DOI:10.2214/AJR.04.1488
AJR 2006; 186:895-897
© American Roentgen Ray Society
Papillary Adenocarcinoma in a Small-Bowel Duplication in a Pregnant Woman
George A. Radich1,
Deniz Altinok1,
N. Volkan Adsay2 and
Renate L. Soulen1
1 Department of Radiology, Wayne State University School of Medicine, DRH 3L8,
4201 St. Antoine St., Detroit, MI 48201.
2 Department of Pathology, Wayne State University School of Medicine, Detroit,
MI 48201.
Received September 21, 2004;
accepted after revision January 31, 2005.
Address correspondence to D. Altinok
(altinokd{at}yahoo.com).
Keywords: abdominal imaging • congenital • MRI • pelvic imaging
Introduction
Enteric duplication cysts are uncommon congenital anomalies containing a
normal gastrointestinal mucosal lining that can occur throughout the digestive
tract, most commonly in the small bowel. Malignancies arising from duplication
cysts are less common, but present most often in the colon
[1]. Malignant tumors arising
from small-bowel duplications are rare, but have been previously reported
without preoperative MRI studies
[2-7].
MRI is particularly helpful in evaluating large, complex masses in a pregnant
woman since X-ray exposure is undesirable, and the limited field of view of
sonography limits interpretation. We present, to our knowledge, the first MRI
study of a malignant lesion arising in a small-bowel duplication and show the
value of this technique even in the absence of specific imaging diagnosis.
Case Report
A 39-year-old, asymptomatic, 24-week-pregnant African-American woman too
large for dates presented for an obstetric sonogram. The sonogram showed an
appropriately sized fetus in a normal-appearing uterus and an unexpected
abdominal mass of uncertain origin. Dedicated abdominal sonography confirmed a
left-upper-quadrant mass, initially interpreted as an enlarged spleen. This
finding was discordant with the limited visualization on the obstetric
sonogram. To better define the mass, MRI was requested.
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Fig. 1A —39-year-old pregnant woman presenting as too large for dates.
Coronal (A) and sagittal (B) T1-weighted breath-hold,
gradient-echo (TR/TE, 160/2.3) images of the abdomen demonstrate a
well-defined, thin-walled cystic mass containing dependent and nondependent
polypoid filling defects extending inferiorly and laterally from pancreatic
head/uncinate process. Note that fluid is hyperintense and filling defects are
hypointense relative to myometrium and that there is no evidence of myometrial
invasion. Single fetus commensurate with gestational age is seen in breech
position.
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Fig. 1B —39-year-old pregnant woman presenting as too large for dates.
Coronal (A) and sagittal (B) T1-weighted breath-hold,
gradient-echo (TR/TE, 160/2.3) images of the abdomen demonstrate a
well-defined, thin-walled cystic mass containing dependent and nondependent
polypoid filling defects extending inferiorly and laterally from pancreatic
head/uncinate process. Note that fluid is hyperintense and filling defects are
hypointense relative to myometrium and that there is no evidence of myometrial
invasion. Single fetus commensurate with gestational age is seen in breech
position.
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Fig. 1C —39-year-old pregnant woman presenting as too large for dates. Axial
(C) breath-hold, gradient-echo T1-weighted (160/2.3) and (D)
axial breath-hold half-Fourier fast spin-echo T2-weighted (8,000/82) images at
same level in midabdomen show that fluid component decreases whereas solid
component increases in signal amplitude on T2-compared with T1-weighted
sequence. Gallstones (arrow, D) are also apparent in
T2-weighted image.
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Fig. 1D —39-year-old pregnant woman presenting as too large for dates. Axial
(C) breath-hold, gradient-echo T1-weighted (160/2.3) and (D)
axial breath-hold half-Fourier fast spin-echo T2-weighted (8,000/82) images at
same level in midabdomen show that fluid component decreases whereas solid
component increases in signal amplitude on T2-compared with T1-weighted
sequence. Gallstones (arrow, D) are also apparent in
T2-weighted image.
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Fig. 1E —39-year-old pregnant woman presenting as too large for dates.
Histologic features show cyst wall itself composed of well-organized layers of
smooth muscle with distinct internal and external layers typical for
gastrointestinal tract. Lining epithelium is mostly composed of
pseudostratified columnar cells with prominent cilia (white arrow)
but shows some transformation into malignant neoplastic cells (black
arrow).
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Fig. 1F —39-year-old pregnant woman presenting as too large for dates.
Photomicrographs shows high-grade adenocarcinoma with areas of necrosis and
complex papillary architecture lined by multilayered cuboidal cells with
high-grade cytology.
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Unenhanced axial, coronal, and sagittal imaging of the abdomen and pelvis
was performed on a 1.5-T magnet (Vision Plus, Siemens Medical Solutions).
