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GE-Portuguese Journal of Gastroenterology

versão impressa ISSN 2341-4545versão On-line ISSN 2387-1954

GE Port J Gastroenterol vol.28 no.5 Lisboa out. 2021  Epub 20-Mar-2022

https://doi.org/10.1159/000511808 

Images in Gastroenterology and Hepatology

Ball-Valve Syndrome, an Unusual Presentation of a GIST

Síndrome de ball-valve, uma apresentação incomum de GIST

Maria Margarida Carvalhoa 

Ana Margarida Laranjoa 

Andreia Reia 

Lurdes Gonçalvesa 

Carlos Quintanab 

Isabel Medeirosa 

aGastroenterology Department, Hospital Espírito Santo - Évora, Évora, Portugal

bAnatomopathology Department, Hospital Espírito Santo - Évora, Évora, Portugal


Keywords Gastrointestinal stromal tumor; Gastric outlet obstruction; Ball-valve syndrome; Scarf-ring sign

Palavras Chave Tumor do estroma gastrointestinal; Obstrução ao esvaziamento gástrico; Síndrome de ball-valve; Sinal do cachecol

We present the case of an 87-year-old woman admitted to the emergency room due to persistent vomiting in the previous 4 days. She also complained of an intermittent abdominal pain and a weight loss of 10 kg with months of evolution. On physical examination, she had audible bowel sounds, painful palpation in the epigastrium without signs of peritoneal irritation. The laboratory panel showed only isolated neutrophilia, and the abdominal X-ray had no evidence of bowel distension.

The upper gastrointestinal endoscopy showed a distended gastric body and complete torsion of the gastric folds in the antropyloric region (“scarf-ring sign” shown in Fig. 1) causing obstruction to the passage of the endoscope.

Fig. 1 Initial endoscopic image of complete torsion and invagination of gastric folds in the antropyloric region, compatible with the “scarf-ring sign.” 

After several attempts of aspiration, lavage and torque, it was possible to completely revert the torsion and reveal a bulky, semipediculated polypoid lesion with a large base of implantation on the anterior face of the distal body. Its surface was covered by congestive gastric mucosa with central erosion, suggestive of a submucosal lesion (Fig. 2, 3).

Fig. 2 After endoscopic resolution of gastric torsion, a bulky semipediculated polypoid lesion was observed. 

Fig. 3 Polypoid lesion with surface covered by congestive gastric mucosa with central erosion, suggestive of a submucosal lesion. 

Following the endoscopy, the patient tolerated progressive diet with no recurrence of vomiting. She was discharged and referred to a surgical consultation. The patient later underwent a laparoscopic atypical gastrectomy. The pathological specimen was compatible with a 4 × 3.2 cm gastrointestinal stromal tumor (GIST), with a low mitotic index (1/50 HPF), suggestive of a low-risk tumor by the modified National Institute of Health classification [1] (Fig. 4).

Fig. 4 Histology of the resected specimen with immunohistochemical study revealed CD117 positivity. Magnification ×20. 

“Ball-valve syndrome” is a condition caused by intermittent prolapse through the pylorus of a gastric lesion, first described in 1946 by Hobbs and Cohen [2]. The clinical manifestations are variable according to the degree of obstruction, ranging from abdominal discomfort or intermittent abdominal pain, nausea, vomiting and loss of appetite. The diagnosis is supported by imaging and/or endoscopic findings. The observation of a “scarf-ring sign” on endoscopy is a valuable clue to the diagnosis [3]. This is a rare GIST presentation, with only 18 cases previously reported in the literature [4].

References

1 Joensuu H. Risk stratification of patients diagnosed with gastrointestinal stromal tumor. Hum Pathol. 2008 Oct;39(10):1411-9. [ Links ]

2 Hobbs WH, Cohen SE. Gastroduodenal invagination due to a submucous lipoma of the stomach. Am J Surg. 1946 Apr;71(4):505-18. [ Links ]

3 Yazumi S, Nakase H, Matsushima Y, Chiba T, Shirahase I, Yoshizaki S, et al. The “scarf-ring sign” of ball valve syndrome. Gastrointest Endosc. 2002 Apr;55(4):560. [ Links ]

4 Đokić M, Novak J, Petrič M, Ranković B, Štabuc M, Trotovšek B. Case report and literature review: patient with gastroduodenal intussusception due to the gastrointestinal stromal tumor of the lesser curvature of the gastric body. BMC Surg. 2019 Oct;19(1):158. [ Links ]

Statement of Ethics Informed consent was obtained from the patient for the case publication

Funding Sources The authors have no funding sources to declare

Received: June 07, 2020; Accepted: August 15, 2020

Corresponding author Maria Margarida Água Morna Braga de Carvalho Gastroenterology Department, Hospital Espírito Santo - Évora Largo Senhor da Pobreza PT-7000-811 Évora (Portugal) mmargaridadecarvalho@gmail.com

Conflict of Interest Statement

The authors have no conflicts of interest to declare

Author Contributions

Dr. Maria Margarida Carvalho wrote the paper. Dr. Carlos Quintana made and provided histological images. Dr. Ana Laranjo and Dr. Andreia Rei revised the paper. Dr. Lurdes Gonçalves and Dr. Isabel Medeiros revised the paper and approved the final version

Creative Commons License This is an open-access article distributed under the terms of the Creative Commons Attribution License