Case Reports

Primary Extra Mammary Paget’s Disease of Vulva, With Apocrine Adenocarcinoma, Signet Ring Cell Differentiation and Distant Metastasis

Abstract

Objective: Extramammary Paget's disease (EMPD) with invasive carcinoma and distant metastasis is extremely rare. In vulva EMPD associated apocrine carcinoma with signet ring cell differentiation has not been described in the literature so far. Its slow evolution, varied clinical presentation and histological appearances, lead to difficulty in diagnosis of this disease.
Case report: We hereby report a case of primary EMPD with invasive carcinoma and distant metastasis in a 59-year-old female who presented with erythematous indurated plaque over vulva. Histopathology revealed Paget cell infiltration throughout the epidermis with invasive carcinoma in dermis and liver metastasis on CECT. The immunohistochemical expressions of CK7, CK20, GCDFP-15, CEA, p40, CDX 2, Her-2/ neu, AR, ER, were examined to explicate the cellular differentiation of this carcinoma. According to the histological assessment, this case was diagnosed as primary EMPD with apocrine adenocarcinoma, signet ring cell differentiation, vulva.
Conclusion: Owing to poor prognosis, a high index of clinical suspicion along with histological and immunohistochemical assessment is of utmost importance in arriving at final diagnosis.

1. Zhou S, Zhong W, Mai R, Zhang G. Mammary and Extramammary Paget's Disease Presented Different Expression Pattern of Steroid Hormone Receptors. Biomed Res Int 2017; 2017: 3768247.
2. Lopes Filho LL, Lopes IM, Lopes LR, Enokihara MM, Michalany AO, Matsunaga N. Mammary and extramammary Paget's disease. An Bras Dermatol 2015; 90: 225-31.
3. Hsieh GL, English DP, Powen Tu, Folkins AK, Karam AK. Case of Metastatic Extramammary Paget Disease of the Vulva Treated Successfully with Trastuzumab Emtansine. JCO Precision Oncology 2018; 2: 1-8.
4. Uchimiya H, Yonekura K, Hashiguchi T, Matsushita S, Kanekura T. Extramammary Paget's disease with prominent signet-ring cells. J Dermatol 2009; 36: 557-8.
5. Grelck KW, Nowak MA, Doval M. Signet ring cell perianal paget disease: loss of MUC2 expression and loss of signet ring cell morphology associated with invasive disease. Am J Dermatopathol 2011; 33: 616-20.
6. Terada T. Extramammary Paget Disease of Signet Ring Cell Carcinoma Type: A Case Report. Am J Dermatopathol 2016; 38: 249-51.
7. Suthiwartnarueput W. Primary perianal Paget's disease with focal adenocarcinoma, signet-ring cell differentiation and unusual immunohistochemical expression: a case report. J Med Assoc Thai 2015; 98: S162-8.
8. Paget J. On the disease of the mammary areola preceding cancer of the mammary gland. St Bartholomew’s Hosp Rep 1874; 10: 87–9.
9. Martin VG, Pellettiere EV, Gress D, Miller AW. Paget's disease in an adolescent arising in a supernumerary nipple. J Cutan Pathol 1994; 21: 283-6.
10. Wilkinson EJ, Brown HM. Vulvar Paget disease of urothelial origin: a report of three cases and a proposed classification of vulvar Paget disease. Hum Pathol 2002; 33: 549-54.
11. Kurman RJ, Ellenson LH, Ronnett BM. Blaustein's Pathology of the Female Genital Tract. 6th ed. New York. Springer-Verlag, 2019; 7: 81-6.
12. Delport ES. Extramammary Paget's disease of the vulva: An annotated review of the current literature. Australas J Dermatol 2013; 54: 9-21.
13. Karam A, Dorigo O. Treatment outcomes in a large cohort of patients with invasive Extramammary Paget's disease. Gynecol Oncol 2012; 125: 346-51.
14. Ohno T, Nakano T, Abe A, Sano T, Niibe Y, Oka K. Mucinous adenocarcinoma of Bartholin gland treated with radiation therapy: a case report. Jpn J Clin Oncol 2001; 31: 226-30.
15. Warkel RL, Helwig EB. Apocrine gland adenoma and adenocarcinoma of the axilla. Arch Dermatol 1978; 114: 198-203.
16. Jung HR, Kwon SY, Son D. Apocrine Carcinoma of the Axilla Associated with Extramammary Paget's Disease: A Case Report and Review of the Literature. J Pathol Transl Med 2015; 49: 535-7.
17. Berdugo J, Dumont-Mackay V, Brissy-Lachery S, Leclerc YE, Doyon J, Mitchell A, et al. Cutaneous Apocrine Carcinoma with an in Situ Component and Histiocytoid and Signet-Ring Cells. Am J Dermatopathol 2017; 39: e76-8.
18. Iwaya M, Uehara T, Yoshizawa A, Kobayashi Y, Momose M, Honda T, et al. A case of primary signet-ring cell/histiocytoid carcinoma of the eyelid: immunohistochemical comparison with the normal sweat gland and review of the literature. Am J Dermatopathol 2012; 34: e139-45.
19. Piris A, Peng Y, Boussahmain C, Essary LR, Gudewicz TM, Hoang MP. Cutaneous and mammary apocrine carcinomas have different immunoprofiles. Hum Pathol 2014; 45: 320-6.
20. Vasilakaki T, Skafida E, Moustou E, Grammatoglou X, Arkoumani E, Koulia K, et al. Primary cutaneous apocrine carcinoma of sweat glands: a rare case report. Case Rep Oncol 2011; 4: 597–601.
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IssueVol 14, No 4 (December 2020) QRcode
SectionCase Reports
DOI https://doi.org/10.18502/jfrh.v14i4.5213
Keywords
Extramammary Paget's Disease Vulva Apocrine Carcinoma Signet Ring Cell
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How to Cite
1.
Rathore R, Yadav D, Agarwal S, Dudani P, Verma K, Mathur S. Primary Extra Mammary Paget’s Disease of Vulva, With Apocrine Adenocarcinoma, Signet Ring Cell Differentiation and Distant Metastasis. J Family Reprod Health. 2020;14(4):276-280.