Malignant glomus tumor of the lung with multiorgan metastases: case report and literature review

Abstract

Glomus tumor is an exceedingly rare neoplasm that is derived from cells of the neuromyoarterial glomus or glomus body. It rarely occurs in the visceral organs where glomus body may be sparse or even absent, such as the stomach, intestines, mediastinum, and respiratory tract. It is unusual for a glomus tumor to demonstrate atypical or malignant histopathological characteristics. It is also rare for such a tumor to express clinically aggressive behavior. However, when metastasis does occur, this disease is often fatal. We herein report an interesting case of a middle-age woman admitted due to progressive cough and hemoptysis. A polypoid mass was found to occlude the left lingular lobar bronchus. Final histopathologic examination showed the presence of malignant glomus tumor, confirmed by immunoreactivity for smooth muscle actin and vimentin. Two months later, the patient developed abdominal distension and gastrointestinal bleeding. Further evaluation lead to the discovery of widespread metastatic disease to the gastrointestinal tract, spleen, and the left adrenal gland. We further entail a review of the literature on the clinicopathologic features and diagnosis of this uncommon tumor.

Primary pancreatic glomus tumor invading into the superior mesenteric vein: a case report

“…However, glomus tumors could also occur in regions where glomus bodies do not normally exist [ 1 ]. A small number of case reports have illustrated visceral glomus tumors involving the stomach [ 5 – 7 ], small intestine [ 8 , 9 ] and lung [ 10 ]. Miliauskas et al .…”

“…Regarding biological behavior, the great majority of glomus tumors are benign. However, approximately 1% of glomus tumors are reported to be malignant [ 1 , 10 ]. In our present patient, the tumor had aggressive invasion into the SMV, penetrating the venous wall and even exhibiting intraluminal tumor growth.…”

Primary pulmonary glomus tumor of uncertain malignant potential: A case report with literature review focusing on current concepts of malignancy grade estimation

“…The vast majority of glomus tumors occur in the dermis and subcutis of the extremities, with the single most common site of the subungual region of the finger; however, rare tumors have been reported in visceral organs [1] , [2] . Primary glomus tumors of the lung are exceedingly rare, with only 36 previously reported cases in the English literature ( Table 1 ) [5] , [7] , [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] , [16] , [17] , [18] , [19] , [20] , [21] , [22] , [23] , [24] , [25] , [26] , [27] , [28] , [29] , [30] , [31] , [32] , [33] , [34] , [35] , [36] . Almost the same number of cases with primary tracheal glomus tumors have also been reported to date [37] .…”

“…However, glomus tumors showing histological atypia have been recognized, and furthermore, fatal cases with distant metastases are occasionally reported, comprising less than 1% of all glomus tumors [3] . For primary bronchopulmonary glomus tumors, 10 cases with malignancy have been previously reported [5] , [10] , [13] , [26] , [30] , [32] , [33] , [35] , [36] . Criteria for the diagnosis of malignancy in glomus tumors was proposed by Folpe et al.…”

Low-Grade Malignancy Glomus Tumor in a Setting of Multiple Glomus Tumors – Case Report

“…Glomus tumors (GTs) comprise less than 1,5-2% of soft tissue tumors, and less than 10% of them consist of multiple GTs (MGTs) [1[, [2], [3]. Solitary GT most frequently appear as small nodules in specific locations such as the subungual regions of digits or the deep dermis of the palm, wrist, forearm, and foot.…”