ABSTRACT
INTRODUCTION: Vaginal agenesis is a rare congenital condition, with an incidence of 1 in 4500 female births. CASE REPORT: We present a clinical case of vaginal aplasia with cervical atresia in a 31-year-old woman with primary amenorrhea. We aim to report the diagnostic process and provide a comprehensive outline of different possible treatments. DISCUSSION: The most common etiology of these agenesis cases is Mayer-Rokitansky-Küster-Hauser syndrome associated with uterine aplasia. However, vaginal aplasia can occur in 9 % of cases where the uterus is present. During embryogenesis, the Müllerian ducts give rise to the fallopian tubes, uterus, and upper two-thirds of the vagina, while the lower portion of the vagina develops from the urogenital sinus. Vaginal aplasia arises from a failure in the development of the terminal portion of the paramesonephric ducts. Abdominal pain, especially periodic pain, is the most common symptom, followed by primary amenorrhea. MRI is considered the gold standard for the diagnosis and precise description of female genital tract anomalies. CONCLUSION: Total hysterectomy remains a preferred option for cases of complete vaginal atresia to mitigate the risk of cervical or vaginal stenosis, adhesions, and pelvic inflammation resulting from poor menstrual blood drainage.
ABSTRACT
Torsion occurs as a complication in 10% of cases of ovarian tumors. It predominantly occurs in benign ones, while malignant tumors are less prone to torsion. Sertoli-Leydig cell tumors are highly unusual sex cord-stromal tumors of the ovary, accounting for less than 0.2% of all ovarian cancers. A 39-year-old patient presented to the emergency department with a Sertoli-Leydig cell tumor diagnosed due to ovarian torsion. The clinical presentation was characterized by abdominal pain. Ultrasound indicated signs of torsion, and torsion of the right ovary was subsequently confirmed during laparotomy. A salpingo-oophorectomy was performed, and histological examination revealed a moderately differentiated Sertoli-Leydig cell tumor. Sertoli-Leydig cell tumors often present with hormone-related or non-hormonal symptoms. Surgery plays a crucial role in both diagnosis and treatment. Postoperative treatment is not necessary for well-differentiated Sertoli-Leydig cell tumors in stage IA-IB. However, patients with grade 2-3 disease, advanced stage, or heterologous elements may consider adjuvant treatment. As these tumors are rare, this case contributes to the documentation of Sertoli-Leydig cell tumors, with a case diagnosed due to ovarian torsion. The case highlights the importance of establishing international registries of Sertoli-Leydig cell tumor cases for standardized management.
