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Trichoepithelioma

MedGen UID:: 138112
•Concept ID:: C0349658
•: Neoplastic Process

Synonym:	Trichoblastoma
SNOMED CT:	Trichoblastoma (878881002); Brooke's tumor (274898000); Epithelioma adenoides cysticum (274898000); Trichoepithelioma (274898000); Trichoblastoma (277942005); Trichoepithelioma (59186007); Brooke's tumor (59186007); Epithelioma adenoides cysticum (59186007)

HPO:	HP:0025367
Monarch Initiative:	MONDO:0020593

Definition

A benign hair follicle tumor whose tumor cells form rudimentary hair follicles but not actual hair shafts. A trichoepithelioma is usually less than one centimeter, firm, round, and shihy with yellow, pink, brown, or bluish color. They may occur multiply, usually on the face, and may gradually increase in number with age. [from HPO]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVTrichoepithelioma

Benign epithelial skin neoplasm
- Benign Germinative Follicular Epithelium Neoplasm
  - Trichoepithelioma
    - Desmoplastic Trichoepithelioma

Conditions with this feature

Follicular atrophoderma and basal cell epitheliomata

MedGen UID:: 87539
•Concept ID:: C0346104
•: Neoplastic Process

Bazex-Dupre-Christol syndrome (BDCS) is an X-linked dominant disorder characterized by a triad of congenital hypotrichosis, follicular atrophoderma affecting the dorsa of the hands and feet, the face, and extensor surfaces of the elbows or knees, and the development of basal cell neoplasms, including basal cell nevi and basal cell carcinomas from the second decade onward (Yung and Newton-Bishop, 2005). Rombo syndrome (180730) has similar features, but shows autosomal dominant inheritance.

See: Condition Record

Follicular atrophoderma and basal cell epitheliomata

Professional guidelines

PubMed

Management of multiple trichoepithelioma: A review of pharmacological therapies.

Lobo Y, Blake T, Wheller L
Australas J Dermatol 2021 May;62(2):e192-e200. Epub 2021 Jan 5 doi: 10.1111/ajd.13537. PMID: 33403677

Differential diagnostic significance of epithelial and stromal CD10 expression in tumors of trichogenic origin.

Kneitz H, Fetzer S, Goebeler M, Bröcker EB
J Dtsch Dermatol Ges 2018 Jun;16(6):719-728. doi: 10.1111/ddg.13547. PMID: 29873924

Phenotype-genotype correlations for clinical variants caused by CYLD mutations.

Nagy N, Farkas K, Kemény L, Széll M
Eur J Med Genet 2015 May;58(5):271-8. Epub 2015 Mar 14 doi: 10.1016/j.ejmg.2015.02.010. PMID: 25782638

See all (11)

Recent clinical studies

Etiology

Cd34+ Stromal Cells/Telocytes in Normal and Pathological Skin.

Díaz-Flores L, Gutiérrez R, García MP, González-Gómez M, Rodríguez-Rodriguez R, Hernández-León N, Díaz-Flores L Jr, Carrasco JL
Int J Mol Sci 2021 Jul 8;22(14) doi: 10.3390/ijms22147342. PMID: 34298962 Free PMC Article

Microcystic Adnexal Carcinoma and a Summary of Other Rare Malignant Adnexal Tumours.

Aslam A
Curr Treat Options Oncol 2017 Aug;18(8):49. doi: 10.1007/s11864-017-0491-z. PMID: 28681209

Desmoplastic trichoepithelioma.

Mamelak AJ, Goldberg LH, Katz TM, Graves JJ, Arnon O, Kimyai-Asadi A
J Am Acad Dermatol 2010 Jan;62(1):102-106. doi: 10.1016/j.jaad.2009.06.066. PMID: 20082889

Congenital desmoplastic trichoepithelioma.

Carter JJ, Kaur MR, Hargitai B, Brown R, Slator R, Abdullah A
Clin Exp Dermatol 2007 Sep;32(5):522-4. Epub 2007 Apr 24 doi: 10.1111/j.1365-2230.2007.02431.x. PMID: 17459070

Cutaneous lymphadenoma.

Inalöz HS, Chowdhury MM, Knight AG
J Eur Acad Dermatol Venereol 2001 Sep;15(5):481-3. doi: 10.1046/j.1468-3083.2001.00302.x. PMID: 11763398

See all (58)

Diagnosis

Trichoepithelioma: A Comprehensive Review.

Karimzadeh I, Namazi MR, Karimzadeh A
Acta Dermatovenerol Croat 2018 Jun;26(2):162-165. PMID: 29989874

Histologic Mimics of Basal Cell Carcinoma.

Stanoszek LM, Wang GY, Harms PW
Arch Pathol Lab Med 2017 Nov;141(11):1490-1502. doi: 10.5858/arpa.2017-0222-RA. PMID: 29072946

Trichoepithelioma.

Johnson H, Robles M, Kamino H, Walters RF, Lee A, Sanchez M
Dermatol Online J 2008 Oct 15;14(10):5. PMID: 19061604

Cutaneous lymphadenoma.

Inalöz HS, Chowdhury MM, Knight AG
J Eur Acad Dermatol Venereol 2001 Sep;15(5):481-3. doi: 10.1046/j.1468-3083.2001.00302.x. PMID: 11763398

Desmoplastic trichoepithelioma.

