Aggressive angiomyxoma is a benign mesenchymal tumor with very low potential to metastasize. The tumor was termed as aggressive due to its slow growth with extensive local invasion [1]. Most commonly it is diagnosed only on histopathology. Etiology is unknown and usually affects vulva, perineal region, buttocks of women in reproductive age. It is a rare and recurrent tumor with less than 250 cases reported in the literature [2]. Considering its nature of high local recurrence and extensive invasion, proper diagnosis, management and long-term follow-up are very essential. No treatment modality has proven to be of any benefit for complete cure for this condition. However, total excision of the mass should be opted to prevent the chance of recurrence. Despite complete surgical removal, recurrence rate of aggressive angiomyxoma is reported as high as 72%.
Case Report
We present a case of a 28-year-old unmarried girl with a large, fleshy, pedunculated painless mass on the left labia majora with difficulty in walking. She underwent local surgical excision four times in the past with history of recurrence within 3–6 months following every surgery. Last surgery was done 3 years back and swelling appeared 3 months following surgery and gradually progressed to present size. The previous histopathology reports were not available. There was a huge soft non-tender swelling in left labia majora around 18 × 10 cm involving labia minora, fourchette distorting the vulval introitus and urethra. It was ill-defined having variegated appearance with smooth surface. We decided to proceed with MRI in view of recurrent soft tissue mass. MRI suggested dumbbell-shaped mass in the left vulva with pelvic extension with thick vascular pedicle. Hemangioma was ruled out, and hence, we did not do MRI angiogram/ venogram. FNAC done showed hemorrhagic material. In view of benign nature of huge mass, recurrence following multiple local excision and MRI showing pelvic extension, combined laparoscopic and vulval excision was planned for the patient to achieve complete removal of the mass. Patient was followed for 12 months and had no recurrence (Fig. 1).
Surgical Technique
Laparoscopic findings: uterus normal size, tubes and ovaries were normal. With the help of the vaginal assistant, vulval mass was pushed inside. A diffuse vague mass was seen lateral to left uterosacral ligament. As MRI suggested a large vascular pedicle supplying the mass-unilateral (left) temporary occlusion of anterior division of internal iliac artery ligation was done before proceeding with excision. Peritoneum was incised lateral to left ureter and anterior division of internal iliac artery was ligated with No 1-0 PDS. Incision was extended down along left lateral pelvic wall and posterior leaf of broad ligament up to left uterosacral ligaments. Left para-rectal space was developed by dissecting between left internal iliac artery and left ureter. Anteriorly uterovesical fold was opened, and bladder was dissected down. On deep dissection between left ureter and uterosacral ligament, a thin-walled cyst of 6 × 6 cm was noted which drained mucinous fluid. Cyst wall was peeled from adjacent structures. Ureterolysis was done. Another illdefined soft tissue mass 8 × 8 cm was noted posterior to uterine vessels and lateral to uterosacral ligament in left parametrium on the lower part of cervix extending into left para-colpos. With blunt and sharp dissection using harmonic, mass was released from adjacent structures and multiple feeding vessels were coagulated/clipped. Ill-defined mass 12 × 8 cm extending into para-colpos and left lateral border of vagina and inferior to ureter and uterine vessels extending to floor of para-rectal space into levator ani and into left vulva was excised with vulval skin with the aid of vaginal assistant by pushing the mass inside through the rent in the levator ani. Levator ani was sutured laparoscopically with No 2-0 PDS continuous sutures. Excess vulval skin was excised, and vulval reconstruction was done (Fig. 2).
Clinically, it may be misdiagnosed as angiomyxolipoma,
hamartoma, hemangioma, Bartholin cyst, lipoma, Gartner
cyst, angiomyofibroblastoma and other smooth muscle tumors. Grossly, they are soft, partly circumscribed masses
with gelatinous appearance on cut section. Microscopic
appearance of these lesions shows numerous thick-walled
vessels in a loose collagenous stroma with stellate-shaped
neoplastic cells. Exact pathogenesis is not known. MRI is
the gold standard for diagnosis [3]. It is superior to a CT
when determining the extent of the tumor and its relation to
the pelvic floor.
Partial excision can be opted in view of high operative
morbidity. It requires multimodal treatment combining surgical
and medical management to treat recurrent angiomyxoma.
In general, excision of these lesions is challenging as
they have the same consistency as that of normal connective
tissue and hence have a high propensity for local recurrence
(36–72%) [4]. Recurrences usually occur in first 5 years after
surgery in which about 70% recurrence in first 3 years and
late recurrences up to 14 years have been reported.
Wide surgical excision is the best treatment of choice. To
achieve complete excision, we may need to remove adjacent
normal fascia and muscles. However, major organs such as
rectum or bladder are spared as this highly morbid extensive
surgery cannot be justified for such recurrent tumors. If the
tumor extends above the pelvic diaphragm, an abdominalperineal
approach is the best treatment option. The high
recurrence is commonly due to incomplete excision mainly because of improper diagnosis. Radiation and chemotherapy
are less effective due to its low mitotic activity.
This tumor is common during reproductive age due to
hormonal dependency. This is explained by its positive estrogen
and progesterone receptors. Hormonal treatments with
gonadotropin-releasing hormone analogues and tamoxifen
have been tried, but its role is not clearly defined. Another
modality of treatment is angiographic embolization of the
tumor. This helps by shrinking the tumor size before surgery.
However, recurrences after initial embolization are common.
As late recurrences are well known, these patients should be
counseled about the need for long-term follow-up. Periodic
clinical examination may be insufficient to detect recurrence.
Imaging studies such as MRI can detect recurrence
early, but there are no guidelines about their follow-up. This
woman has been advised regular checkups. After follow-up
for 1 year, she had no recurrence.
Conflict of interest Dr. Kavitha Yogini Duraisamy, Dr. Devi Balasubramaniam,
Dr. Malathi Ezhilmani and Dr. Palanivelu Chinnusamy
declare that they have no conflict of interest.
Human Rights Statement There is no violation of human rights.
Informed Consent Informed written consent for publication of this
article was obtained from patient and her relative.
