AJR 2004; 182:1485-1491
© American Roentgen Ray Society
Abdominal Lymphangiomas: Imaging Features with Pathologic Correlation
Angela D. Levy1,2,
Vito Cantisani3 and
Markku Miettinen4
1 Department of Radiologic Pathology, Armed Forces Institute of Pathology, 6825
16th St., NW, Washington, DC 20306-6000.
2 Department of Radiology and Nuclear Medicine, Uniformed Services University of
the Health Sciences, Bethesda, MD 20814.
3 Institute of Radiologic Sciences, University "La Sapienza," Viale
Regina Elena, 324, Rome 00161, Italy.
4 Department of Soft Tissue Pathology, Armed Forces Institute of Pathology,
Washington, DC 20306.
Received September 4, 2003;
accepted after revision October 7, 2003.
The opinions and assertions contained herein are the private views of the
authors and are not to be construed as official or as reflecting the views of
the Department of the Army or the Department of Defense.
Address correspondence to A. D. Levy
(levya{at}afip.osd.mil).
Introduction
Lymphangiomas are benign lesions of vascular origin that show lymphatic
differentiation. They occur in many anatomic locations and may have a
pediatric or adult clinical presentation. Most (95%) occur in the neck and
axillary regions; the remaining 5% are located in the mesentery,
retroperitoneum, abdominal viscera, lung, and mediastinum
[1]. Lymphangiomatosis is a
rare disease with multifocal lymphatic proliferation that typically presents
during childhood and involves multiple parenchymal organs including the lung,
liver, spleen, bone, and skin. Because lymphangiomas present across a wide age
range of patient ages and occur in many sites, they are associated with a
broad spectrum of clinical and radiologic manifestations.
This article summarizes our experience with 107 cases of abdominal
lymphangiomas (58 mesenteric, 11 retroperitoneal, five pancreatic, nine
splenic, eight colonic, eight small intestinal, two renal, one hepatic, one
hepatosplenic, one biliary, one adrenal, one bladder, and one ovarian)
accessioned in the radiologic pathology archive of the Armed Forces Institute
of Pathology over a 22-year period. The purpose of this essay is to describe
and illustrate the imaging features of abdominal lymphangiomas with pathologic
correlation.
Histopathologic Features
Lymphangiomas are thin-walled cystic masses with a smooth gray, pink, tan,
or yellow external surface. On cut section, they vary in appearance and may
contain large macroscopic interconnecting cysts (often referred to as cystic
hygroma or cystic lymphangioma) or microscopic cysts (cavernous lymphangioma)
[2]. The cysts may contain
chylous, serous, hemorrhagic, or mixed fluid
[3]. Histologically, dilated
lymphatic spaces are lined with attenuated endothelial cells resembling the
cells that line normal lymphatics (Fig.
1). The lymphatic spaces are usually filled with proteinaceous
eosinophilic fluid. The supporting stroma is composed of collagen and may
contain lymphocytes and lymphoid aggregates
[2].
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Fig. 1. —Photomicrograph of histopathologic specimen of mesenteric
lymphangioma from 1-year-old girl shows thin-walled cystic spaces
(asterisk) filled with proteinaceous fluid. Cysts are lined with
attenuated endothelia (arrow). Cyst walls contain irregular
smooth-muscle elements, and foci of lymphocyte clusters appear inside and
between some cystic spaces. (H and E, x40)
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Mesentery and Retroperitoneum
Abdominal lymphangiomas are reported to occur most commonly in the
mesentery, followed by the omentum, mesocolon, and retroperitoneum. Mesenteric
lymphangiomas may produce complications such as intestinal obstruction or
volvulus, and infarction may occur (Fig.
2A,
2B,
2C).
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Fig. 2A. —10-year-old girl with 3-day history of epigastric pain and
bilious vomiting caused by small-bowel volvulus secondary to mesenteric
lymphangioma. IV contrast–enhanced CT scan shows circular arrangement of
mesenteric vessels with surrounding small bowel (arrows). Note
fluid-containing mass (asterisk) to right of small-bowel
volvulus.
