Author
Information
Devalla
A*, Warke HS**, Mayadeo NM
(*
Second Year Resident, **Associate Professor; *** Professor; Department of
Obstetrics and Gynaecology, Seth G S Medical College & KEM
Hospital , Mumbai , India
Abstract
Vulval
leiomyomas are rare in occurrence with as few as 300 cases reported in the
literature. Smooth muscle tumors may arise from the erector pili muscle, the
erectile tissue or at the insertions of the round ligaments and are usually
well- circumscribed. Purely epithelioid lesions are identified only in the
vulva and their hyalinisation is more common. We present a case of a 46 year
old woman with bilateral multiple vulval masses with symptoms of discomfort
while sitting and walking. Clinically it was diagnosed as a labial lipoma due
to its soft consistency and surgical excision was done. Histopathology report
was suggestive of leiomyoma with hyaline changes on microscopic examination.
Introduction
Extrauterine
leiomyomas commonly affect the urinary bladder, labia majora, labia minora,
ovaries and urethra; labia majora being the most common. Presence of concurrent
uterine leiomyomas or a history of hysterectomy done for the same may be
suggestive of the diagnosis.[1] Among those affected by these tumors, the mean
age at presentation varies from 13 to 71 years. The average tumor size varies
from 0.5 to 15 cm. With regard to pathologic origin, the tumors are thought to
arise from the smooth muscle cells within erectile tissue or blood vessel walls
or the remnants of the round ligament. These may also originate from the dartos
muscle of the genitalia.[2] Vulval leiomyomas may be clinically misdiagnosed as
Bartholin’s cysts, lipomas or fibromas.[3] Although considered benign tumors,
they can be locally aggressive and can also metastasize. Most tumors are
solitary and well circumscribed masses. Symptoms include pain and difficulty in
walking, discomfort while sitting or difficulty in micturition. Owing to the
size and appearance of the tumor, the patient may suffer from stress, anxiety
and cosmetic issues. Treatment options include surgical excision and removal of
the entire tumor, when symptomatic. Follow-up care with regular screening and
examination are very important as the chances of recurrence are not uncommon.
Excision of the labial masses was performed in our patient admitted with
complaints of increase in the size of the swelling causing her discomfort while
walking. The patient was discharged on day 7 and followed up after 2 weeks and
6 weeks.
Case
Report
A 46
year old, married since 26 years, Para 3 was admitted with the complaints of
multiple vulval masses since 1 year which gradually increased, largest mass
being 5x4 cm and smallest being 3x2 cm,
causing discomfort while sitting and walking with occasional bleeding from the
mass. There was no history of fever, vaginal discharge and bladder or bowel
disturbances. The patient gave history of undergoing total abdominal
hysterectomy 5 years ago for fibroid uterus with abnormal uterine bleeding. On
general examination, vital parameters of the patient were normal. On local
examination, multiple masses (grossly four masses) were present on both sides
(two on either side) of the labia majora and smaller masses on the lateral
walls of vagina near labia majora, the smallest being 3x2 cm and largest 5x5
cm. Clinically, the masses appeared to be lipomas or degenerated fibromyomas
due to its soft consistency. All the routine investigations were normal.
Ultrasonography of the pelvis was done which indicated hyperechoic lesions in
the labia majora and essentially a normal pelvic study. On clinical
examination, the innermost extent of one of the vulval mass on the left side
could not be appreciated. Hence, a multiplanar-multiecho magnetic resonance
imaging (MRI) with post-contrast imaging was done which was suggestive of a
fairly large space occupying lesion involving the lower pelvis and perineal
region measuring 10x7x5 cm in the vagina extending into the peri-urethral
region and distinct from the rectum. The fat planes from the rectum were
well-preserved. Antero-inferiorly, it was seen extending up to the vulva and
superiorly till the vault. The patient was planned for excision of the vulval
mass with an abdomino-perineal approach with surgical stand by. Procedure was
done under spinal and epidural anesthesia. A 3 cm incision was made at the
muco-cutaneous junction and a firm to cystic encapsulated mass was enucleated
after dissection along its capsular plane. The size of the cavity formed after
removal of the mass was reduced with interrupted sutures followed by closure of
the overlying skin. Similar procedure was done for the other vulval masses.
