Vulvo-vaginal stromal tumours – Case series of a rare entity from an oncology centre in India

• Published in: Gynecologic oncology reports 2024-10, Vol.55, p.101464, Article 101464
• Main Authors: Dash, Biswajit, Rath, Sushmita, Rekhi, Bharat, Mittal, Neha, Kulkarni, Rohini, Shylasree, T.S., Maheshwari, Amita
• Format: Article
• Language: English
• Subjects: Angiomyofibroblastoma; Angiomyxoma; Myofibroblastoma; Stromal; Tumours; Vulvo-vaginal
• ISSN: 2352-5789; 2352-5789
• DOI: 10.1016/j.gore.2024.101464

•Vulvo-vaginal stromal tumours are rare unique site-specific mesenchymal tumours.•Although usually benign, except deep angiomyxoma which can be locally aggressive, they mimic other malignant tumours of vulva.•There is a morphologic and immunohistochemical overlap among these tumours.•Surgery remains the primary approach for managing localized disease, with an emerging role for hormonal therapy.•A subset of these tumours may have genetic links, indicating potential areas for further research and understanding. Vulvo-vaginal stromal tumours are a rare and diverse group of mesenchymal neoplasms unique to hormone-responsive stroma of the vulva and vagina. These tumours are mostly benign, except for the locally aggressive deep angiomyxomas. Often these tumours pose diagnostic challenges, resembling certain malignant vulvo vaginal tumours. This case series highlights clinicopathological features of four angiomyxomas; a single angiomyofibroblastoma, and another superficial myofibroblastoma, including their clinical outcomes. All patients were in their 4th or 5th decade of life. Only 1/4 angiomyxomas was correctly diagnosed at the referring hospitals. Three out of four patients harbouring angiomyxomas achieved clinical remission post-surgery, while one patient was lost to follow-up. By immunohistochemistry, tumor cells showed variable positivity for desmin, SMA, ER, and PR, and negativity for S100P and CD34. The angiomyofibroblastoma was initially misdiagnosed as a liposarcoma, and the patient was lost to follow-up after diagnosis. Immunohistochemically, the tumor cells were diffusely positive for SMA and ER; weakly and focally positive for desmin, and negative for AE1/AE3, CD34, and S100P. The patient with superficial myofibroblastoma is in clinical remission post-excision with an 18-month follow-up. Immunohistochemically, the tumor cells showed CD34 positivity. Therapeutically, none of the patient received adjuvant treatment, except for a single patient with angiomyxoma, who underwent chemoradiation for a synchronous cancer cervix post-surgery. This case series provides valuable insights into the clinical heterogeneity, diagnostic intricacies, and outcomes of vulvo-vaginal stromal tumours from an oncology centre in India, further contributing to a better understanding of these rare tumours.