Clinical Image

A unique case of isolated vulvar endometriosis

Ayesha Aziz Ali1
,  
Kelsey Musselman2
,  
Marie Smithgall3
,  
Kristen Pepin4

1 MD, Medical Resident, Department of Obstetrics and Gynecology, New York Presbyterian Hospital/Weill Cornell Medical Center, New York, NY, USA

2 MD, Assistant Clinical Professor, Department of Obstetrics and Gynecology, New York Presbyterian Hospital/Weill Cornell Medical Center, New York, NY, USA

3 Fellow in Pathology and Laboratory Medicine, Division of Pathology and Laboratory Medicine, New York Presbyterian Hospital/Weill Cornell Medical Center, New York, NY, USA

4 Assistant Attending and Assistant Professor, Department of Obstetrics and Gynecology, New York Presbyterian Hospital/Weill Cornell Medical Center, New York, NY, USA

Address correspondence to:

Ayesha Aziz Ali

MD, 525 E 68th St, Suite M7, New York, NY 10065,

USA

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Article ID: 100124Z08AA2022

doi: 10.5348/100124Z08AA2022CI

How to cite this article

Ali AA, Musselman K, Smithgall M, Pepin K. A unique case of isolated vulvar endometriosis. J Case Rep Images Obstet Gynecol 2022;8(2):4–6.

ABSTRACT

No Abstract

Keywords: Endometriosis, Vulvar endometriosis, Vulvar mass

Case Report

A 40-year-old nulligravida woman with longstanding dysmenorrhea and heavy menstrual bleeding underwent computed tomography (CT) and magnetic resonance imaging (MRI), and alongside findings of uterine fibroids was incidentally found to have a 4.0×1.8×1.6 cm right labial soft tissue structure anterior to the right pubic bone (Figure 1A, Figure 1B, Figure 1C). The patient noted that the mass enlarged during her menses, and her exam revealed a palpable, firm, semi-mobile right vulvar mass slightly adherent to the pubic symphysis with no overlying skin abnormalities or lymphadenopathy. Notably, the patient had an inguinal hernia repair and diagnostic gynecologic laparoscopy performed five years prior during which no endometriosis or fibroids were identified.

The patient underwent laparoscopic myomectomy, hysteroscopic endometrial polypectomy, and vulvar mass resection. To excise the vulvar mass, an incision was made with a scalpel, and extended to the level of the mass with electrosurgery. The mass was grasped with allis clamps, and was caully separated from surrounding tissue using electrosurgery. The wound was copiously irrigated and then closed in three layers using vicryl suture. The specimen was sent for permanent pathologic evaluation. Microscopically, the specimen showed fibroadipose tissue containing endometrial stroma and glands with associated chronic inflammation consistent with extensive endometriosis (Figure 2A and Figure 2B). No other lesions suspicious for endometriosis were identified on laparoscopy (Figure 3A, Figure 3B, Figure 3C). On the patient’s post-operative visit, her vulvar incision was noted to be well healed, with no recurrent mass or swelling palpable.

Figure 1: CT and MRI findings. (A) Axial CT with 2 cm soft tissue mass (yellow arrow) anterior to right pubic bone. No enlarged inguinal lymph nodes were identified. (B) T2-weighted axial MRI with ill-defined but mass-like enhancing soft tissue (yellow arrow) in the subcutaneous tissues of the right anterior–superior labia majora and mons pubis measuring 4.0 cm×1.8 cm×1.6 cm. (C) T2-weighted sagittal MRI with labial mass (yellow arrow) and fibroid uterus (blue arrow).
Figure 2: Histologic findings confirming endometriosis. (A) Section of the vulvar mass at low power (2×) shows fibroadipose tissue with extensive endometriosis. (B) High power (10×) shows endometrial stroma and glands within the tissue with associated chronic inflammation.
Figure 3: Laparoscopic findings. (A) No endometriosis identified in posterior cul de sac. (B) Uterus with multiple anterior subserosal and intramural fibroids. No anterior cul de sac endometriosis identified. (C) Uterus status post laparoscopic myomectomy.

