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Glomus tumor

Tumor biology and incidence

  • Rare (~1.5% of soft tissue tumors of the hand), generally benign neoplasms
    • In isolated cases, may be locally aggressive and even metastasize (then called glomangiosarcoma)
  • Characterized by proliferation of glomus cells (specialized smooth muscle cells that surround small vessels and play a role in regulation of peripheral blood flow); etiology unknown
  • Arise from arterial part of glomus body -- specifically, Sucquet-Hoyer canal (arteriovenous shunt in dermis that helps regulate temperature)
  • Primarily solitary tumors, although multiple tumor variant (inherited in an autosomal-dominant pattern with invariable penetrance) occurs in 10% to 25% of cases
    • Multiple tumor variant subdivided into three types: regional (localized to specific area), disseminated (multiple lesions widely distributed throughout body), congenital (very rare; coalesce into plaques)

Age

  • Most common between 30 and 50
  • Multiple tumor variant tends to present in second or third decade of life

Gender

  • Solitary tumor: F > M
  • Multiple tumor variant: F = M, or slight male predominance

Presentation

  • Classic triad of paroxysmal pain, tenderness to palpation, and cold sensitivity
  • Pain often excruciating, though less severe in multiple tumor variant
  • Can have nailbed ridging and bluish discoloration at base of nail

Physical findings

  • Solitary tumors usually bluish or purplish in color, smaller than 1 cm, and located in acral areas (especially subungual)
  • Highly tender to palpation
  • Range from nodular to faintly papular
  • Rare malignant tumor tends to be larger, deeper, and more rapidly growing

Plain films

May show bony erosion in up to half of subungual lesions

Site

Can present in various locations but most commonly found in hand (75%), particularly in subungual region

Size

<1 cm

Tumor effect on bone / bone response to tumor
  • Bone eroded by tumor in some cases
  • Osteolytic appearance, usually with sclerotic, well-defined border due to slow growth
Matrix

None

Cortex

Cortical erosion in 20-50% of cases

Soft tissue mass

Often see increased distance between nailplate and dorsal soft tissue, indicating presence of mass

CT scan

  • Shows non-specific subungual mass
  • Delineates bony erosion if present

MRI

  • Appear as dark, well-delineated lesions on T1 images
  • Very high and homogeneous signal on intensity on T2-weighted images
  • Useful in detecting small fingertip lesions
  • High-resolution MRI better for delineating extent of lesion

Differential diagnosis

  • Blue nevi
  • Hemangioma
  • Neuroma
  • AVM
  • Angiolipoma
  • Tufted angioma
  • Angioleiomyoma
  • Melanoma
  • Epidermal inclusion cyst
  • Metastatic carcinoma
  • Osteoid osteoma
  • Enchondroma
  • Chronic osteomyelitis.

Natural history

  • Exquisitely painful until treated
  • Excision usually cures solitary lesion, with very low recurrence rate
  • Malignant tumors extremely rare, locally aggressive, prone to recur, and can metastasize (few case reports)

Pathology 

Solitary lesion
  • Gross: Well-circumscribed nodules surrounded by fibrous tissue
  • Micro: Clusters of glomus cells, which have rounded, regular shape with sharply punched-out, rounded nucleus and scant eosinophilic cytoplasm
    • Glomus cells and fine, nonmyelinated nerve endings located between endothelial-lined vascular spaces.
Multiple lesions

Grossly and histologically appear more like hemangiomas, with larger vascular channels and smaller aggregates of glomus cells, compared to solitary lesions

Malignant glomus tumor

Similar to benign glomus tumor, but tends to show mild atypia, shorter and more spindly nuclei, various rate of mitosis, and more invasive growth pattern

Diagnosis and treatment

  • Hildreth sign: Disappearance of pain after application of tourniquet proximally; reported sensitivity of 77.4% and specificity near 100%.
  • Love test: Pain elicited by applying pressure to precise area with pin or tip of a sharp pencil; sensitivity 100% according to some sources
  • Cold sensitivity: Jighly sensitive and specific as well
  • Excision is treatment of choice for solitary glomus tumor
    • Tumor fairly well encapsulated and can be shelled out
    • Can be approached via lateral approach or directly under nailbed
    • Pain relief typically immediate following surgery
  • For multiple glomus tumors, surgical treatment usually limited to most symptomatic lesion(s)
    • Other methods -- including sclerotherapy, electron beam radiation, argon lasers, and carbon dioxide lasers -- have been used to treat multiple lesions, with good results reported in literature

Complications

  • Recurrence (as high as 20%)
  • If symptoms continue longer than 3 months post-op, re-exploration indicated to look for missed/recurrent lesion

Recommended reading

Bhaskaranand K and BC Navadgi. Glomus tumour of the hand. J Hand Surg Eur, 2002, 27: 229.

Carroll RE and AT Berman. Glomus tumors of the hand: review of the literature and report on twenty-eight cases. JBJS 1972, 54: 691-703.

Green DP et al, ed. Green's Operative Hand Surgery. Fifth edition. Elsevier. Philadelphia, PA: 2005.

Hiruta N et al. Malignant glomus tumor: a case report and review of the literature. Am J Surg Path 21(9), September 1997, 1096-1103.

Parsons ME et al. Multiple glomus tumors. International Journal of Dermatology 36(12): 894-900, Dec. 1997.

Vasisht B, HK Watson, J Emmanuella, GT Lionelli. Digital glomus tumors: a 29-year experience with a lateral subperiosteal approach. Plastic and Reconstructive Surgery 114(6): 1486-9, Nov. 2004.

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