The Journal of Foot & Ankle Surgery xxx (2014) 1–3

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Case Reports and Series

Subungual Glomus Cell Proliferation in the Toe: A Case Report Jeong Ho Seo, MD 1, Ho Seong Lee, MD, PhD 2, Sang Woo Kim, MD 3, Jae Jung Jeong, MD 4, Young Rak Choi, MD 5 1

Clinical Fellow, Department of Orthopedic Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea Professor, Department of Orthopedic Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea 3 Assistant Professor, Department of Orthopaedic Surgery, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, South Korea 4 Assistant Professor, Department of Orthopaedic Surgery, Daejeon St Mary’s Hospital, Catholic University of Korea College of Medicine, Daejeon, South Korea 5 Assistant Professor, Department of Orthopaedic Surgery, CHA Bundang Medical Center, CHA University College of Medicine, Seongnam-Si, Gyeonggi-do, South Korea 2

a r t i c l e i n f o

a b s t r a c t

Level of Clinical Evidence: 4

Glomus tumors are rare benign neoplasms. They typically present with a triad of severe pain, point tenderness, and cold sensitivity. Most glomus tumors will form well-circumscribed nodules, and excisional biopsy of masses detected by ultrasonography or magnetic resonance imaging of the lesion can relieve the symptoms. We describe a 30-year-old female patient with nontumorous glomus cell proliferation with clinical symptoms similar to those of glomus tumors. The lesion was treated by exploration and excision, with a satisfactory clinical outcome. Ó 2014 by the American College of Foot and Ankle Surgeons. All rights reserved.

Keywords: biopsy gadolinium glomus body nontumorous proliferation Suquet-Hoyer canal tumor ultrasonography

Glomus tumors are rare benign neoplasms derived from the normal glomus body. These tumors account for less than 1.6% of soft tissue tumors, and approximately 1% of the tumors will be in the hands (1,2). Glomus tumors typically present with a triad of severe pain, point tenderness, and cold sensitivity (3). Most glomus tumors will form well-circumscribed nodules, and excisional biopsy of masses detected by ultrasonography or magnetic resonance imaging (MRI) of the lesion can relieve the symptoms. We describe a patient with nontumorous glomus cell proliferation with clinical symptoms similar to those of glomus tumors. Case Report A 30-year-old female patient presented with pain in her right hallux. She had received pharmacologic treatment and physiotherapy at several different private clinics but had had no improvement in her symptoms. Physical examination of the right hallux showed diffuse tenderness on the toenail but no evidence of nail color changes or hypersensitivity to cold. Financial Disclosure: None reported. Conflict of Interest: None reported. Address correspondence to: Ho Seong Lee, MD, PhD, Department of Orthopaedic Surgery, Asan Medical Center, 388-1 Pungnap-2 Dong, Songpa-Gu, Seoul 138-736, South Korea. E-mail address: [email protected] (H.S. Lee).

The radiologic examination showed no abnormalities; however, because the patient had presented with clinical symptoms similar to those of a glomus tumor, she was assessed by MRI. T1-weighted MRI showed diffuse hypointense signals in the nail bed, and T2-weighted MRI revealed hyperintense signals. However, no typical signs of glomus tumors, such as the presence of nodules, were present, leading to a radiologic diagnosis of the lesion as inflammation or edema of the nail bed (Fig. 1). The patient was treated with nonsteroidal anti-inflammatory drugs, analgesics and tricyclic antidepressants for 1 year; however, the symptoms did not improve. Because we could not rule out the presence of a glomus tumor, we explored the nail bed to ensure the diagnosis was accurate. After digital block anesthesia and elevation of the nail, we observed blue-reddish color changes in the nail bed. Edematous white-pinkish soft tissue without a definite border was also noted in a superficial location and was excised and examined (Fig. 2). Histologic examination of the specimen showed that the mass measured 3 mm by 2 mm at the greatest dimensions, with a circular proliferation of rounded cells with rounded nuclei around the blood vessels, findings characteristic of glomus cells. Because the numbers of glomus cells were not large enough to be characterized as nodules, we could not diagnose a classic glomus tumor. In addition, no evidence of necrosis, high mitotic activity, or nuclear atypia was found; therefore, the mass did not fulfill the criteria for a glomus tumor with atypical features as defined by Folpe et al (17). Therefore, the only

1067-2516/$ - see front matter Ó 2014 by the American College of Foot and Ankle Surgeons. All rights reserved. http://dx.doi.org/10.1053/j.jfas.2014.03.004

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Fig. 1. Magnetic resonance imaging (MRI) of the hallux, showing (A) an area of high signal intensity on T2-weighted MRI, (B) an area of low signal intensity on T1-weighted MRI, and (C) a diffuse, enhanced pattern of the nail bed lesion (arrow) on T1-weighted gadolinium-enhanced images.

