|
OJHAS Vol. 10, Issue 1:
(Jan-Mar 2011) |
|
|
|
Villar’s
Nodule |
|
Mallika Sengupta, Assistant Professor, Department of Obst. & Gynaecology,
Animesh Naskar,
Assistant Professor, Department of Obst. & Gynaecology,
Sonia Gon, Assistant Professor, Department of Pathology,
Bipasa Majumdar, Assistant Professor, Department of Pathology,
RG Kar Medical College & Hospital, Kolkata-700004, India |
|
|
| |
|
|
|
|
|
|
Address for Correspondence |
Department
of Obst & Gynaec,
R G Kar Medical
College & Hospital,
1, Khudiram
Bose Sarani,
Kolkata-700004, India.
E-mail:
drmallikasengupta@gmail.com |
|
|
|
|
|
Sengupta M, Naskar A, Gon S, Majumdar B. Villar's Nodule. Online J Health Allied Scs.
2011;10(1):19 |
|
|
|
|
Submitted: Feb 5,
2011; Accepted: March 31, 2011; Published:
April 15, 2011 |
|
|
|
|
|
|
|
| |
|
| Abstract: |
|
Villar’s
Nodule or primary umbilical endometrioma is a rare condition, with an
estimated incidence of 0.5 to 1% of all patients with endometrial ectopia.
Several aetiological theories have been proposed for its pathogenesis
with coelomic metaplasia being the favoured one and also, umbilicus
may acts as a physiological scar with a predilection for endometrial
tissue in the development of spontaneous umbilical endometriosis. Since
endometriosis remains a diagnostic and therapeutic enigma even today,
largely due to its variable presentations, we, hereby report a case
of primary umbilical endometrioma due to its rarity and unusual presentation.
Key Words:
Umbilical; Endometriosis
|
|
Primary umbilical
endometrioma, also known as Villar’s Nodule is a rare condition, with
an estimated incidence of 0.5 to 1% of all patients with endometrial
ectopia.(1) It was first discovered by Villar in 1886, hence the name
(2) and is defined as presence of ectopic endometrial glands within
umbilicus without prior history of pelvic endometriosis.(3)
Endometriosis
remains a diagnostic and therapeutic enigma even today, largely due
to its variable presentations and in spite of its classical presentation,
misdiagnosis is not uncommon. We, hereby report a case of primary umbilical
endometrioma due to its rarity and unusual presentation.
A 29 years
old unmarried, nulliparous female presented in the outpatient department
of Gynaecology, R G Kar Medical College & Hospital, Kolkata, with
chief complaint of a painful nodule in the umbilical region since one
year. The pain was dull in nature but tend to become severe during menstruation.
Her menstrual cycles were regular with no history of dysmenorrhoea.
On local examination,
a hyperpigmented, dark brown umbilical nodule was seen measuring 1.5
cm in its greatest dimensions. The nodule was firm in consistency and
tender.(Figure 1) It was non-reducible, non-pulsatile and with no
impulse on coughing. The systemic examination was unremarkable.
Patient’s
routine haematological and biochemical profile was within normal limits
apart from mild anaemia. Ultrasonography of whole abdomen was done which
revealed a lesion of variable echogenecity, measuring 32x18mm, with
mild adhesion to the underlying parites and no relation to the abdominal
viscera.(Figure 2) The uterus along with its adnexa was normal. Excision
biopsy of the umbilical nodule was done and the specimen was sent to
the department of pathology for histopathological examination. During
excision biopsy, the umbilical nodule was found to be densely adhered
to the underlying rectus muscle.
 |
 |
|
Figure 1: Hyperpigmented,
dark brown umbilical nodule measuring 3x2 cm |
Figure 2: Umbilical
lesion of variable echogenecity, measuring 32x18mm |
Pathological
Examination:
On gross, specimen
was partly skin covered, nodular and measured 3.5x2.0 cm. Cut surface
was brownish with areas of haemorrhages. Microscopic examination showed
numerous endometrial glands surrounded by compact endometrial stroma
embedded in the subepithelial connective tissue stroma. (figure 3 &
4) The overlying stratified squamous epithelium was unremarkable. A
few haemosedrin containing macrophages were seen at places and a histopathological
diagnosis of umbilical endometriosis was made.
