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Glomus tumor

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Glomus tumor
SpecialtyOncology Edit this on Wikidata
Glomus tumor was also the name formerly (and incorrectly) used for a tumor now called a paraganglioma.

A glomus tumor (also known as a "solitary glomus tumor,"[1] "solid glomus tumor,"[1] or glomangioma is a rare benign neoplasm arising from the glomus body.[2]: 670  Glomus tumors were first described by Hoyer in 1877, while the first complete clinical description was given by Masson in 1924.[3]

As stated above, these lesions should not be confused with paragangliomas, which were formerly also called glomus tumors in now-antiquated clinical usage.

Familial glomangiomas have been associated with a variety of deletions in the glomulin gene, and are inherited in an autosomal dominant manner, with incomplete penetrance.[3]

Presentation

They are usually solitary and small, and can be found under the fingernails.[4] They can also be found on the tympanic membrane.[5]

They are often painful, and the pain is reproduced when the lesion is placed in cold water.

These tumors tend to have a bluish discoloration, although a white appearance is also noted. Elevation of the nail bed can occur

In rare cases, the tumors may present in other body areas, such as the gastric antrum or glans penis. Treatment is essentially the same. [6]

Frequency

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.

Malignant glomus tumors

Malignant glomus tumors, histologically and/or clinically are exceedingly rare. Criteria for the diagnosis of malignancy in glomus tumors are:[7]

  1. Tumor size of more than 2 centimeters and subfascial or visceral location.
  2. Atypical mitotic figures.
  3. Marked nuclear atypia and any level of mitotic activity.
    • Pericytes of Zimmerman

Malignant glomus tumors have been subdivided into three categories based on their histologic appearance: locally infiltrative glomus tumors (LIGT), glomangiosarcomas arising in benign glomus tumors (GABG), and glomangiosarcomas arising de novo (GADN). [8]

A few cases of malignant glomus tumors have been reported; however, they are usually only locally invasive, and metastases are exceedingly rare. Brathwaite et al in 1996 reported widespread metastases of a malignant glomus tumor involving the skin, lungs, jejunum, liver, spleen, and lymph nodes.[9] Another report of a malignant glomus tumor with metastases is that of Watanabe et al., who reported a cutaneous malignant glomus tumor with widespread metastases.[10] A few other cases of malignant glomus tumors have been reported in the literature usually occur in the older age group, and have been described at several locations, primarily the soft tissues and gastrointestinal tracts.

Recently, Lamba G et al reported the first case of malignant glomus tumor arising from the kidneys. [11]

Treatment

Photograph of surgical extraction of a glomus tumor from a female fingertip. The tumor is the translucent oblate spheroid in the center of the incision, approximate horizontal dimension is 4 millimeters. Before surgery, the tumor resided close to the bone in the right ring finger and was not visible externally. The presence of the tumor was confirmed prior to surgery via MRI. Symptoms prior to surgery were present for approximately ten years, getting progressively more severe. When subjected to cold temperature, or local stimulation, the tumor caused intense pain that radiated up the arm to the elbow.

Radiation therapy and surgery can be involved in the treatment of these tumors.[12]

See also

References

  1. ^ a b Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.{{cite book}}: CS1 maint: multiple names: authors list (link)
  2. ^ Freedberg, et. al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.
  3. ^ a b Gombos, Z (2008 Sep). "Glomus tumor". Archives of pathology & laboratory medicine. 132 (9): 1448–52. doi:10.1043/1543-2165(2008)132[1448:GT]2.0.CO;2. PMID 18788860. {{cite journal}}: Check date values in: |date= (help); Unknown parameter |coauthors= ignored (|author= suggested) (help)
  4. ^ Koç O, Kivrak AS, Paksoy Y (2007). "Subungual glomus tumour: magnetic resonance imaging findings". Australasian radiology. 51 Spec No.: B107–B109. doi:10.1111/j.1440-1673.2007.01797.x. PMID 17875128.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  5. ^ De Candia A, Como G, Passon P, Pedace E, Bazzocchi M (2002). "Sonographic findings in glomus tympanicum tumor". Journal of Clinical Ultrasound. 30 (4): 236–240. doi:10.1002/jcu.10058. PMID 11981934.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  6. ^ Macaluso JN, Sullivan JW, Tomberlin S: Glomus tumor of the glans penis. Urology. 25:409-410, 1985
  7. ^ Folpe AL, Fanburg-Smith JC, Mietinen M, Weiss SW (2001): 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.
  8. ^ Lamba G, Rafiyath SM, Kaur H, Khan S, Singh P, Hamilton AM, Ang DC. Malignant glomus tumor of kidney: the first reported case and review of literature. Hum Pathol. 2011 Aug;42(8): 1200-3.
  9. ^ Brathwaite CD and Poppiti RJ Jr., Malignant glomus tumor. A case of widespread metastasis in a patient with multiple glomus body hamartomas. Am J Surg Pathol. 1996 Feb;20(2):233-8.
  10. ^ Watanabe K, Hoshi N, Tsu-Ura Y, Suzuki T. A case of glomangiosarcoma. Fukushima Journal of Medical Sciences 1995 Jun;41(1):71–77
  11. ^ Lamba G, Rafiyath SM, Kaur H, Khan S, Singh P, Hamilton AM, Ang DC. Malignant glomus tumor of kidney: the first reported case and review of literature. Hum Pathol. 2011 Aug;42(8): 1200-3.
  12. ^ Kaylie DM, O'Malley M, Aulino JM, Jackson CG (2007). "Neurotologic surgery for glomus tumors". Otolaryngol. Clin. North Am. 40 (3): 625–649. doi:10.1016/j.otc.2007.03.009. PMID 17544699.{{cite journal}}: CS1 maint: multiple names: authors list (link)


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