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Malignant Vulvar Sweat Gland Neoplasm

MedGen UID:
268626
•Concept ID:
C1512976
•
Neoplastic Process

Definition

A malignant neoplasm that arises from sweat glands in the vulva. Representative examples include eccrine adenocarcinoma, apocrine adenocarcinoma, and Paget disease. [from NCI]

Professional guidelines

PubMed

Topical imiquimod in the treatment of extramammary Paget's disease: A 10 year retrospective analysis in an Asian tertiary centre.
Liau MM, Yang SS, Tan KB, Aw CW
Dermatol Ther 2016 Nov;29(6):459-462. Epub 2016 Aug 16 doi: 10.1111/dth.12394. PMID: 27526657
Depth and Patterns of Adnexal Involvement in Primary Extramammary (Anogenital) Paget Disease: A Study of 178 Lesions From 146 Patients.
Konstantinova AM, Shelekhova KV, Stewart CJ, Spagnolo DV, Kutzner H, Kacerovska D, Plaza JA, Suster S, Bouda J, Pavlovsky M, Kyrpychova L, Michal M, Guenova E, Kazakov DV
Am J Dermatopathol 2016 Nov;38(11):802-808. doi: 10.1097/DAD.0000000000000552. PMID: 26863064
The treatment of syringomas by CO(2) laser using a multiple-drilling method.
Park HJ, Lee DY, Lee JH, Yang JM, Lee ES, Kim WS
Dermatol Surg 2007 Mar;33(3):310-3. doi: 10.1111/j.1524-4725.2007.33065.x. PMID: 17338688

Recent clinical studies

Etiology

Primary adenocarcinomas of the vulva and related structures: An enigmatic and diverse group of tumors(✰).
Desouki MM, Fadare O
Semin Diagn Pathol 2021 Jan;38(1):71-84. Epub 2020 Sep 25 doi: 10.1053/j.semdp.2020.09.011. PMID: 33032903
Vulval hidradenoma papilliferum: a clinical and dermoscopic study.
Tosti G, Salvini C, Barisani A, Grazi R, Pirola S, Cantisani C, Vaccari S, Madnani N
Clin Exp Dermatol 2020 Dec;45(8):1035-1039. Epub 2020 Oct 6 doi: 10.1111/ced.14254. PMID: 32356582
Syringoma: A clinicopathological study of 244 cases.
Ciarloni L, Frouin E, Bodin F, Cribier B
Ann Dermatol Venereol 2016 Aug-Sep;143(8-9):521-8. Epub 2016 Apr 21 doi: 10.1016/j.annder.2015.06.025. PMID: 27112697
Vulvar adnexal lesions: a 32-year, single-institution review from Massachusetts General Hospital.
Baker GM, Selim MA, Hoang MP
Arch Pathol Lab Med 2013 Sep;137(9):1237-46. doi: 10.5858/arpa.2012-0434-OA. PMID: 23991738
Clinicopathologic characteristics of 12 patients with vulvar sweat gland carcinoma.
Hou JL, Wu LY, Zhang HT, Lv NN, Huang Y, Yu GZ
Int J Gynecol Cancer 2010 Jul;20(5):874-8. doi: 10.1111/IGC.0b013e3181e1c167. PMID: 20606537

Diagnosis

Digital Papillary Adenocarcinoma in Nonacral Skin: Clinicopathologic and Genetic Characterization of 5 Cases.
Kervarrec T, Imbeaud S, Veyer D, Pere H, Puech J, Pekár-Lukacs A, Markiewicz D, Coutts M, Tallet A, Collin C, Berthon P, Bravo IG, Seris A, Jouary T, Macagno N, Touzé A, Cribier B, Battistella M, Calonje E
Am J Surg Pathol 2023 Oct 1;47(10):1077-1084. Epub 2023 Jul 31 doi: 10.1097/PAS.0000000000002096. PMID: 37505796
Benign Tumors and Tumor-like Lesions of the Vulva.
Heller DS
Clin Obstet Gynecol 2015 Sep;58(3):526-35. doi: 10.1097/GRF.0000000000000133. PMID: 26125957
Benign vulvar tumors.
Haley JC, Mirowski GW, Hood AF
Semin Cutan Med Surg 1998 Sep;17(3):196-204. doi: 10.1016/s1085-5629(98)80014-x. PMID: 9759677
Benign vulvar tumors.
Hood AF, Lumadue J
Dermatol Clin 1992 Apr;10(2):371-85. PMID: 1318812
Vulvar sweat gland carcinomas.
Wick MR, Goellner JR, Wolfe JT 3rd, Su WP
Arch Pathol Lab Med 1985 Jan;109(1):43-7. PMID: 2982338

