AJR 2005; 184:S129-S131
© American Roentgen Ray Society
Pre- and Postoperative MRI of Esophageal and Gastric Leiomyomatosis in a Pediatric Patient
A. Kristina Kilian1,
Thomas Ringle2,
Karl-Ludwig Waag2,
Christoph Düber1 and
K. Wolfgang Neff1
1 Department of Clinical Radiology, University of Heidelberg,
Universitätsklinikum Mannheim, Theodor-Kutzer-Ufer 1-3, Mannheim 68167,
Germany.
2 Department of Pediatric Surgery, University of Heidelberg,
Universitätsklinikum Mannheim, Mannheim 68167, Germany.
Received March 25, 2004;
accepted after revision June 8, 2004.
Address correspondance to A. K. Kilian.
Introduction
Esophageal leiomyomatosis is a rare benign tumor that can be
associated with leiomyomas at other sites or with nephropathy, defective
hearing, astigmatism, and myopia (Alport's syndrome). The most common
diagnostic procedures for detecting and diagnosing esophageal leiomyomatosis
are barium studies, sonography, and CT. In children, except for sonography,
MRI seems to be the best diagnostic tool because the patient is not exposed to
radiation and MRI offers multiplanar imaging possibilities. It is the optimal
method for follow-up investigations after tumor resection. In this report, the
pre- and postoperative MRI findings in a case of familial esophageal and
gastric leiomyomatosis are presented.
Case Report
A 9-year-old girl presented with dysphagia over several months. She had a
long-standing history of gastroesophageal reflux, bronchial asthma, and IgA
nephritis. The patient also had defective hearing and a familial vision
disorder (astigmatism and hyperopia). At physical examination, the girl
weighed 24 kg and was 131 cm tall, which correspond to the 10-25th percentile.
The remainder of the examination findings were unremarkable. The girl's
14-year-old brother and the children's mother had shown the same symptoms of
dysphagia 1 year (brother) and 20 years (mother) previously. In both the
mother and brother, an esophageal tumor was found and histopathologic analysis
after surgery revealed esophageal leiomyomatosis. The girl's 14-year-old
brother also has associated diseases such as IgA nephritis, bronchial asthma,
defective hearing, and reduced vision.
On the barium study, the girl's distal esophagus was dilated and showed an
abnormal peristaltis and delayed esophageal-gastric passage. Endoscopy
revealed a circular reddening of the mucosa with fibrinoid layers. Biopsy
showed chronic esophagitis. On percutaneous transabdominal sonography, the
wall of the distal esophagus and the proximal parts of the stomach were
markedly thickened.
An MR study (1.5-T unit [Magnetom Vision, Siemens]) using axial breath-hold
fast spin-echo T2-weighted and gradient-echo T1-weighted sequences with and
without fat saturation was then performed. In addition, breath-hold
T2-weighted multiplanar HASTE imaging was performed. All sequences showed a
homogeneous tumor of the lower esophagus that extended to the gastric fundus
with extensive wall thickening of up to 3 cm and corresponding lumen
reduction. On T2-weighted images (Fig.
1A), the tumor was moderately hyperintensive compared with the
liver; on T1-weighted images (Fig.
1B), the tumor was hypointense in comparison with the liver
parenchyma. On T2-weighted HASTE images (Figs.
1C,
1D and
1E), the tumor was similar to
the liver in signal intensity; on T1-weighted images with fat saturation, the
signal intensity was decreased compared with that of the liver.
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Fig. 1A. —9-year-old girl with esophageal and gastric leiomyomatosis.
Breath-hold T2-weighted axial turbo spin-echo MR image obtained at level of
distal esophagus shows extensive homogeneous wall thickening.
|
|
After the administration of 0.1 mmol/mL/kg of gadopentetate dimeglumine
(Magnevist, Schering), a moderately homogeneous contrast enhancement of the
tumor was noticed and esophageal and gastric leiomyomatosis was diagnosed.
After esophagectomy and gastric pull-through and after a histologic analysis
of leiomyomatosis, postoperative MRI (Fig.
1F) confirmed complete tumor resection and normal postoperative
findings at the esophageal-gastric anastomosis.
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Fig. 1F. —9-year-old girl with esophageal and gastric leiomyomatosis.
Postoperative T2-weighted coronal HASTE image obtained after esophagectomy and
gastric pull-through shows normal postoperative findings.
