Endoscopy 2022; 54(12): E726-E727
DOI: 10.1055/a-1759-2256
E-Videos

A forethought about obscure gastrointestinal bleeding: an unusual ileal mass

Yujen Tseng
1   Department of Digestive Diseases, Huashan Hospital, Fudan University, Shanghai, China
2   Huashan Rare Disease Center, Huashan Hospital, Fudan University, Shanghai, China
,
Lishuang Lin
3   Department of Pathology, Huashan Hospital, Fudan University, Shanghai, China
,
Tiansheng Huang
4   Department of Digestive Diseases, Shanghai Guanghua Hospital of Integrated Traditional Chinese and Western Medicine, Shanghai University of Traditional Chinese Medicine, Shanghai, China
,
Zhongguang Luo
1   Department of Digestive Diseases, Huashan Hospital, Fudan University, Shanghai, China
› Author Affiliations
Supported by: Huashan Hospital http://dx.doi.org/10.13039/501100010120 2020QD008

The patient was a 32-year-old woman admitted for hematochezia, with a hemoglobin of 67 g/L and fecal occult blood test of 4+. Emergency gastroscopy and colonoscopy were unremarkable. A double balloon enteroscopy revealed a lobulated ileal mass located 70 cm proximal from the ileocecal valve ([Fig. 1], [Video 1]). Positron emission tomography/computed tomography (PET/CT) showed that the lesion was located in the pelvic segment of the small intestine, with a standardized uptake value index of 3.6 ([Fig. 2]). Surgery was recommended owing to the patient’s low hemoglobin levels and risk of recurrent gastrointestinal bleeding. A gross specimen revealed a lobulated, polypoid mass in the ileum, measuring up to 1.5 cm × 0.8 cm ([Fig. 3]).

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Fig. 1 Endoscopic view of the lesion.

Video 1 Retrograde double-balloon enteroscopy was performed using the water-exchange method.


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Fig. 2 Positron emission tomography/computed tomography revealed a lesion with increased uptake in the pelvic segment of the small intestine.
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Fig. 3 Macroscopic view of the lesion after surgical resection.

A final diagnosis of neuromuscular and vascular hamartoma of the small intestine was made. Postoperative pathology revealed a mixed component of blood vessels, nerve fibers, and smooth muscle consistent with that of a hamartoma ([Fig. 4]). The haphazard arrangement of the vascular structures and muscular tissues were confirmed by CD31 and desmin staining, respectively. Aberrant nerve bundles and ganglion cells were highlighted by S100 staining ([Fig. 5]).

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Fig. 4 HE staining showed an aberrant arrangement of vascular structures, muscular tissues and nerve bundles.
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Fig. 5 Immunohistochemistry confirmed the presence of vascular, muscular and nerve tissues

Neuromuscular and vascular hamartoma is a rare gastrointestinal lesion first described in 1982 by Fernando and McGovern [1]. Clinical symptoms can be non-specific and can range from chronic abdominal pain and intermittent intestinal obstruction to gastrointestinal bleeding. This condition can occur as single or multiple strictures or a polypoid mass. It is mainly composed of disorganized fascicles of blood vessels, smooth muscle, and bundles of non-myelinated nerve fibers with scattered abnormal ganglion cells, and occurs focally within a segment of the small intestine [2]. Given that similar histological features seen in cryptogenic multifocal ulcerous stenosing enteritis or diaphragm disease of the small bowel, the hamartomatous nature of neuromuscular and vascular hamartoma has been argued [3]. However, our case demonstrated a rare single, lobulated polypoid lesion of the small intestine with histological features consistent with neuromuscular and vascular hamartoma and cannot be attributed to other reactive diseases.

Endoscopy_UCTN_Code_TTT_1AP_2AD

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Publication History

Article published online:
10 March 2022

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  • References

  • 1 Fernando S, Mcgovern V. Neuromuscular and vascular hamartoma of small bowel. Gut 1982; 23: 1008-1012
  • 2 Hemmings C. Neuromuscular and vascular hamartoma arising in a Meckelʼs diverticulum. Pathology 2006; 38: 173-174
  • 3 Setaffy L, Osuna M, Plieschnegger W. et al. Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE), and neuromuscular and vascular hamartoma (NMVH): two sides of the same coin?. Endoscopy 2015; 47: 345-348