|Year : 2021 | Volume
| Issue : 2 | Page : 287-289
Rare clinical presentation of umbilical endometriosis
Aditi Shivcharan Agrawal1, Tuhina Mishra2
1 Department of Surgery, Wockhardt Hospital, Mira-Bhayandar, Mumbai, Maharashtra, India
2 Department of Surgery, Grant Government Medical College and Sir J.J. Group of Hospitals, Mumbai, Maharashtra, India
|Date of Submission||31-Jan-2021|
|Date of Acceptance||03-May-2021|
|Date of Web Publication||31-May-2021|
Dr. Aditi Shivcharan Agrawal
Wockhardt Hospital, Mira Road, Mira-Bhayandar, Maharashtra
Source of Support: None, Conflict of Interest: None
Primary umbilical endometriosis is a rare disorder accounting for 0.5%–1% of extragenital endometriosis. It is defined as the presence of ectopic endometrial tissue within the umbilicus. Although it mostly presents as a typical cyclic umbilical discharge coincidental with a palpable mass, the diagnosis is often delayed due to its low prevalence and abnormal presentation, as in this case. The possibility of subcutaneous endometriosis should not be ruled out when an umbilical mass is detected, even if there is no history of previous surgery. Other differential diagnoses of umbilical mass include paraumbilical hernia, surgical scar (laparoscopy), primary umbilical tumor, granuloma, Sister Mary Joseph's nodule, and urachal duct cyst. In our report, we present the case of a 40-year-old female who came with a painless mass in the umbilicus for the past 10 years.
Keywords: Abnormal presentation, endometriosis, extragenital, primary endometriosis, umbilicus
|How to cite this article:|
Agrawal AS, Mishra T. Rare clinical presentation of umbilical endometriosis. Indian J Health Sci Biomed Res 2021;14:287-9
| Introduction|| |
First described by Rokitansky in 1860, endometriosis is characterized by the presence of endometrial tissue outside of the uterine cavity. It commonly occurs in the pelvic organs, especially the ovaries, the uterosacral ligaments, and the pouch of Douglas. Extragenital endometriosis is rare but has been described in almost every area of the female body, including the bowel, bladder, lungs, brain, umbilicus, and surgical scars. The condition is associated with infertility in 30%–40% of women who leave childbirth after the age of 30. The incidence is 1%–7%, and it is not associated with lifestyle. We report a rare case of spontaneous, primary umbilical endometriosis.
| Case Report|| |
A 40-year-old female with primary infertility presented to a clinic with pain in the lower abdomen and bleeding from the umbilical nodule, which coincided with her menstrual cycle. She had irregular menstrual cycle but did not take any medications for the same. There was no history of endometriosis and of previous uterine or pelvic surgery. She also complained of severe dysmenorrhea. She was experiencing the symptoms for the last 8–10 years, but she did not get herself treated as it did not affect her routine life. Abdominal examination revealed a 2 cm × 2 cm nodule at the umbilicus [Figure 1]. The nodule was not painful on palpation, was irreducible by gentle pressure, and showed negative cough impulse, and there was no other abdominal mass, regional lymphadenopathy, or hepatosplenomegaly. The uterus and ovary appeared normal on pelvic examination. Computed tomography (CT) scan confirmed the presence of a nodule however could not reveal the type. Fine needle aspiration cytology (FNAC) was performed which revealed the presence of endometrial cells in the umbilicus, thus confirming the diagnosis of umbilical endometriosis.
|Figure 1: A superficial nodule, located in the umbilicus of approximately 2 cm|
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The patient underwent wide local excision of the umbilical nodule with umbilicus along with reconstruction of neoumbilicus. Since the patient did not want to lose her umbilicus, so reconstruction was done with purse string method [Figure 2].
