Glomus tumors are rare and the hand is classically the most commonly affected site. The present study performed a literature review on the topic and reported on an unusual case of neck glomangiomyoma in a female adult patient. A 31-year-old woman presented with a 4-year history of a growing submandibular tumor with localized non-irradiated pain. A previous fine needle aspiration biopsy had suggested a glomus tumor, and immunohistochemical analysis showed positive staining for smooth muscle actin, H-caldesmon, muscle-specific actin and collagen type IV. The MRI revealed a well-defined nodular lesion measuring 38x28x33 mm. The patient underwent surgery with no complications. After the histopathological examination and immunohistochemical staining, a diagnosis of glomangiomyoma was established. Glomangiomyomas are extremely rare but should be considered in the differential diagnosis of an adult presenting with a neck mass and localized tenderness, especially if no other risk factors for head and neck tumors are present.
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