Gastric duplication cyst lined by pseudostratified columnar ciliated epithelium: a case report and literature review

Gastric duplication cyst lined by pseudostratified columnar ciliated epithelium: a case report and literature review 1 first-author 2 3 4 2 corresp ‡Corresponding Author ‡Corresponding Author †E-mail:gaoshunliangzju@163.com †E-mail:gaoshunliangzju@163.com gaoshunliangzju@163.com 1 2 Department of Surgical Oncology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, China Department of Surgical Oncology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, China Department of General Surgery, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, China Department of General Surgery, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, China Department of Pathology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, China Department of Pathology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, China Department of Radiology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, China Department of Radiology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, China Gastric duplication cyst (GDC) lined by pseudostratified columnar ciliated epithelium (PCCE) is an uncommon lesion stemming from a foregut developmental malformation. Its clinical and radiological presentation is usually nonspecific. In this study, we reported a 76-year-old man who presented with an incidentally found perigastric mass. An exploratory laparotomy revealed a non-communicating cyst below the gastroesophageal junction, measuring 4 cm×4 cm in size. Microscopically, the gastric cyst was lined merely by PCCE. Although rare, GDC lined by PCCE should be included in the differential diagnosis of gastric wall masses. Surgical intervention is warranted in patients who have clinical symptoms, or who are aged more than 50 years. Gastric duplication cyst (GDC) Foregut Pseudostratified columnar ciliated epithelium (PCCE) Endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) Introduction 1 Gastric duplication cyst (GDC) is an uncommon congenital malformation and is usually lined by gastrointestinal mucosa. GDC lined by pseudostratified columnar ciliated epithelium (PCCE) is extremely rare, and only 17 cases have been reported up to Mar. 2010 (Table 1 1 ). Due to the nonspecific clinical and radiological presentation, GDC lined by PCCE is easily misdiagnosed as a gastrointestinal stromal tumor (GIST). In this paper, we describe a unique case of GDC lined merely by PCCE, and the available English literature is reviewed. M: male; F: female; AGIJ: anterior of gastrointestinal junction; NGEJ: near gastroesophageal junction; LC: lesser curvature; GC: greater curvature; PW: posterior wall; NA: not available References Age (year) Gender Complaint Location Size (cm) Mardi et al., 2010 2010 42 M Left lumbar pain AGIJ, LC 4.5×5.2 Jiang et al., 2010 2010 25 F Epigastric pain Gastric fundus 3.0×2.5×2.0 Shibahara et al., 2009 2009 43 M Epigastric pain Cardia, LC NA Sato et al., 2008 2008 60 F No Cardia, LC 3 Murakami et al., 2008 2008 72 F No Middle body, LC 2.0×1.5 Wakabayashi et al., 2007 2007 37 M Epigastric pain NGEJ, LC 4×4 Hall et al., 2007 2007 40 M Epigastric discomfort NGEJ, LC 6×5 Theodosopoulos et al., 2007 2007 46 F Vomiting (1) PW of fundus; 8×5.5 (2) Gastrosplenic ligament 3 Lee et al., 2006 2006 38 F No Cardia, LC 7×5 Cunningham et al., 2006 2006 63 F Fever, abdominal pain PW of fundus 10×7.6 Melo et al., 2005 2005 39 F No Fundus 4×2.5×1 Rubio et al., 2005 2005 26 M Epigastric pain NA NA Song et al., 2005 2005 62 F No NGEJ, LC 3.5×2.5×1.5 Hedayati et al., 2003 2003 59 F No PW of stomach, LC 7×5 Kim et al., 2003 2003 35 M Epigastric pain NGEJ, LC 7×6×5 Takahara et al., 1996 1996 25 M No PW of fundus 6.5×5×5 Gensler et al., 1966 1966 46 F No NGEJ, GC 6×8 Present 76 M No NGEJ, LC 4×4 Case report 1 A 76-year-old man was referred to our hospital for evaluation of a perigastric mass found incidentally on abdominal ultrasonography. He had been in a good state of health. Physical examination findings and laboratory studies were within normal ranges. An abdominal computed tomography (CT) demonstrated a homogeneous, low-density mass, measuring 4 cm×4 cm, on the lesser curvature of stomach without contrast enhancement (Fig. 1 1 ). Gastroscopy revealed a submucosal type elevation with overlying normal mucosa below the gastroesophageal junction. Endoscopic ultrasound (EUS) showed this to be a hypoechoic lesion, arising within the tunica muscularis (Fig. 2 2 ). Preoperative diagnosis was a GIST and surgical excision was performed. The patient had an uneventful recovery and remained healthy to date. Pathologic examination reported that the cyst was embedded within the gastric muscular layer, and did not communicate with the gastric lumen. Microscopically, the cyst wall was lined merely by PCCE, with two or three complete layers of smooth muscle bundles. However, cartilaginous tissue, seromucous gland, or gastric epithelium was not identified (Fig. 3 3 ). Cystic structures are lined