Vaginal Endometrial Stromal Sarcoma - A Case Report and Literature Review

von Solodkoff M1*, Battista MJ1, Gaßmann P2, Schmidt M1, Schad A3, Hasenburg A1 and Anic K1

1Department of Gynecology and Obstetrics, University Medical Center of the Johannes Gutenberg University Mainz, Germany
2Department of General and Visceral Surgery, “Städtisches Krankenhaus” Pirmasens, Germany
3Department of Pathology, University Medical Center of the Johannes Gutenberg University Mainz, Germany

^*Correspondance to: Michail von Solodkoff 

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Abstract:

Vaginal Endometrial Stromal Sarcoma (VESS), an extrauterine endometrial stromal sarcoma in the absence of a primary uterine lesion, is an extremely rare disease. Currently only fourteen cases have been documented. VESS may be correlated with endometriosis and chronic high-dose estrogen levels. It is usually diagnosed by a pathological examination. Specific clinical symptoms are often not present. In most cases, VESS is a low-grade endometrial stromal sarcoma. The malignant transformation of endometrial stroma is marked by uniform stromal cells with minimal cellular pleomorphism, mild nuclear atypia and variable mitoses. We report the case of a 33-year-old woman who presented with increasing pelvic pain and was diagnosed with a low-grade VESS. On examination, a smooth-bordered 2.0 cm × 2.1 cm lesion between the right levator muscle and the right posterior vaginal wall was found. There are no guidelines based on large prospective-randomized studies for the treatment of Endometrial Stromal Sarcomas and existing recommendations are mostly derived from case reports due to the rarity of these tumors. Our patient received a local excision of the tumor and an adjuvant treatment with a GnRH analogue combined with an aromatase inhibitor. In conclusion, this case report clearly shows that LG-ESS must also be considered in women with rectovaginal fistula, even though it is very rare. Due to the rarity of VESS, the creation of a register for one is also useful.

Keywords:

Vaginal endometrial stromal sarcoma; Malignant transformation of endometriosis; Extrauterine sarcoma

Cite the Article:

von Solodkoff M, Battista MJ, Gaßmann P, Schmidt M, Schad A, Hasenburg A, et al. Vaginal Endometrial Stromal Sarcoma - A Case Report and Literature Review. Ann Clin Case Rep. 2023; 8: 2486..