Therapy

Topical imiquimod in the treatment of extramammary Paget's disease: A 10 year retrospective analysis in an Asian tertiary centre.
Liau MM, Yang SS, Tan KB, Aw CW
Dermatol Ther 2016 Nov;29(6):459-462. Epub 2016 Aug 16 doi: 10.1111/dth.12394. PMID: 27526657
Eccrine porocarcinoma with carcinomatous lymphangitis in a patient with history of arsenic exposure: a case report.
Ramirez Y, Fellegara G, Bugiani M
Int J Surg Pathol 2012 Oct;20(5):515-8. Epub 2011 Nov 21 doi: 10.1177/1066896911428738. PMID: 22108500
Multiple eccrine hidrocystomas of the vulva.
Ozkan Z
Int J Gynaecol Obstet 2009 Apr;105(1):65. Epub 2008 Dec 25 doi: 10.1016/j.ijgo.2008.11.009. PMID: 19110246
Vulvar syringoma: a clinicopathologic and immunohistologic study of 18 patients and results of treatment.
Huang YH, Chuang YH, Kuo TT, Yang LC, Hong HS
J Am Acad Dermatol 2003 May;48(5):735-9. doi: 10.1067/mjd.2003.285. PMID: 12734503
Apocrine carcinoma of the vulva in a band-like arrangement with inflammatory and telangiectatic metastasis via local lymphatic channels.
Kiyohara T, Kumakiri M, Kawami K, Kouraba S, Takeuchi A, Sawai T, Lao LM, Ansai S, Hashimoto K
Int J Dermatol 2003 Jan;42(1):71-4. doi: 10.1046/j.1365-4362.2003.01568.x. PMID: 12581149

Prognosis

Adenoid Cystic Carcinoma (ACC) of the Bartholin's gland misdiagnosed for three times: a case report.
Alhashemi M, Oubari S, Mohamad AH, Alhashemi M, Kabel O, Alhelue A, Fattal F
BMC Womens Health 2023 Apr 3;23(1):156. doi: 10.1186/s12905-023-02320-4. PMID: 37013513Free PMC Article
Eccrine porocarcinoma of the vulva: a case report and review of the literature.
Fujimine-Sato A, Toyoshima M, Shigeta S, Toki A, Kuno T, Sato I, Watanabe M, Niikura H, Yaegashi N
J Med Case Rep 2016 Nov 10;10(1):319. doi: 10.1186/s13256-016-1106-1. PMID: 27832810Free PMC Article
Clinicopathologic characteristics of 12 patients with vulvar sweat gland carcinoma.
Hou JL, Wu LY, Zhang HT, Lv NN, Huang Y, Yu GZ
Int J Gynecol Cancer 2010 Jul;20(5):874-8. doi: 10.1111/IGC.0b013e3181e1c167. PMID: 20606537
Vulvar syringoma: a clinicopathologic and immunohistologic study of 18 patients and results of treatment.
Huang YH, Chuang YH, Kuo TT, Yang LC, Hong HS
J Am Acad Dermatol 2003 May;48(5):735-9. doi: 10.1067/mjd.2003.285. PMID: 12734503
Microcystic adnexal carcinoma of the vulva.
Buhl A, Landow S, Lee YC, Holcomb K, Heilman E, Abulafia O
Gynecol Oncol 2001 Sep;82(3):571-4. doi: 10.1006/gyno.2001.6300. PMID: 11520158

Clinical prediction guides

The treatment of syringomas by CO(2) laser using a multiple-drilling method.
Park HJ, Lee DY, Lee JH, Yang JM, Lee ES, Kim WS
Dermatol Surg 2007 Mar;33(3):310-3. doi: 10.1111/j.1524-4725.2007.33065.x. PMID: 17338688
Vulvar syringoma, report of a case and review of the literature.
Miranda JJ, Shahabi S, Salih S, Bahtiyar OM
Yale J Biol Med 2002 Jul-Aug;75(4):207-10. PMID: 12784970Free PMC Article
Mammary-like glands of the vulva and their disorders.
van der Putte SC
Int J Gynecol Pathol 1994 Apr;13(2):150-60. doi: 10.1097/00004347-199404000-00009. PMID: 8005737
Benign pigmented apocrine vulvar hamartomas.
Stephens-Groff SM, Hansen RC, Bangert J
Pediatr Dermatol 1993 Jun;10(2):123-4. doi: 10.1111/j.1525-1470.1993.tb00035.x. PMID: 8346101
Anogenital "sweat" glands. Histology and pathology of a gland that may mimic mammary glands.
van der Putte SC
Am J Dermatopathol 1991 Dec;13(6):557-67. PMID: 1666822

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