AJR 2003; 180:1335-1337
© American Roentgen Ray Society
Primary Hodgkin's Lymphoma of the Esophagus
Emmanuel Coppens1,
Issam El Nakadi2,
Nathalie Nagy3 and
Marc Zalcman1
1 Department of Radiology, Hôpital Erasme, Université Libre de
Bruxelles, 808 Route de Lennik, 1070 Brussels, Belgium.
2 Department of Abdominal Surgery, Hôpital Erasme, Université Libre
de Bruxelles, 1070 Brussels, Belgium.
3 Department of Pathology, Hôpital Erasme, Université Libre de
Bruxelles, 1070 Brussels, Belgium.
Received July 18, 2002;
accepted after revision September 10, 2002.
Address correspondence to M. Zalcman.
Introduction
Esophageal involvement by lymphoma is rare and represents approximately 1%
of the cases of lymphomatous involvement of the gastrointestinal tract
[1,
2]. Non-Hodgkin's lymphomas
account for most of the cases
[2]. As with other digestive
tract lymphomas, few of the esophageal lymphomas are of the primary type;
esophageal location as the first site of Hodgkin's disease is exceptional
[2,
3,
4,
5]. The radiographic
manifestations of esophageal lymphoma have shown a diverse spectrum of
abnormalities similar to those of lymphoma elsewhere in the gastrointestinal
tract [6,
7], except for the aneurysmal
dilatation pattern that, to our knowledge, has never been described
previously. We report an exceptional case of primary Hodgkin's lymphoma of the
esophagus with a unique radiologic description of progressive aneurysmal
dilatation.
Case Report
A 61-year-old man presented with odynophagia associated with dysphagia for
solids and liquids. His history included a gastric ulcer and reflux
esophagitis treated for 1 year. The findings at physical examination were
normal. Complete blood cell count and routine serum chemistry levels were
within normal limits. Findings of double-contrast esophagography showed an
irregular luminal narrowing of the proximal two thirds of the esophagus due to
multiple submucosal nodules. These nodules coalesced and presented as enlarged
tortuous and ulcerated longitudinal folds
(Fig. 1A) that mimicked
esophageal varices. Varicoid carcinoma was considered, but this diagnosis was
excluded after the biopsy analyses.
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Fig. 1A. —61-year-old man with odynophagia and progressive dysphagia.
At initial workup, double-contrast esophagogram shows submucosal nodules with
confluent areas appearing as enlarged tortuous and ulcerated longitudinal
folds that mimic varices in upper and mid esophagus.
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Endoscopic biopsies of the esophageal mucosa showed granulation tissue
composed of a mixed lymphoplasmocytic–polynuclear infiltrate with
neovascularity, associated with a fibrinonecrotic exudate containing bacteria,
spores of Candida organisms, and mycelium filaments in large amounts
with no sign of dysplasia or malignancy. CT of the chest showed a nonspecific
circumferential wall thickening (Fig.
1B) of the proximal two thirds of the esophagus without
mediastinal lymphadenopathy and showed incidentally a lung mass later found to
be a poorly differentiated lung adenocarcinoma of the right upper lobe (stage
T3 N0 M0 [8]), treated by right
upper lobectomy. Mediastinal exploration during lung surgery showed no
enlarged lymph nodes. All resected periesophageal lymph nodes were normal at
pathologic examination.
For 6 months, the patient underwent several therapeutic trials for
esophagitis of unknown origin. Ten additional esophagoscopic examinations were
performed. Results of mucosal and deep submucosal biopsies showed nonspecific
inflammatory infiltrates and remained negative for malignancy. Results of
repeated double-contrast esophagograms and CT scans of the esophagus showed a
progressive aneurysmal dilatation of the diseased proximal two thirds of the
esophagus with distal extension of the submucosal nodules (Figs.
1C and
1D).
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Fig. 1C. —61-year-old man with odynophagia and progressive dysphagia.
Double-contrast esophagogram obtained 5 months after right upper lobectomy
shows aneurysmal dilatation in proximal and mid esophagus.
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A fistula developed between the diseased esophagus and both the cavity of
lobectomy and the right mainstem bronchus, leading to abscess formation of the
right upper lobectomy cavity, right middle lobe pneumonia, and empyema, which
was treated with antibiotics and pleural drainage
(Fig. 1E). CT revealed enlarged
lymph nodes in the gastrohepatic ligament.
