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      Apocrine Adenocarcinoma of the Vulva

      case-report
      , , ,
      Rare Tumors
      PAGEPress Publications, Pavia, Italy
      medicine, pathology, tumors, neoplasia, carcinoma

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          Abstract

          Cutaneous vulvar carcinomas are predominantly of squamous cell carcinoma type. Primary vulvar adenocarcinomas are rare with a poorly understood histogenesis. They are classified into extramammary Paget’s disease, sweat gland carcinomas, and breast-like adenocarcinomas of the vulva. Adenocarcinomas, originating from Bartholin glands, can also present as vulvar adenocarcinoma. Rare adenocarcinomas with apocrine features have been described in the literature. The origin of these neoplasms from the native apocrine sweat glands or from anogenital mammary-like glands is still debatable. We report herein a case of a 67 year old female with a rare primary apocrine carcinoma of the vulva.

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          Most cited references11

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          Mammary-like glands of the vulva and their disorders.

          Supernumerary mammary glands derived from rudiments of the embryonic milk lines or mammary ridges in the vulva are considered a source of a series of unusual tumors resembling lesions of the breasts. This phenomenon was reevaluated in light of recent observations of mammary-like anogenital glands (MLG), which are a normal constituent of the vulva and appear to be closely related to eccrine glands. An analysis of the literature reveals that the concept of "milk lines" originated in a mixture of phylogenetic and ontogenetic theories at the beginning of this century and was not supported by observations in human embryos, which show that primordia of the mammary glands do not extend beyond the axillary-pectoral area. The breasts and the vulva are so widely separated by time and space that the vulvar MLG can not be derived from the mammary ridges or milk lines. The profile of the MLG, which can also reveal some eccrine or apocrine features, makes these glands the most likely source of a series of lesions occurring in the anogenital region and comprising lactating glands, lactating adenoma, fibroadenoma, hidrocystoma, hidradenoma papilliferum, and most cases of extramammary Paget's disease and invasive adenocarcinoma.
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            Primary cutaneous apocrine carcinoma: a clinico-pathologic analysis of 24 cases.

            Primary cutaneous apocrine carcinoma is a rare malignancy. This study of 24 examples suggests that the prognosis is not always poor and that grading criteria devised for breast carcinoma may have utility in this group of malignancies. Furthermore, steroid receptor expression should be investigated in these tumors, particularly if a tumor is unlikely to be controlled by surgery alone.
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              Adenocarcinoma of the mammary-like glands of the vulva: a concept unifying sweat gland carcinoma of the vulva, carcinoma of supernumerary mammary glands and extramammary Paget's disease.

              An unusual case of primary adenocarcinoma of the vulva is described. It combined features of the three different types of adenocarcinoma of the skin of the vulva which are currently recognized, i.e. sweat gland carcinoma, adenocarcinoma derived from supernumerary mammary glands, and extramammary Paget's disease (EMPD). Central in this tumor was a recently recognized type of cutaneous gland which appeared special for the anogenital region and was distinguished because it combined morphological features of eccrine, apocrine and mammary glands. As it most resembles mammary glands, it is named "mammary-like gland". On the basis of the case presented and of a critical review of the literature, it was concluded that, with the exception of a few sweat gland carcinomas similar to those elsewhere in the skin, adenocarcinomas of the skin of the vulva form a single category of neoplasms with a variable expression of features reminiscent of eccrine, apocrine and mammary gland carcinomas. The data strongly suggested a common derivation from the mammary-like gland or, in cases of EMPD, its related germinative cells in the epidermis.
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                Author and article information

                Journal
                Rare Tumors
                Rare Tumors
                RT
                Rare Tumors
                PAGEPress Publications, Pavia, Italy
                2036-3605
                2036-3613
                09 September 2013
                01 July 2013
                : 5
                : 3
                : e40
                Affiliations
                Department of Pathology and Molecular Medicine, Mc Master University , Hamilton, Ontario, Canada
                Author notes
                Department of Pathology and Molecular Medicine, Mc Master University, 1280 Main St W, Hamilton, ON L8S 4L8, Canada. Tel. +1.905.525.9140. E-mail: babita.kajal@ 123456medportal.ca
                Contributions: the authors contributed equally.
                Conflict of interests: the authors declare no potential conflict of interests.
                Article
                10.4081/rt.2013.e40
                3804815
                24179652
                8fc02f31-79d1-49db-9228-2ee4c794b37d
                ©Copyright B. Kajal et al.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 5 December 2012
                : 27 May 2013
                : 28 May 2013
                Page count
                Figures: 1, Tables: 0, Equations: 0, References: 13, Pages: 2
                Categories
                Case Report

                Oncology & Radiotherapy
                medicine,pathology,tumors,neoplasia,carcinoma
                Oncology & Radiotherapy
                medicine, pathology, tumors, neoplasia, carcinoma

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