Extradigital Symplastic Glomus Tumor of the Hand: Report of 2 Cases and Literature Review Mauricio Sandoval, MD,* Juan Carrasco-Zuber, MD,* and Sergio Gonzalez, MD† CASE REPORT

Abstract: Glomus tumor is a rather common tumor of the hand, which presents with a classic triad of symptoms of high temperature, sensitivity and pain, and localized tenderness. Most glomus tumors are located in the digits, especially in subungueal areas. However, extradigital tumors are less frequent, and little is known about their clinical and histopathological features. Two cases of extradigital symplastic glomus tumor are presented. An active clinical and pathological awareness to avoid misdiagnosis or delayed diagnosis of this uncommon lesion is highlighted. Key Words: symplastic glomus tumor, extradigital glomus tumor, atypical glomus tumor (Am J Dermatopathol 2015;37:560–562)

INTRODUCTION A glomus body is a neuromyoarterial body found within the reticular dermis that functions as a specialized form of arteriovenous anastomosis. It is composed of an afferent arteriole and an efferent venule with multiple communications. The arterial end, or Sucquet–Hoyer canal, is surrounded by uniform glomus cells. Glomus cells have properties similar to smooth muscle cells, accounting for their contractility.1 The specific clinical features of digital glomus tumors, including their location and the presence of pain, enable about 90% to be easily clinically diagnosed.2 However, extradigital glomus tumors are more difficult to diagnose, resulting in long delayed diagnosis and/or misdiagnosis. Although extradigital glomus tumors have been reported to be more common than previously thought, there have been few detailed studies of their clinicopathological features.3 Based on morphologic appearances, the 2002 World Health Organization classification describes typical glomus tumors, glomangiomatosis, and symplastic and malignant glomus tumors.4 We report two cases of symplastic glomus tumor located on the hand and a brief review of current facts of this kind of tumor.

From the Departments of *Dermatology, and †Pathology, Faculty of Medicine, Pontificia Universidad Catolica de Chile, Santiago, Chile. The authors declare no conflicts of interest. Reprints: Sergio Gonzalez, MD, Department of Pathology, Marcoleta 377, 10 P, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile 01 (e-mail: [email protected]). Copyright © 2014 Wolters Kluwer Health, Inc. All rights reserved.


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Case 1 A 38-year-old previously healthy Indian female (a nun radicated in Chile for 10 years) consulted with an approximately 7-month history of pain in the middle hypothenar eminence of the left hand. The pain was characterized as progressive, localized, well delimited, and exquisite. Clinical examination showed a circumscribed painful area in the hypothenar eminence of left hand about 0.5 mm in diameter without erythema or swelling (Fig. 1). No local or axillar lymphadenopathies were noticed. A soft tissue echotomography was performed. The images showed a solid nodular lesion in the dermohypodermic area without deep extension (Fig. 2). The echotomography did not inform vascular components. The lesion was excised with the clinical diagnosis of foreign body granulomatous reaction. Histopathologic examination disclosed a wellcircumscribed nonencapsulated dermal nodule with a vascular component and epithelioid perivascular cells in extended nests (Fig. 3); nuclear pleomorphism was present and no mitoses were visible (Fig. 4). Immunostaining showed a positive reaction for CD34 in endothelia and a positive reaction for smooth muscle actin and vimentin in the perivascular cells (Fig. 5).

Case 2 This case was seen in consultation by one of the authors (S.G.). A 66-year-old female presented with a nodule on the hand with several months of evolution. The patient related an increase in volume without pain. A partial excision was performed with the clinical diagnosis of nodular tumor. Histopathologic examination disclosed fragments of a nonencapsulated nodule (Fig. 6) with

FIGURE 1. Case 1. Clinical picture showing an ill-defined erythematous area on the hypothenar region. Am J Dermatopathol  Volume 37, Number 7, July 2015

Am J Dermatopathol  Volume 37, Number 7, July 2015

FIGURE 2. Case 1. Echotomography showing a well-demarcated shadow corresponding to a dermal nodule.

FIGURE 3. Case 1. Panoramic view showing a vascularized solid dermal nodular (hematoxylin-eosin, ·80).

FIGURE 4. Case 1. Higher magnification showing mononucleated and multinucleated perivascular cells with moderate nuclear pleomorphism (hematoxylin-eosin, ·40). Copyright  2014 Wolters Kluwer Health, Inc. All rights reserved.

Extradigital Symplastic Glomus Tumor of the Hand

FIGURE 5. Case 1. Intense staining of perivascular cells with actin antibodies (diaminobenzidine, ·80).

FIGURE 6. Case 2. Tumor fragment showing dense cellularity and nuclear pleomorphism (hematoxylin-eosin, ·100).

