Glomus tumor of the esophagus

Abstract

Here we report a case of primary glomus tumor of the esophagus, which is extremely rare, and review the corresponding literature. A 69-year-old man underwent screening endoscopy during a medical checkup and a submucosal tumor was incidentally found in the midthoracic esophagus. Endoscopic ultrasonography revealed that the tumor originated from the muscle layer of the esophagus. Computed tomography and 18 F-fluorodeoxyglucose positron emission tomography revealed neither lymphadenopathy nor distant metastasis. Endoscopic ultrasonography-guided fine needle aspiration biopsy indicated epithelioid mesenchymal tumor. Immunohistochemically, the tumor cells were positive for alpha smooth muscle actin, and focally positive for Discovered on GIST1 but negative for c-kit and protein kinase C theta, suggesting that the lesion was a KIT-negative epithelioid gastrointestinal stromal tumor or glomus tumor. The tumor was successfully enucleated through right thoracotomy. Pathological examination confirmed that the tumor was a glomus tumor of the esophagus. The patient remains healthy without any recurrence to date one year after surgery.

Malignant glomus tumor with oncocytic features: an unusual presentation of dysphagia

“…The vast majority of glomus tumors localized in subcutaneous tissues are benign, but other unusual sites carry uncertainty regarding their biologic behaviors. Two cases from the literature described extremely large glomus tumors with > 5 mitosis/50 HPF; based on the tumors' deep locations, they were considered malignant glomus tumors according to the classification of Folpe et al. .…”

“…Miettinen (5) reviewed 32 cases of glomus tumors in the gastrointestinal tract, most of which were located in the stomach (n:31) and intestines (n:1). Fewer than 10 cases of glomus tumors in the esophagus have been described in the literature (11)(12)(13)(14)(15)(16)(17).…”

“…In the literature of case reports describing glomus tumors located at the esophagus, the patients consisted of four women and three men, ranging in age from 28 to 80 years (mean, 56 years) (11)(12)(13)(14)(15)(16)(17). The clinicopathologic findings of these cases are summarized in Table 1.…”

“…The full text of these seven case reports accounts for five patients, with two assumed to have also benefited based on the abstract. Three patients presented with dysphagia and weight loss; whereas, the remaining four patients were detected during an incidental endoscopy (11)(12)(13)(14)(15)(16)(17). In one case, emesis, regurgitation, and bloody stool were present in addition to dysphagia and weight loss (15).…”

“…If all of the cases in the literature are considered, presenting symptoms occurred when the tumor diameter rose above 30 mm. Nuclear atypia was present in four cases, and the mitotic activity was between 0 to 10/50 HPF (11)(12)(13)(14)(15)(16)(17). One patient had lymph node metastasis; in this case, the diameter of the tumor was 58 mm, and the mitotic activity was 10/50 HPF (13).…”