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Esophageal Cancer in Epiphrenic Diverticulum With Fistulization to the Lung

Vanstraelen, Stijn MD1,2; Depypere, Lieven MD, PhD1,2; Sagaert, Xavier MD, PhD3; Nafteux, Philippe MD, PhD1,2; Ceulemans, Laurens J. MD, PhD1,2

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doi: 10.14309/crj.0000000000000781
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A 56-year-old man with a history of smoking and alcohol dependence presented to the emergency department with a 3-day history of epigastric pain and fever. The laboratory results were consistent with infection (white blood cell count: 27.8 × 103/mm3; C-reactive protein: 287 mg/L). Computed tomography showed an esophageal perforation in continuity with a right lower lobe abscess (Figure 1). Esophagogastroduodenoscopy demonstrated an ulcerative mass originating from a large epiphrenic diverticulum concealing a fistula at the inferior aspect (Figure 2).

Figure 1.:
Axial section of computed tomography showing the epiphrenic diverticulum with perforation (*) and pulmonary abscessation of the right lower lobe (arrow).
Figure 2.:
(A) Endoscopic view on the epiphrenic diverticulum (*) with an ulcerative wall and (B) endoscopic view inside the diverticulum showing the fistula (arrow) on an ulcerative base.

Pathological examination confirmed the presence of a squamous cell carcinoma (Figure 3). After a 10-day antibiotic treatment (penicillin 4 × 1 g), an esophagectomy and segment-6-sparing lower lobectomy through right thoracotomy were performed. Owing to inflammation, the reconstruction was postponed, and a cervical esophagostomy was created. Twelve days later, a retrosternal laparoscopic-assisted esophagogastric reconstruction was performed.

Figure 3.:
Histopathological image (H&E coloring, ×25) on the edge of the diverticulum showing the origin of the squamous cell carcinoma (arrow).

Pathological examination demonstrated transmural invasion into the right lower lobe and 3 positive lymph nodes (pT4b N2). The patient was discharged after 10 days after an uneventful postoperative course. Adjuvant chemotherapy (6 courses of a combination of Oxaliplatin, 5- Fluorouracil and Leucovorin (FOLFOX)) was administered for 3 months, followed by paclitaxel for local lymph node recurrence. A follow-up positron emission tomography with computed tomography, 9 months after surgery, revealed disease progression in mediastinal lymph nodes and metastases in the kidney and liver. Despite salvage immunotherapy with nivolumab, the patient died 3 months later.

Epiphrenic diverticula are very rare and account for only 10% of all esophageal diverticula.1 The incidence of the latter is estimated between 0.06% and 3.6% and is the result of the herniation of mucosa and submucosa through the muscular wall of the esophagus.2,3 The development of cancer in an esophageal diverticulum is seen in only 0.3%–3% and believed to be the consequence of chronic irritation of retained food and its subsequent inflammation.4 Most tumors are detected in an advanced stage given the absence of the muscularis propria. In a literature overview of Yoshida et al, 1 in 3 patients (7/19) was diagnosed with invasion in the surrounding organs and had a 1-year mortality rate of 66% (4/6).5 Therefore, early detection is paramount and could be realized with regular endoscopic and radiologic follow-up and subsequent treatment.


Author contributions: LJ Ceulemans, MD, PhD is the article guarantor. All authors of this article have substantially contributed to the conception and design, drafting and revision of its content, and approval of the final version. All authors agree to the correctness and are accountable for all the content of this article.

Financial disclosure: None to report.

Informed consent was obtained for this case report.


1. Guerra JM, Zuil M, Garcia I, Moreno E. Epiphrenic diverticula, esophageal carcinoma and esophagopleural fistula. Hepatogastroenterology. 2001;48:718–9.
2. Hoghooghi D, Coakley FV, Breiman RS, Qayyum A, Yeh BM. Frequency and etiology of midesophageal diverticula at barium esophagography. Clin Imaging. 2006;30:245–7.
3. Honda H, Kume K, Tashiro M, et al. Early stage esophageal carcinoma in an epiphrenic diverticulum. Gastrointest Endosc. 2003;57:980–2.
4. Fujita H, Kakegawa T, Shima S, Kumagaya Y. Carcinoma within a middle esophageal (parabronchial) diverticulum: A case report and the review of the literature. Jpn J Surg. 1980;10:142–8.
5. Yoshida T, Hashimoto S, Mizuno KI, et al. Advanced squamous cell carcinoma in an asymptomatic, large, epiphrenic esophageal diverticulum. Clin J Gastroenterol. 2020;13(4):477–82.
© 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.