Lee 2010
Lee 2010
Lee 2010
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Hypervascular Subepi-
thelial Gastrointestinal
Masses: CT-Pathologic
Correlation1
Nam Kyung Lee, MD • Suk Kim, MD • Gwang Ha Kim, MD • Tae Yong
ONLINE-ONLY
CME Jeon, MD • Dae Hwan Kim, MD • Ho Jin Jang, MD • Do Youn Park, MD
See www.rsna
.org/education Although the vast majority of gastrointestinal (GI) masses are epi-
/rg_cme.html thelial neoplasms, a variety of subepithelial masses are infrequently
encountered during endoscopic or radiologic examination. A subepi-
LEARNING thelial mass, which was previously called a submucosal mass, is de-
OBJECTIVES fined as a mass covered with normal-appearing mucosa, whether the
After reading this underlying process is intramural or extramural in origin. At contrast
article and taking
the test, the reader material–enhanced computed tomography (CT), hypervascular sub-
will be able to: epithelial masses are usually detected more easily than isoattenuating
■■Describe the vari-
ous types of hyper-
or hypovascular masses. Entities that appear as intramural hypervas-
vascular subepitheli- cular subepithelial lesions include neuroendocrine tumors, GI stromal
al masses that occur
in the GI tract.
tumor, glomus tumor, hemangioma, angiosarcoma, Kaposi sarcoma,
■■List the CT fea- nerve sheath tumors, hypervascular metastases, heterotopic tissues,
tures of intramural and vascular structures. Entities that appear as extramural hypervas-
hypervascular sub-
epithelial masses in cular subepithelial lesions include Castleman disease, solitary fibrous
the GI tract. tumor, inflammatory myofibroblastic tumor, and actinomycosis. Some
■■Discuss potential rare gastric cancers resemble subepithelial tumors. In comparison
pitfalls in CT diag-
nosis of intramural with endoscopic ultrasonography, CT is of limited value in differen-
hypervascular sub- tiating the layers of the GI wall and determining the origin of mass
epithelial masses in
the GI tract. lesions. However, recent advances in multidetector CT with multi-
planar reformation allow one to determine whether a GI mass is of
epithelial, intramural subepithelial, or extramural subepithelial origin.
TEACHING
POINTS Furthermore, the full extent of tumors can be delineated, and local
See last page invasion and distant metastases can be identified. Familiarity with the
characteristic CT appearances of hypervascular subepithelial masses
of the GI tract will help radiologists make a more confident diagnosis.
©
RSNA, 2010 • radiographics.rsna.org
Abbreviations: AIDS = acquired immunodeficiency syndrome, GI = gastrointestinal, GIST = GI stromal tumor, SFT = solitary fibrous tumor
the normal GI wall and the detection of subtle gastric and rectal disease has increased due to
disease. Water (500 mL) is given approximately increased endoscopic surveillance (9,10).
15 minutes before CT, and an additional 500 mL Some neuroendocrine tumors release vasoac-
is given immediately before the study. Water as an tive amines, and the liver effectively metabolizes
oral neutral agent provides excellent distention in and inactivates these substances. Carcinoid syn-
the upper GI tract. drome occurs in less than 10% of patients with
However, water is rapidly resorbed within the small bowel neuroendocrine tumors and occurs
GI mucosa, leading to insufficient distention in almost exclusively in the presence of a liver me-
the distal parts of the small bowel. Dilute 0.1% tastasis. Classic carcinoid syndrome (eg, cutane-
barium sulfate (Volumen; E-Z-Em, Lake Success, ous flushing and diarrhea) does not generally oc-
NY) or polyethylene glycol electrolyte solution cur in the absence of hepatic metastases, because
is an available neutral agent that can distend vasoactive substances released by the hepatic
the small bowel better than water (8). Nonionic metastasis enter the systemic circulation directly,
intravenous contrast material is administered thus avoiding hepatic inactivation (9–12).
simultaneously and enhances the GI walls. Dy- According to the most recent classification
namic contrast-enhanced imaging, including the of the World Health Organization, neuroendo-
late arterial and portal venous phases, is usually crine neoplasms are classified into three gen-
performed. eral categories based on histologic features and
In many patients, conventional CT is limited malignant potential: well-differentiated neuroen-
by poor distention of the small and large bowels. docrine tumors (typical carcinoid tumors), which
Therefore, CT enteroclysis has been introduced are low-grade malignancies; well-differentiated
to overcome the disadvantages of both CT and neuroendocrine carcinomas (atypical carcinoid
conventional enteroclysis in evaluation of the tumors), which are more aggressive because of
small bowel. CT enteroclysis is performed after the presence of metastases; and poorly differenti-
placement of a nasojejunal tube in conjunction ated neuroendocrine carcinomas, which are high-
with large volumes of ingested neutral enteric grade malignancies with a poor prognosis (9,11).