Histopathological examination revealed fibro-adipose connective tissue with widespread prevalence of multiple endometrial glands and endometrial stroma. No signs of hyperplasia, atypia, or malignancy were found. The findings were consistent with diagnosis of umbilical endometriosis [Figure 3].
|Figure 3: (a) Excised specimen of the umbilicus with surrounded tissue. (b) Histological microscopic view of this lesion demonstrated endometrial gland and stroma|
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| Discussion|| |
Despite being a rare phenomenon (0.5%–1%), the deposition of endometrium on the umbilicus is well recognized. Originally described by Viller in 1886, less than 150 cases have been reported. There are two types of umbilical endometriosis: Primary endometriosis in patients without any past surgical history and secondary endometriosis occurring after gynecological operations. Umbilical endometriosis without surgical history is a rare phenomenon.
Several theories have been formulated regarding pathogenesis of extragenital endometriosis. Latcher has classified these theories into three main categories: The embryonal rest theory, which explains endometriosis adjoining the pelvic viscera by Wollfian or Mullerian remnants; the coelomic metaplasia theory, which states that in the presence of stimulus such as inflammation or trauma, the embryonic coelomic mesothelium dedifferentiates into endometrial tissue; and the migratory pathogenesis theory, which explains the dispersion of endometrial tissue by direct extension, vascular and lymphatic channels, and surgical manipulation. Some other theories suggest the cellular proliferation of endometrial cells from initial extraperitoneal disease along the urachus. The real mechanism still remains a mystery.
Histological confirmation is the current golden standard for diagnosing umbilical endometriosis, while initial diagnosis for treatment workup is primarily clinical. Transcutaneous ultrasound, magnetic resonance imaging, and/or CT scan are the various diagnostic tools that can be used to investigate the relationship of the nodule with the surrounding tissue and to differentiate between other umbilical lesions. FNAC can be supplementary, but inconclusive results have been reported to be as high as 75%.
Histological findings are characterized by irregular endometrial glandular structures in basophilic cytoplasm, accompanied by high cellular and vascular stroma, which have a spindle-cell appearance. The differential diagnoses include melanocytic nevus, endosalpingiosis presenting as periumbilical papules, pyogenic/foreign body granuloma, umbilical polyp, seborrheic keratosis, epithelial inclusion cyst, desmoid tumor, hemangioma, granular cell tumor, umbilical hernia, omphalitis, and keloid. Primary or secondary metastatic neoplasms, such as melanoma or Sister Mary Joseph's nodule, should be ruled out., Nevertheless, the risk of malignancy is considerably low. Only three cases of umbilical endometriosis associated with malignancy have been reported. Surgical management involving a local excision (respecting margins of 1 cm) with/without resection and repair of the underlying fascia is the most preferred and definitive treatment modality. In our case, the lesion was excised and histology confirmed the diagnosis. Simultaneous laparoscopy was not done in the above case because our patient was asymptomatic. Although local recurrence is uncommon, the patient has been warned of the risk of scar endometriosis and recurrence.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Giudice LC, Kao LC. Endometriosis. Lancet 2004;364:1789-99.
Markham SM, Carpenter SE, Rock JA. Extrapelvic endometriosis. Obstet Gynecol Clin North Am 1989;16:193-219.
Zollner U, Girschick G, Steck T, Dietl J. Umbilical endometriosis without previous pelvic surgery: A case report. Arch Gynecol Obstet 2003;267:258-60.
Rubegni P, Sbano P, Santopietro R, Fimiani M. Case 4. Umbilical endometriosis. Clin Exp Dermatol 2003;28:571-2.
Latcher JW. Endometriosis of the umbilicus. Am J Obstet Gynecol 1953;66:161-8.
Razzi S, Rubegni P, Sartini A, De Simone S, Fava A, Cobellis L, et al
. Umbilical endometriosis in pregnancy: A case report. Gynecol Endocrinol 2004;18:114-6.
Ghosh A, Das S. Primary umbilical endometriosis: A case report and review of literature. Arch Gynecol Obstet 2014;290:807-9.
Victory R, Diamond MP, Johns DA. Villar's nodule: A case report and systematic literature review of endometriosis externa of the umbilicus. J Minim Invasive Gynecol 2007;14:23-32.
Purvis RS, Tyring SK. Cutaneous and subcutaneous endometriosis. Surgical and hormonal therapy. J Dermatol Surg Oncol 1994;20:693-5.
[Figure 1], [Figure 2], [Figure 3]