merely by pseudostratified columnar ciliated epithelium (PCCE), with two or three complete layers of smooth muscle bundles (hematoxylin and eosin staining; original magnification: (a) 100×; (b) 400×) Discussion 1 Gastric duplication cyst is an uncommon lesion that accounts for 2%–8% of all alimentary duplications (Kim et al., 2000 2000 ). It is suggested that GDC lined by PCCE is derived from the caudad-most portion of the laryngotracheal outgrowth, which remains attached to the portion of the primitive foregut destined to become the stomach (Gensler et al., 1966 1966 ), and migrates into the abdominal cavity before the fusion of the pleuroperitoneal membrane in the 7th week of gestation (Song et al., 2005 2005 ). When PCCE is seen in a cyst, we designate those containing cartilage or seromucous respiratory glands as bronchogenic cysts, those containing two well-developed layers of smooth muscle without cartilage as esophageal cysts, and those without these distinguishing features as foregut cysts (Murakami et al., 2008 2008 ). Moreover, it is advocated that the nomenclature for these duplicated cysts should involve the location as the prefix and the differentiation of the cyst suggested by the epithelium as the suffix (Sharma et al., 2009 2009 ). Our present case includes none of those distinguishing features mentioned above. Therefore, we describe it as gastric duplication cyst with foregut differentiation. GDC lined by PCCE is a late onset disease (from 25 to 76 years in age) with little difference between genders (10 women and 8 men). The most common location is on the lesser curvature, near the gastroesophageal junction. It is usually asymptomatic and is discovered incidentally as a gastric wall mass. However, some patients complain of epigastric pain which may be caused by compression on adjacent structures or ulceration due to the ectopic gastric mucosa in the cysts (Kim et al., 2000 2000 ). Malignant change of GDC lined by PCCE is rare. Shibahara et al. ( 2009 2009 ) reported a case of bronchogenic cyst of the stomach located at the opposite side wall to the gastric adenocarcinoma, and attributed the adenocarcinoma to the chronic inflammation from the bronchogenic cyst to the gastric mucosa. Worth mentioning, because of persistent exposure to carcinogenic substances, alimentary duplication cyst should be recognized as a lesion at risk for malignant transformation in patients aged more than 50 years (Fukumoto et al., 2008 2008 ). CT can detect the presence of abdominal mass, but it frequently fails to identify the cystic nature of the GDC lined by PCCE due to the thick, proteinaceous cyst fluid (Lee et al., 2006 2006 ). EUS is helpful in identifying the intramural or extramural relation of the gastrointestinal tract (Eloubeidi et al., 2004 2004 ). Moreover, EUS-guided fine needle aspiration (EUS-FNA) provides cytological diagnosis of the GDC. It is believed that the presence of detached ciliary tufts and absence of neoplastic cells confirm the benign nature of the cyst (Eloubeidi et al., 2004 2004 ). In addition, 99 Tc m -pertechnetate scintigraphy can diagnose ectopic functioning gastric mucosa, which is often associated with complications like ulceration in the GDC (Kumar et al., 2005 2005 ). However, in our case, we misdiagnosed the mass as a GIST, which is much more common than a congenital cyst, and it is not advised to perform biopsy to confirm the diagnosis of a resectable GIST as it can lead to tumor dissemination or hemorrhage (Chaudhry and DeMatteo, 2009 2009 ). Therefore, we failed to make an accurate diagnosis before operation. The recommended management of symptomatic GDC is complete excision of the cyst without violation of the gastric lumen. If this is not possible, segmental gastrectomy is an alternative (Holcomb et al., 1989 1989 ). When malignant change is seen in the cyst, a surgical procedure without rupture of the cyst is required (Shibahara et al., 2009 2009 ). Management of asymptomatic cases remains controversial. Eloubeidi et al. ( 2004 2004 ) suggested watchful waiting after confirming the benign nature of these cysts by EUS-FNA. However, considering the potential for complications and malignant change, we believe, for asymptomatic cases, surgical intervention is warranted in patients aged more than 50 years, especially when ectopic functioning gastric mucosa was detected. Conclusions 1 In summary, GDC lined by PCCE is an uncommon lesion stemming from foregut developmental malformation. Its clinical presentation is usually nonspecific and it is easily misdiagnosed as a GIST radiologically. EUS-FNA can provide cytological diagnosis and confirm the benign nature of the cyst. Although rare, GDC lined by PCCE should be included in the differential diagnosis of gastric wall mass. Surgical intervention is warranted in patients with clinical symptoms, or who are aged more than 50 years. References UI Chaudhry , RP DeMatteo . Management of resectable gastrointestinal stromal tumor . 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