The Rare Sertoli Leydig Ovarian Cell Tumours

The Sertoli leydig cell tumours are quite unusual, accounting to 0.2-0.5% of all ovarian cancers. These are nothing but the sex cord stromal tumours of the ovary that may be present as a grossly appearing mass dependant on the histological grade.

These Sertoli leydig cell tumors are highly malignant and have been shown to have ancestral occurrence and associated thyroid irregularities that are characterized by existence of testicular structure.

The women with this atypical condition experienced nonappearance, irregularities of periods, post-menopausal bleeding, pain experienced in the lower part of the abdomen, unusual hair growth distributions over the body and notable alterations like roughness in voice noted.

A generalised physical exam of such women showed characteristic features of hirsutism in the form of atypical thick, coarse hair distribution noticed on the areas like the chin, sides of the cheeks, abdominal area and also on both limbs, acne, raised libido and balding in the temporal region. Such women undergo masculinisation that is evident by the abnormal enlargement of the clitoris, pubic hair distribution resembling men and a wastage or decrease in size of breast tissue.

An abdominal exam undertaken in such women pointed to the presence of singular, distinct, even-surfaced, movable, firm mass measuring sizes of close to ten centimetres in size located in the region where the three merged bones that make up the hip bone. The rectal exam also corroborated the above findings and displayed a normal, firm, regular sized uterus. The chest x-rays and hemograms of such women show normal results. The pelvic sonography usually point to the presence of an ovarian mass with internal echoes and an empty cavity. These women displayed elevated levels of serum testosterone.

Following the clinical analysis of the ovarian stromal tumour, laparotomy is undertaken wherein the peritoneal fluid is extracted from several sites for purpose of cytology and oophorectomy undertaken. Following freeze segment report of the sertoli leydig cell tumour, several biopsies of the peritoneum and omental biopsy are undertaken.  Following the operative phase, these women are given additional chemotherapy sessions that entailed the use of BEP Bleomycin, Etoposide and Cisplatin for every three weeks for three menstrual cycles along with dosage of oral contraceptives.

The undesirable symptoms were drastically reduced following six months of this treatment with hirsutism and clitoral enlargement disappearing within a period of two months and periods normalising after six months. The serum testosterone levels also normalized following the foremost chemotherapy cycle.

The management of this rare disease is an individualised approach with staging laparotomy and total surgical removal being the basis of treatment. In younger women, fertility conserving surgery is employed as most of the tumours are unilateral in nature, in youngsters. In aged patients who are way past their fertile phase, a complete abdominal hysterectomy, cytoreductive operation, oophorectomy and infracolic omentectomy are undertaken. Relapse is rare in well-distinguished tumours, but in cases of less defined type there are 20% chances of relapse within a year’s time. Majority of patients with relapse within two years normally die.

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