Glomus body (glomus in Latin means ball) is a specific structure in the dermis part of the skin regulating the body's temperature and blood pressure. It consists of a peripheral capsule, afferent arterioles, collecting venules, arteriovenous anastomoses known as Sucquet-Hoyer canal, nerve fibers, and modified smooth muscle cells named glomus cells [figure 5](2, 12). The pathologic proliferation of the glomus cells either due to an underlying hamartoma or as a reaction to previous trauma may lead to GT formation (13).
The GTs frequently (90%) present solitary. Multiple GT presentation known as glomangiomatosis is seen mostly in children and is associated with either autosomal dominant inherited mutation in chromosome 1 or neurofibromatosis type 1(2, 11). The lesions in glomangiomatosis may not be painful, unlike isolated glomus tumor cases (14).
The average presenting age of GT is usually between 30 to 50 years old and the tumor starts to become atrophied after the age of 60(11, 15). Its prevalence in both genders is nearly similar(16). However the GT is more likely to be extra-digital in male patients(1, 17).
The GTs can be found in any location, even in visceral organs. There are reported cases of GT affecting breast (18), thyroid(6, 10), heart(7, 19), trachea(2, 20), lung(8), mediastinum(21, 22), esophagus(5, 23), stomach(24, 25), liver(26), small intestine(9) and colon(27). Extra-digital cases may be missed frequently as a result of affecting less common sites and their atypical presentation. Similarly, our GT case involving the chest wall at the mid-axillary line had long been missed and maltreated. To the best of our knowledge, only 7 cases of GT with chest wall involvement have been reported in the literature [Table 1]. Most reported chest-wall GT, interestingly, are from the east and southeast Asia, suggesting a possible role of genetics in this specific variety of the tumor.
Table 1
Reported cases of chest wall glomus tumor in the literature.
Study
|
Patient’s Gender
|
Patient’s Age
(Y/O)
|
Duration of symptoms
(Y)
|
Location of mass
|
Relapse
|
Temiz et al(31)
|
Not reported
|
Not reported
|
Not reported
|
Subcutaneous
Sternum
|
No
|
Kambhampati et al(14)
|
Male
|
47
|
2
|
Subcutaneous
Left anterior chest wall
|
No
|
Neelaiah et al(33)
|
Male
|
46
|
2
|
Subcutaneous
Anterior chest wall
|
Not reported
|
Tsuruta et al(34)
|
Female
|
19
|
5
|
Dosal side of pectoralis major
Right anterior chest wall
|
No
|
Yim et al(35)
|
Male
|
41
|
Not reported
|
Deep in the chest wall muscles
Right lateral chest wall
|
Yes
|
Uchiyama et al(36)
|
male
|
50
|
10
|
Right 3rd intercostal space
|
No
|
Schneller J(37)
|
Male
|
30
|
10
|
Multifocal in Intercostal spaces
Left posterior chest wall(largest one)
|
Not reported
|
Van Geertruyden et al. demonstrated that the average time between the beginning of GT’s symptoms and its diagnosis is ten years(28). This time was 15 years in our patient.
History of paroxysmal pain, which appears spontaneously or following direct compression and exposure to cold temperature, is the main clue to reach the diagnosis. (11). Upon physical examination, Love's test and Hildreth ischemia test are helpful(29). In Love's test, pain ensues from blunt point pressure on the lesion, and in the Ischemia test, pain is relieved following tourniquet inflation. Love's test has up to 100 percent sensitivity but is not specific in diagnosing the lesion. However, the Hildreth test has up to 92 percent sensitivity, and up to 100 percent reported specificity in diagnosing the GT(11, 30).
Plain radiography generally does not show any abnormality unless in chronic lesions, in which bony erosion may be seen. Although Ultrasonography can help in the diagnose, the imaging modality of choice is MRI(15). Like other vascular tumors, typical GTs have low signal on T1 weighted MRI and high signal on T2 weighted and T1 post-gadolinium MRI views (16). Definitive diagnosis can be established only after histopathologic studies. Upon histologic evaluation of GT, nests of epithelioid cells with eosinophilic cytoplasm and round nuclei are detected. Immunohistochemistry distinguishes the GT from many differential diagnoses like schwannoma as GT's immunohistochemistry is positive for smooth muscle actin and vimentin and negative for neuroendocrine markers (e.g., S100, chromogranin, and synaptophysin)(2).
Surgical excision remains the management of choice with a 10–30% recurrence rate in the literature (11). Laser ablation and sclerotherapy are demonstrated to have encouraging results in some cases, such as small and multiple lesions (31).
The GT mostly has a benign nature, and it has less than 1 percent risk of transformation into malignancy (11). However, deep tumor location, diameter more than 2cm, atypical mitotic figures, and high nuclear grade with more than five mitotic figures per 50 high power fields are proposed to hint toward a possible malignant variety (i.e., glomangiosarcoma)(2, 32). Depending on the predominant tumor cells, whether vascular, smooth muscle cells, and glomus cells, the benign lesions may be classified as glomangioma, glomangiomyoma, and glomus tumor proper, respectively(2, 16).
In our case, the histopathologic findings were in favor of glomangioma, and no significant nuclear atypia or mitosis was identified. However, the size and deep location of the tumor overlapped with the malignancy criteria. Close observation of the patient and 19-month follow-up has shown neither metastasis nor relapse.
In conclusion, a glomus tumor may affect any part of the human body and be associated with significant functional impairment. Meanwhile, the tumor is curable with surgical excision in most cases. Therefore, early correct diagnosis is of paramount importance. A high index of suspicion would be needed in order not to miss GT cases.