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Fig. 2B. —10-year-old girl with 3-day history of epigastric pain and
bilious vomiting caused by small-bowel volvulus secondary to mesenteric
lymphangioma. CT scan obtained through lower abdomen shows multilocular cystic
mass (asterisk). Ascites is present in right paracolic gutter.
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Fig. 2C. —10-year-old girl with 3-day history of epigastric pain and
bilious vomiting caused by small-bowel volvulus secondary to mesenteric
lymphangioma. Photograph of cut surface of resected surgical specimen shows
opened small bowel (sb) and multiloculated cystic lymphangioma
(arrows) arising from adjacent small-bowel mesentery.
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Sonographically, lymphangiomas are most often multilocular cystic masses
that are anechoic or contain echogenic debris (Figs.
3 and
4). IV contrast–enhanced
CT may show enhancement of the cyst wall and septa (Fig.
5A,
5B). The fluid component is
typically homogeneous with low attenuation values. Occasionally, negative
attenuation values occur in the presence of chyle. Calcification may occur but
is uncommon [4]. The signal
pattern of lymphangiomas on MRI resembles that of fluid: low signal intensity
on T1-weighted images and high signal intensity on T2-weighted images (Fig.
6A,
6B). The presence of
hemorrhage or infection in the lesion may alter the CT attenuation and MRI
signal pattern to give a more solid appearance
[4] (Fig.
7A,
7B).
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Fig. 5A. —Retroperitoneal lymphangioma in 32-year-old woman with
increasing abdominal girth. IV and oral contrast–enhanced CT scan shows
large multiseptate cystic mass with enhancing septa arising from anterior
pararenal space.
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Fig. 5B. —Retroperitoneal lymphangioma in 32-year-old woman with
increasing abdominal girth. Photograph of cut surface of resected surgical
specimen shows that some locules contain milky chylous fluid.
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Fig. 6A. —Mesenteric lymphangioma in 42-year-old woman with chronic
abdominal pain. IV and oral contrast–enhanced CT scan shows well-defined
cystic mass in small-bowel mesentery that insinuates around mesenteric
vessels. Note punctate calcifications in cyst.
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Fig. 7A. —Mesenteric lymphangioma in 70-year-old woman with painless
abdominal distension. IV and oral contrast–enhanced CT scan shows
complex mixed-attenuation mass arising from small-bowel mesentery. Foci of
high attenuation in mass (arrow) correspond to areas of hemorrhage in
resected surgical specimen.
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Fig. 7B. —Mesenteric lymphangioma in 70-year-old woman with painless
abdominal distension. Photograph of cut surface of resected specimen shows
well-circumscribed mass composed of numerous small locules. Some locules
(arrows) contain hemorrhagic fluid.
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Accurate anatomic localization and definition of the lesions are important
in preoperative planning because lymphangiomas have an insinuating nature that
makes complete surgical excision difficult in some cases. The diagnostic
challenge on imaging is the differentiation of lymphangiomas from other
fluid-containing masses and ascites in the abdomen. Retroperitoneal
lymphangiomas tend to be large elongated lesions that traverse adjacent
anatomic compartments [5].
Large mesenteric lymphangiomas can be differentiated from ascites by the
presence of septa, compression on adjacent intestinal loops, and lack of fluid
in the dependent recesses of the peritoneum such as the paracolic gutters and
subhepatic spaces and between the leaves of the small-bowel mesentery
[1]
(Fig. 8). Differentiating
lymphangiomas from enteric duplications, mesothelial cysts, and pseudocysts
may be difficult because the imaging features of these lesions overlap.
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Fig. 8. —Lymphangioma arising from greater omentum in 16-month-old boy
with abdominal distention. Oral and IV contrast–enhanced CT scan shows
lobular fluid-filled mass following anterior surfaces of intestine.
