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Description
A 37-year-old woman presented with vague upper abdominal pain and dyspepsia. Clinical examination was unremarkable. Gastroscopy showed a smooth ‘bulge’ at the junction of the gastric body and fundus along the greater curvature with normal overlying mucosa. Biopsy revealed inflammatory changes but no dysplasia or malignancy. CT scan showed well-defined gastric body wall mass (figure 1). She underwent a wedge resection of the mass with normal gastric margin without regional lymphadenectomy.
CT scan (A, coronal and B, axial views) demonstrating well-defined rounded heterogeneous exophytic mass lesion in the greater curvature of the stomach with no associated lymphadenopathy.
Histopathology showed a well-circumscribed lesion (4.5×3.0 cm) composed of spindled cells arranged in interlacing fascicles, whorls and palisades (figure 2). Very rare mitoses (1/50 HPF) were seen and the overlying mucosa was normal. The appearance was those of gastric autonomic nerve tumour (GANT). This was confirmed by immunohistochemistry. The patient remained well with no evidence of local recurrence or metastases at 5-year follow-up.
High-power histomicrograph showing bland spindle cell nuclei with occasional large hyper chromatic nuclei with very rare mitoses and no necrosis (A). High-power photography, which shows the neoplasm as composed of spindle cells arranged in interlacing fascicles, whorls and palisades (B; H&E stain).
GANTs arise from the autonomic nerve plexuses. They are extremely rare accounting for 1% of all malignant gastrointestinal tumours. They occur at any age, but most commonly in younger patients.1 Histologically, they resemble other gastrointestinal stromal tumours.2 The diagnosis is made on electron microscopy and immunohistochemistry.3 Although considered benign, they are slowly growing with an aggressive clinical course and poor prognosis that leads to death.3 However, like this case, GANT may run a benign course if it exhibits rare mitotic figures. Surgical resection offers the only chance of cure as the response to chemotherapy and radiotherapy is ineffective. However, in spite of radical excision, patients die a few months after surgery with local recurrences and distant metastases to lymph nodes, liver and bones.4
Learning points
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Gastric autonomic nerve tumours are extremely rare that arise from the autonomic nerve plexuses.
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The diagnosis is often made by electron microscopy and immunohistochemistry.
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Although considered benign tumours, gastric autonomic nerve tumour may run an aggressive course with poor prognosis even after radical surgical resection.
Footnotes
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Contributors AA wrote the initial draft and searched the literature. AA-A performed the surgery and participated in drafting the manuscript. A-WNM searched the literature and wrote the final draft.
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Competing interests None.
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Patient consent Obtained.
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Provenance and peer review Not commissioned; externally peer reviewed.