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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 9  |  Issue : 1  |  Page : 49-51

Primary vulval mucinous adenocarcinoma of intestinal type masquerading as Bartholin's cyst


1 Department of Pathology, Sri Venkateswara Medical College, Hospital and Research Center, Puducherry, India
2 Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
3 Department of Surgical Oncology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India

Date of Submission31-Aug-2021
Date of Decision09-Feb-2022
Date of Acceptance10-Apr-2022
Date of Web Publication14-Jun-2022

Correspondence Address:
Dr. Jinkala Sreerekha
Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry - 605 006
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijamr.ijamr_190_21

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  Abstract 

Vulval carcinomas are rare and account for 3%–5% of female genital tract malignancies. Primary vulval adenocarcinoma of intestinal type is an extremely rare tumor which is considered metastatic until otherwise proven with very few case reports available in the literature. A 58-year-old woman presented with recurrent swelling in the genital region associated with pain. She had a past history of surgery done for Bartholin's cyst which was reported as adenocarcinoma. She underwent radical vulvectomy for the recurrent tumor which showed features of adenocarcinoma with glandular and papillary pattern with abundant extracellular mucin production and immunohistochemical (IHC) features favoring a diagnosis of primary vulval mucinous adenocarcinoma of intestinal type after excluding metastasis from other sites. As morphology cannot differentiate it from metastasis, a thorough investigation to rule out any primary in the gastrointestinal tract with a minimum IHC panel of markers including cytokeratin (CK) 20, CK7, carcinoembryonic antigen, and caudal-related homeobox 2 can help in confirming the diagnosis.

Keywords: Bartholin's cyst, immunohistochemistry, intestinal type, primary vulval mucinous adenocarcinoma


How to cite this article:
Subramanian KS, Sreerekha J, Badhe BA, Penumadu P. Primary vulval mucinous adenocarcinoma of intestinal type masquerading as Bartholin's cyst. Int J Adv Med Health Res 2022;9:49-51

How to cite this URL:
Subramanian KS, Sreerekha J, Badhe BA, Penumadu P. Primary vulval mucinous adenocarcinoma of intestinal type masquerading as Bartholin's cyst. Int J Adv Med Health Res [serial online] 2022 [cited 2022 Oct 7];9:49-51. Available from: https://www.ijamhrjournal.org/text.asp?2022/9/1/49/347468


  Introduction Top


Vulval carcinoma is rare and accounts for 3%–5% of female genital tract malignancies.[1] About 2/3rd of the cases affect older women of postmenopausal age. Squamous cell carcinomas are the most common type (86%–90%) of vulval carcinomas; adenocarcinoma is rare and accounts for 8%–9% of all such tumors.[2],[3] Primary vulval adenocarcinoma of intestinal type is extremely rare and should be considered metastatic until proven otherwise.[4] Different theories have been postulated to explain the origin of vulval intestinal-type adenocarcinoma. Only very few cases of primary vulval adenocarcinomas of intestinal type have been described in the literature.[3] We report a rare case of intestinal type primary vulval mucinous adenocarcinoma in a 58-year-old postmenopausal woman.


  Case Report Top


A 58-year-old woman with no known comorbidities presented to our hospital with chief complaints of recurrent swelling in the genital region associated with pain in the perineal region. There were no associated urinary complaints. She had been previously operated for a swelling at the same site, with preoperative diagnosis of Bartholin's cyst, and wide local excision was done. Histopathology of the wide local excision specimen showed an adenocarcinoma. She received postoperative radiotherapy for 1 month following which the patient presented to our hospital with complaints of pain and recurrent swelling in the genital region. On examination, there was an ulcerated lesion with surrounding induration in the left labia majora and minora measuring 3 cm × 4 cm. Magnetic resonance imaging showed an ill-defined 3.4 cm × 3.6 cm × 2.4 cm lesion centered over the left vulva. Radical vulvectomy with primary closure and bilateral inguinal block dissection was done. The vulvectomy specimen showed an ulceroproliferative growth involving the left posterior labia majora and minora measuring 3 cm × 3 cm × 2.5 cm extending to the right side. Microscopy of the tumor showed features of adenocarcinoma with glandular and papillary patterns with abundant extracellular mucin production [Figure 1]a and [Figure 1]b. The surface of the tumor showed ulceration, and the continuity with the surface epithelium could not be identified. On immunohistochemistry, these tumor cells were positive for cytokeratin (CK) 7, CK20, CK8, caudal-related homeobox 2 (CDX2), and carcinoembryonic antigen (CEA), suggesting an intestinal differentiation [Figure 1]c, [Figure 1]d, [Figure 1]e, [Figure 1]f. p63, calponin, estrogen receptor, and progesterone receptor were negative, thus ruling out tumors of adnexal origin and malignancy arising in ectopic breast tissue or metastasis from the breast. Single left inguinal lymph node showed metastasis. Since the tumor cells showed immunohistochemical (IHC) profile of intestinal origin, a thorough investigation was done to rule out primary in the gastrointestinal tract. Upper and lower gastrointestinal endoscopy was done and did not reveal any abnormality. Thus, a final diagnosis of primary vulval mucinous adenocarcinoma of intestinal type was considered. The patient is currently on follow-up and doing well.
Figure 1: (a) vulval adenocarcinoma with glandular and focal papillary pattern (H and E × 40); (b) Excess extracellular mucin production by the tumor (H and E × 40); (c) Strong CD20 positivity (IHC × 200); (d) focal CK7 positivity (IHC × 100); (e) strong CDX2 positivity (IHC × 100); (f) strong CEA positivity (IHC × 100). IHC = Immunohistochemical, CDX2 = Caudal-related homeobox 2

