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.