Multiple Glomus Tumors: An Unusual Presentation of Chronic Regional Pain

Abstract

To the Editor: Multiple glomus tumors are extremely rare and can differ from the more common solitary glomus tumors in their clinical presentation and histological features. A 63-yr-old man presented with an exquisitely painful nodule in his right supraclavicular fossa that had clinically just become apparent. He initially sought help 20 years ago complaining of constant right-sided shoulder and chest discomfort worsened by movement of his arm. This morbidity had a significant effect upon both his social and professional life. He reported that intercourse with his wife had become impossible; he had been made redundant; was depressed and expressed suicidal tendencies. Despite multiple hospital admissions, no cause for his pain had been found and he had been under review by the pain team for 10 years. Unsuccessful treatments included simple analgesia, carbamazepine, diazepam, thoracic epidurals, transcutaneous electrical nerve stimulation, acupuncture, and increasingly large doses of morphine. On presentation of the nodule, further investigation via whole body CT and MRI revealed multiple tumors within the right supraclavicular fossa, posterior triangle, axilla, right paravertebral gutter, apex of the right lung posterior to the parietal pleura, and in the vicinity of the right lobe of the thyroid (Fig. 1). Excision of this supraclavicular mass, confirmed a glomus tumor 22 mm in diameter located in the subcutaneous fat. Following this histological diagnosis, the patient underwent five additional surgical procedures to remove all identified tumors. In all cases, glomus tumor was excised, the largest measuring 45 mm in diameter in the axilla. Several had an infiltrative margin; however, none showed significant atypia and mitoses were scarce (2 per 50 high power fields).Figure 1.: MRI scan showing multiple glomus tumors. (a, b) Glomus tumors of the supraclavicular fossa, and perithyroid lymph node. (c) Glomus tumor of the axilla. (d) Glomus tumor of the paravertebral gutter.Complete surgical excision of these tumors resulted in alleviation of the patients’ symptoms. Twenty-five years after the onset of his symptoms he is now pain free and is resuming a normal lifestyle. A glomus tumor arises from the modified smooth muscle cells of the glomus body, a neuromyoarterial receptor that is sensitive to variations in temperature and regulates arteriolar flow (1). Glomus bodies, normally located in the subcutis or dermis, are most commonly found in the distal portion of the digits, especially in the subungal region. These masses are exquisitely painful to touch and pressure. Infrequently, atypical glomus tumors have been reported to involve other sites, including muscles, joints, head and neck, stomach, penis, and rarely the mediastinum (2,3). Multiple glomus tumors often differ from the more common solitary nodules in their atypical clinical presentation and histological features. This is the first case where tumors were seen to involve the neck, axilla, and mediastinum in a synchronous unilateral distribution. Malignant glomus tumors are exceeding rare and account for <1% of glomus tumors. There have only been a few cases reported of documented metastases (4). Because of location, size, and histological features, it may be that the apparent multiplicity of tumors in this case represents locoregional metastasis. Surgical excision is the treatment of choice, although radiotherapy has been advocated as a primary or adjunctive modality (5,6). This patient had a 20-yr history of pain before a surgical cure was forthcoming. With the correct diagnosis and treatment he remains pain free. E. J. Longdon, BM BS, BmedSci, MRCS T. A. McCulloch, BM BS, MRCP, FRCPath A. G. B. Perks, MB BS, FRCS, FRACS C. S. Ubhi, MB ChB, FRCS W. E. Morgan, MB ChB, FRCS Nottingham City Hospital Nottingham, United Kingdom [email protected]