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  Discussion Top


Adenocarcinoma of vulva is rare and accounts for 8%–9% of vulval carcinomas.[4] Primary adenocarcinomas in vulva most often arise from the Bartholin's glands but may also originate in the cutaneous eccrine or apocrine glands, Skene's glands, minor vestibular glands, ectopic mammary tissue, or even from endometriotic implants. Metastases to vulva are commonly from gastrointestinal tract, breast, and pancreas. Before diagnosing primary vulval adenocarcinoma, metastasis from these sites should be excluded.

The etiology of an adenocarcinoma of the intestinal type in the female genital tract is still under debate. Several theories have been described; they hypothesize that adenocarcinoma of vulva can arise from (1) intestinal metaplasia in foci of adenosis arising in tissues of Mullerian origin, (2) heterotopic intestinal tissue, (3) cloacal remnants, (4) mesonephric remnants, and (5) endometriosis.[5] Adenocarcinoma arising from cloacal remnants is usually localized at the posterior border of the introitus. Mesonephric remnants are most often situated deep in the lateral walls of the vagina.[6]

As the lesion was situated in the posterior region of introitus, a possible origin from cloacal remnants was favored. Histologically, the adenocarcinoma of cloacogenic origin will be in direct continuity with the epidermis and appear morphologically similar to enteric adenocarcinoma with occasional or abundant goblet or Paneth cells. Adenocarcinoma arising from ectopic mammary tissue or from endometriosis will show adjacent normal breast tissue/ductal carcinoma in situ or endometriotic foci, respectively. Adenocarcinoma of adnexal origin can arise from eccrine or apocrine glands. However, morphologically, it is very difficult to differentiate primary vulval adenocarcinoma of intestinal type from metastatic enteric adenocarcinomas and from adenocarcinoma of other origins.

Immunohistochemistry to some extent can help in differentiating these tumors. CDX2, a nuclear transcription factor, is characteristic of enteric differentiation and is found to be positive in both metastatic and primary adenocarcinomas of intestinal type. However, it differentiates adenocarcinomas of other origins. A combination of immunomarkers CEA, CK20, and CK7 can help in the differentiation of metastatic and primary adenocarcinomas of intestinal type.[7] The details of expression of IHC markers in primary vulval adenocarcinoma of various origins are illustrated in the flowchart [Chart 1]. Kurita et al. also have highlighted the use of IHC staining for CK20 and polyclonal CDX2 in the diagnosis of adenocarcinoma of intestinal type.[8] Ugwu et al. also have reported a case of primary vulval adenocarcinomas of intestinal type and insisted on the importance of differentiating from metastatic tumors.[9]



Adenocarcinoma of vulva arising from cloacal remnants is most commonly encountered. Thus far, seven cases have been reported in the posterior region favoring the hypothesis of cloacogenic origin.[3] It is postulated that during embryogenesis, the anorectal tissue is incorporated into the posterior vaginal wall during the division process of the cloaca. These remnants are capable of undergoing dysplastic changes in the presence of a hostile vaginal environment; this may progress to adenocarcinoma.[5]

Vulval adenocarcinoma can be clinically misinterpreted as Bartholin's gland infection, as in our case. Liu et al. have also reported a case of cloacogenic adenocarcinoma of the vulva presenting as recurrent Bartholin's gland infection.[10] Hence, any swelling or recurrent infection of Bartholin's gland should not be ignored. Presentation with lymph node metastasis is rare; to our knowledge, only one case with lymph node metastasis has been reported thus far by Cormio et al.[11]

