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Desmoplastic Trichoepithelioma

MedGen UID:
105114
•Concept ID:
C0432526
•
Neoplastic Process
Synonyms: Desmoplastic trichoepithelioma; Sclerosing Epithelial Hamartoma; Sclerosing epithelial hamartoma
SNOMED CT: Sclerosing epithelial hamartoma (254696000); Desmoplastic trichoepithelioma (254696000)

Definition

A benign adnexal tumor occurring in the face of young female subjects. It is characterized by the presence of epithelial neoplastic cells and keratinous cysts in a desmoplastic stroma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDesmoplastic Trichoepithelioma

Professional guidelines

PubMed

A Systematic Review of the Epidemiology, Clinical Characteristics, Treatment, and Outcomes for Desmoplastic Trichoepithelioma: Underscoring Mohs Micrographic Surgery in Management.
Nanda R, Srivastava D, Nijhawan RI
Dermatol Surg 2024 Aug 1;50(8):695-698. Epub 2024 Apr 9 doi: 10.1097/DSS.0000000000004194. PMID: 38595132
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
Desmoplastic trichoepithelioma versus morphoeic basal cell carcinoma: a critical reappraisal of histomorphological and immunohistochemical criteria for differentiation.
Costache M, Bresch M, Böer A
Histopathology 2008 Jun;52(7):865-76. Epub 2008 May 6 doi: 10.1111/j.1365-2559.2008.03036.x. PMID: 18462369

Recent clinical studies

Etiology

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
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
Does the panel of cytokeratin 20 and androgen receptor antibodies differentiate desmoplastic trichoepithelioma from morpheaform/infiltrative basal cell carcinoma?
Katona TM, Perkins SM, Billings SD
J Cutan Pathol 2008 Feb;35(2):174-9. doi: 10.1111/j.1600-0560.2007.00783.x. PMID: 18190441
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

Diagnosis

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
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
Desmoplastic trichoepithelioma.
Zuccati G, Massi D, Mastrolorenzo A, Urbano FG, Paoli S, Reali UM
Australas J Dermatol 1998 Nov;39(4):273-4. doi: 10.1111/j.1440-0960.1998.tb01491.x. PMID: 9838731
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

Therapy

A Systematic Review of the Epidemiology, Clinical Characteristics, Treatment, and Outcomes for Desmoplastic Trichoepithelioma: Underscoring Mohs Micrographic Surgery in Management.
Nanda R, Srivastava D, Nijhawan RI
Dermatol Surg 2024 Aug 1;50(8):695-698. Epub 2024 Apr 9 doi: 10.1097/DSS.0000000000004194. PMID: 38595132
Diagnostic utility of cytokeratin 17 immunostaining in morpheaform basal cell carcinoma and for facilitating the detection of tumor cells at the surgical margins.
Anderson-Dockter H, Clark T, Iwamoto S, Lu M, Fiore D, Falanga JK, Falanga V
Dermatol Surg 2012 Aug;38(8):1357-66. Epub 2012 Jun 12 doi: 10.1111/j.1524-4725.2012.02417.x. PMID: 22691048Free PMC Article
Desmoplastic trichoepithelioma: report of a unique periocular case.
Kirzhner M, Jakobiec FA, Borodic G
Ophthalmic Plast Reconstr Surg 2012 Sep-Oct;28(5):e121-3. doi: 10.1097/IOP.0b013e318245535a. PMID: 22366669

Prognosis

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
Expression of p75 neurotrophin receptor in desmoplastic trichoepithelioma, infiltrative basal cell carcinoma, and microcystic adnexal carcinoma.
Jedrych J, McNiff JM
Am J Dermatopathol 2013 May;35(3):308-15. doi: 10.1097/DAD.0b013e31826281f2. PMID: 23598854
Microcystic adnexal carcinoma versus desmoplastic trichoepithelioma: a comparative study.
Tse JY, Nguyen AT, Le LP, Hoang MP
Am J Dermatopathol 2013 Feb;35(1):50-5. doi: 10.1097/DAD.0b013e31825988df. PMID: 22722464
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

Clinical prediction guides

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
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
Expression of p75 neurotrophin receptor in desmoplastic trichoepithelioma, infiltrative basal cell carcinoma, and microcystic adnexal carcinoma.
Jedrych J, McNiff JM
Am J Dermatopathol 2013 May;35(3):308-15. doi: 10.1097/DAD.0b013e31826281f2. PMID: 23598854
Microcystic adnexal carcinoma versus desmoplastic trichoepithelioma: a comparative study.
Tse JY, Nguyen AT, Le LP, Hoang MP
Am J Dermatopathol 2013 Feb;35(1):50-5. doi: 10.1097/DAD.0b013e31825988df. PMID: 22722464
Immunohistochemical and ultrastructural observations of desmoplastic trichoepithelioma with a special reference to a morphological comparison with normal apocrine acrosyringeum.
Yamamoto O, Hamada T, Doi Y, Sasaguri Y, Hashimoto H
J Cutan Pathol 2002 Jan;29(1):15-26. doi: 10.1034/j.1600-0560.2002.290104.x. PMID: 11841513

Recent systematic reviews

A Systematic Review of the Epidemiology, Clinical Characteristics, Treatment, and Outcomes for Desmoplastic Trichoepithelioma: Underscoring Mohs Micrographic Surgery in Management.
Nanda R, Srivastava D, Nijhawan RI
Dermatol Surg 2024 Aug 1;50(8):695-698. Epub 2024 Apr 9 doi: 10.1097/DSS.0000000000004194. PMID: 38595132

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