Villar’s Umbilical Nodule: A Rare Case Of Umbilical Endometriosis

Author Information

DAlmeida J*, Backthan L**, Rao SV***.

(*Assistant Professor and Consultant Gynaecologist , *** Professor and Head of department of Obstetrics and Gynecology, ** Associate Professor, Department of pathology, Father Muller Medical college hospital, Mangalore, India.)

Abstract

Endometriosis ( term coined by Sampson ) is the presence of endometrial glands and stroma outside the uterine cavity and  musculature and is a very common gynecological condition. Primary cutaneous endometriosis or umbilical endometriosis is a rare localization of extragenital endometriosis. It was first described by Villar in 1886.

Introduction

Endometriosis ( term coined by Sampson ) is the presence of endometrial glands and stroma outside the uterine cavity and  musculature and is a  very common gynecological condition. The  Common sites of occurrence of endometriosis are pelvic organs especially ovaries , uterosacral ligaments and pouch of Douglas. Its average  incidence  is 7 to 10% in the reproductive age. Patients usually present with dysmenorrhea ,  menorrhagia , pelvic pain and infertility.^[1] Primary cutaneous endometriosis or umbilical endometriosis is a rare localization of extragenital endometriosis .It was first described by Villar in 1886.

Case Report

A 38 year old parous lady presented to the gynecology outpatient with abdominal pain and severe dysmenorrhea for a duration of nine months. She also had menorrhagia for  the last six months. The lady also described a  slow progressive, tender bluish umbilical swelling that had been growing over ten years. She complained that serosanguinous discharge from the nodule coincident with her menstrual period and tenderness around the nodule increased during this time. She attained menarche at 12 years and had previous regular menstrual cycles, with spasmodic dysmenorrhea, which was relieved after consuming analgesics and rest. Her obstetric, past medical and surgical history was unremarkable.

On examination it was seen that she had  2x2 cm nodule at the umbilical ring, which had a blackish blue hue and was  firm, tender  and nonreducible. Surface of the nodule was smooth and there was no cough impulse. The lesion appeared to be attached to the anterior abdominal wall. Her uterus was enlarged to the size of a 24 week gravid uterus and was  irregular and firm.

  

Figure 1. Umbilical nodule.

  

Figure 2. Magnified view of umbilical nodule.

On further investigation an ultrasound revealed an echogenic subcutaneous nodule associated with multiple uterine fibroids intrabdominally. A fine needle aspiration cytology (FNAC) of the lesion  was done and the cytology smears revealed the smears to be cellular and with clusters and  sheets of hyperchromatic cells. The background of the smear showed plenty of hemosiderin laden macrophages. This finding of hemosiderin laden macrophages was highly suggestive of endometriosis.

Figure 3 FNAC: Sheets of hyperchromatic cells & hemosiderin laden macrophages.

Surgical removal of the lesion, umbilical reconstruction and a  total abdominal hysterectomy with a bilateral salphingo oophorectomy  were done. Post operative  course of the patient was uneventful.

When a Histopatholgical examination of the excised nodule was done, it showed fragmented endometrial glands with sub epithelial stroma. It confirmed the diagnosis of umbilical endometriosis. The histopathology of the specimen of the uterus showed adenomyosis 

Figure 4 Fragmented endometrial glands with subepithelial stroma. (4XH&E)

Figure 5. Cystic dilated endometrial glands containing red blood cells.

Figure 6. Endometrial glands lined by columnar cells.

Discussion

Umbilical endometriosis is endometriosis  involving the subcutaneous tissue of the umbilicus.  The actual incidence of umbilical  endometriosis is  0.5-1%.^[2] Endometriosis of the umbilicus is rare, atypical and presents with diagnostic difficulties. It occurs frequently with suspicions of malignancy.  There are two theories that suggest the etiopathogenesis  of umbilical endometriosis.

1. Metaplasia theory: it arises from embryonic celomic mesothelium, which due to some stimuli differentiates into endometrial tissue.
2. Metastasis theory: it occurs due to vascular and lymphatic spread of endometrial fragments and ectopic implantation.

The most common symptom of umbilical endometriosis is  cyclical pain at the site of the nodule. The other symptoms are the presence of a pigmented umbilical nodule  and umbilical weeping or cyclical bleeding occurring at the umbilicus. Umbilical endometriosis is commonly associated with  pelvic endometriosis and very often with endometriosis of the surgical scar (which occurs due to  direct seeding of endometrial tissue  after laparoscopy or laparotomy). Rarely the occurrence is of a primary umbilical endometriosis in which there is a  single implant of endometrial tissue, with no associated evidence of pelvis endometriosi.^[3.4] Our case was a case of primary umbilical endometriosis or Villar’s nodule.

The following protocol is suggested in differential diagnosis of an umbilical nodule.^[5] 

• If present with discharge consider a patent urachus, endometriosis of umbilicus and patent vitellointestinal duct.
• If there is no discharge at umbilicus and no signs of infection are present consider granuloma, umbilical inclusion cyst, endometriosis or adenoma.
• If there is no discharge but  signs of infection are present, consider dermatitis, omphalitis or pilonidal sinus.

If an umbilical nodule is found, the following investigations are of relevance in diagnosis: magnetic resonance imaging, epiluminescence microscopy, histology (features of irregular gland lumina in stroma – functional endometrium) and or dermascopy (shows homogeneous red coloration, regularly distributed, fading to the periphery, characteristically called red atolls).^[6] The only treatment for the Villar’s nodule is  surgical excision of the umbilical nodule, which is universally recommended. The complications of umbilical endometriosis are recurrence after excision (rare) and  malignant transformation of the umbilical endometriotic nodule into endometrial carcinoma.

Conclusion

Umbilical endometriosis is a rare surgical entity. It should  be considered in the differential diagnosis for an umbilical lesion  and also in the diagnosis  for cyclical pain, emanating in the vicinity of the umbilicus. The key to diagnosis  is the temporal association of bleeding  from the umbilical  lesion, that is related to the menstrual period.

References

1. Edmonds DK. Endometriosis. In Whitfield CR, editor. Dewhurst's Textbook of Obstetrics and Gynaecology for Postgraduates, 5th ed. Oxford: Blackwell Science, 1995;577-89.
2. Bergqvist A. Different types of extragenital endometriosis: a review. Gynecol Endocrinol 1993 Sep;7(3):207-21.
3. Latcher JW: Endometriosis of the umbilicus. Am J Obstet Gynecol 1953; 66:161-168.  OpenURL
4. Mann LS, Clarke WR: Endometriosis of umbilicus. Ill Med J 1964;125:335-336. OpenURL
5. Techapongsatorn S, Techapongsatorn S. Primary Umbilical Endometriosis. J Med Assoc Thai 2006;89 (10):1753-5.
6. Giorgi VD, Massi D, Mannone F, Stante M, Carli P. Cutaneous endometriosis: noninvasive analysis by epiluminescence microscopy. Clin Exp Dermatol. 2003;28:315–7. 

Citation

Joylene DAlmeida J, Backthan L, Rao SV. Villar’s Umbilical Nodule: A Rare Case Of  Umbilical Endometriosis. JPGO 2015. Volume 2 No. 8. Available from: http://www.jpgo.org/2015/08/villars-umbilical-nodule-rare-case-of.html