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      Fibroadenoma como causa de tumor en la vulva. Presentación de caso Translated title: Fibroadenoma as the cause of tumor in the vulva. A case report

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          Abstract

          RESUMEN Introducción: El fibroadenoma es una lesión bastante común en mama, pero su localización en la región vulvar es extremadamente rara, con alrededor de sesenta casos, descritos en la literatura. Se presenta como una masa unilateral, subcutánea y asintomática, de crecimiento insidioso. El tamaño de este tipo de lesión varía entre 1 y 12 cm en los casos reportados hasta la fecha. A pesar de ser infrecuentes, las tumoraciones a nivel vulvar continúan siendo importante motivo de consulta. La histogénesis de este tipo de lesiones ha sido tema de discusión en los últimos años, sobresalen dos teorías: la presencia de tejido mamario ectópico, y la degeneración tumoral de un grupo de glándulas similares a tejido mamario, descritas como componente habitual de la región. Objetivo: Aportar un diagnóstico diferencial polémico de tumores vulvares. Presentación del caso: Se presenta una paciente de 22 años con diagnóstico histopatológico de fibroadenoma de vulva. Se realiza exéresis de la lesión, la cual al examen macroscópico midió aproximadamente 15 cm en su eje longitudinal (la más grande reportada en la literatura). Conclusiones: A pesar de su baja frecuencia de presentación, el fibroadenoma de vulva es una entidad a tener en cuenta como diagnóstico diferencial de los tumores subcutáneos de localización vulvar.

          Translated abstract

          ABSTRACT Introduction: Fibroadenoma is a fairly common lesion in the breast tissue, but its location in the vulvar region is extremely rare, with about 60 specific cases in the literature. It is presented as a unilateral, subcutaneous and asymptomatic mass, with insidious growth. The size of this type of lesion is between 1 and 12 cm in the cases reported to date. Despite being infrequent, vulvar tumors continue to be important reasons for medical consultation. The histogenesis of this type of lesions has been the subject of discussion in recent years; two theories stand out: the presence of ectopic breast tissue, and tumor degeneration of a group of glands similar to breast tissue described as a common component of the region. Objective: To providea polemic differencial diagnosis of vulvar tumors. Case presentation: We present a 22-year-old patient with histopathological diagnosis of vulvar fibroadenoma. Excision of the lesion was performed, which at the macroscopic examination measured approximately 15 cm in its longitudinal axis (the largest reported in the literature). Conclusions: Despite its low frequency of presentation, vulvar fibroadenoma needs to be considered as differencial diagnosis of vulvar tumors.

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          Most cited references20

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          Mammary-like gland adenoma of the vulva: review of 46 cases.

          Hidradenoma papilliferum (HP) of the anogenital region, which was previously thought to be an apocrine tumour, is now believed to be derived from anogenital mammary-like glands (MLG) and is more accurately termed MLG adenoma. We sought to explore any ramifications that may have resulted from the perceived change in histogenesis of this tumour. We performed a clinicopathological audit of 46 cases. The mean age was 52 years and the range 31-90 years. Symptoms occurred in 23%, comprising nodules of increasing size 9%, pruritus 9% and bleeding 6%. Tumours occurred in the known distribution of mammary-like glands. The labia minora accounted for 50%, labia majora 40%, fourchette 7% and clitoris 3%. Tumours were described clinically as cystic in 42%, ulcerated 33% and solid 25%. Histologically, adjacent normal MLG were often present. There was striking diversity in histology. Tubular, papillary, cystic and solid areas were seen in various combinations. Two cell types, epithelial and myoepithelial, were present. The most common epithelial cell, the ductal cell, was seen alone in 43% or associated with apocrine metaplasia (57%) and/or foam cells (13%) and/or squamous cells (13%). Myoepithelial cells were usually flattened, but were prominent and clear cell in type in 11%. Stroma was variable in amount and either desmoplastic or sclerotic. Inflammatory cells were particularly associated with tumours involving the surface. Unusual architectural patterns resembled breast lesions such as erosive adenomatosis, sclerosing adenosis and ductal adenoma. No recurrence or association with malignancy was recorded. MLG adenomas demonstrate a marked diversity in histological pattern and cell morphology. The ductal cell and a site compatible with and/or the presence of adjacent normal MLG are the most characteristic features. Unusual vulvar tumours, which have been previously reported as erosive adenomatosis, sclerosing adenosis, papillary adenofibroma, syringocystadenoma papilliferans, etc., are variants of MLG adenomas.
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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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              Primary ectopic breast cancer of the vulva, treated with local excision of the vulva and sentinel lymph node biopsy: a case report

              Primary breast cancer fairly infrequently occurs in ectopic breast tissue, and primary ectopic breast cancer of the vulva is particularly rare. Only 26 cases have been published in the English-language literature, and there has been no report of primary breast carcinoma of the vulva in Japan. We report a rare case of primary ectopic breast cancer of the vulva that was treated with local excision of the vulva and sentinel lymph node biopsy (SLNB). The patient was a 72-year-old woman who had noticed a right vulvar tumor 10 years earlier. The tumor was excised by the Department of Plastic Surgery of our hospital. The histology of the vulvar tumor revealed an invasive ductal carcinoma of the breast, and immunohistochemical staining of the vulvar specimen showed the tumor cells to be 100% estrogen-receptor-positive and 100% progesterone-receptor-positive. All margins of resection were positive for neoplastic involvement. An additional local excision of the vulva and right inguinal SLNB were performed in our department. The intraoperative frozen section was negative for metastasis, and lymph node dissection was not performed. The final pathology was negative for residual disease, and a partially normal ductal component was present. Adjuvant hormonal therapy with an aromatase inhibitor was indicated post-operatively. The patient was asymptomatic and free of detectable disease at a 6-month follow-up. Due to the rarity of this diagnosis, there are no established guidelines for treatment. Although cases in which SLNB was performed are rare, we consider SLNB to be an effective alternative to inguinal node dissection for ectopic primary breast cancer of the vulva.
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                Author and article information

                Journal
                rhcm
                Revista Habanera de Ciencias Médicas
                Rev haban cienc méd
                Universidad de Ciencias Médicas de la Habana (La Habana, , Cuba )
                1729-519X
                June 2020
                : 19
                : 3
                : e3094
                Affiliations
                [2] La Habana La Habana orgnameUniversidad de Ciencias Médicas de La Habana orgdiv1Instituto de Ciencias Básicas y Preclínicas “Victoria de Girón” Cuba
                [3] Villa Clara orgnameUniversidad de Ciencias Médicas de Villa Clara orgdiv1Facultad de Ciencias Médicas “Dr. Serafín Ruiz de Zárate” Cuba
                [1] Holguín orgnameUniversidad de Ciencias Médicas de Holguín orgdiv1Facultad de Ciencias Médicas “Mariana Grajales Coello” Cuba
                Article
                S1729-519X2020000300008 S1729-519X(20)01900300008
                0990c124-8da3-49c1-9773-84a37e5fb18e

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

                History
                : 01 April 2020
                : 16 December 2019
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 20, Pages: 0
                Product

                SciELO Cuba

                Categories
                CIENCIAS QUIRÚRGICAS

                glándulas anogenitales similares a tejido mamario,vulvar mass,anogenital mammary-like glands,ectopic breast tissue.,Vulvar fibroadenoma,tejido ectópico mamario,masa vulvar,Fibroadenoma de vulva

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