I Chakrabarti, A De, M Gangopadhyay, P Bera
heterologous elements, ovary, sertoli-leydig cell tumor, virilisation
I Chakrabarti, A De, M Gangopadhyay, P Bera. Sertoli-Leydig cell tumour of ovary with heterologous elements—A case report. The Internet Journal of Gynecology and Obstetrics. 2009 Volume 13 Number 1.
Sertoli-Leydig tumors are rare tumors of the ovary, occurring in young women in the majority of cases. They constitute less than 0.5 % of all ovarian neoplasms and 1% of sex-cord tumors. Here, we report a case of a 26 year old multiparous woman presenting with abdominal lump, pain, secondary amenorrhea and virilizing signs and symptoms. A diagnosis of Sertoli-Leydig cell tumors of the ovary of intermediate differentiation with heterologous elements was given. Unilateral salpingo-oophorectomy followed by Bleomycin, Etoposide and Cisplatin combined chemotherapy was given. The patient was healthy and symptom-free at the 6 month follow-up.
Sertoli-Leydig cell tumors of the ovary have been defined by World Health Organization (WHO) as tumors composed of variable proportions of Sertoli cells, Leydig cells and in the case of intermediate and poorly differentiated neoplasms, primitive gonadal stroma and sometimes heterologous elements1. These are rare tumors accounting only for about 1% of sexcord-stromal tumors2 and constitute < 0.5% of all the ovarian neoplasms; intermediate and poorly differentiated forms being the most common. SLCTs have been reported in females from 2-75 years of age with a mean age of 23-25 years in different studies1.75% of the cases present within 30 years. The majority of these tumors are benign, and most are localized unilaterally. Bilaterality is reported in only 2% cases3.
It has been reported that about one-third of the patients present with virilizing symptoms and signs like amenorrhoea, hirsutism, breast atrophy, clitoral hypertrophy and hoarseness. Some patients may have estrogenic manifestations like isosexual pseudo-precocity and menometrorrhagia. It has been seen that the androgenic manifestations are more common in the poorly differentiated tumors. 20% cases have been reported to have slightly raised AFP. However, 50% of these patients may have no endocrine symptomatology and instead have abdominal pain or swelling. Heterologous elements are seen in 20% of the SLCTs4 .
A 26 year old married woman with 4 children, presented with secondary amenorrhea, pain abdomen and lower abdominal fullness for the last 2 years. On examination, hirsutism, hoarseness, breast atrophy and clitoromegaly were noted. Abdominal examination revealed a hard mass of about 20 weeks gestation size in the pelvic region. Ascites was absent. USG revealed a 12 × 8 cm cystic SOL with multiple septae in the left ovarian region. Chest X-ray and ECG were within normal limits. US guided FNAspiration yielded blood mixed mucoid material and microscopically seen to have poor cytological yield with only thick mucinous material. Estimation of androstenedione, by Radio-ImmunoAssay (RIA), showed a high value of 5.38 ng/ml (the normal value for adult female being 0.47-2.68 ng/ml.) The free testosterone level was 5.2 pg/ml. The normal level of free testosterone in cases of adult females usually ranges from 0.7 to 3.6 pg/ml. The estrogen and progesterone levels were within normal limits. A left sided salpingo-oophorectomy was done. Following the histopathology report, combination chemotherapy, usually with Bleomycin, Etoposide and Cisplatin (BEP) was started.
Inset shows Leydig cells at a higher magnification (H & E stain, 400x magnification)
Following the histopathology report, combination chemotherapy with Bleomycin, Etoposide and Cisplatin (BEP) was started. At 6 months follow-up, the virilizing symptoms and signs were found to have receded to a large extent. There was neither clinical nor sonographical evidence of recurrence and the sex hormone levels had returned to within normal range.
Sertoli-Leydig cell tumors are rare tumors of the ovary occurring more commonly in young women. The term Sertoli-Leydig cell tumors have replaced the previous terminologies like arrhenoblastomas and androblastomas. Pure Sertoli cell tumors are also included in this group but pure Leydig cell tumors are included under the heading of lipid cell tumors6. It is also interesting to note that the Sertoli and not the Leydig cells form the neoplastic component of these tumors7. Heterologous elements are seen in 20% of the SLCTs predominantly belonging to the moderately and poorly differentiated categories. The heterologous components may be in the form of mucinous glands, cartilage, bone, skeletal muscle, smooth muscle and fat4. However, smooth muscle element is described as rare occurrence5. Heterologous mesenchymal elements are usually found in the tumors that have sarcomatoid background. Several theories have been proposed to explain the histogenesis of heterologous elements in Sertoli-Leydig cell tumor. According to Young RH, Kleimen GM and Scully RE, the histogenesis involves neometaplasia, in which the cells undergoing neoplasia change into other types of cells that are not normally present in the tissue of origin of the tumor8. According to Hartz9, these tumors are of teratogenic origin. Heterologous mesenchymal elements (skeletal muscle or cartilage) or neuroblastoma imply a poor prognosis while gastrointestinal epithelium or carcinoid as the heterologous element does not have prognostic significance. Some authors opine that although overall SertoliLeydig cell tumors have a relatively good prognosis, intermediate tumors with retiform pattern and those containing heterologous elements have poorer prognosis. Tumor staging is extremely important from the prognostic point of view and guides the treatment too. For young women with stage I tumors, unilateral salpingo-ophorectomy is usually done and if poorly differentiated elements or heterologous elements are present, adjuvant therapy may be indicated with radiation or combination chemotherapy, usually with Bleomycin, Etoposide and Cisplatin (BEP). For tumors of Stage II or higher, a total abdominal hysterectomy with bilateral salpingo-oophorectomy is done and adjuvant therapy is often considered. Our case was treated with left sided salpingo-oophorectomy and BEP chemotherapy. At 6-month follow up, it was seen that the virilizing symptoms and signs had receded, sex hormone levels normalized and there was no complaint.