Introduction/Background Epithelioid leiomyomas have been well studied in the gastrointestinal tract however, its presence in the female genital tract is extremely rare. Epithelioid leiomyomas include leiomyoblastoma, clear cell and plexiform leiomyoma. Consensus is lacking on their differentiating features and malignant potential.

Methodology A 48-year-old P2 lady with BMI 50 presented to Emergency Department with 3-week history of vaginal bleeding and passing grape-like products vaginally. Clinical examination elicited a cystic lesion measuring 2 cm in diameter on the anterior vaginal wall that was also noted on pelvic TAS. Histology of the tissues passed vaginally suggested microscopic features of malignancy suggestive of Embryonal rhabdomyosarcoma. Her case was discussed at the regional Sarcoma MDT. MRI showed a 2.3 cm hypo-intense vaginal mass with no evidence of fat-stranding or lymphadenopathy and CT scan ruled out metastatic disease. Although the morphological features mimicked sarcoma with strong expression of muscle-related antigens however immunophenotyping did not support malignancy and confirmed a cellular epithelioid leiomyoma. Due to the diagnostic complexity, excisional biopsy and hysteroscopy was decided for. The entire tumour was excised through transvaginal route with hydro-dissection of its planes and enucleation of its capsule leaving clear margins around. Hysteroscopic finding was normal. Tumour biopsy showed lack of necrosis or mitotic activity thereby excluding neoplastic process and confirmed the diagnosis of an epithelioid vaginal wall leiomyoma. MDT recommended close post-operative surveillance.

Results Wherever possible TVS is superior to TAS for vaginal lesions. MRI is excellent in tissue characterisation of the lesion and essential to evaluate relation / invasion to adjacent structures. Histological confirmation remains the gold standard for diagnosis. Tumour recurrence or rapid enlargement alongwith microscopic features of >5 mitoses/10 HPF with atypia suggests sarcomatous change and removal en-bloc is recommended.

Conclusion Sarcomatous transformation and recurrence after 11 months and 10 years have been reported hence close surveillance is essential.

Disclosure Nothing to disclose