AUTHOR AND EDITOR INFORMATION

Section 1 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Multimedia
• References

INTRODUCTION

Section 2 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Multimedia
• References

Background

Glomus tumors are relatively uncommon benign neoplasms that differentiate to become modified smooth muscle cells called glomus cells. Two variants exist: solitary glomus tumors and multiple glomus tumors, which are also known as glomangiomas or glomulovenous malformations. Each variant has distinct clinical and histopathologic characteristics. The most common location for these tumors is the distal extremities, especially in subungual areas.

Pathophysiology

Glomus tumors arise from the arterial portion of the glomus body, or the Sucquet-Hoyer canal, which is an arteriovenous shunt in the dermis that contributes to temperature regulation. Although glomus tumors are thought to arise from glomus cells, these tumors have been observed in extracutaneous locations that are not known to contain glomus cells. One explanation for this finding is that these tumors may arise from perivascular cells that can differentiate into glomus cells. Multiple glomus tumors, especially the disseminated variant, are inherited in an autosomal-dominant pattern with incomplete penetrance.

Frequency

United States

The exact incidence of glomus tumors is unknown. The multiple variant is rare, accounting for less than 10% of all cases. The probable misdiagnosis of many of these lesions as hemangiomas or venous malformations also makes an accurate assessment of incidence difficult.

Mortality/Morbidity

The most common adverse effect is pain, which is usually associated with solitary lesions. Multiple tumors are less likely to be painful. In one report, a patient with more than 400 glomus tumors had thrombocytopenia as a result of platelet sequestration (ie, Kasabach-Merritt syndrome). Malignant glomus tumors, or glomangiosarcomas, are extremely rare and usually represent a locally infiltrative malignancy. However, metastases do occur and are usually fatal.

Sex

Solitary glomus tumors, particularly subungual lesions, are more common in females than in males. Multiple lesions are slightly more common in males.

Age

Solitary glomus tumors are more frequent in adults than in others. Multiple glomus tumors develop 10-15 years earlier than single lesions; about one third of the cases of multiple tumors occur in those younger than 20 years. Congenital glomus tumors are rare; they are plaquelike in appearance and are considered a variant of multiple glomus tumors.

CLINICAL

Section 3 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Multimedia
• References

History

• Patients with solitary glomus tumors usually have paroxysmal pain, which can be severe and exacerbated by pressure or temperature changes, especially cold.
• Multiple glomus tumors can also be painful, but this feature is less common, and the pain usually is not severe.
• Patients with multiple lesions often seek medical attention because they are worried or have cosmetic concerns.
• Because multiple glomus tumors are inherited as an autosomal-dominant condition, a family history of similar lesions may be helpful for diagnosis.

Physical

• Solitary glomus tumors have the following characteristics:
• • Blue or purple
  • Papules or nodules that can be blanched
  • Size usually smaller than 1 cm
  • Located most commonly in acral areas, especially subungual areas of fingers and toes

• The multiple variant is subdivided into regional or localized, disseminated, and congenital plaquelike forms.
• • The regional variant consists of blue-to-purple partially compressible papules or nodules that are grouped and limited to a specific area, most commonly an extremity.
  • The disseminated type consists of multiple lesions distributed over the body with no specific grouping. This form is less common than the regional variant.
  • Congenital plaquelike glomus tumors consist of either grouped papules that coalesce into indurated plaques or clusters of discrete nodules. This form is the rarest variant of multiple glomus tumors.

• Two useful items for diagnosing glomus tumors, particularly solitary painful glomus tumors (especially those under a nail) are the following:
• • Hildreth sign, which is disappearance of pain after application of a tourniquet proximally on the arm
  • Love test, which consists of eliciting pain by applying pressure to a precise area with the tip of a pencil

• Features of glomangiosarcomas may include the following:
• • Size larger than 1 cm
  • Rapid growth
  • Deep soft tissue involvement

Causes

• Glomus tumors are neoplasms caused by a proliferation of glomus cells, which make up a portion of the glomus body.
• The initiating event for glomus cell proliferation is unknown.
• Some authors have postulated that trauma induces solitary subungual glomus tumors, although this theory is not well studied.
• Most multiple glomus tumors, especially those of the disseminated form, are inherited in an autosomal dominant pattern with incomplete penetrance. Most hereditary glomangiomas are associated with defects in the glomulin gene, located on chromosome 1.

DIFFERENTIALS

Section 4 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Multimedia
• References

Other Problems to be Considered

Angioleiomyoma
Angiolipoma
Arteriovenous (AV) malformations
Congenital plaquelike blue nevus
Hemangioma
Melanoma
Spiradenoma
Tufted angioma

WORKUP

Section 5 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Multimedia
• References

Lab Studies

• Routine laboratory studies are not helpful.
• The rare exception is in cases with widely disseminated lesions in which platelet sequestration is a concern. In these cases, a complete blood cell count is indicated.

Imaging Studies

• Imaging may aid in diagnosis, especially for subungual lesions.


• In subungual glomus tumors, radiographs show bony erosion in 14-60% of patients.


• In addition, radiographs may show an increased distance between the dorsum of the phalanx and the underside of the nail.


• High-resolution MRI is probably the criterion standard for the imaging of glomus tumors, although nearly all tumors can be detected with standard MRI.


• Ultrasonography has also been shown to be helpful.

Histologic Findings

Solitary and multiple glomus tumors have distinct histopathologic features.

Solitary lesions appear mostly as solid well-circumscribed nodules surrounded by a rim of fibrous tissue. They contain endothelium-lined vascular spaces surrounded by clusters of glomus cells. The glomus cells are monomorphous round or polygonal cells with plump nuclei and scant eosinophilic cytoplasm.