contrast material. This technique permits vi- At endoscopy, neuroendocrine tumors usu-
sualization of the small bowel wall and lumen, ally appear as subepithelial lesions that resemble
improving the depiction and characterization of other more common subepithelial tumors, be-
small bowel diseases. Neutral agents (water) or cause neuroendocrine tumors usually arise from
negative agents (air) administered via a rectal enteroendocrine cells, which are usually located
tube can provide excellent contrast for colonic relatively deep in the mucosal layer of the GI
imaging; however, CT enteroclysis and the rectal wall (Fig 5). The diagnosis is based on results of
administration of water or air are not performed histologic analysis with immunohistochemical
routinely at our institution. staining. However, the histologic features may
not allow prediction of the tendency for aggres-
Neuroendocrine Tumors sive behavior. The presence of metastasis or local
Enteroendocrine cells (enterochromaffin cells) spread to adjacent organs establishes the malig-
are located throughout the GI tract, pancreatico- nant nature of the tumor. The risk of metastasis is
biliary tract, and lung. The most common loca- related to tumor size. The main sites of metastasis
tion for neuroendocrine tumors in the GI tract are the lymph nodes, liver, peritoneum, lung,
is the appendix, followed by the small bowel, and, less frequently, bone (9–12).
rectum, colon, and stomach. Recently, the preva-
lence of appendiceal neuroendocrine tumors
has decreased, while the apparent prevalence of
1920 November-December 2010 radiographics.rsna.org
Mesenchymal Tumors
Figure 10. GIST of the stomach in a 44-year-old man. (a) Coronal reformatted contrast-
enhanced CT image shows a 1.8-cm, well-defined hypervascular intraluminal mass (arrow) with
central ulceration in the lesser curvature of the gastric body. However, the intact mucosa overlying
the mass is not well depicted. (b) Endoscopic US image shows the 1.8-cm, well-defined, homoge-
neous hypoechoic subepithelial mass (*) with central ulceration arising from the second and third
layers of the stomach.
Figure 13. Glomus tumor of the stomach in a 56-year-old woman. (a) Contrast-enhanced CT image
obtained during the late arterial phase shows a 2.5-cm, well-defined, intra- and extraluminal subepithe-
lial mass (arrow) with homogeneous strong enhancement in the gastric antrum. Note the liver hemangi-
oma (*). (b) Contrast-enhanced CT image obtained during the hepatic venous phase shows prolonged
enhancement of the mass (arrow). * = liver hemangioma.
Figure 15. Angiosarcoma of the jejunum in a 55-year-old man. (a) Contrast-enhanced CT im-
age obtained during the late arterial phase shows an asymmetric circumferential mass (arrow)
with slightly heterogeneous enhancement in the jejunum. Note the prominent vessels around the
mass and the bizarre enhancing foci within the inhomogeneous mass. (b) Intraoperative photo-
graph shows blood-filled jejunal tumors (arrows) directly invading the adjacent jejunum.
or early childhood and involute during childhood. Angiosarcoma.—Angiosarcomas are rare, aggres-
Most patients present with hemorrhage, abdomi- sive, malignant vascular tumors that are ex-
nal pain, and intussusception (20,21). tremely rare in the GI tract. Although the precise
Histologically, hemangiomas may be classi- cause of these tumors remains unclear, factors
fied as cavernous, capillary, or mixed. Cavern- that have been implicated in their pathogen-
ous hemangiomas are large blood-filled vascular esis include trauma; exposure to vinyl chloride,
channels with an endothelial lining. Capillary thorium dioxide (Thorotrast), or arsenic com-
hemangiomas are a proliferation of small capillar- pounds; long-standing lymphedema; and radiation
ies usually with a hyperplastic endothelium. Most therapy. The presenting symptoms of small bowel
GI hemangiomas arise from the submucosal angiosarcomas are nonspecific and include GI
vascular plexus and often form a polypoid, intra- hemorrhage and anemia, perforation, and pain
luminal subepithelial mass, although they may (22).
