Pilomatricoma

Pilomatricoma

Micrograph of a pilomatricoma showing the characteristic "ghost" cells (anucleate squamous cells), benign viable squamous cells and multi-nucleated giant cells. H&E stain.
Classification and external resources
Specialty oncology
ICD-10 D23
DiseasesDB 32128
eMedicine article/1058965

Pilomatricoma, also known as a calcifying epithelioma of Malherbe,[1] Malherbe calcifying epithelioma, and Pilomatrixoma, is a benign skin tumor derived from the hair matrix.[2]:670[3]

Histologic features

Pilomatricomas consist of anucleate squamous cells (called "ghost cells"), benign viable squamous cells and multi-nucleated giant cells. The presence of calcifications is common.

Pathogenesis

Pilomatricoma is associated with high levels of beta-catenin caused by either a mutation in the APC gene or beta-catenin gene. These high levels of beta-catenin can aid cell proliferation, inhibit cell death, and ultimately lead to cancer.[4]

Associations

Pilomatricomas are associated with Turner syndrome, with a prevalence of 2.6%.[5] In addition to Turner syndrome, multiple pilomatricomas are associated with myotonic dystrophy, Rubinstein-Taybi syndrome, and trisomy 9.[6]

See also

References

  1. ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
  2. ↑ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: Clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.
  3. ↑ Levy J, Ilsar M, Deckel Y, Maly A, Anteby I, Pe'er J (2008). "Eyelid pilomatrixoma: a description of 16 cases and a review of the literature". Surv Ophthalmol 53 (5): 526–35. doi:10.1016/j.survophthal.2008.06.007. PMID 18929763.
  4. ↑ Hassanein AM, Glanz SM, Kessler HP, Eskin TA, Liu C (2003). "beta-Catenin is expressed aberrantly in tumors expressing shadow cells. Pilomatricoma, craniopharyngioma, and calcifying odontogenic cyst". Am J Clin Pathol (5):732-6. PMID 14608900.
  5. ↑ Handler MZ, Derrick KM, Lutz RE, Morrell DS, Davenport ML, Armstrong AW (2013). "Prevalence of pilomatricoma in Turner syndrome: findings from a multicenter study". JAMA Dermatol 149(5):559-64. PMID 23426075.
  6. ↑ James, William D.; Berger, Timothy G.; Elston, Dirk. (2015). Andrews' Diseases of the Skin: Clinical Dermatology. Saunders Elsevier. p. 666. ISBN 978-0323319676.
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