T1-weighted images were obtained with a breath-hold gradient-echo sequence
(TR/TE, 160/2.3) and T2-weighted images with breath-hold half Fourier fast
spin-echo sequence (8,000/82).
Images showed a 14.8 x 8.5 x 8.0 ovoid, sharply marginated, and
thin-walled cystic retroperitoneal mass extending inferiorly and to the left
of the pancreatic head/uncinate process, clearly separate from the normal
spleen and the uterus (Figs. 1A
and 1B). It contained multiple
dependent and nondependent lobulated nodules. The fluid component was
hyperintense on T1- and hypointense on T2-weighted images, while the nodules
were hypointense on T1 and increased in signal amplitude on T2 (Figs.
1C and
1D). No pancreatic duct
dilatation, vascular encasement, or small-bowel invasion was evident. The
uterus contained a single fetus appropriate for gestational age. Given the
patient's age and race, the large size and signal characteristics of the mass,
and its proximity to the pancreas, the diagnosis considered most likely was a
solid and papillary epithelial neoplasm of the pancreas.
Fine-needle aspiration revealed a high-grade carcinoma, most likely of
pancreatic origin. However, at surgery the well-encapsulated cystic mass was
found to abut, but not invade, normal pancreas and normal spleen. Described as
attached to the retroperitoneum by loose fibroareolar tissue, it was resected
in entirety. The fluid content, brown and considered typical of hemorrhagic
fluid, was not analyzed further. Histologic examination of the surgical
specimen revealed a high-grade papillary adenocarcinoma with areas of
sarcomatoid transformation arising within a congenital cyst. The cyst was
composed of well-organized layers of smooth muscle typical of the
gastrointestinal tract and, where not involved by tumor, lined by benign
epithelium of columnar cells (Figs.
1E and
1F).
Discussion
The high signal of the fluid component of the mass on T1-weighted images,
with a relative decrease on T2, is typical of blood, as is the brown color of
the fluid found in the specimen. Mucinous/proteinaceous fluid may have similar
characteristics on MRI. Simple fluid in a duplication cyst would be expected
to be hypointense on T1 and hyperintense on T2
[8]. The increase in signal
amplitude of the solid component on T2-weighted images argued against debris
or thrombus and suggested neoplasm, further supported by the nondependence of
some of the nodules. Gadolinium was not given because of the pregnancy and the
belief that, since the mass was thought to be clearly neoplastic,
contrast-enhanced images would not alter management decisions.
Most solid and papillary epithelial neoplasms of the pancreas are found in
young African-American women (10-50 years old, mean 24 years). Most arise from
the pancreatic tail, but involvement of any portion of the pancreas has been
described [9]. They are usually
large (> 10 cm) at presentation with internal hemorrhage/gelatinous
degeneration but low-grade and cured by resection. Thus, this diagnosis fits
both clinical and imaging findings in our patient. Mucinous cystadenoma or
cystadenocarcinoma could have similar signal intensities but more commonly
present as multicystic lesions in a slightly older age group
[10]. Though enteric
duplication cysts occur most often in the small bowel, malignancy arising in
duplication cysts is rare and is most often associated with colonic or rectal
cysts rather than the proximal small bowel
[1]. We found six other reports
of malignancy arising from small-bowel duplication cysts, none with MRI
studies
[2-7].
MRI features of simple bowel duplication cysts have been described and
include heterogeneous signal intensity on T1- and homogeneous high-signal
intensity on T2-weighted images
[11,
12]; fat-suppressed
T1-weighted images were found particularly helpful in separating the
duplication cyst from the pancreas
[12]. The imaging approach in
this patient was dictated by the need for rapid imaging to avoid fetal motion
artifact and the desire to avoid contrast material if possible.
The misinterpretation of the abdominal sonogram speaks to the difficulties
of sonography in evaluating large masses because of the limited field of view
and underscores the advantage of MRI with its ability for large field-of-view
multiplanar imaging unimpeded by intervening air or tissue. The lack of
reported symptoms with such a large duplication cyst with malignant
transformation may be due to the patient's pregnancy. Any symptoms, such as
weight change, early satiety, nausea, or vomiting could have been attributed
to the patient's pregnancy.
In conclusion, MRI in this pregnant patient with a large indeterminate
abdominal mass established the retroperitoneal origin of the mass, defined its
borders, cleared uterus and spleen as distinctly separate from the mass, and
characterized the mass as a cystic neoplasm with hemorrhagic or proteinaceous
content. Though the specific diagnosis was not made, MRI defined the mass and
determined resectability, thus guiding management, all without exposure to
X-rays or use of contrast medium. The additional lesson learned is to consider
the possibility of neoplasm within a duplication cyst in the differential
diagnosis of such masses.
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Introduction
Case Report