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Discussion
Benign tumors of the esophagus are very rare. Only 0.4%
[1,
2] of all esophageal neoplasms
are benign. Of the benign tumors, 80% are esophageal leiomyomatoses
[1]. In a study conducted by
Levine et al. [3], a diagnosis
of familial leiomyomatosis was made in half of the patients with esophageal
leiomyomatosis and one diagnosis of Alport's syndrome was made in a third.
Other studies report similar results
[3-6].
Federici et al. [7] described
one case of esophageal leiomyomatosis, but they were able to review only 24
cases of esophageal leiomyomatosis found in the literature in children less
than 14 years old.
Esophageal leiomyomatosis usually appears as a solitary tumor (97%)
[2], but multilocular forms can
also be found. In the pediatric population, a multilocular or diffuse
appearance is more common than a unilocular manifestation
[3]. Esophageal leiomyomatosis
can occur as an isolated tumor or in association with leiomyomas at other
sites (bronchi, uterus, vulva)
[8].
In contrast to malignant esophageal tumors, patients with esophageal
leiomyomatosis usually present with a slowly progressive dysphagia over years.
Levine et al. [3] reported an
average duration of dysphagia of 3.5 years; other studies describe patients
with a history of about 20 years of dysphagia
[2,
6]. Dysphagia is the result of
circumferential wall thickening that is caused by proliferation of the
circular and longitudinal smooth muscle mostly located in the lower third of
the esophagus, extending to the cardia of the stomach in some cases, and
obstructing the lumen. A small number of patients develop prestenotic
dilatation of the esophagus.
Esophageal ulceration and bleeding caused by esophageal leiomyomatosis are
uncommon. Minimal to moderate vascularization is seen in esophageal
leiomyomatosis. To our knowledge, no study has reported neoplastic
transformation.
The diagnostic methods for detecting esophageal leiomyomatosis are barium
studies, sonography, CT, and MRI. The advantage of the barium swallow in
diagnosing esophageal leiomyomatosis is that luminal narrowing and the
motilinity of the esophagus can be precisely delineated, resembling findings
in achalasia. Levine et al. [3]
described an average length of 6.3 cm of the narrowed segment in
leiomyomatosis. However, the narrowed segment is much longer than the
short-segment stenosis in achalasia.
CT can distinguish between leiomyomatosis and achalasia or peptic stricture
because the space-occupying tumor with extensive wall thickening can be imaged
and compared with the almost normal esophageal wall thickness in achalasia and
peptic stricture. CT can show the complete extraluminal size of the tumor with
the delimitation to mediastinal and supra- and infradiaphragmal
structures.
Like CT, MRI can depict the thickness of the esophageal wall and illustrate
the tumor involvement in the chest and abdomen. To our knowledge, MRI findings
of pediatric esophageal and gastric leiomyomatosis have not been described to
date. In children, the appropriate diagnostic imaging technique is MRI because
there is no radiation exposure and because of the multiplanar imaging
possibilities. In particular, multiplanar high-resolution T2-weighted HASTE
imaging can depict tumor extension in detail. For these reasons and in
addition to depicting the tumor, MRI is the optimal method for follow-up
investigations. The MR characteristics of esophageal leiomyomatosis have been
described in the literature in a small number of cases in adult patients
[8]. The typical MRI findings
of leiomyomatosis are very similar to the findings in our pediatric
patient.
As we stated earlier, esophageal leiomyomatosis was diagnosed 1 year
previously in the patient's 14-year-old brother. In the children's mother,
esophageal leiomyomatosis had been found 20 years earlier. Both relatives also
received surgical therapy. Like in our patient, other studies also showed a
familial presentation of esophageal leiomyomatosis
[3]. Therefore, it might be
useful to establish screening investigations—in particular,
examiniations based on sonography and MRI in affected families. In addition,
the diagnosis of esophageal leiomyomatosis should lead to a prompt search for
other features—for example, Alport's syndrome—in both the patient
and his relatives.
In conclusion, the ultimate diagnosis of esophageal leiomyomatosis must be
made by histopathologic analysis, but the characteristic findings of MRI
correlated with the clinical symptoms of long-standing dysphagia over years in
children can give a preoperative diagnosis of esophageal leiomyomatosis. In
the preoperative patient evaluation, multiplanar T2-weighted HASTE MRI, in
particular, is able to depict the exact extent of leiomyomatosis.
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Introduction
Case Report
