal polypoid component of hemangioma.
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Abdominal radiographs may show phleboliths. Barium examination of the small
intestine typically reveals a compressible polypoid intraluminal mass (Fig.
7A,7B)
or a nodular filling defect. Occasionally, when hemangiomas originate from the
mesentery, large segments of the small intestine may be involved. In patients
with this involvement, evidence of small-intestine displacement is seen on
radiographs and barium examinations. Mucosal or fold-pattern irregularities
may be present in barium studies. CT can identify a large mass contiguous with
the small intestine in these patients, but small lesions may be
radiographically occult and difficult to detect before surgery.
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Fig. 7B. —62-year-old man with iron deficiency anemia due to polypoid
intraluminal small-bowel capillary hemangioma. Photograph of resected specimen
shows 3-cm polypoid mass in lumen of jejunum.
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The differential diagnosis for hemangiomas that manifest as a focal mass
includes benign and malignant small-intestine tumors as well as metastatic
disease. Lymphoma, metastatic disease, primary peritoneal malignancies,
fibromatosis, and inflammatory pseudotumor should be considered in the
differential diagnosis for lesions that infiltrate the mesentery and small
intestine.
Colon
The colon is the second most common site of gastrointestinal hemangiomas,
and the rectosigmoid is the most commonly involved colonic segment. Most
patients with hemangiomas of the colon are young men and all patients present
with rectal bleeding [1]. The
presence of phleboliths is common in colorectal hemangiomas and is a useful
sign in young patients. Phleboliths that occur in clusters and those that have
an atypical distribution within the pelvis should raise concern for a
hemangioma (Fig.
8A,8B).
Soft, serpentine masses (Figs.
9A,9B
and 10), polypoid lesions, and
circumferential lesions (Fig.
11A,11B,11C)
may be seen on barium studies; some patients may have features of rigid
luminal narrowing [1].
Hemangiomas of the rectosigmoid may widen the retrorectal space. CT may show
transmural thickening of the involved segment of colon
(Fig. 11B), phleboliths,
vascular engorgement within the adjacent mesentery, and enhancement with IV
contrast material [4]. The MR
imaging features of hemangiomas of the rectosigmoid have been described as
focal thickening of the colonic wall with high signal intensity on T2-weighted
images in both the lesion and perirectal fat
[5].
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Fig. 8A. —Cavernous hemangioma of rectum in 20-year-old man with rectal
bleeding who was given diagnosis of hemorrhoids at age 5. Radiograph of pelvis
obtained with patient in supine position shows clusters of phleboliths.
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Fig. 8B. —Cavernous hemangioma of rectum in 20-year-old man with rectal
bleeding who was given diagnosis of hemorrhoids at age 5. Single-contrast
barium enema shows attenuation of rectal caliber and lobulated mass effect
containing phleboliths (arrows).
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Fig. 9A. —Cavernous hemangioma of descending colon in 62-year-old man
with 6-month history of rectal bleeding. Air-contrast barium enema shows
serpentine mass effect along lateral aspect of distal descending colon
(arrows).
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Fig. 9B. —Cavernous hemangioma of descending colon in 62-year-old man
with 6-month history of rectal bleeding. Photograph of descending colon
outside of the abdomen during surgery shows blood-filled masses along serosal
surface of colon (arrows).
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Fig. 10. —29-year-old man with history since birth of multiple
hemangiomas involving left lower extremity, scrotum, and rectum. Air-contrast
barium enema shows hemangioma that has lobulated mass effect along entire
sigmoid colon.
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Fig. 11A. —Cavernous hemangioma of colon in 9-month-old girl who
presented with blood-filled diaper. Single-contrast barium enema shows 6-cm
annular mass of hepatic flexure with lacelike barium-filled crevices. Air
bubbles are also present in lumen of proximal transverse colon.
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Fig. 11B. —Cavernous hemangioma of colon in 9-month-old girl who
presented with blood-filled diaper. Contrast-enhanced CT scan shows
circumferential infiltration of colon (arrows) by soft-tissue
attenuation mass.