Discussion

Endometriosis is a gynecologic disease that involves endometrial glands and stroma occurring outside the uterine cavity, with most common sites including the ovaries, anterior and posterior cul-de-sac, posterior broad ligaments, uterosacral ligaments, uterus, fallopian tubes, sigmoid colon, appendix, and round ligaments [1],[2]. Endometriosis of the vulva is extremely rare, often presenting as vulvodynia or dyspareunia. Very few cases have been reported in the literature, and in most of these case reports either concomitant pelvic disease is present or it is undetermined whether more extensive disease is present as laparoscopy was not performed [3],[4],[5],[6]. In one systematic review, it was noted that 95.3% of patients presenting with vulvo-perineal endometriosis have undergone either episiotomy, perineal trauma, vaginal injury, or vaginal surgery [7]. In most cases, patients presented with cyclical vulvar pain, and were initially thought to have a Bartholin gland cyst. In three reported cases, during marsupialization of what was thought to be a Bartholin cyst, chocolate colored fluid drained from the mass, and the diagnosis was confirmed by pathology [3],[6],[8]. In all reported cases, surgical excision was the management of choice. In one report, the patient received six months of treatment with a luteinizing hormone releasing hormone analogue with a good evolution [8]. As there are few such cases reported in literature, there is no good data to comment on complication or recurrence rates.

Conclusion

Our patient presents with a unique, spontaneous case of isolated vulvar endometriosis unrelated to prior trauma or surgery, with intra-abdominal sites of disease ruled out laparoscopically.

REFERENCE

1.

Gustofson RL, Kim N, Liu S, Stratton P. Endometriosis and the appendix: A case series and comprehensive review of the literature. Fertil Steril 2006;86(2):298–303. [CrossRef] [Pubmed] Back to citation no. 1  

2.

Jenkins S, Olive DL, Haney AF. Endometriosis: Pathogenetic implications of the anatomic distribution. Obstet Gynecol 1986;67(3):335–8. [Pubmed] Back to citation no. 1  

3.

Gocmen A, Inaloz HS, Sari I, Inaloz SS. Endometriosis in the Bartholin gland. Eur J Obstet Gynecol Reprod Biol 2004;114(1):110–1. [CrossRef] [Pubmed] Back to citation no. 1  

4.

Brug P, Gueye NA, Bachmann G. Vulvar endometriosis presenting with dyspareunia: A case report. J Reprod Med 2012;57(3–4):175–7. [Pubmed] Back to citation no. 1  

5.

Eyvazzadeh AD, Smith YR, Lieberman R, Quint EH. A rare case of vulvar endometriosis in an adolescent girl. Fertil Steril 2009;91(3):929.e9–11. [CrossRef] [Pubmed] Back to citation no. 1  

6.

Heijink T, Bogers H, Steensma A. Endometriosis of the Bartholin gland: A case report and review of the literature. J Med Case Rep 2020;14(1):85. [CrossRef] [Pubmed] Back to citation no. 1  

7.

Maillard C, Cherif Alami Z, Squifflet JL, et al. Diagnosis and treatment of vulvo-perineal endometriosis: A systematic review. Front Surg 2021;8:637180. [CrossRef] [Pubmed] Back to citation no. 1  

8.

Hakimi I, Benabdejlil Y, Kouach J, Moussaoui D, Dehayn M. Endometriosis in the Bartholin gland: A case report. J Obstet Gynaecol 2014;2(5):75–6. [CrossRef] Back to citation no. 1  

SUPPORTING INFORMATION

Acknowledgments

The authors would like to thank Dr. Evelyn Cantillo, MD, MPH, Gynecologic Oncologist and Assistant Professor at New York Presbyterian Hospital/Weill Cornell Medical Center for her diligent care of this patient.

Author Contributions

Ayesha Aziz Ali - Acquisition of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Kelsey Musselman - Acquisition of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Marie Smithgall - Analysis of data, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Kristen Pepin - Conception of the work, Design of the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Data Availability Statement

The corresponding author is the guarantor of submission.

Consent For Publication

Written informed consent was obtained from the patient for publication of this article.

Data Availability

All relevant data are within the paper and its Supporting Information files.

Competing Interests

Disclosure statement: The authors declare that they have no conflicts of interest and nothing to disclose. These data have not been published or presented elsewhere and the manuscript is not under review at any other journal.

Copyright

© 2022 Ayesha Ali et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.