ANGIOMYXOMA - A RARE CAUSE OF RECURRENT PERINEAL SWELLING

OBJECTIVE- Aggressive Angiomyxoma (AAM) is a rare mesenchymal benign myxoid tumour of the pelvis and perineum which occurs most commonly in adult females. Only about 180 cases were reported in medical literature. We report a middle aged female patient with recurrent perineal swelling whose histopathology was suggestive of aggressive angiomyxoma.METHOD- Case report and review of medical literature. CASE REPORT- A 40 year old women patient presented with a recurrent swelling in the perineum. Examination showed a pedunculated swelling of size 5x4x3 cm protruding just lateral to the right labia majora, firm in consistency. Swelling was compressible, irreducible and there was no cough impulse .Cytology was inconclusive. MRI revealed 12 cm long and 2 cm thick sinus tract extending from right perineal region coursing right ischiorectal fossa and tracking between the vagina and rectum with extension of sinus tract into roof of right ischiorectal fossa. Tract showed hyperintensity in T2 weighted image. Planned for exploration and encapsulated tubular firm swelling was excised. HPE reported as Angiomyxoma. No local recurrence on 6 month followup. CONCLUSION- Aggressive Angiomyxoma (AAM) is a rare mesenchymal benign myxoid tumour of the pelvis and perineum which occurs most commonly in adult females with female to male ratio of 61. They are large, slow growing with tendency for local recurrence. MRI finding consist of iso-intense or low-signal intensity findings on T1-weighted images and high-signal-intensity findings on T2-weighted Images. Histologically they are hypocellular and highly vascular tumour with a myxoid stroma containing cytologically bland stellate or spindled cells. The tumour cells are characteristically positive for oestrogen and progesterone receptors, desmin, muscle specific actin and vimentin and negative for S-100. Complete excision of the tumour is the treatment of choice. Follow-up MRI 2 yearly intervals should suffice for recurrence.Trials of various sorts of chemotherapy like Tamoxifen, Leuprolide and even full blown chemotherapy were on.