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The differential diagnosis of colorectal hemangiomas includes benign and
malignant masses, inflammatory and infectious disorders. Atypical lesions that
manifest as rigid luminal narrowing may have radiologic appearances similar to
that of carcinoma and strictures from diverticulitis, ischemia, or radiation.
Serpentine borders and the presence of phleboliths may be helpful signs in the
diagnosis of hemangiomas.
Stomach
Gastric hemangiomas are rare and are reported to represent 1.6% of benign
tumors of the stomach [6]. To
our knowledge, there are only a few case reports of gastric hemangiomas in the
radiology literature. Gastrointestinal bleeding or signs and symptoms of
anemia are often the presenting complaint. The presence of phleboliths within
a gastric mass is virtually pathognomonic (Fig.
12A,12B,12C).
Evidence that the phleboliths can change position relative to each other and
the absence of gastric rigidity on fluoroscopic examination have also been
described as pathognomonic features
[2]. The differential diagnosis
includes gastric masses that may contain calcifications, such as carcinomas,
stromal tumors, and metastatic disease.
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Fig. 12A. —Cavernous hemangioma of stomach in 84-year-old woman with
abdominal pain and black, tarry stools. Radiograph of upper abdomen obtained
with patient in supine position shows cluster of large phleboliths in left
upper quadrant. Residual contrast material is present in colon. Reprinted with
permission from [2].
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Fig. 12B. —Cavernous hemangioma of stomach in 84-year-old woman with
abdominal pain and black, tarry stools. Left posterior oblique air-contrast
upper gastrointestinal series shows circumferential mass effect in body of
stomach containing phleboliths (arrows).
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Fig. 12C. —Cavernous hemangioma of stomach in 84-year-old woman with
abdominal pain and black, tarry stools. Photograph of portion of resected
specimen shows blood-filled cavities (arrows) beneath mucosa and
phlebolith in vascular space (curved arrow).
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Esophagus
Esophageal hemangiomas are rare, with a reported frequency of 3.3% of
benign esophageal tumors [7].
They may occur at any level within the esophagus and often present with
symptoms of dysphagia. Hematemesis and melena may also occur. A barium
esophagram may show a well-defined lobulated intramural mass, a pedunculated
intraluminal mass, or an infiltrating annular mass (Fig.
13A,13B,13C,13D,13E,13F).
CT typically reveals a well-defined soft-tissue density mass that enhances
with IV contrast material [8].
Phleboliths may be more apparent on CT
(Fig. 13B), particularly if
they are small. The differential diagnosis includes other benign lesions such
as leiomyomas, duplication cysts, polyps, lipomas, neurofibromas, and varices.
Malignant mucosal lesions such as carcinoma should also be considered in the
differential diagnosis.
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Fig. 13A. —Cavernous hemangioma of esophagus in 60-year-old man with
1-year history of worsening dysphagia. Barium esophagram shows masslike
impression and subtle varicoid fold thickening in distal esophagus. Several
phleboliths are located to left of esophagus (arrow).
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Fig. 13B. —Cavernous hemangioma of esophagus in 60-year-old man with
1-year history of worsening dysphagia. Contrast-enhanced CT scan shows diffuse
esophageal-wall thickening with phleboliths (arrows).
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Fig. 13C. —Cavernous hemangioma of esophagus in 60-year-old man with
1-year history of worsening dysphagia. Sagittal T1-weighted MR image shows
low-signal-intensity irregular thickening of esophageal wall with compressed
esophageal lumen (arrow).
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Fig. 13D. —Cavernous hemangioma of esophagus in 60-year-old man with
1-year history of worsening dysphagia. Axial T2-weighted MR image shows
high-signal-intensity wall thickening. Signal void is present at site of
phlebolith (arrow).
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Fig. 13E. —Cavernous hemangioma of esophagus in 60-year-old man with
1-year history of worsening dysphagia. Posterior chest view from
99mTC-labeled RBC scan shows radiotracer uptake along right and
left lateral aspects of spine (arrows).
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Fig. 13F. —Cavernous hemangioma of esophagus in 60-year-old man with
1-year history of worsening dysphagia. Endoscopic image of esophagus shows
bulging intraluminal mass (asterisk) containing prominent
vessels.