FIGURE 7. Case 2. Higher magnification showing larger hyperchromatic nuclei without mitoses (hematoxylin-eosin, ·200). www.amjdermatopathology.com |


Am J Dermatopathol  Volume 37, Number 7, July 2015

Sandoval et al

TABLE 1. Summary of Reported Cases of Symplastic Glomus Tumor Author

Patient Sex/Age, yrs





F/52 F/62 F/78 F/83 F/44 F/38 F/66

No No No No No Yes Yes

Total excision Total excision Total excision Total excision Total excision Total excision Partial excision

15 mo 6 mo NR 6 mo NR 12 mo .5 yrs

No No NR No NR No NR

Park et al5 Falleti et al6 Kamarashev et al7 Arsenovic et al8 Chong et al9 Sandoval et al (current case 1) Sandoval et al (current case 2) NR, not reported.

a vascular component and epithelioid perivascular cells; marked nuclear pleomorphism was present but no mitoses were visible (Fig. 7). The original histopathologic diagnosis was rule out of malignant glomus tumor.


current evidence consistently shows no malignant behavior for this variant. This variant should be regarded as a benign tumor. The term atypical glomus tumor should be avoided because it is indistinctly applied to extradigital location and/or histopathologic uncommon features. Symplastic nuclear atypia is a well-recognized phenomenon in glomus tumors and other lesions such as hemangioma, peripheral neural tumors, and others, and recognition of this rare morphologic variant should avoid the mistaken impression of malignancy.8

In our literature revision, all published cases showed actin-positive staining, and 3 cases were also studied for CD34 depicting endothelial positive reaction; 3 cases showed negative proliferation index with Ki-67. Table 1 summarizes all published cases of symplastic glomus tumor. No extradigital variants have been published before. Follow-up in most cases shows no evidence of local recurrence and/or metastasis. Despite that digital-subungueal area is the most frequent localization reported for glomus tumors, these lesions could appear almost in any terminal vascularized location of the human body; they appear sometimes as multiple lesions,10 in internal organs,8,11–14 and can be found in other unusual localizations such as the tongue.12 In the literature, the word atypical, as applied to these lesions, often means both extradigital and histopathologic infrequent features. A small number of purportedly malignant glomus tumors have been described but histologic criteria for malignancy have never been elaborated. In 2001, Folpe et al13 studied 52 unusual glomus tumors previously diagnosed as “atypical” or “malignant” by virtue of nuclear atypia, infiltrative growth, or mitotic activity. They evaluated size, depth, growth pattern, cellularity, nuclear grade, number of mitotic figures per 50 high-power fields, atypical mitotic figures, vascular space involvement, and necrosis to define criteria for malignancy in glomus tumors. Because of their results, they propose 4 patterns of histologically atypical glomus tumors: malignant glomus tumor, symplastic glomus tumor, glomus tumor of uncertain malignant potential, and glomangiomatosis. They reported aggressive biological behavior only in the malignant form with 38% of metastases in their series. A symplastic glomus tumor is a glomus tumor showing high nuclear grade in the absence of any other malignant feature including mitosis, necrosis and invasive margins, and

1. Venkatachalam MA, Greally JG. Fine structure of glomus tumor: similarity of glomus cells to smooth muscle. Cancer. 1969;23:1176–1184. 2. Van Geertruyden J, Lorea P, Goldschmidt D, et al. Glomus tumours of the hand. A retrospective study of 51 cases. J Hand Surg Br. 1996;21: 257–260. 3. Schiefer TK, Parker WL, Anakwenze OA, et al. Extradigital glomus tumors: a 20-year experience. Mayo Clin Proc. 2006;81:1337–1344. 4. Fletcher CDM, Unni KK, Mertens F, eds. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon, France: IARC Press; 2002. 5. Park GH, Kang SM, Lee WJ, et al. Symplastic glomus tumor. Int J Dermatol. 2012;51:1007–1009. 6. Falleti J, Vita G, De Cecio R, et al. Symplastic glomus tumor: report of a challenging lesion with literature review. Pathol Res Pract. 2012;208: 372–375. 7. Kamarashev J, French LE, Dummer R, et al. Symplastic glomus tumor—a rare but distinct benign histological variant with analogy to other “ancient” benign skin neoplasms. J Cutan Pathol. 2009;36:1099–1102. 8. Arsenovic N, Ramaiya A, Moreira R. Symplastic glomangioma: information review and addition of a new case. Int J Surg Pathol. 2011;19:499–501. 9. Chong Y, Eom M, Min HJ, et al. Symplastic glomus tumor: a case report. Am J Dermatopathol. 2009;31:71–73. 10. Anakwenze OA, Parker WL, Schiefer TK, et al. Clinical features of multiple glomus tumors. Dermatol Surg. 2008;34:884–890. 11. Jaiswal VR, Champine JG, Sharma S, et al. Primary glomangioma of the liver: a case report and review of the literature. Arch Pathol Lab Med. 2004;128:e46–e49. 12. Quesada R, González-Lagunas J, Raspall G. Aggressive glomus tumor of the tongue: report of a case. Med Oral. 2004;9:350–354. 13. Folpe AL, Fanburg-Smith JC, Miettinen M, et al. Atypical and malignant glomus tumors: analysis of 52 cases, with a proposal for the reclassification of glomus tumors. Am J Surg Pathol. 2001; 25:1–12. 14. Xu XD, Lu XH, Ye GX, et al. Immunohistochemical analysis and biological behaviour of gastric glomus tumours: a case report and review of the literature. J Int Med Res. 2010;38:1539–1546.


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Extradigital Symplastic Glomus Tumor of the Hand: Report of 2 Cases and Literature Review.

Glomus tumor is a rather common tumor of the hand, which presents with a classic triad of symptoms of high temperature, sensitivity and pain, and loca...
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