Management of primary tumor is by surgical resection. For early lesions (primary tumor <2 cm and no inguinal node involvement), wide local resection is the treatment of choice. For primary lesions >2 cm, radical vulvectomy may be necessary, with or without inguinofemoral lymphadenectomy. Chemotherapy is not commonly used to treat vulvar carcinoma, but it is increasingly used in neoadjuvant and palliative settings. Several authors have highlighted the role of neoadjuvant chemotherapy followed by radical surgery as a compelling option for vulvar cancer patients.[12],[13] Radiation has a role if there are two or more inguinal lymph nodes or retroperitoneal lymph node involvement. The clinical behavior of this tumor is unpredictable, with progression-free intervals ranging from 12 to 120 months.[3],[14]


  Conclusion Top


Primary vulval adenocarcinoma of intestinal type is a rare tumor and can mimic Bartholin's gland cyst/infection. All women more than 40 years of age with swelling of the Bartholin's gland need biopsy and evaluation. As morphology cannot differentiate it from metastasis, a thorough investigation to rule out any primary in the gastrointestinal tract with a minimum immunohistochemistry panel of markers including CK20, CK7, CEA, and CDX2 can help in confirming the diagnosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Judson PL, Habermann EB, Baxter NN, Durham SB, Virnig BA. Trends in the incidence of invasive and in situ vulvar carcinoma. Obstet Gynecol 2006;107:1018-22.  Back to cited text no. 1
    
2.
Fuh KC, Berek JS. Current management of vulvar cancer. Hematol Oncol Clin North Am 2012;26:45-62.  Back to cited text no. 2
    
3.
Musella A, Marchetti C, Salerno L, Vertechy L, Iadarola R, Pecorella I, et al. An unexpected complete remission of advanced intestinal-type vulvar adenocarcinoma after neoadjuvant chemotherapy: A case report and a literature review. Case Rep Obstet Gynecol 2013;2013:427141.  Back to cited text no. 3
    
4.
Ren K, Ma X, Wang F, Guo F, Jiang Y, Liu L. Metachronous metastasis from the right colon adenocarcinoma to the vulva: An unusual report and literature review. Int J Clin Exp Pathol 2015;8:1000-6.  Back to cited text no. 4
    
5.
van Wessel S, Van Kerrebroeck H, Van Bogaert V, Tummers P, Van den Broecke R. Primary intestinal type adenocarcinoma of the female genital tract, arisen from a tubulo-villous adenoma: Case report. Gynecol Oncol Case Rep 2013;4:63-5.  Back to cited text no. 5
    
6.
Tjalma WA, Colpaert CG. Primary vaginal adenocarcinoma of intestinal type arising from a tubulovillous adenoma. Int J Gynecol Cancer 2006;16:1461-5.  Back to cited text no. 6
    
7.
Peña-Fernández M, Abdulkader-Nallib I, Novo-Domínguez A, Turrado-Sánchez EM, Brea-Fernández A, Sebio-Lago L, et al. Vaginal tubulovillous adenoma: A clinicopathologic and molecular study with review of the literature. Int J Gynecol Pathol 2013;32:131-6.  Back to cited text no. 7
    
8.
Kurita T, Matuura Y, Hisaoka M, Hachisuga T. Adenocarcinoma of intestinal type of the vulva. Int Cancer Conf J 2019;8:89-93.  Back to cited text no. 8
    
9.
Ugwu AO, Haruna M, Okunade KS, Ohazurike E, Anorlu RI, Banjo AA. Primary vaginal adenocarcinoma of intestinal-type: Case report of a rare gynaecological tumour. Oxf Med Case Reports 2019;2019:omz088.  Back to cited text no. 9
    
10.
Liu SH, Ho CM, Huang SH, Shih BY, Lee FK. Cloacogenic adenocarcinoma of the vulva presenting as recurrent Bartholin's gland infection. J Formos Med Assoc 2003;102:49-51.  Back to cited text no. 10
    
11.
Cormio G, Carriero C, Loizzi V, Gissi F, Leone L, Putignano G, et al. 'Intestinal-type' mucinous adenocarcinoma of the vulva: A report of two cases. Eur J Gynaecol Oncol 2012;33:433-5.  Back to cited text no. 11
    
12.
Woelber L, Trillsch F, Kock L, Grimm D, Petersen C, Choschzick M, et al. Management of patients with vulvar cancer: A perspective review according to tumour stage. Ther Adv Med Oncol 2013;5:183-92.  Back to cited text no. 12
    
13.
Domingues AP, Mota F, Durão M, Frutuoso C, Amaral N, de Oliveira CF. Neoadjuvant chemotherapy in advanced vulvar cancer. Int J Gynecol Cancer 2010;20:294-8.  Back to cited text no. 13
    
14.
Ghamande SA, Kasznica J, Griffiths CT, Finkler NJ, Hamid AM. Mucinous adenocarcinomas of the vulva. Gynecol Oncol 1995;57:117-20.  Back to cited text no. 14
    


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