There was no extension of the vulval masses till the vault and into the pelvis.
The vulval mass on the upper left side, clinically and on MRI thought to be
extending into the pelvis was also enucleated completely through the vaginal
approach. On cut section of the largest mass a whorl-like appearance with
central degenerative changes were noted and our intra-operative diagnosis was
that of a vulval leiomyoma. She had an uneventful postoperative recovery and
was discharged on the seventh postoperative day. At 6 weeks follow up she was
completely asymptomatic.
Histopathology
report showed tissue bits greyish white in color with whorled pattern in gross
appearance that was confirmed on microscopic examination that revealed features
of leiomyoma with hyaline changes with no evidence of malignancy.
Figure 1. Vulval leiomyomas (arrows).
Figure 2. Vulval leiomyomas after removal. One leiomyoma is bisected (arrow) to show its archtecture.
Discussion
Leiomyomas
are an important health concern as they are the most frequent indication for
the performance of hysterectomy, accounting for nearly 2,40,000 such procedures
in the United States.[4] About 40% of the patients with fibroids have
chromosomal abnormalities that include deletions of chromosome 7 and trisomy of
chromosome 12. The genes known to play an important role in their occurrence
are CLAD1 and PLAG1 gene.[5] Vaginal leiomyomas were first described by Denys
de Leyden in 1733.[6] Vulval leiomyomas are most commonly seen in the anterior
vaginal wall. The size of the tumor may vary from 2 to 10 cm, the most common
site being the labia majora. Myxoid changes and hyalinization are common over
labia majora. Majority of the patients are asymptomatic though some may present
with vaginal bleeding, dyspareunia, discomfort while sitting and urinary
disturbances. Most tumors occur as solitary, well circumscribed and
slow-growing masses.[7] Diagnosis may involve complete clinical examination
with thorough evaluation of medical history. Ultrasound and MRI are most
commonly used for diagnosis though histopathology of the surgical specimen is
the gold standard for diagnosis. Treatment of choice would be surgical excision
of the tumor with urethral catheterisation (to avoid damaging the urethra) if
limited to the vulva. In cases of large tumors, abdomino-perineal approach is
preferred. The patient needs to be explained about the chances for recurrence
and hence the need for regular follow up.[8]
Conclusion
Vulvar
leiomyomas are rare, seen more commonly in middle aged women. Concurrent
uterine leiomyomas or a past history of abdominal hysterectomy for the same
helps in its diagnosis. These tumors rarely metastasize but chances of their
recurrence are known. Clinical examination, USG and MRI help in the diagnosis.
However, histopathology of the surgical specimen is the gold standard. Surgical
excision of the tumors is the treatment of choice.
References
- Szklarukj, Tamm EP, Choi H, Varavithya V. MR imaging of common and uncommon large pelvic masses. Radiographics 2003; 23(2): 403-24.
- Fasih N, Prasad SAK, Macdonald DB, Fraser-Hill MA, Papadatos D, Kielar AZ et al. Leiomyomas beyond the uterus: unusual locations, rare manifestations. Radiographics 2008, Nov-Dec;28(7): 1931-48.
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- Leppert PC, Catherino WH, Segars JH. A new hypothesis about the origin of uterine fibroids based on gene expression profiling with microarrays. Am J Obstet Gynecol 2006;195(2): 415-20.
- Young SB, Rose PG, Reuter KL. Vaginal fibromyomata: two cases with pre-operative assessment, resection and reconstruction.Obstet Gynecol. 1991; 78: 972-974.
- Cobanoğlu O, Gürkan Zorlu C, Ergun Y, Kutluay L. Leiomyosarcoma of the vagina.Eur J Obstet Gynecol Reprod Biol. 1996 Dec 27; 70(2): 205-7.
- Bae
J H,
Devalla
A, Warke HS, Mayadeo NM