Pseudoachalasia as the first manifestation of signet-ring cell duodenal carcinoma


Nouha Trad
Emna Bel Hadj Mabrouk
Asma Mensi
Yosra Zaimi
Mariem Ksantini
Yosra Said


Background: Pseudoachalasia is a rare clinical condition, often caused by malignancy. Rarely, this entity can reveal the underlying
neoplasia. To the best of our knowledge, we report the first case of pseudoachalasia revealing a metastatic signet-ring cell carcinoma of
the non-ampullary duodenum.
Case Report: A previously healthy 67-year-old patient presented with a 3-month history of rapidly progressive intermittent dysphagia with
20kg weight loss. An upper endoscopy showed multiple duodenal ulcerations on congestive mucosa. Duodenal biopsies revealed tumor
proliferation formed by independent cells with atypical nuclei. In immunohistochemistry, tumor cells expressed pan-cytokeratin. Esophageal
manometry revealed an aspect in favor of achalasia type II. The CT scan showed ascites and pleural effusion. Their punctures confirmed the
presence of neoplastic cells. The diagnosis of metastatic signet-ring cell duodenal carcinoma revealed by pseudoachalasia was retained.
Conclusion: In the presence of rapidly progressive symptoms with significant weight loss, especially in the elderly patients, malignancyassociated pseudoachalasia should be suspected.


Esophageal achalasia, Signet ring cell carcinoma, Duodenum



  1. Gergely M, Mello MD, Rengarajan A, Gyawali CP. Duration of symptoms and manometric parameters offer clues to diagnosis of pseudoachalasia. Neurogastroenterol Motil 2021;33:13965.
  2. Schizas D, Theochari NA, Katsaros I, Mylonas KS, Triantafyllou T, Michalinos A, et al. Pseudoachalasia: a systematic review of the literature. Esophagus 2020;17:216‑22.
  3. Fabian E, Eherer AJ, Lackner C, Urban C, Smolle-Juettner FM, Krejs GJ. Pseudoachalasia as first manifestation of a malignancy. Dig Dis 2019;37:347‑54.
  4. Iturrino J, Katzka D, Farrugia G, Arora A. Achalasia secondary to neoplasia: A disease with a changing differential diagnosis. Am J Gastroenterol 2011;106:S4.
  5. Campo S, Zullo A, Scandavini C, Frezza B, Cerro P, Genoveffa B. Pseudoachalasia: A peculiar case report and review of the literature. World J Gastrointest Endosc 2013;5:450‑4.
  6. Anderson J, Sharma N, Holfinger S, Adamic B, Castor J, Lo DY. Duodenal adenocarcinoma associated with achalasia: 1633. Am J Gastroenterol 2016;111:S770.
  7. Mochizuki K, Kondo T, Tahara I, Inoue T, Kasai K, Oishi N, et al. Signet ring cell carcinoma of the non-ampullary duodenum: A case report. Pathol Res Pract 2015;211:801‑4.
  8. Caraşca C, Simion G, Nechifor-Boilă AC, Boeriu AM, Dobru ED. A rare case of signet-ring cell carcinoma associated with poorly differentiated adenocarcinoma of the non-ampullary duodenum. Rom J Morphol Embryol 2018;59:311‑5.
  9. Abubakar U, Bashir MB, Kesieme EB. Pseudoachalasia: A review. Niger J Clin Pract 2016;19:303‑7.
  10. Li YD, Cheng YS, Li MH, Chen NW, Chen WX, Zhao JG. Temporary self-expanding metallic stents and pneumatic dilation for the treatment of achalasia: a prospective study with a long-term follow-up. Dis Esophagus 2010;23:361‑7.