Lymphangioma compresses all intraabdominal organs. Unlike ascites,
lymphangioma does not pool in recesses of small-bowel mesentery and subhepatic
spaces.
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Intestine
Lymphangiomas are infrequently located in the esophagus, stomach, small
intestine, and colorectum. Most intestinal lymphangiomas are mural masses
discovered incidentally at endoscopy or on radiologic studies performed for
other reasons [6]. Intramural
lymphatic obstruction, disturbed endothelial permeability, inflammation,
congenital absence of lymphatics, and aging of the bowel wall have been
suggested as causes for the development of intestinal lymphangiomas.
Barium studies show smoothly marginated mural masses (Fig.
9A,
9B,
9C) that deform and alter
shape when compression is applied. Endoscopic sonography and CT show evidence
of cystic mass in the intestinal wall (Fig.
10A,
10B). Endoscopic
"unroofing" or polypectomy has become the treatment of choice for
small to intermediate colorectal lymphangiomas
[6].
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Fig. 9A. —Two colonic lymphangiomas in 43-year-old woman with
pancreatic cancer and no colonic symptoms. Single-contrast enema-enhanced CT
scan shows two well-defined oval filling defects (arrows) in
ascending colon.
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Fig. 9B. —Two colonic lymphangiomas in 43-year-old woman with
pancreatic cancer and no colonic symptoms. Photograph of open resected
surgical specimen shows two masses (arrows) covered with normal
mucosa bulging into lumen of ascending colon. Appendix (a) and ileocecal valve
(ic) appear in lower portion of specimen.
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Fig. 9C. —Two colonic lymphangiomas in 43-year-old woman with
pancreatic cancer and no colonic symptoms. Photomicrograph of histopathologic
specimen shows multiple interconnecting cysts in submucosa. (H and E,
x4)
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Kidney
Renal lymphangiomas are rare and are thought to arise from dilated
lymphatic channels or developmental malformations of the lymphatic system.
Most renal lymphangiomas are focal lesions, but diffuse renal
lymphangiomatosis may occur. Most renal lymphangiomas appear as unilocular or
multilocular cysts on cross-sectional imaging (Fig.
11A,
11B). Case reports have
described lymphangiomas that appear solid on sonography
[7]. In these cases, the masses
are sonographically echogenic due to the reverberations that occur between
microscopic lymph spaces and surrounding connective tissue.
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Fig. 11A. —Renal lymphangioma in 8-month-old male infant who presented
with urinary tract infection. IV contrast–enhanced CT scan shows left
renal cyst (arrow) with lobulated contours and mural enhancement.
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Fig. 11B. —Renal lymphangioma in 8-month-old male infant who presented
with urinary tract infection. Photograph of bisected surface of nephrectomy
specimen shows thin-walled lower pole cyst (arrow) containing
septa.
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Imaging differential diagnosis in adult patients includes benign renal
cysts, cystic renal cell carcinoma, multilocular cystic nephroma, and abscess.
Isolated simple renal cysts are uncommon in pediatric patients, for whom
multilocular cystic nephroma is the principal differential diagnosis.
Liver, Spleen, and Extrahepatic Bile Ducts
Primary hepatic and splenic lymphangiomas are rare. They may occur in
isolation, as a manifestation of systemic lymphangiomatosis, or as a feature
of hepatosplenic lymphangiomatosis. The literature suggests that isolated
hepatic lymphangiomas tend to occur primarily in patients older than 25 years,
whereas hepatosplenic or systemic lymphangiomatosis presents during infancy,
childhood, or adolescence
[8].
Cross-sectional imaging studies may show single or multiple hepatic cysts
of variable size. Sonography may reveal debris, septa, or fluid–fluid
levels in the cysts, similar to mesenteric lymphangiomas. Homogeneously
hyperechoic lymphangiomas have been reported; it has been suggested that they
may be lesions composed of small- to medium-size lymphatic spaces separated by
fibrous septa.