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Fig. 1E. —61-year-old man with odynophagia and progressive dysphagia.
CT scan obtained at same level as B shows marked dilatation of
esophageal lumen (e) with nodular thickening of esophageal wall,
fluid–gas level with barium residue (arrow) in lobectomy
cavity, and paraesophageal hypodense lymphadenopathy (arrowhead).
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The patient underwent esophagectomy. Findings of the pathologic examination
(Fig. 1F) showed Hodgkin's
lymphoma of the esophagus (stage IIIE according to Ann Arbor classification
[9]) with involvement of a
single noncontiguous retroesophageal lymph node and of numerous perigastric
lymph nodes. Septic shock developed postoperatively as a result of
bronchopneumonia of the right middle and lower lobes.
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Fig. 1F. —61-year-old man with for odynophagia and progressive dysphagia.
Photograph of esophagectomy specimen shows ulcerated burgeoning masses
partially covered by whitish fibrinous exudate. Diagnosis was Hodgkin's
lymphoma of mixed cellularity subtype.
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The patient died on day 25 after esophagectomy. Autopsy showed numerous
tracheobronchial lymph nodes involved by Hodgkin's disease and a single
lymphomatous hepatic metastasis. No other gastrointestinal location of the
disease was found.
Discussion
To our knowledge, Hodgkin's disease involving the esophagus has been
reported rarely in the literature. Most cases of supposed primary esophageal
Hodgkin's disease that were previously reported had concomitant peripheral
lymphadenopathy at the time of lesion detection, arose secondarily by
extension from involved adjacent lymph nodes or by contiguous spread from the
gastric fundus, or recurred after a previously treated Hodgkin's disease. On
the basis of these findings, true primary Hodgkin's lymphoma of the esophagus
is exceptional, being reported in only four cases since the 1920s
[2,
3,
4,
5]. Lymphoma of the esophagus
occurs more often in the distal esophagus
[6,
7]; however, Hodgkin's disease
involves predominantly the upper or mid esophagus
[2].
Hodgkin's disease accounts for most cases of lymphoma with
tracheoesophageal fistula
[10]. Contiguous lymphomatous
involvement of the distal esophagus and gastric fundus appears less frequently
in Hodgkin's disease than in non-Hodgkin's lymphoma. As with lymphoma
elsewhere in the gastrointestinal tract, the radiographic findings of
esophageal lymphoma are somewhat nonspecific and have been reported as
multiple submucosal nodules sometimes appearing as diffuse fine nodularity
[7] or as enlarged varicoid
tortuous longitudinal folds, a single large intramural mass, polypoid masses
with or without ulceration, an achalasialike tapered narrowing of the distal
esophagus, and irregular and sometimes infiltrating strictures
indistinguishable from esophageal carcinoma
[6,
7].
The focal dilatation in the diseased esophagus that we reported resembled
that of the aneurysmal dilatation, well described in small-bowel lymphoma
[11], in which the mechanism
of dilatation in the esophagus could be explained by extensive neoplastic
invasion and destruction of the wall, including the muscle layers and neural
plexus, resulting in mural atonicity and luminal dilatation. The fistula
formation also supported the presence of transmural growth of the tumor, which
was confirmed at pathologic examination.
This case highlights the difficulty of diagnosing primary esophageal
lymphoma, even when symptomatic. Because primary lymphoma arises typically in
the submucosal or lamina propria lymphoid patches of the gut wall, findings of
routine endoscopic biopsies, which sample the mucosa, are often normal or show
nonspecific inflammatory changes
[12]. Even deep biopsies with
macroforceps at rigid endoscopy are sometimes nondiagnostic because
lymphomatous changes in the esophageal wall may be patchy and missed because
of sampling error [2,
12]. The repeated negative
biopsies in the present case were responsible for the delayed diagnosis,
despite the concern caused by the radiologic and endoscopic appearances.
Some authors stress the necessity of an early diagnosis because the
response to local or systemic treatment is often good
[2,
11]; therefore, clinicians and
radiologists should be aware of this disease. Despite its rarity, primary
esophageal Hodgkin's disease and lymphoma in general should be included in the
differential diagnosis of atypical lesions of the esophagus, even in the
asymptomatic patient.
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Introduction
Case Report