After the disgnosis
of endometriosis was established, no recurrence has been noted at the
site till now and patient has no other complaints.
 |
 |
|
Figure 3 &
4: Numerous endometrial glands surrounded by compact endometrial stroma
embedded in the subepithelial connective tissue stroma (H&E, 400x) |
Umbilical
endometriosis is rare, especially in patients who do not give a history
of previous pelvic surgery or who do not have clinical evidence of preexisting
pelvic endometriosis. Mean
age of presentation of patient with umbilical endometriosis is 37.7
+/- 0.98 years with swelling (90.9%), pain (81.5%), and bleeding (49.2%)
as the common mode of presentation. Most umbilical lesions are brown
in color, followed by blue, purple, black and red.(4) This case also
presented with brownish black swelling and prominent cyclical pain.
The pathogenesis
of spontaneous cutaneous endometriosis is yet unknown. Several aetiological
theories have been proposed. These include coelomic metaplasia, congenital
presence of developmentally-displaced endometrial tissue, direct extension
through the round ligament or the patent omphalomesenteric duct, or
mechanical seeding of endometrial tissues via the lymphatic or venous
system transfer via lymphatics or blood vessels.(5)
In the development
of spontaneous umbilical endometriosis, as in the case presented, it
is possible that the umbilicus acts as a physiological scar with a predilection
for endometrial tissue.(6) However, in our opinion, it is still not
clear how the endometrial cells has traveled to umbilical location and
the other above mentioned theories might have played a role in the development
of villar’s nodule of present case.
Umbilical endometriosis
can pose a diagnostic dilemma as it can simulate a malignant melanoma
or the “sister Mary Joseph nodule”—a manifestation of intra-abdominal
malignancy.(5) Any other condition that presents with a subcutaneous
mass or discoloration of the umbilical skin, such as a benign nevus,
a lipoma, an abscess, a cyst, or a hernia, as well as a metastatic deposit
from a systemic malignancy should be considered in the differential
diagnosis.(6)
Though the
most important key feature of diagnosis is intermittent bleeding in
accordance with the menstruation cycle,(4) magnetic resonance imaging, epiluminescence microscopy or, fine-needle aspiration cytology
may help resolve the diagnostic dilemma.(5) However, histopathology
is the main stay of diagnosis. Because of the classical symptoms and ultrasonography picture, and also the poor socioeconomic background
of the patient, excision biopsy with histopathological examination was
done in the present case to reach at a confirmatory diagnosis rather
than undergoing all the above mentioned diagnostic investigations.
The
treatment of choice of Villar’s Nodule remains surgical excision with
sparing of the umbilicus where possible, and recurrences, though reported,
are rare.(8) Malignant
transformation of umbilical endometriosis is extremely rare with only
one case reported in the literature where umbilical endometriosis was
present since age of thirty.(9)
To conclude,
endometriosis is important to consider in cases of unclear skin lesions
of the umbilicus, even in cases with no previous abdominal surgery.
And also, excisional surgery is the treatment of choice so as to avoid
lesion relapse and the risk of oncogenic transformation.
- Dessy LA, Buccheri
EM, Chiummariello S, Gagliardi DN, Onesti MG. Umbilical endometriosis,
our experience. In Vivo. 2008;22:811–815.
Michovitz
M, Baratz M, Stavorovsky M. Endometriosis of the umbilicus. Dermatologica
1983;167:326-330.
Yuan-Yu Hsueh, Shyh-Jou
Shieh. Primary Umbilical Endometriosis- Case Report and Literature Review.
JPSAROC. 2009;8(1):66-69
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.
Agarwal A, Fong
YF. Cutaneous endometriosis. Singapore Med J. 2008;49(9):704-709
Yu CY, Perez-Reyes
M, Brown JJ, Borrello JA. MR appearance of umbilical endometriosis.
J Comput Assist Tomogr. 1994;18:269–271.
Chatzikokkinou P, Thorfinn
J, Angelidis IK, Papa G, Trevisan G. Spontaneous
umbilical endometriosis. Acta Dermatoven APA 2009;18(3):126-130
Purvis
RS, Tyring SK. Cutaneous and subcutaneous endometriosis surgical and
hormonal therapy. Journal of Dermatologic Surgery and Oncology 1994;20:693-695.
Lauslahti K. Malignant
external endometriosis. A case of adenocarcinoma of umbilical endometriosis.
Acta pathologica et microbiologica Scandinavica 1972;233:98-102.
|
