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Papillary hidradenoma

MedGen UID:
87221
•Concept ID:
C0334348
•
Neoplastic Process
Synonyms: Anogenital Papillary Hidradenoma; Anogenital Papillary Hidradenomas; Hidradenoma Papilliferum; Hidradenoma Papilliferums; Hidradenoma, Anogenital Papillary; Hidradenoma, Papillary; Papillary Hidradenoma; Papillary Hidradenoma, Anogenital; Papillary Hidradenomas; Papilliferum, Hidradenoma
SNOMED CT: Papillary hidradenoma (89791006); Hidradenoma papilliferum (89791006); Hidradenoma papilliferum (307597000); Papillary hidradenoma (307597000)
 
Monarch Initiative: MONDO:0003446

Definition

A benign neoplasm arising from the sweat glands. It presents as a slow growing cystic nodular lesion most often in the skin of the vulva and the perianal region. It is characterized by the presence of cystic and large papillary structures. The papillary structures contain connective tissue and are covered by two layers of epithelium. Complete excision is curative. [from NCI]

Professional guidelines

PubMed

Several genotypes, one phenotype: PIK3CA/AKT1 mutation-negative hidradenoma papilliferum show genetic lesions in other components of the signalling network.
Pfarr N, Allgäuer M, Steiger K, Weichert W, Schirmacher P, Noske A, Stenzinger A
Pathology 2019 Jun;51(4):362-368. Epub 2019 Apr 19 doi: 10.1016/j.pathol.2019.01.010. PMID: 31010589

Recent clinical studies

Etiology

Vulvar hidradenoma papilliferum (HP) is located on the sites of mammary-like anogenital glands (MLAGs): Analysis of the photographs of 52 tumors.
El-Khoury J, Renald MH, Plantier F, Avril MF, Moyal-Barracco M
J Am Acad Dermatol 2016 Aug;75(2):380-4. Epub 2016 Mar 2 doi: 10.1016/j.jaad.2016.01.034. PMID: 26944596
Differential expression of calcyclin and its accessible ligands in various types of cutaneous tumors.
Brinck U, Gabius HJ, Zeng FY, Gerke V, Lazarou D, Zografakis C, Tsambaos D, Berger H
J Dermatol Sci 1995 Nov;10(3):181-90. doi: 10.1016/0923-1811(95)00402-e. PMID: 8593259

Diagnosis

Vulvar hidradenoma papilliferum (HP) is located on the sites of mammary-like anogenital glands (MLAGs): Analysis of the photographs of 52 tumors.
El-Khoury J, Renald MH, Plantier F, Avril MF, Moyal-Barracco M
J Am Acad Dermatol 2016 Aug;75(2):380-4. Epub 2016 Mar 2 doi: 10.1016/j.jaad.2016.01.034. PMID: 26944596
Hidradenoma Papilliferum: A Clinicopathologic Study of 264 Tumors From 261 Patients, With Emphasis on Mammary-Type Alterations.
Konstantinova AM, Michal M, Kacerovska D, Spagnolo DV, Stewart CJ, Kutzner H, Zelger B, Plaza JA, Denisjuk N, Hejda V, Shelekhova K, Bisceglia M, Danis D, Zamecnik M, Kerl K, Guenova E, Kazakov DV
Am J Dermatopathol 2016 Aug;38(8):598-607. doi: 10.1097/DAD.0000000000000495. PMID: 26863059
Papillary hidradenoma of the eyelid margin: clinical and immunohistochemical observations further supporting an apocrine rather than an eccrine origin.
Jakobiec FA, Rai R, Lefebvre DR
Surv Ophthalmol 2014 Sep-Oct;59(5):540-7. Epub 2014 Jan 28 doi: 10.1016/j.survophthal.2014.01.002. PMID: 24661803
Papillary hidradenoma: immunohistochemical analysis of steroid receptor profile with a focus on apocrine differentiation.
Offidani A, Campanati A
J Clin Pathol 1999 Nov;52(11):829-32. doi: 10.1136/jcp.52.11.829. PMID: 10690173Free PMC Article
Papillary hidradenoma of the vulva: a clinicopathologic study of 69 cases.
Woodworth H Jr, Dockerty MB, Wilson RB, Pratt JH
Am J Obstet Gynecol 1971 Jun 15;110(4):501-8. doi: 10.1016/0002-9378(71)90691-0. PMID: 4325819

Prognosis

Hidradenoma Papilliferum: A Clinicopathologic Study of 264 Tumors From 261 Patients, With Emphasis on Mammary-Type Alterations.
Konstantinova AM, Michal M, Kacerovska D, Spagnolo DV, Stewart CJ, Kutzner H, Zelger B, Plaza JA, Denisjuk N, Hejda V, Shelekhova K, Bisceglia M, Danis D, Zamecnik M, Kerl K, Guenova E, Kazakov DV
Am J Dermatopathol 2016 Aug;38(8):598-607. doi: 10.1097/DAD.0000000000000495. PMID: 26863059
Mutations in genes encoding PI3K-AKT and MAPK signaling define anogenital papillary hidradenoma.
Pfarr N, Sinn HP, Klauschen F, Flechtenmacher C, Bockmayr M, Ridinger K, von Winterfeld M, Warth A, Lorenz K, Budczies J, Penzel R, Lennerz JK, Endris V, Weichert W, Stenzinger A
Genes Chromosomes Cancer 2016 Feb;55(2):113-9. Epub 2015 Oct 23 doi: 10.1002/gcc.22315. PMID: 26493284
Cystic papillary hidradenoma of the vulva: case report and review of the literature.
Kaufmann T, Pawl NO, Soifer I, Greston WM, Kleiner GJ
Gynecol Oncol 1987 Feb;26(2):240-5. doi: 10.1016/0090-8258(87)90280-0. PMID: 3026933

Clinical prediction guides

Several genotypes, one phenotype: PIK3CA/AKT1 mutation-negative hidradenoma papilliferum show genetic lesions in other components of the signalling network.
Pfarr N, Allgäuer M, Steiger K, Weichert W, Schirmacher P, Noske A, Stenzinger A
Pathology 2019 Jun;51(4):362-368. Epub 2019 Apr 19 doi: 10.1016/j.pathol.2019.01.010. PMID: 31010589
Hidradenoma Papilliferum: A Clinicopathologic Study of 264 Tumors From 261 Patients, With Emphasis on Mammary-Type Alterations.
Konstantinova AM, Michal M, Kacerovska D, Spagnolo DV, Stewart CJ, Kutzner H, Zelger B, Plaza JA, Denisjuk N, Hejda V, Shelekhova K, Bisceglia M, Danis D, Zamecnik M, Kerl K, Guenova E, Kazakov DV
Am J Dermatopathol 2016 Aug;38(8):598-607. doi: 10.1097/DAD.0000000000000495. PMID: 26863059
Papillary hidradenoma of the eyelid margin: clinical and immunohistochemical observations further supporting an apocrine rather than an eccrine origin.
Jakobiec FA, Rai R, Lefebvre DR
Surv Ophthalmol 2014 Sep-Oct;59(5):540-7. Epub 2014 Jan 28 doi: 10.1016/j.survophthal.2014.01.002. PMID: 24661803

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