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 ]).
Fig. 1 Endoscopic view of the lesion.
Video 1 Retrograde double-balloon enteroscopy was performed using the water-exchange method.
Fig. 2 Positron emission tomography/computed tomography revealed a lesion with increased
uptake in the pelvic segment of the small intestine.
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 ]).
Fig. 4 HE staining showed an aberrant arrangement of vascular structures, muscular tissues
and nerve bundles.
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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