Multiple lesions are less well circumscribed and less solid-appearing than their solitary counterparts. Multiple lesions have the overall appearance of a hemangioma. They contain multiple irregular, dilated, endothelium-lined vascular channels that contain red blood cells. The vascular spaces are larger than those in solitary glomus tumors. Small aggregates of glomus cells are present in the walls of these channels and in small clusters in the adjacent stroma. Multiple glomus tumors have more narrow and focal aggregates of glomus cells than solitary lesions. The overall appearance of multiple glomus tumors accounts for their alternate name, glomangiomas.

Glomangiosarcomas resemble benign glomus tumors. However, glomangiosarcomas have more atypia, pleomorphism, and mitotic figures, and they have an invasive growth pattern. Most often, foci of benign glomus tumor are correlated with malignant lesions.

TREATMENT

Section 6 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Multimedia
• References

Surgical Care

• The treatment of choice for solitary glomus tumors is surgical excision.
• • For multiple glomus tumors, excision may be more difficult because of their poor circumscription and the large number of lesions.
  • Excision should be limited to symptomatic lesions.

• Other reported treatment modalities include argon and carbon dioxide laser therapy and sclerotherapy with hypertonic saline or sodium tetradecyl sulfate. These are most useful in treating multiple lesions.
• The treatment of glomangiosarcoma is based on a few case reports.
• • Wide local excision is adequate treatment and probably the treatment of choice.
  • However, geometric excision is probably a reasonable alternative in cosmetically sensitive areas.

FOLLOW-UP

Section 7 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Multimedia
• References

Prognosis

• The prognosis is excellent.
• Excision of painful lesions most often results in cure, with a low recurrence rate for solitary lesions.
• Malignant glomus tumors are extremely rare and usually locally aggressive.
• Their overall prognosis is good when they are treated with wide excision. However, metastases do occur and are associated with a very poor prognosis.

MULTIMEDIA

Section 8 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Multimedia
• References

Media file 1:  Multiple glomus tumors.
	View Full Size Image
Media type:  Photo

REFERENCES

Section 9 of 9

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Multimedia
• References

• Barnes L, Estes SA. Laser treatment of hereditary multiple glomus tumors. J Dermatol Surg Oncol. Sep 1986;12(9):912-5. [Medline].
• Belanger SM, Weaver TD. Subungual glomus tumor of the hallux. Cutis. Jul 1993;52(1):50-2. [Medline].
• Boon LM, Mulliken JB, Enjolras O, Vikkula M. Glomuvenous malformation (glomangioma) and venous malformation: distinct clinicopathologic and genetic entities. Arch Dermatol. Aug 2004;140(8):971-6. [Medline].
• Chatterjee JS, Youssef AH, Brown RM, Nishikawa H. Congenital nodular multiple glomangioma: a case report. J Clin Pathol. Jan 2005;58(1):102-3. [Medline].
• Drapé JL, Idy-Peretti I, Goettmann S, Guérin-Surville H, Bittoun J. Standard and high resolution magnetic resonance imaging of glomus tumors of toes and fingertips. J Am Acad Dermatol. Oct 1996;35(4):550-5. [Medline].
• 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. Jan 2001;25(1):1-12. [Medline].
• Glick SA, Markstein EA, Herreid P. Congenital glomangioma: case report and review of the world literature. Pediatr Dermatol. Sep 1995;12(3):242-4. [Medline].
• Goodman TF, Abele DC. Multiple glomus tumors. A clinical and electron microscopic study. Arch Dermatol. Jan 1971;103(1):11-23. [Medline].
• Gould EP. Sclerotherapy for multiple glomangiomata. J Dermatol Surg Oncol. Apr 1991;17(4):351-2. [Medline].
• Hiruta N, Kameda N, Tokudome T, Tsuchiya K, Nonaka H, Hatori T, et al. Malignant glomus tumor: a case report and review of the literature. Am J Surg Pathol. Sep 1997;21(9):1096-103. [Medline].
• Landthaler M, Braun-Falco O, Eckert F, Stolz W, Dorn M, Wolff HH. Congenital multiple plaquelike glomus tumors. Arch Dermatol. Sep 1990;126(9):1203-7. [Medline].
• Laymon CW, Peterson WC Jr. Glomangioma (glomus tumor). A clinicopathologic study with special reference to multiple lesions appearing during pregnancy. Arch Dermatol. Nov 1965;92(5):509-14. [Medline].
• Matsunaga A, Ochiai T, Abe I, Kawamura A, Muto R, Tomita Y, et al. Subungual glomus tumour: evaluation of ultrasound imaging in preoperative assessment. Eur J Dermatol. Jan-Feb 2007;17(1):67-9. [Medline].
• Requena L, Sangueza OP. Cutaneous vascular proliferations. Part III. Malignant neoplasms, other cutaneous neoplasms with significant vascular component, and disorders erroneously considered as vascular neoplasms. J Am Acad Dermatol. Feb 1998;38(2 Pt 1):143-75; quiz 176-8. [Medline].
• Siegle RJ, Spencer DM, Davis LS. Hypertonic saline destruction of multiple glomus tumors. J Dermatol Surg Oncol. May 1994;20(5):347-8. [Medline].
• Wetherington RW, Lyle WG, Sangueza OP. Malignant glomus tumor of the thumb: a case report. J Hand Surg [Am]. Nov 1997;22(6):1098-102. [Medline].
• Yen A, Raimer SS. Multiple painful blue nodules. Multiple glomus tumors (glomangiomas). Arch Dermatol. Jun 1996;132(6):704-5, 707-8. [Medline].

Article Last Updated: Mar 28, 2007