occasionally appear as a diffuse infiltrating mass In our experience, the CT findings of an-
(hemangiomatosis) extending to the surrounding giosarcomas do not differ from those of other
connective tissue (20,21). malignant epithelial and malignant intramural
CT reveals different radiologic features ac- subepithelial masses of the small bowel. Contrast-
cording to the gross appearance: an intramural enhanced CT demonstrates irregular, asymmetric
subepithelial mass with an intraluminal growth wall thickening with heterogeneous enhancement
pattern or transmural circumferential bowel wall (Fig 15). The prominent vessels around the mass
thickening extending into surrounding tissue and the bizarre enhancing portions within the
(Fig 14). Dynamic contrast-enhanced CT images inhomogeneous mass, which reflect increased
demonstrate slow diffuse enhancement, which is vascularity, help identify the vascular origin of the
similar to that seen in hemangiomas elsewhere, mass (22).
and vascular engorgement within the adjacent
mesentery around mass lesions, which reflects Kaposi Sarcoma.—Kaposi sarcomas of the GI
increased lesion vascularity. In addition, CT may tract are common in patients with acquired
reveal phleboliths caused by calcified thrombi immunodeficiency syndrome (AIDS) but also
within the tumor vascular channels, a finding that occur in patients without AIDS. GI involvement
is virtually pathognomonic for a GI hemangioma
(Fig 14) (20,21).
1926 November-December 2010 radiographics.rsna.org
Figure 16. Disseminated AIDS-related Kaposi sarcoma in a 55-year-old man with gastric
compromise. (a) Contrast-enhanced CT image shows a flat polypoid subepithelial mass
(arrow) with increased enhancement in the gastric body. (b) Endoscopic image shows mul-
tiple polypoid lesions with a cherry-red appearance.
occurs in 40%–50% of AIDS-related Kaposi copy and biopsy. The typical endoscopic findings
sarcomas and is almost always associated with include an ulcerated subepithelial mass or masses
cutaneous disease. Kaposi sarcomas are thought with a reddish appearance.
to originate from the endothelial cells of lym- At CT, GI Kaposi sarcomas occasionally ap-
phatic or blood vessels in response to infection pear as one or more small (0.5–3 cm in diameter)
with human herpesvirus 8; this origin explains polypoid subepithelial masses or as irregular fold
the multiplicity of the tumor. The diagnosis of GI thickening (Fig 16). Central ulceration is also
Kaposi sarcomas is usually made by using endos- common, resulting in the appearance of “bull’s-
eye” or “target” lesions in barium studies. At
contrast-enhanced CT, the enhancement of the
masses is greater than that of the adjacent mu-
RG • Volume 30 Number 7 Lee et al 1927
Figure 18. Paraganglioma of the perirectal space in a 67-year-old woman. (a) Contrast-enhanced
CT image shows a well-defined, lobulated, heterogeneous mass with avid enhancement (arrows)
that displaces the rectum. The mass mimics an intramural subepithelial mass of the rectum. Note
the prominent feeding and draining vessels (arrowhead) adjacent to the mass. (b) Photograph of the
resected gross specimen shows the 5.5-cm tumor replacing the left perirectal space and infiltrating
the adjacent rectal wall. Scale is in centimeters. (Case courtesy of J. Y. Oh, MD, Dong-A University
Hospital, Busan, Korea.)
cosa secondary to the high tumor vascularity (Fig Paragangliomas are extremely rare neoplasms
16). CT can also demonstrate other evidence of that may be identified anywhere within or around
Kaposi sarcomas, such as lung nodules, highly the GI tract (Fig 18) (26).
enhanced lymph nodes, or splenomegaly (23).
Hypervascular Metastases
Nerve Sheath Tumors The mechanism of GI metastases includes direct
GI schwannomas are rare mesenchymal tumors extension from a contiguous or noncontiguous
arising from the Schwann cells of the neural primary malignant tumor, peritoneal carcinoma-
plexus within the GI wall. The most common tosis with serosal implants, and embolic metas-
site is the stomach (60%–70% of cases), followed tases with intramural subepithelial masses. GI
by the colon and rectum. Gastric schwannomas metastases closely resemble the primary tumors;
account for only 0.2% of all gastric tumors and therefore, the radiologic appearance of GI metas-
occur most frequently in the 3rd to 5th decades tases depends mainly on the histologic character-
of life. They are usually solitary tumors and istics of the primary or secondary lesions, such
principally involve the submucosa and muscularis as the degree of vascularity relative to the growth
propria. These tumors are usually discovered in- rate.
