Sertoli-Leydig Cell Ovarian Tumors: Is Fertility or Endocrine-Sparing Surgery an Option upon Relapse?

Abstract

Sertoli-Leydig cell ovarian tumors (SLCT) are rare ovarian tumors of the sex cord-stroma subset. Their incidence peaks in the second to third decade of life. Most SCLT are diagnosed at an early stage and have a good prognosis. Fertility-sparing surgery may thus be offered. Adjuvant chemotherapy may be indicated according to prognostic factors. However, outcome in relapsing SLCT is poor. There is no evidence supporting a best treatment option upon relapse, but most publications combine radical surgery, chemotherapy, and rarely radiotherapy. Two years after left adnexectomy for FIGO IA SLCT, a now 22-year-old patient presented with peritoneal recurrence without involvement of the remaining ovary and uterus. Since there is no evidence of a survival benefit in the literature of macroscopically healthy contralateral ovary ablation in relapse and hormonal replacement therapy is contraindicative, we consented to endocrine-sparing surgery with conservation of the contralateral ovary, followed by 3 cycles of BEP chemotherapy regimen. Our patient is disease-free 16 months after relapse diagnosis. Since recurrence of SLCT has a very poor prognosis and hormonal treatment is contraindicated, endocrine-sparing surgery for young patients with a normal contralateral ovary might be a legitimate option. This is one of the first reported cases of conservative surgery in SLCT recurrence, we therefore aimed to illustrate its management in a young patient with considerations of contraception, fertility-and then endocrine-sparing surgery, and quality of life.

Ovarian Sertoli–Leydig cell tumours: A systematic review of relapsed cases

“…SLCT relapses tend to be systemic and multi-focal. The peritoneum is a very common site for relapse of this tumour [3,41,42]. The present results confirm this trend, as shown in Table 3 [52% were distant and 20/75 (26.7%) had peritoneal carcinomatosis].…”

“…The results show a trend (p = 0.117) for better survival with radical surgery (conservative surgery -2 years: 61.0%, 5 years: 20.3%; radical surgery -2 years: 78.5%, 5 years: 43.3%). To date, there are no reports in the literature with strong evidence of survival benefit of radical surgery in patients with SLCT relapses and a remaining macroscopically normal ovary [3].…”

“…They belong to a rare and heterogeneous subgroup of ovarian neoplasms, the sex cord stromal tumours (SCSTs). The peak incidence of women diagnosed with SLCT occurs in the second and third decade of life, but can occur in all age groups [1][2][3][4][5]. Women with SLCT develop signs of androgen excess in 40-50% of cases [1,6].…”

“…In contrast to other SCST such as ovarian granulosa cell tumours, SLCT tend to relapse early, with a relapse rate of 95% within 5 years [6,10]. The literature also reports a very poor prognosis for relapse, with a salvage rate of <20% for clinically malignant and recurrent disease [2,3]. SLCT relapses tend to be unifocal or multi-focal and localized in the pelvis or the abdomen [11,12].…”

“…It is mainly based on a few case reports and small retrospective series. When possible, surgery remains the cornerstone of treatment upon relapse, and appears to be the most effective approach [2,3,13]. Platinumbased chemotherapy such as bleomycin + etoposide + cisplatin (BEP) and paclitaxel + carboplatin (tax-carb) regimens have shown the best results, with overall response rates of 63-80% for advanced stage and recurrent disease [2].…”