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Wu F, Andaleeb U, Ahmed I. Extramammary breast cancer of the vulva. BMJ Case Rep 2024; 17:e259567. [PMID: 38955387 DOI: 10.1136/bcr-2023-259567] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 07/04/2024] Open
Abstract
A woman in her 70s was seen in the gynaecology outpatient clinic with a swelling on the right side of the vulva. Surgical excision of the lesion revealed unexpectedly an extensive ductal carcinoma in situ with a focus of a grade 2 invasive ductal carcinoma arising in extramammary breast tissue of the vulva. Postoperative staging studies showed normal breasts, with no evidence of disease elsewhere. The patient underwent a wider excision of the right vulva and sentinel node biopsy of the right inguinal region, which revealed no further disease. The patient is currently taking adjuvant hormonal therapy and has remained disease free at 2-year follow-up. This case underscores the importance of considering rare presentations of vulvar malignancies and the necessity for a multidisciplinary approach in managing such cases.
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Affiliation(s)
- Fiona Wu
- Department of Surgery, Medway NHS Foundation Trust, Gillingham, UK
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Mansour M, Zahra O, Nabulsi D, Alhamwi A, Chahin M, alani WR, Yousef D, Kanbour I, Kurdi B, Mohammad Deeb A. Ectopic primary ductal breast carcinoma of the vulva: a case report and literature review. Ann Med Surg (Lond) 2023; 85:5138-5144. [PMID: 37811111 PMCID: PMC10553189 DOI: 10.1097/ms9.0000000000001160] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/20/2023] [Accepted: 08/02/2023] [Indexed: 10/10/2023] Open
Abstract
Background Ectopic breast tissue (EBT) is normally found along the rudimentary mammary line, which is located from the axilla to the inguinal region and disappears during embryogenesis. Up to 6% of females around the world suffer from EBT. EBT cancer is rare, with only a few reports in the literature. The diagnosis, and treatment of these rare cases can be very difficult and complex. Case presentation The authors report an interesting case of a 74-year-old female patient with a vulvar lump located on the mons pubis associated with ulceration, pain, and size increase. The histopathological study demonstrated an invasive ductal carcinoma grade 2 arising from EBT in the vulva. Within 4 months of follow-up, the patient's condition was stable. Clinical discussion and conclusions Throughout this paper, the authors aim to highlight the diagnostic and therapeutic challenges since there are no current guidelines for treatment for such cases. Furthermore, this article describes this rare disease and includes surgical details, difficulties, diagnostic methods, and treatment options.
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Affiliation(s)
- Marah Mansour
- Department of Colorectal Surgery, Mayo Clinic, Rochester, MN
- Faculty of Medicine, Tartous University, Tartous
| | - Orgwan Zahra
- Faculty of Medicine, Ankara University, Ankara, Turkey
| | - Dalia Nabulsi
- Faculty of Medicine, Kuwait University, Jabriya, Kuwait
| | | | | | | | - Diaa Yousef
- Faculty of Medicine, Aleppo University, Aleppo
| | - Ilda Kanbour
- Department of Gynecology, Obstetrics University Hospital, Damascus
| | - Bashar Kurdi
- Department of Gynecology, Obstetrics University Hospital, Damascus
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Buitrago-Flechas SM, Barrera-Latorre SJ, Morante-Caicedo C. Ectopic mammary tissue in vulva: case report and systematic literature review. REVISTA COLOMBIANA DE OBSTETRICIA Y GINECOLOGIA 2021; 72:271-290. [PMID: 34851570 PMCID: PMC8629372 DOI: 10.18597/rcog.3593] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 08/13/2020] [Accepted: 09/04/2021] [Indexed: 11/26/2022]
Abstract
Objective To report the case of a patient diagnosed with ectopic mammary tissue in the vulva, and to conduct a literature review of the diagnosis, treatment and prognosis of this condition in that location. Materials and Methods A 49-year-old patient who presented with a painful vulvar mass to a private intermediate complexity center in Bogotá, Colombia. The lesion was assessed on ultrasound and then surgically excised; histopathology showed ectopic mammary tissue with absence of malignancy. A search was conducted in the PubMed, Embase, Cochrane, LILACS and Scielo databases using the keywords “Vulva,” “Breast” and “Ectopic.” Case reports and case series of women with histopathology-confirmed mammary tissue in the vulva were included. Results Overall, 184 titles were identified and, of these, 94 were ultimately included, for a total of 126 cases, with 57.9% being benign tumors, 95% in women under 50 years of age, and 42.06% being malignant tumors, 92% in women over 50 years of age. Diagnosis was made on the basis of the clinical findings, with ancillary diagnostic imaging, tumor markers and immunohistochemistry in some cases. Local excision was performed in 91% of cases with benign pathology and in 43% of cases with malignant pathology, with the diagnostic method being therapeutic. Conclusions Ectopic mammary tissue in the vulva must be considered as part of the differential diagnosis of vulvar masses, prognosis being different in pre and postmenopausal women. Further studies are needed to enhance the characterization of this condition and define the ideal course of treatment in terms of relapse and survival.
