Bilateral retiform variant of sertoli leydig cell tumour of ovary: An uncommon tumor with review of literature.

Sertoli-leydig cell tumors are the uncommon sex-cord stromal tumors of the ovary. We report a case of 42-year-old female with retiform variant of sertoli-leydig cell tumour. She presented with the complaint of mass in abdomen for 7 years. Ultrasound revealed bilateral ovarian mass suggestive of malignancy. Bilateral salpingo-oopherectomy with surgical staging was done. The tumor was diagnosed as stage I retiform variant of sertoli-leydig cell tumor on histopathology and immunohistochemistry.

INTRODUCTION

Sertoli-leydig cell tumours (SCLTs) are rare sex-cord stromal tumors of the ovary. Retiform SCLT's are rare histological variant of SCLT with an average age of presentation being 16 years.[1] To the best of our knowledge the retiform variant of sertoli leydig tumors reported so far are seen in less than 10 years of age. We present a rare case of retiform variant of SCLT's presenting in a 42-year-old female.

CASE REPORT

A 42-year-old female presented with painless lump in the lower abdomen for 7 years. The lump was now increasing in size progressively from last 4 months. All hematological investigations were normal. Ultrasound showed bilateral ovarian mass suggestive of malignancy. The patient had two alive children and had completed family. So bilateral salpingo-oopherectomy with surgical staging was done. On gross examination, multiple grayish-white solid-cystic pieces of tissue were received. Largest measured 14 × 12 cm, smallest measured 3 × 3 cm. On cutting fleshy, firm tissue was found within the cyst wall [Figure 1]. Bilateral fallopian tissue were also seen. Microscopic examination showed ovarian tumor with tubular, cord like pattern lined by bland cuboidal to columnar cells. Also seen is retiform pattern at few places and scattered cells with abundant eosinophilic cytoplasm suggestive of leydig cells. Bilateral fallopian tubes were normal [Figures 2–6]. Immunohistochemistry showed that sertoli cells were strongly positivity for cytokeratin and negative for vimentin. Leydig cells showed focal positivity for vimentin and negativity for leydig cells [Figures 7 and 8]. Histological diagnosis of retiform variant of sertoli leydig cell tumor of ovary stage 1 (T1N0M0) was made. The patient was followed up to 2 years, she did not have any complaints.

Figure 1

Gross appearance of sertoli leydig cell tumor

Figure 2

Sertoli cells arranged in cords, sheets and aggregates (HE 100×)

Figure 6

Sertoli cells showing atypia (HE 400×)

Figure 7

Cytokeratin positivity in Sertoli component of sertoli leydig cell tumor of ovary (100×)

Figure 8

Vimentin positivity in Leydig component of sertoli leydig cell tumor of ovary (HE 100×)

Retiform pattern in serrtoli- leydig tumor (HE 100×)

Sertoliform cells with clear cytoplasm (HE 400×)

Leydig cells showing eosinophilic cytoplasm (HE 100×)

DISCUSSION

SLCTs of the ovary comprise less than 0.1% of ovarian neoplasms.[2] The average age of presentation is 24 years. However, retiform sertoli-leydig cell tumors present in the average age of 16-year-old. Clinically, 40% of the patients present with virilisation. Non-virilised patients present with non-specific symptoms like abdominal mass, pelvic pain.[1] It is seen that 80% of the patients with ovarian SLCTs and virilising manifestations present with elevated serum levels of testosterone and androstenedione.[34] Ultrasound is considered as the best imaging technique for the initial assessment of mass.[56] However, SCLTs are unilateral and are confined to ovary at the time of diagnosis.[1] Most of the tumors are stage I at the time of diagnosis.[7] Only less than 5% patients present with extra-ovarian spread.[1] Computerised tomography (CT), magnetic resonance imaging (MRI), and positron imaging tomography (PET) scans can provide us a better visualisation of the extra-ovarian or metastatic spread of the tumor.[7]

Grossly, they are predominantly solid but may show cystic areas also.[2] Well-differentiated SLCT have an average diameter of 5 cm whereas intermediate type and poorly differentiated types have an average diameter of 15 cm.[1] Microscopically, they are divided into following categories:

Well-differentiated or Meyer type 1: They constitute 11% of SLCT.

Intermediate type or meyer type 2: They constitute 54% of SLCT.

Poorly differentiated (sarcomatoid or undifferentiated or meyer type 3]: They constitute 13% of SLCT.

SCLT with heterologous elements like skeletal muscle, cartilage.

Retiform type (15%): They resemble rete of ovary or testes. They have blunt papillae with hyalinised or edematous cores.[2] Staging wise T1 means tumor limited to the ovaries, T2 means tumor involves one or both ovaries with pelvic extention, T3 means involvement of one or both ovaries with microscopically confirmed peritoneal metastasis outside the pelvis. N0 means no regional lymph node metastasis. N1 refers to regional lymph node metastasis. M0 refers to no distant metastasis. M1 refers to distant metastasis as per TNM classification of ovarian tumors.[8]

Immunohistochemically sertoli cels are positive for cytokeratin, CD99 and WT 1. Stromal cells and leydig cells are vimentin positive. Both sertoli cells and leydig cells are positive for calretinin and inhibin.[1]

Unilateral salpingo-oopherectomy is the adequate treatment of choice. A total abdominal hysterectomy is considered as the treatment of choice in patients with unfavourable findings like rupture, extra-ovarian spread, poorly differentiated neoplasm, heterologous mesenchymal differentiation.[1] Post-operative chemotherapy, radiotherapy or a combination of both, may also be considered in the patients with above mentioned poor prognostic factors.[9]

Well-differentiated SLCTs does not recur after excision. Intermediate and poorly differentiated tumors have 80% survival rates. Retiform SLCT has a slightly worse prognosis than rest of the group.[1] Similarly the 5 year survival rates for stage 1 tumor is 95% and that for stage III and Stage IV is zero percent.[910]