Case report:
A 56 years old, debilitating lady presented to the gynecology OPD of KMC
Manipal, with pain and dismay, accompanied by her son and was walking
with an antalgic gait. She complained of a painful mass in the private
area. She gave a history about her visit to a local doctor, where it was
diagnosed as an infection and was incised to drain cheesy pus like
material and was also treated with antibiotics. She was at ease after
the intervention for about a month and a half but the mass reappeared
and it was more painful than the previous episode.
She gave a history of this recurrent mass being as almond sized which
she noticed for the first time after having continuous itch due to dry
skin from a couple of months. There was no history of trauma or history
of Bartholin’s abscess drainage. She has never noticed any sort of blood
discharge. There was no history of trauma and no other swelling on her
body anywhere else. There was no history of associated fever,
folliculitis or burning micturation. She had no complaints of altered
bowel and bladder habits or any symptoms of Crohn’s disease and no past
history of tuberculosis in her or her family. She had no history of
weight loss or loss of appetite.
On examination, she was moderately built and nourished and was a little
agitated with the pain. After an oral pain killer she was relaxed and
allowed examination. Clinical examination showed the patient in a good
general condition with normal vital signs. Abdominal examination showed
no tenderness, or mass, and inspection of the perineum showed no anal
fistula, ulcer, abscesses, or scarring. On local gynecological
examination a firm to hard, palpable clitoral mass involving the
clitoral hood of 3x2cm was noted. It was indurated and tender to touch
with a small ulcerative area of 1 cm with pus-like deposit on the base
of the ulcer. Urethra appeared free from the lesion and there were no
abnormalities of vulva or vagina. No associated skin lesions or enlarged
lymph nodes were palpable. (Image 1)
She was evaluated further and blood investigation revealed normal ESR
and other serological markers. To confirm the diagnosis of cancer, a
biopsy of the lesion was taken and sent for a histopathological
evaluation and also, to rule out infectious cause, a pus culture was
sent. (Image 3)
MRI pelvis also performed due to high suspicion was suggestive of Vulval
Malignancy with FIGO stage II (Image 2). MRI showed a bulky
clitoris with a lobulated lesion of 2.4x 2.1x 1.8 cm in the right half
of the body of the clitoris with no invasion of the urethra, with few
subcentimetric ovoid bilateral inguinal lymph nodes.
To our surprise, the histopathology was not consistent with imaging
diagnosis that came after 2 weeks. It revealed that the tumor mass
contained a fibrocollagenous stroma with dense lymphoplasmacytic
infiltrate and suggested a myofibroblastic tumor of unknown etiology.
Further immunohistochemistry studies were done, but could not confirm
inflammatory myofibroblastic tumor. Pus culture was reported to show
infection with heavy growth of E.Coli and Proteus, which was treated
with higher antibiotics as per sensitivity pattern by IFD team and she
recovered.
Patient was scared of this tumor recurrence and malignancy and after
discussion she underwent a wide excision of the tumor and this time the
histopathology report suggested a granulomatous infection with tissues
showing epithelioid cells, Langhan’s giant cells, extensive necrosis
with inflammatory debris, suggestive of ulcer with granulomatous
inflammation. Further investigations were done to rule out causes of
granulomatous infection. Tuberculosis and superficial mycotic infection
was ruled out by Genexpert, Mantoux, immunochemistry with Grocott-Gomori
Methenamine silver, Periodic acid Schiff, and Ziehl-Neelsen and cultures
all reported negative.
However even after repeat biopsy and histopathological evaluation we
could not find a cause of the recurrent growth. Patient was therefore
counseled and she is now on regular follow up both OPD and telephonic
basis and doing good. Surgical wound is healing with secondary intention
and no discharge or discomfort after 2year of routine follow-up.