cidentally during endoscopic evaluation, although GI metastases are further classified as hypo-
larger tumors may occasionally manifest as upper vascular or hypervascular (27). Hypervascular
GI bleeding, abdominal pain, or discomfort. embolic GI metastases are often multifocal and are
Schwannomas are benign, and malignant trans- most frequently seen in patients with malignant
formation is extremely rare. melanoma or breast cancer. Uncommon hyper-
The most consistent CT feature of GI schwan- vascular GI metastases include those from renal
nomas is homogeneous attenuation for their cell carcinoma, choriocarcinoma, neuroendocrine
relatively large size. Although large tumors may carcinoma, and mesenchymal sarcoma (28).
undergo cystic degeneration, they rarely appear CT can be useful in detecting metastatic
cystic. Calcification is uncommon. Nonenhanced lesions, in determining the growth patterns of
CT shows low attenuation due to the dense metastasis (eg, intramural subepithelial lesions
spindle cell composition. Contrast-enhanced CT with intraluminal or extraluminal growth, which
may show no or minimal enhancement during are frequent in embolic metastasis, or extramural
the arterial phase but delayed enhancement dur- lesions, which are common in peritoneal carcino-
ing the equilibrium phase (Fig 17). Therefore, matosis with serosal implants), and in evaluating
a hypervascular mass may not be a common
feature of GI schwannomas at CT (24,25).
1928 November-December 2010 radiographics.rsna.org
Figure 19. Small bowel metastases from malignant melanoma in a 70-year-old woman. (a) Coronal refor-
matted contrast-enhanced CT image shows multiple hypervascular intraluminal polypoid masses (arrows) in
the ileum. (b) Image from a small bowel follow-through study shows multiple nodular filling defects (arrows)
in the ileum. (Case courtesy of J. Y. Oh, MD, Dong-A University Hospital, Busan, Korea.)
Figures 20, 21. (20) Heterotopic pancreas of the stomach in a 47-year-old woman. Contrast-
enhanced CT image shows a 3-cm, ill-defined, ovoid intraluminal subepithelial mass (arrow) in
the gastric antrum. The lesion has homogeneous enhancement similar to that of the pancreas.
(21) Heterotopic pancreas of the jejunum in a 46-year-old woman. Contrast-enhanced CT image
shows a 2.8-cm, ill-defined, lobulated mass (arrow) in the jejunum. The mass demonstrates en-
hancement similar to that of the orthotopic pancreas (*). The ascites in the peritoneal cavity is due
to recent surgery.
Heterotopic Tissues
Heterotopic pancreas is pancreatic tissue in aber-
rant sites that lacks a vascular and ductal connec-
tion to the main pancreatic body. The reported
prevalence at autopsy ranges from 1% to 15%.
Since heterotopic pancreas arises as a detach-
ment of one or more branching buds during
embryonic rotation and results in fusion of the
dorsal and ventral pancreatic buds, heterotopic
pancreas is most often found in the upper part
of the GI tract (90% of cases) near the pancreas,
particularly in the prepyloric region of the stom-
ach along the greater curvature (7,31–33).
Heterotopic pancreas is usually located in the
submucosa or muscularis propria and appears as
an intramural subepithelial nodule less than 3 cm
Figure 22. Heterotopic gastric tissue in a in diameter, usually with central umbilication that
Meckel diverticulum in a 20-year-old man. Con- represents a rudimentary pancreatic duct and its
trast-enhanced CT image shows a 2-cm, well-de- orifice. Heterotopic pancreas is usually asympto-
fined, hypervascular intraluminal masslike lesion matic, but patients may occasionally present with
(arrows) in a blind-ending tubular structure (*). abdominal pain, GI bleeding, and obstruction.
Pathologic analysis demonstrated heterotopic
Complications that can occur in the pancreas can
gastric tissue in a Meckel diverticulum.
also develop in the heterotopic pancreas, including
pancreatitis, pseudocysts, cystic dystrophy, insu-
tional barium fluoroscopy and permits detection linomas, and malignant transformation, although
of extraluminal abnormalities in patients with these are rare (7,31–33).
intestinal melanoma, the sensitivity of CT for de- Contrast-enhanced CT usually demonstrates
tection of intestinal melanoma is only 60%–70%. a homogeneously enhancing mass with attenua-
The use of CT enteroclysis or multidetector CT tion similar to that of the pancreas (Figs 20, 21).
