ABSTRACT
It represents 1% only of all malignancies that mostly affects young women. Its histological diagnosis is difficult and unpredictable. Trough this observation and literature review, we will outline the difficulties of management of this tumour. Mrs. I.R 28 years old had a secondary amenorrhea of 4 months with a huge abdominopelvic mass. CT examination described a mars occupying the right adnexa of 23 cm diameter orienting suspected teratoma or other tumours of ovarian origin. During surgery, we discovered an enormous fibrocyotic necrotic and friable mass, at the expense of the right ovary, so a right ophorectomy was done. The pathological examination confirmed the diagnosis. The patient was clinically manifested by an abdominal distension, with signs of hyperandrogenism and or hyperestrogenism. So a hormonal examination was essential in case of virilization to eliminate abnormal adrenal anomaly. The imaging and macroscopie study showed a solid avarian mass with some well-demarcated intra mural cysts of varying size. Histological examination defines the tumour grade. There are six sub-types that are valued by the degree of tubular differentiation of the Sertoli cell component and the abundance of primitive gonadal stroma. Recurrences are early. The reported prognostic factors are: histologic grade, tumor rupture and the presence of a heterologous mesenchymal elements. The differential diagnosis poses a problem with other ovarian tumours. Treatment consists of a lumpectomy or unilateral oophorectomy for the benign forms than a bilateral oophorectomy and hysterectomy if the tumour is poorly differentiated or greater than the stage of IFGO. This tumour is characterized by its rarity and difficulty of diagnosis. It occurs in a nulliparous or paucipare, young women. The unpredictable evolution causes a radical problem in the management of this entity