Brownstein MH, Shapiro L
Cancer 1977 Dec;40(6):2979-86. doi: 10.1002/1097-0142(197712)40:6<2979::aid-cncr2820400633>3.0.co;2-8. PMID: 589563

See all (191)

Therapy

Differentiation and risk stratification of basal cell carcinoma with deep learning on histopathologic images and measuring nuclei and tumor microenvironment features.

Lan X, Guo G, Wang X, Yan Q, Xue R, Li Y, Zhu J, Dong Z, Wang F, Li G, Wang X, Xu J, Jiang Y
Skin Res Technol 2024 Jan;30(1):e13571. doi: 10.1111/srt.13571. PMID: 38196164 Free PMC Article

Management of multiple trichoepithelioma: A review of pharmacological therapies.

Lobo Y, Blake T, Wheller L
Australas J Dermatol 2021 May;62(2):e192-e200. Epub 2021 Jan 5 doi: 10.1111/ajd.13537. PMID: 33403677

Treatment of cutaneous tumors with topical 5% imiquimod cream.

Alessi SS, Sanches JA, Oliveira WR, Messina MC, Pimentel ER, Festa Neto C
Clinics (Sao Paulo) 2009;64(10):961-6. doi: 10.1590/S1807-59322009001000005. PMID: 19841702 Free PMC Article

Brooke-Spiegler syndrome.

Layegh P, Sharifi-Sistani N, Abadian M, Moghiman T
Indian J Dermatol Venereol Leprol 2008 Nov-Dec;74(6):632-4. doi: 10.4103/0378-6323.45109. PMID: 19171990

Sporadic trichoepithelioma demonstrates deletions at 9q22.3.

Matt D, Xin H, Vortmeyer AO, Zhuang Z, Burg G, Böni R
Arch Dermatol 2000 May;136(5):657-60. doi: 10.1001/archderm.136.5.657. PMID: 10815860

See all (15)

Prognosis

Morphologically high-grade trichoblastic carcinoma: a clinicopathological study with long-term clinical follow-up.

Mehta A, Davey J, Wiedemeyer K, Brenn T
Histopathology 2021 Jun;78(7):970-975. Epub 2021 Apr 6 doi: 10.1111/his.14325. PMID: 33393120

Trichoepithelioma Misdiagnosed as Basal Cell Carcinoma.

Kang KJ, Seo BF, Shin J, Kwon H, Byeon JH, Jung SN
J Craniofac Surg 2019 May/Jun;30(3):e197-e199. doi: 10.1097/SCS.0000000000005089. PMID: 30394977

Microcystic Adnexal Carcinoma and a Summary of Other Rare Malignant Adnexal Tumours.

Aslam A
Curr Treat Options Oncol 2017 Aug;18(8):49. doi: 10.1007/s11864-017-0491-z. PMID: 28681209

Microcystic Adnexal Carcinoma: A Review of the Literature.

Gordon S, Fischer C, Martin A, Rosman IS, Council ML
Dermatol Surg 2017 Aug;43(8):1012-1016. doi: 10.1097/DSS.0000000000001142. PMID: 28654578

Congenital desmoplastic trichoepithelioma.

Carter JJ, Kaur MR, Hargitai B, Brown R, Slator R, Abdullah A
Clin Exp Dermatol 2007 Sep;32(5):522-4. Epub 2007 Apr 24 doi: 10.1111/j.1365-2230.2007.02431.x. PMID: 17459070

See all (40)

Clinical prediction guides

Expression of Early Growth Response 3 in Skin Cancers.

Zhou KY, Deng LJ, Luo SY, Wang QX, Fang S
Appl Immunohistochem Mol Morphol 2024 Apr 1;32(4):169-175. Epub 2024 Mar 14 doi: 10.1097/PAI.0000000000001191. PMID: 38478384

Does Immunohistochemistry Add to Morphology in Differentiating Trichoepithelioma, Desmoplastic Trichoepithelioma, Morpheaform Basal Cell Carcinoma, and Microcystic Adnexal Carcinoma?

Mohanty SK, Sardana R, McFall M, Pradhan D, Usmani A, Jha S, Mishra SK, Sampat NY, Lobo A, Wu JM, Balzer BL, Frishberg DP
Appl Immunohistochem Mol Morphol 2022 Apr 1;30(4):273-277. doi: 10.1097/PAI.0000000000001002. PMID: 35384877

Expression of Hedgehog signalling molecules in microcystic adnexal carcinoma.

Gambichler T, Hartenstein I, Dreißigacker M, Stockfleth E, Stücker M, Schaller J, Schulze HJ, Becker JC, Käfferlein HU, Brüning T, Lang K
Clin Exp Dermatol 2021 Aug;46(6):1052-1057. Epub 2021 May 1 doi: 10.1111/ced.14634. PMID: 33714217

Induction of Desmoplastic Trichoepithelioma in a Dermatofibroma.

Georgescu M, Blödorn-Schlicht N, Böer-Auer A
Am J Dermatopathol 2018 Dec;40(12):924-926. doi: 10.1097/DAD.0000000000000982. PMID: 29053493

Phenotype-genotype correlations for clinical variants caused by CYLD mutations.

Nagy N, Farkas K, Kemény L, Széll M
Eur J Med Genet 2015 May;58(5):271-8. Epub 2015 Mar 14 doi: 10.1016/j.ejmg.2015.02.010. PMID: 25782638

See all (62)