confirmed characteristic of the mass was the presence of glomus cell proliferation (Fig. 3). After the procedure, the patient experienced no further pain in the right hallux. Follow-up examinations during the 3 years after the procedure showed no signs of recurrence. Discussion Glomus tumors are neoplasms of the normal glomus body, located in the subcutaneous tissue (5). A glomus body is composed of an afferent arteriole, an anastomotic Suquet-Hoyer canal, an efferent venule, actincontaining glomus cells surrounding the canals, the intraglomerular reticulum, and a capsular portion (6). The normal glomus body is a contractile neuromyoarterial receptor that controls blood pressure and temperature by regulating the peripheral blood flow (5). Wood (7) first described the clinical symptoms of glomus tumors in 1812 as a “painful subcutaneous tubercle.” In 1924, Masson (8) described the pathologic features as tumors derived from the subungual tissue of the fingers and named it “glomus tumor.” Compared with the adventitious formation of the veins, glomus tumors tend to be nodules (9). About 75% of glomus tumors will arise in the hands, with 75% to 90% of these in subungual locations (3). Glomus tumors have been categorized into solitary and multiple types. Most have been solitary, with up to 25% the multiple type (10,11). Solitary glomus tumors will be encapsulated, will usually be found in subungual locations, and will contain numerous small vascular lamina (6). In contrast, multiple type tumors will be unencapsulated, will rarely be subungual, will have larger shaped vascular spaces, and will often be asymptomatic (12). Glomus tumors have been more common in females than in males and have been the most prevalent during the fourth decade of life (3,13).

Carroll and Berman (3) have defined the classic triad of symptoms of glomus tumors as severe pain, point tenderness, and cold sensitivity, and these symptoms form the key to the diagnosis. However, not all glomus tumors will present with this classic triad of symptoms. Van Geertruyden et al (14) have reported that in patients with solid glomus tumors, pain, point tenderness, and cold sensitivity will occur at a rate of 80%, 100%, and 63%, respectively. Our patient had pain and diffuse tenderness across the nail bed but did not present with cold sensitivity. Histologically, glomus tumors will typically consist of 3 components: glomus cells, vessels, and smooth muscle cells. Glomus tumors can be subcategorized as solid glomus tumors, glomangiomas, and glomangiomyomas, depending on the proportion of these 3 components. Solid glomus tumors are well-circumscribed lesions consisting of small amounts of smooth muscle and tight convolutes of capillary size vessels that are surrounded by glomus cells set in a hyalinized or myxoid stroma (15,16). Glomus tumors were once thought to be hyperplastic or hamartomatous lesions; however, they are now regarded as true neoplasms arising from the glomus body (16). The patient we have described had a lesion that resembled a solid glomus tumor that was not well circumscribed. The proliferation of cells did not lead to the formation of nodules, and, therefore, the lesion did not present with typical tumorous characteristics. No previous reports have been published regarding whether this type of glomus cell proliferation represents a transition state before glomus tumor formation or is a separate variant. Malignant glomus tumors comprise approximately 1% of all glomus tumors (17). Folpe et al (4) defined the histopathologic criteria to identify malignant glomus tumors, including a large size (greater than 2 cm) and deep location, a moderate-to-high nuclear grade, and

Fig. 2. Intraoperative findings showing (A) blue-reddish discoloration of the nail bed after nail elevation (arrow) and (B) edematous white-pinkish soft tissue without defined borders.

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nodules on MRI could have underlying nontumorous glomus cell proliferation. Because the procedure is relatively simple, exploration and excision of the tissue in question can aid in the diagnosis and improve the clinical symptoms. In conclusion, we observed nontumorous glomus cell proliferation in a patient who presented with symptoms similar to those of glomus tumors. The MRI findings, unlike typical nodules, were of diffuse signal changes similar to an edematous nail bed in our patient. The lesion was treated by exploration and excision, with a good clinical outcome. References

Fig. 3. Histologic appearance of the glomus tumor showing glomus cell proliferation surrounding the vessels (arrows). Tumorous changes such as capsules or nodules were not observed (hematoxylin and eosin stain, original magnification 200).