Splenic lymphangiomas tend to occur in subcapsular locations, reflecting
the anatomic distribution of splenic lymphatics. Thin-walled subcapsular or
parenchymal cysts have been described, as well as global splenic enlargement
by a diffusely infiltrating lesion. Mural or septal calcifications may be
present (Fig. 12A,
12B).
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Fig. 12A. —Splenic lymphangioma in 70-year-old man with asymptomatic
splenomegaly. Unenhanced CT scan shows multiple circumscribed low-attenuation
lesions throughout spleen. Subtle calcification (arrow) is
present.
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Fig. 12B. —Splenic lymphangioma in 70-year-old man with asymptomatic
splenomegaly. Photograph of cut surface of resected spleen shows multiple
thin-walled cystic structures replacing splenic parenchyma.
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The differential diagnosis for hepatic lymphangiomas includes bile duct
cysts, polycystic liver disease, cystic metastasis, echinococcal cysts, and
mesenchymal hamartoma. True splenic cysts, pyogenic abscesses, parasitic
cysts, infarction, peliosis, hemangioma, lymphoma, and cystic metastasis
should be considered in the differential diagnosis for splenic
lymphangiomas.
Lymphangiomas of the extrahepatic biliary tract are rare. Those involving
the gallbladder are most often reported to manifest as multilocular cystic
masses that compress the gallbladder lumen (Fig.
13A,
13B,
13C,
13D,
13E). No communication appears
between the biliary tract and lymphangioma on cholangiographic studies
[9].
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Fig. 13A. —Gallbladder lymphangioma in 36-year-old woman with dull
intermittent right upper quadrant pain. Axial T1-weighted MR image shows
high-signal-intensity gallbladder (arrow) surrounded by
low-signal-intensity lymphangioma.
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Fig. 13B. —Gallbladder lymphangioma in 36-year-old woman with dull
intermittent right upper quadrant pain. Axial T2-weighted MR image shows
low-signal-intensity gallbladder (arrow) surrounded by
high-signal-intensity lymphangioma. Altered signal pattern of gallbladder is
caused by inspissated bile.
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Fig. 13C. —Gallbladder lymphangioma in 36-year-old woman with dull
intermittent right upper quadrant pain. Coronal T2-weighted MR
cholangiopancreatograph shows teardrop-shaped high-signal-intensity
lymphangioma (arrow) extending upward toward hepatic hilum and
compressing low-signal-intensity gallbladder.
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Fig. 13D. —Gallbladder lymphangioma in 36-year-old woman with dull
intermittent right upper quadrant pain. Photograph of unopened resected
specimen shows lymphangioma (arrow) encircling bile-colored
gallbladder.
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Fig. 13E. —Gallbladder lymphangioma in 36-year-old woman with dull
intermittent right upper quadrant pain. Photograph of partially opened
surgical specimen shows thin septa in lymphangioma (arrow)
surrounding gallbladder.
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Pancreas
Lymphangiomas may arise in the retroperitoneal or mesocolic fat adjacent to
the pancreas. However, they are considered to be of pancreatic origin if they
are located in the pancreatic parenchyma, are immediately adjacent to the
pancreas, or are connected by a pedicle
[10] (Figs.
14 and
15). The differential
diagnosis for cystic masses arising in the pancreas is broad and includes
pseudocysts, mucinous cystic neoplasms, microcystic adenomas, solid and
papillary epithelial neoplasms, intraductal papillary mucinous neoplasms, and
cystic neuroendocrine neoplasms. Because the imaging appearance of pancreatic
lymphangiomas overlaps with these cystic masses, the diagnosis can be made
only by histopathologic examination. Lymphangiomas should particularly be
considered in the differential diagnosis for a thin-walled cystic pancreatic
mass with fine septations.
Conclusion
Lymphangiomas are uncommon benign lymphatic lesions that may occur at
virtually any anatomic location in the abdomen. Knowledge of the imaging and
pathologic spectrum of abdominal lymphangiomas is necessary when evaluating
pediatric and adult patients with intraabdominal cystic masses.
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Introduction
Histopathologic Features
