Glomus tumors usually occur in the acral soft tissue and rarely in visceral locations, such as the stomach, intestines, mediastinum, lung, pancreas, bladder, and vagina. The authors present a 74-year-old woman with an exceptionally large glomus tumor of the mesentrium with malignant features. Previously reported cases of intraabdominal glomus tumor in the abdominal cavity exhibited benign behavior and few cases with metastatic disease. Criteria for malignancy in acral glomus tumors, such as unusually large size, infiltrative growth, necrosis, nuclear atypia, and mitotic activity, seem not to translate to abdominal glomus tumors. As very few intraabdominal glomus are described, the malignant potential of these tumors stays uncertain for longer period.

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