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Ananthula A, Lockwood B, Savage J, Malak S, Chen C, Makhoul I, Pennisi A. Primary Breast Carcinoma of the Vulva Metastatic to Lymph Nodes and Bones: A Case Report and Literature Review. Perm J 2020; 24:19.084. [PMID: 32097114 DOI: 10.7812/tpp/19.084] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Abstract
INTRODUCTION Primary breast carcinoma can occur at ectopic sites. The axilla is the most common site of ectopic primary breast cancer, but presentation in the vulva is rare. We discuss a rare presentation of primary breast carcinoma of the vulva with distant lymph node and bone metastases in a premenopausal woman. CASE PRESENTATION A vulvar malignancy consistent with adenocarcinoma of the mammary gland type was diagnosed in a 47-year-old premenopausal woman. The patient underwent radical vulvectomy with bilateral superficial and deep inguinal lymphadenectomy. The tumor was positive for estrogen receptor and negative for progesterone receptor and human epidermal growth factor receptor 2/neu on immunohistochemical findings. A positron emission tomography-computed tomography scan demonstrated lymph node and bone metastases. Her disease was treated as stage IV breast cancer with metastases to the bone. Palliative treatment with ovarian suppression, aromatase inhibitor, and cyclin-dependent kinase 4/6 inhibitor was recommended. DISCUSSION For a diagnosis of primary breast cancer of the vulva, a thorough metastatic workup should be performed, with attention directed toward detecting a breast primary disease by results of the history, physical examination, and radiologic examination of the breasts mainly to help confirm that the vulvar lesion is the primary site as opposed to metastasis from a breast primary cancer and also for staging. Management of this rare entity is challenging because of a lack of specific guidelines, and treatment, therefore, is similar to that of breast cancer.Treatment should consist of an individualized combination of surgery, radiotherapy, chemotherapy, and antiestrogen hormonal therapy.
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Affiliation(s)
- Aneesha Ananthula
- Department of Internal Medicine, University of Arkansas for Medical Sciences, Little Rock
| | - Blake Lockwood
- Department of Hematology/Oncology, University of Arkansas for Medical Sciences, Little Rock
| | - John Savage
- Department of Gynecologic Oncology, University of Arkansas for Medical Sciences, Little Rock
| | - Sharp Malak
- Department of Radiology and Epidemiology, University of Arkansas for Medical Sciences, Little Rock
| | - Chien Chen
- Department of Pathology, University of Arkansas for Medical Sciences, Little Rock
| | - Issam Makhoul
- Department of Hematology/Oncology, University of Arkansas for Medical Sciences, Little Rock
| | - Angela Pennisi
- Department of Hematology/Oncology, University of Arkansas for Medical Sciences, Little Rock
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Matak L, Dukić B, Tupek T, Lisica-Šikić N, Mikuš M. Primary ectopic lobular breast cancer of the vulva: case report and review of literature. J OBSTET GYNAECOL 2019; 40:727-730. [PMID: 31352845 DOI: 10.1080/01443615.2019.1623182] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/26/2022]
Affiliation(s)
- Luka Matak
- Department of Gynecology and Obstetrics, Zadar General Hospital, Zadar, Croatia
| | - Branko Dukić
- Department of Gynecology and Obstetrics, Zadar General Hospital, Zadar, Croatia
| | - Tvrtko Tupek
- Department of Gynecology and Obstetrics, Clinical Hospital Sveti Duh, Zagreb, Croatia
| | | | - Mislav Mikuš
- Department of Gynecology and Obstetrics, University Hospital Centre Zagreb, Zagreb, Croatia
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Ishigaki T, Toriumi Y, Nosaka R, Kudou R, Imawari Y, Kamio M, Nogi H, Shioya H, Takeyama H. Primary ectopic breast cancer of the vulva, treated with local excision of the vulva and sentinel lymph node biopsy: a case report. Surg Case Rep 2017; 3:69. [PMID: 28510222 PMCID: PMC5433958 DOI: 10.1186/s40792-017-0343-x] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/15/2017] [Accepted: 05/05/2017] [Indexed: 02/07/2023] Open
Abstract
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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Affiliation(s)
- Takayuki Ishigaki
- Department of Breast and Endocrine Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-shinbashi, Minato-ku, Tokyo, 105-8461, Japan.
| | - Yasuo Toriumi
- Department of Breast and Endocrine Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Ryouko Nosaka
- Department of Breast and Endocrine Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Rei Kudou
- Department of Breast and Endocrine Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Yoshimi Imawari
- Department of Breast and Endocrine Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Makiko Kamio
- Department of Breast and Endocrine Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Hiroko Nogi
- Department of Breast and Endocrine Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Hisashi Shioya
- Department of Breast and Endocrine Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Hiroshi Takeyama
- Department of Breast and Endocrine Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-shinbashi, Minato-ku, Tokyo, 105-8461, Japan
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Kredentser AM, Kredentser DC. Adenocarcinoma of the Vulva Arising in Ectopic Breast Tissue: A Case Series and Literature Review. J Gynecol Surg 2017. [DOI: 10.1089/gyn.2016.0025] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022] Open
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Cripe J, Eskander R, Tewari K. Sentinel lymph node mapping of a breast cancer of the vulva: Case report and literature review. World J Clin Oncol 2015; 6:16-21. [PMID: 25866706 PMCID: PMC4390890 DOI: 10.5306/wjco.v6.i2.16] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/24/2014] [Revised: 01/29/2015] [Accepted: 02/12/2015] [Indexed: 02/06/2023] Open
Abstract
Ectopic breast tissue is rare and typically presents as an axillary mass. Previous reports have identified ectopic breast tissue in the vulva, but malignancy is exceedingly uncommon. We present a 62 years old with locally advanced breast carcinoma arising in the vulva demonstrates the utilization of sentinel lymph node mapping to identify metastatic lymph nodes previously unable to be identified via traditional surgical exploration. Our case supports the principles of adjuvant therapy for breast cancer to be applied to ectopic breast cancer arising in the vulva. A literature review highlights common key points in similar cases to guide management.
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