an increased mitotic rate (greater than 5:50 high power fields) or the presence of atypical mitotic figures. Glomus tumors of uncertain malignant potential will have high mitotic activity and a superficial location, a large size only, or a deep location only. In contrast, symplastic glomus tumors will only have high nuclear grades. Because the lesion in our patient showed no evidence of necrosis, high mitotic activity, or nuclear atypia, it could not be categorized as a glomus tumor with atypical features. Glomus tumors are diagnosed by their clinical symptoms, with confirmation by ultrasonography or MRI. MRI can be useful because not all glomus tumors will present with the classic triad of symptoms (18). Glomus tumors will typically be seen as small, wellcircumscribed nodules on MRI, presenting as hypointense signals on T1-weighted MRI, hyperintense signals on T2-weighted MRI, and with strong enhancement on gadolinium-enhanced T1-weighted images (19,20). However, some glomus tumors will not show any abnormalities on MRI; therefore, MRI cannot be used to identify all glomus tumors. Dahlin et al (19) have reported the case of a patient who presented with the classic triad of symptoms (pain, point tenderness, cold sensitivity) but with normal ultrasound and MRI findings. However, the lesion was confirmed to be a glomus tumor by the biopsy findings. Because our patient presented with atypical clinical symptoms, we performed MRI to confirm the diagnosis. Although the MRI scan showed changes in signal intensity, it did not show nodules or tumorous lesions, making it difficult to form a definite diagnosis. Patients with clinical symptoms of glomus tumors but without

1. Soule EH, Ghormley RK, Bulbulian AH. Scientific exhibits: primary tumors of the soft tissues of the extremities exclusive of epithelial tumors: an analysis of five hundred consecutive cases. AMA Arch Surg 70:462–474, 1955. 2. Tuncali D, Yilmaz AC, Terzioglu A, Aslan G. Multiple occurrences of different histologic types of the glomus tumor. J Hand Surg 30:161–164, 2005. 3. Carroll RE, Berman AT. Glomus tumors of the hand: review of the literature and report on twenty-eight cases. J Bone Joint Surg Am 54:691–703, 1972. 4. Folpe AL, Fanburg-Smith JC, Miettinen M, Weiss SW. Atypical and malignant glomus tumors: analysis of 52 cases, with a proposal for the reclassification of glomus tumors. Am J Surg Pathol 25:1–12, 2001. 5. Sorene ED, Goodwin DR. Magnetic resonance imaging of a tiny glomus tumour of the fingertip: a case report. Scand J Plast Reconstr Surg Hand Surg 35:429–431, 2001. 6. Drape JL, Idy-Peretti I, Goettmann S, Wolfram-Gabel R, Dion E, Grossin M, Benacerraf R, Guerin-Surville H, Bittoun J. Subungual glomus tumors: evaluation with MR imaging. Radiology 195:507–515, 1995. 7. Wood W. On painful subcutaneous tubercle. Edinb Med J 8:283–291, 1812. 8. Masson P. Le Glomus neuromyo-arteriel des Regions tactiles et ses Tumeurs. Lyon Chir 21:257–280, 1924. 9. Anakwenze OA, Parker WL, Schiefer TK, Inwards CY, Spinner RJ, Amadio PC. Clinical features of multiple glomus tumors. Dermatol Surg 34:884–890, 2008. 10. Maxwell GP, Curtis RM, Wilgis EF. Multiple digital glomus tumors. J Hand Surg 4:363–367, 1979. 11. McDermott EM, Weiss AP. Glomus tumors. J Hand Surg 31:1397–1400, 2006. 12. Moor EV, Goldberg I, Westreich M. Multiple glomus tumor: a case report and review of the literature. Ann Plast Surg 43:436–438, 1999. 13. Fleegler EJ, Zeinowicz RJ. Tumors of the perionychium. Hand Clin 6:113–133, 1990. discussion 135, 116. 14. Van Geertruyden J, Lorea P, Goldschmidt D, de Fontaine S, Schuind F, Kinnen L, Ledoux P, Moermans JP. Glomus tumours of the hand: a retrospective study of 51 cases. J Hand Surg 21:257–260, 1996. 15. Fletcher CD, Unni K, Meretens F. Pathology and Genetics of Tumours of the Nervous System, IARC Press, Lyon, France, 2002, pp. 136–137. 16. Weiss SW, Goldblum JR. Enzinger and Weiss’ Soft Tissue Tumors, Mosby Elsevier, St Louis, 2008, p. 754. 17. Khoury T, Balos L, McGrath B, Wong MK, Cheney RT, Tan D. Malignant glomus tumor: a case report and review of literature, focusing on its clinicopathologic features and immunohistochemical profile. Am J Dermatopathol 27:428–431, 2005. 18. Assmus H, Dombert T. [Glomus tumours of the extremities: localisation and operative treatment in 36 cases]. Handchir Mikrochir Plast Chir 34:103–107, 2002. 19. Dahlin LB, Besjakov J, Veress B. A glomus tumour: classic signs without magnetic resonance imaging findings. Scand J Plast Reconstr Surg Hand Surg 39:123–125, 2005. 20. Schiefer TK, Parker WL, Anakwenze OA, Amadio PC, Inwards CY, Spinner RJ. Extradigital glomus tumors: a 20-year experience. Mayo Clin Proc 81:1337–1344, 2006.

Subungual glomus cell proliferation in the toe: a case report.

Glomus tumors are rare benign neoplasms. They typically present with a triad of severe pain, point tenderness, and cold sensitivity. Most glomus tumor...
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