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Vulvar Adenocarcinoma of Sweat Gland Origin

MedGen UID:
925544
•Concept ID:
C4288012
•
Neoplastic Process
Synonym: Vulvar Adenocarcinoma of Sweat Gland Type

Definition

A rare adenocarcinoma arising from vulvar skin sweat glands. [from NCI]

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
Extramammary Paget's disease of the vulva. A clinicopathologic study of 13 cases.
Lee SC, Roth LM, Ehrlich C, Hall JA
Cancer 1977 Jun;39(6):2540-9. doi: 10.1002/1097-0142(197706)39:6<2540::aid-cncr2820390635>3.0.co;2-1. PMID: 194667

Diagnosis

Malignant spiradenoma/cylindroma of the vulva.
Emam EE, Sawan AS, Al-Tamimi SR, Molah RM
Saudi Med J 2012 Nov;33(11):1229-33. PMID: 23147883
Vulvar apocrine adenocarcinoma: a case with nodal metastasis and intranodal mucinous differentiation.
Alsaad KO, Obaidat N, Dube V, Chapman W, Ghazarian D
Pathol Res Pract 2009;205(2):131-5. Epub 2008 Oct 7 doi: 10.1016/j.prp.2008.07.012. PMID: 18842349
"Pagetoid" eccrine carcinoma of the vulva: report of an unusual case with review of the literature.
Grin A, Colgan T, Laframboise S, Shaw P, Ghazarian D
J Low Genit Tract Dis 2008 Apr;12(2):134-9. doi: 10.1097/LGT.0b013e31815b2156. PMID: 18369309

Therapy

Cytoreductive combination chemotherapy for regionally advanced unresectable extramammary Paget carcinoma.
Voigt H, Bassermann R, Nathrath W
Cancer 1992 Aug 1;70(3):704-8. doi: 10.1002/1097-0142(19920801)70:3<704::aid-cncr2820700327>3.0.co;2-5. PMID: 1320453

Prognosis

Extramammary Paget's disease of the vulva. A clinicopathologic study of 13 cases.
Lee SC, Roth LM, Ehrlich C, Hall JA
Cancer 1977 Jun;39(6):2540-9. doi: 10.1002/1097-0142(197706)39:6<2540::aid-cncr2820390635>3.0.co;2-1. PMID: 194667

Clinical prediction guides

Mammary and extramammary Paget's disease: expression of Ca 15-3, Ka-93, Ca 19-9 and CD44 in Paget cells and adjacent normal skin.
Tsuji T
Br J Dermatol 1995 Jan;132(1):7-13. doi: 10.1111/j.1365-2133.1995.tb08617.x. PMID: 7538778
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
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
Paget's disease of the vulva. A histogenetic study of five cases including ultrastructural observations and review of the literature.
Roth LM, Lee SC, Ehrlich CE
Am J Surg Pathol 1977 Sep;1(3):193-206. doi: 10.1097/00000478-197709000-00001. PMID: 200150
Extramammary Paget's disease of the vulva. A clinicopathologic study of 13 cases.
Lee SC, Roth LM, Ehrlich C, Hall JA
Cancer 1977 Jun;39(6):2540-9. doi: 10.1002/1097-0142(197706)39:6<2540::aid-cncr2820390635>3.0.co;2-1. PMID: 194667

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