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

Autor: Dash B; Department of Surgical Oncology, Gynecologic Oncology Division, Tata Memorial Centre, Homi Bhabha National Institute (HBNI), Mumbai, India., Rath S; Department of Medical Oncology, Tata Memorial Centre, Homi Bhabha National Institute (HBNI), Mumbai, India., Rekhi B; Department of Pathology, Tata Memorial Centre, Homi Bhabha National Institute (HBNI), Mumbai, India., Mittal N; Department of Pathology, Tata Memorial Centre, Homi Bhabha National Institute (HBNI), Mumbai, India., Kulkarni R; Department of Surgical Oncology, Gynecologic Oncology Division, Tata Memorial Centre, Homi Bhabha National Institute (HBNI), Mumbai, India., Shylasree TS; Department of Gynaecological Oncology, Aberdeen Royal Infirmary, Foresterhill, Aberdeen AB252ZN, UK.; Department of Surgical Oncology, Gynecologic Oncology Division, Tata Memorial Centre, Homi Bhabha National Institute (HBNI), Mumbai, India., Maheshwari A; Department of Surgical Oncology, Gynecologic Oncology Division, Tata Memorial Centre, Homi Bhabha National Institute (HBNI), Mumbai, India.
Jazyk: angličtina
Zdroj: Gynecologic oncology reports [Gynecol Oncol Rep] 2024 Jul 28; Vol. 55, pp. 101464. Date of Electronic Publication: 2024 Jul 28 (Print Publication: 2024).
DOI: 10.1016/j.gore.2024.101464
Abstrakt: 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.
Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
(© 2024 The Authors.)
Databáze: MEDLINE