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DOI: 10.1055/s-0034-1393591
Submucosal carcinoma of the gastroesophageal junction diagnosed after peroral endoscopic myotomy
Publikationsverlauf
Publikationsdatum:
29. Dezember 2015 (online)
Gastroesophageal junction (GEJ) malignancies are found in 4.7 % of patients who fulfil the manometric criteria for achalasia [1]. Such malignancies may manifest as pseudoachalasia because of submucosal infiltration and secondary impairment of the inhibitory neurons of the esophageal myenteric plexus, therefore mimicking the manometric pattern of achalasia [2]. Despite endoscopic biopsies, false-negative rates of 25 % may mask this cause of pseudoachalasia [3].
A 62 year old man with no history of Barrett’s esophagus presented with a 4-month history of dysphagia, vomiting, and weight loss. His initial esophagogastroduodenoscopy (EGD) showed a dilated esophagus and tight GEJ; multiple biopsies were negative. A computed tomography (CT) scan of the thorax and abdomen showed a bulky GEJ with no definite mass lesion. Endoscopic ultrasound (EUS) also showed a circumferential thickening in the area of the GEJ, but no masses suggestive of malignancy or suspicious lymph nodes were seen ([Fig. 1]). High resolution manometry suggested type II achalasia.
As malignancy had been excluded as a cause of the tight GEJ, the patient underwent peroral endoscopic myotomy (POEM). During creation of the submucosal tunnel, thickening and fibrosis of the muscularis propria was encountered near the GEJ ([Fig. 2]); however, intraoperative frozen sections were normal. The remainder of the POEM proceeded uneventfully, the myotomy being performed with a Triangle Tip knife (Olympus) and the tunnel entry being closed with clips ([Video1]). Multiple biopsies of the mucosa and muscularis propria showed cytologic atypia of unknown significance but were negative for malignancy.
Qualität:
The patient was monitored closely in the clinic and his symptoms recurred within a month. Another EGD showed a GEJ stricture 38 cm from the incisors. Biopsies were again indeterminate. A repeat CT scan confirmed a 3.6-cm concentric mural thickening at the GEJ. Surgery was therefore advised. Intraoperatively, a localized tumor was found at the GEJ. A total gastrectomy with D2 lymphadenectomy was performed. Final histology revealed a moderately differentiated submucosal adenocarcinoma of the GEJ with no involvement of the mucosa ([Fig. 3]).
After receiving chemotherapy, the patient remained free of symptoms and disease 1 year postoperatively.
This case highlights the rare occurrence of a submucosal GEJ adenocarcinoma that was not identified on either pre-POEM endoscopy with mucosal biopsies or on EUS in a patient with symptoms and signs suggestive of achalasia. This may have been due to a submucosal tumor and sampling error. After performing POEM, clinicians should maintain a degree of suspicion for malignancy if a patient’s symptoms fail to resolve.
Endoscopy_UCTN_Code_CPL_1AH_2AJ
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References
- 1 Campo SM, Zullo A, Scandavini CM et al. Pseudoachalasia: a peculiar case report and review of the literature. World J Gastrointest Endosc 2013; 5: 450-454
- 2 Rozman RW, Achkar E. Features distinguishing secondary achalasia from primary achalasia. Am J Gastroenterol 1990; 85: 1327-1330
- 3 Woodfield CA, Levine MS, Rubesin SE et al. Diagnosis of primary versus secondary achalasia: reassessment of clinical and radiographic criteria. Am J Roentgenol 2000; 175: 727-731