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Gastrointestinal Hemangiomatosis
Gastrointestinal hemangiomatosis is a complex vascular malformation that
occurs primarily in infancy and childhood. The clinical presentation is
variable, although most patients present with gastrointestinal bleeding.
Patients may also present with intussusception, small-bowel obstruction,
perforation, or malabsorption. Gastrointestinal hemangiomatosis may be
associated with blue rubber bleb nevus syndrome,
Klippel-Trénaunay-Weber syndrome, Maffucci's
syndrome, diffuse neonatal hemangiomatosis
[9], and Proteus syndrome (Fig.
14A,14B,14C).
Hemangiomatosis is manifested by diffuse infiltration of the intestinal wall
(Fig. 14C), the mesentery,
and, occasionally, the retroperitoneum. Solid organs in the abdomen may also
be involved (Fig.
15A,15B,15C,15D).
The radiographic findings of hemangiomatosis include the presence of
phleboliths on abdominal radiographs (Fig.
15A), scattered submucosal small-intestine nodules on barium
examination (Fig. 15C), and
mural thickening with phleboliths on CT
[9].
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Fig. 14A. —Hemangiomatosis of the rectosigmoid in 20-year-old man with
Proteus syndrome. Radiograph of abdomen obtained with patient in supine
position shows innumerable phleboliths scattered throughout abdomen.
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Fig. 14B. —Hemangiomatosis of the rectosigmoid in 20-year-old man with
Proteus syndrome. Single-contrast barium enema shows infiltrating submucosal
mass involving rectosigmoid and descending colon. Portions of mass contain
phleboliths (arrow).
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Fig. 14C. —Hemangiomatosis of the rectosigmoid in 20-year-old man with
Proteus syndrome. CT scan through upper pelvis shows phleboliths in mass that
diffusely thickens sigmoid and descending colon.
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Fig. 15A. —Infantile hemangiomatosis in 3-month-old boy who subsequently
died. At autopsy, hemangiomas were found that involved liver, spleen, upper
gastrointestinal tract, pancreas, retroperitoneum, kidneys, mediastinum, lung,
thyroid, and skin. Radiograph obtained with patient in supine position shows
phleboliths in abdomen and lung base (arrows).
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Fig. 15B. —Infantile hemangiomatosis in 3-month-old boy who subsequently
died. At autopsy, hemangiomas were found that involved liver, spleen, upper
gastrointestinal tract, pancreas, retroperitoneum, kidneys, mediastinum, lung,
thyroid, and skin. Upper gastrointestinal series shows displacement of
gastroesophageal junction and numerous nodular filling defects in small
bowel.
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Fig. 15C. —Infantile hemangiomatosis in 3-month-old boy who subsequently
died. At autopsy, hemangiomas were found that involved liver, spleen, upper
gastrointestinal tract, pancreas, retroperitoneum, kidneys, mediastinum, lung,
thyroid, and skin. Unenhanced CT scan of upper abdomen shows large soft-tissue
mass (asterisk), containing phleboliths, that displaces stomach,
liver masses with phleboliths, and splenic mass.
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Fig. 15D. —Infantile hemangiomatosis in 3-month-old boy who subsequently
died. At autopsy, hemangiomas were found that involved liver, spleen, upper
gastrointestinal tract, pancreas, retroperitoneum, kidneys, mediastinum, lung,
thyroid, and skin. Autopsy photograph of cut section of small bowel shows
numerous hemorrhagic nodules.
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Conclusion
Hemangiomas of the gastrointestinal tract are unusual benign tumors that
most commonly present with bleeding. They occur most often in the small
intestine. Throughout the gastrointestinal tract, they may manifest as
intramural or intraluminal masses and may be associated with a syndrome. In
the colon, they are more common in the rectosigmoid and may have the atypical
appearance of a rigid annular lesion that mimics carcinoma. The radiologic
feature of phleboliths is virtually pathognomonic for gastrointestinal
hemangiomas.
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Introduction
Pathologic Features
