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Vulvar adenocarcinoma of mammary gland type

MedGen UID:
925545
•Concept ID:
C4288013
•
Neoplastic Process
Synonyms: Adenocarcinoma of AGMLGs (anogenital mammary-like glands); Adenocarcinoma of Anogenital Mammary-Like Glands; Adenocarcinoma of anogenital mammary-like glands; Adenocarcinoma of Anogenital Mammary-Type Glands; Adenocarcinoma of the Vulva with Breast Carcinoma Features; Vulvar Adenocarcinoma of Mammary Gland Type
SNOMED CT: Adenocarcinoma of AGMLGs (anogenital mammary-like glands) (1293118009); Adenocarcinoma of anogenital mammary-like glands (1293118009); Adenocarcinoma of AGMLGs (anogenital mammary-like glands) (1293146006); Adenocarcinoma of anogenital mammary-like glands (1293146006)

Definition

A primary invasive malignant epithelial neoplasm of the vulva showing morphological features of recognized breast adenocarcinomas. (WHO, 2014) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Vulvar adenocarcinoma of mammary gland type

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
Study of Selected BRCA1, BRCA2, and PIK3CA Mutations in Benign and Malignant Lesions of Anogenital Mammary-Like Glands.
Konstantinova AM, Shelekhova KV, Imyanitov EN, Iyevleva A, Kacerovska D, Michal M, Kazakov DV
Am J Dermatopathol 2017 May;39(5):358-362. doi: 10.1097/DAD.0000000000000725. PMID: 28291131

Diagnosis

Adenocarcinoma of anogenital mammary gland type arising from encapsulated papillary carcinoma: A rare vulvar tumor mimicking breast carcinoma.
Tang H, Laskin WB, Luan Y, McNiff JM, Zhan H
J Cutan Pathol 2024 Aug;51(8):604-608. Epub 2024 May 6 doi: 10.1111/cup.14639. PMID: 38711196
Case report: Primary vulvar adenocarcinoma of mammary gland type-its genetic characteristics by focused next-generation sequencing.
Hu L, Tiesinga J
Pathol Oncol Res 2024;30:1611376. Epub 2024 Mar 20 doi: 10.3389/pore.2024.1611376. PMID: 38572338Free PMC Article
Nonsquamous Malignancies of Vagina and Vulva: 23-Year Experience at a Tertiary Center in the United Kingdom.
Elshennawy RA, Aird C, Boyle W, Yap J, Williams A, Ganesan R
Int J Gynecol Pathol 2023 Jul 1;42(4):327-337. Epub 2022 Oct 10 doi: 10.1097/PGP.0000000000000924. PMID: 36731045
Diagnosis and management of primary vulvar adenocarcinoma of mammary gland type: report of two distinct cases.
Morais M, Vaz Silva J, Tavares MV
BMJ Case Rep 2022 Jun 7;15(6) doi: 10.1136/bcr-2021-245580. PMID: 35672058Free PMC Article
Primary Breast Carcinoma of the Vulva Metastatic to Lymph Nodes and Bones: A Case Report and Literature Review.
Ananthula A, Lockwood B, Savage J, Malak S, Chen C, Makhoul I, Pennisi A
Perm J 2020;24 Epub 2020 Feb 14 doi: 10.7812/TPP/19.084. PMID: 32097114Free PMC Article

Therapy

Adenocarcinoma of mammary-like glands in the vulva successfully treated by weekly paclitaxel.
Tanaka H, Umekawa T, Nagao K, Ishihara A, Toyoda N
Int J Gynecol Cancer 2005 May-Jun;15(3):568-71. doi: 10.1111/j.1525-1438.2005.15328.x. PMID: 15882189

Prognosis

Study of Selected BRCA1, BRCA2, and PIK3CA Mutations in Benign and Malignant Lesions of Anogenital Mammary-Like Glands.
Konstantinova AM, Shelekhova KV, Imyanitov EN, Iyevleva A, Kacerovska D, Michal M, Kazakov DV
Am J Dermatopathol 2017 May;39(5):358-362. doi: 10.1097/DAD.0000000000000725. PMID: 28291131
Adenocarcinoma of mammary-like glands in the vulva successfully treated by weekly paclitaxel.
Tanaka H, Umekawa T, Nagao K, Ishihara A, Toyoda N
Int J Gynecol Cancer 2005 May-Jun;15(3):568-71. doi: 10.1111/j.1525-1438.2005.15328.x. PMID: 15882189

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