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Adenocarcinoma in EIPD | ||
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1 Specialty
Training Registrar ST5 2 Consultant
Gastroenterologist Summary: Oesophageal intramural pseudodiverticulosis (EIPD) is
a rare condition of unknown aetiology characterised by multiple, flask-shaped
out-pouching with segmental or diffuse involvement of the oesophagus. EIPD has been associated with oesophageal
stricture, candida oesophagitis and oesophageal dysmotility. Our knowledge of long term outcome of this
condition is limited. We report a case
of adenocarcinoma in a patient with EIPD that had multiple oesophageal
dilatations Case report: A 63 year old man with Type 2 diabetes was diagnosed
of oesophageal intramural pseudo-diverticulosis EIPD with recurrent high
oesophageal stricture since 2007. He was
treated successfully with wire-guided through the scope balloon dilatations
(inflated up to 10mm). Multiple
oesophageal biopsies showed evidence of active chronic inflammatory changes
with fungal hyphae on Periodic acid-Schiff stain. See Figs 1, 2 and 3.
Fig 1: Barium swallow showed multiple “flash-shaped” outpouchings involving the whole of the oesopghagus typical of EIPD
Fig 2: Upper gastro-intestinal endoscopy showed oesophageal intramural pseudo-diverticulosis with stricture requiring wire-guided through the scope balloon dilatation
Fig 3: Oesophageal biopsy showed active chronic inflammatory changes with fungal hyphae on Periodic acid-Schiff stain. No evidence of dysplasia or malignancy identified He received oral fluconazole for the treatment of
oesophageal candidiasis as well as long term full dose oral proton pump
inhibitor. He remained asymptomatic for
several years. Patient re-presented with
dysphagia in 2013. A diagnostic upper GI
endoscopy showed stricture at the gastro-oesophageal junction (GOJ) 40cm from
incisor. The stricture was
unsuccessfully treated with wire-guided through the scope balloon dilatations
(inflated up to 12mm). Multiple biopsies
confirmed adenocarcinoma of tubular intestinal type. Positron emission tomography (PET),
Computerised tomography (CT) and Endoscopic Ultrasound scan(EUS) staging showed
T3,N0,M0.
The patient received pre-operative adjuvant chemotherapy followed by
radical total oesophagectomy. See Figs 4 and 5.
Fig 4: UGI endoscopy showed stricture at gastro-oesophageal junction (GOJ) unsuccessfully treated with balloon dilatation
Fig 5: Intense uptake in the distal oesophageal lesion which extended from 40-45cm Discussion: Since first reported EIPD in 1960, there were around
200 cases published world-wide.1,2 Up to 90% of patients with EIPD
have associated oesophageal stenosis of various levels most commonly in the
upper oesophagus secondary to chronic stasis oesophagitis.3,4 EIPD is often regarded as benign oesophageal
disease but there are several reports where EIPD was found in patients with
oesophageal carcinoma. 5 The
aetiology behind EIPD developing oesophageal cancer could be due to metaplastic
changes of the squamous epithelium within the excretory ducts of oesophageal
submucosal glands in EIPD. 6 The pre-malignant nature of metaplastic
epithelium is widely recognised in the upper gastro-intestinal tract. The increased prevalence of EIPD in patients
with oesophageal carcinoma may warrant periodic surveillance in this small
population of patients. References:
1. Mendl K, Tanner CH. Intramural diverticulosis of the
oesophagus and Rokitansky-Aschoff sinuses in the gall-bladder. Br J Radiol.
1960;33:496-501. 2. Levine MS, Moolten DN, Herlinger H, Laufer I. Esophageal intramural pseudodiverticulosis: a reevaluation. Am J Roentgenol. 1986;147:1165-1170.
3. Sabanathan S, Salama FD, Morgan WE. Oesophageal
intramural pseudo-diverticulosis. Thorax 1985;40:849-857. 4. Cho SR, Sanders MM, Turner MA, Liu CI, Kipreos BE.
Esophageal intramural pseudodiverticulosis. Gastrointest Radiol 1981;6:9-16.
5. Plavsic BM, Chen MY, Gelfand DW, Drnovsek VH, Williams
JP 3rd, Kogutt MS, Terry JA, Plenkovich D. Intramural
pseudodiverticulosis of the esophagus detected on barium esophagograms:
increased prevalence in patients with esophageal carcinoma. Am J Roentgenol
1995;165:1381-1385. 6. Kataoka H, Higa T, Koono M. An autopsy case report of diffuse esoophageal intramural pseudodiverticulosis. Acta Pathol Jpn 1992;42:837-840. | ||
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