with multiplanar visualization is likely to improve However, some instances of heterotopic pancreas
the detection rate of small bowel melanomas. enhance poorly; this finding is mainly attributed
Typically, metastatic melanomas manifest to ductal tissue with surrounding hypertrophied
as one or more small intramural subepithelial muscle but with the pancreatic acini as only a
nodules and polypoid masses in the GI tract with minor component (31,32). Differentiation of
increased enhancement at contrast-enhanced heterotopic pancreas from other subepithelial
CT (Fig 19). Embolic metastases are localized to masses is crucial to avoid unnecessary resection.
one wall of the bowel, usually the antimesenteric The typical location (prepyloric antrum), flat-
border; this pattern is related to the intramural ovoid shape, intraluminal growth, and irregular
ramifications of the vasa recta toward the submu- border of heterotopic pancreas are important for
cosal plexus in the antimesenteric border. Central differentiation from other subepithelial masses
ulceration is especially common because metas- (Fig 20) (7,33).
tases tend to outgrow their blood supply, leading Heterotopic gastric mucosa may be found
to the appearance of bull’s-eye or target lesions in in many parts of the GI tract, including the
barium studies (Fig 19). A larger lesion may ap- esophagus, duodenum, small bowel, and Meckel
pear more complex due to necrosis, hemorrhage, diverticulum and in duplication cysts. Although
and degenerative change (28–30). heterotopic gastric mucosa is not a subepithe-
Hematogenous metastases from breast cancer lial lesion but an epithelial lesion, heterotopic
occur in the stomach, small bowel, and colon and gastric mucosa in the Meckel diverticulum
manifest as localized eccentric bowel wall thick- may appear as a nodular or polypoid intralu-
ening or as a diffuse irregular circumferential minal mass at contrast-enhanced CT (Fig 22);
stricture. At contrast-enhanced CT, GI metas- this mass can be mistaken for other common
tases from breast cancer may appear as diffuse subepithelial masses such as carcinoid tumors or
irregular wall thickening with increased attenua- GISTs (34,35).
tion, producing a linitis plastica–like appearance
(28).
1930 November-December 2010 radiographics.rsna.org
Miscellaneous Masses
Vascular Structures
Vascular structures may produce focal submuco-
sal abnormalities. Varices are occasionally seen as
masslike polypoid lesions or as tortuous elevation
of the mucosa at endoscopy. The diagnosis of
varices is usually made with endoscopy. However,
it may be difficult to distinguish between varices
and subepithelial mass lesions at endoscopy. The
vascular nature of varices may not be evident
during endoscopy because of thick overlying
rugal folds.
CT can be helpful in detecting varices and
identifying the underlying cause, such as portal
hypertension. Varices appear as enhanced tubular
vessels at CT. Because they are veins, they will Figure 23. Duodenal varices in a 62-year-old
enhance during the portal venous phase (Fig 23). man with occult GI bleeding. Contrast-enhanced
CT can demonstrate communications of the vari- CT image shows varices (arrows) in the poste-
ces, such as afferent and efferent veins, and other rior wall of the third part of the duodenum.
collateral vessels in patients with portal hyperten-
sion (36).
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This article meets the criteria for 1.0 AMA PRA Category 1 Credit TM. See www.rsna.org/education/rg_cme.html.
Teaching Points November-December Issue 2010
Page 1916
A subepithelial mass, which was previously called a submucosal mass, is defined as a mass covered with
normal-appearing mucosa. The term subepithelial has come to be favored over submucosal because lesions
in this category do not necessarily arise from the submucosa, but can also arise from any layer of the gas-
trointestinal (GI) wall other than the submucosa (intramural) or from extrinsic compression of the wall
by a number of normal or abnormal intraabdominal structures (extramural) (1,2).
Page 1922
When a hypervascular intramural subepithelial mass larger than 3 cm is encountered at contrast-en-
hanced CT, GIST should be considered first in the differential diagnosis. GIST is the most common
subepithelial tumor involving the GI tract, and other intramural subepithelial masses such as carcinoid
tumor, glomus tumor, or ectopic pancreas usually manifest as intraluminal masses, smaller than 3 cm,
with stronger enhancement than GIST. However, the CT features of GISTs smaller than 3 cm overlap
with those of other intramural subepithelial masses.
Page 1927
GI metastases closely resemble the primary tumors; therefore, the radiologic appearance of GI metas-
tases depends mainly on the histologic characteristics of the primary or secondary lesions, such as the
degree of vascularity relative to the growth rate.