|Year : 2015 | Volume
| Issue : 1 | Page : 35-38
Bilateral retiform variant of sertoli leydig cell tumour of ovary: An uncommon tumor with review of literature
Monika Rathi1, Satish Kumar Budania2, Mohammad Khalid3, Ankur Mittal4
1 Department of Pathology, Lala Lajpat Rai Memorial Medical College, Meerut and Department of Pathology, Teerthanker Mahaveer Medical College and Research Center, Moradabad, Uttar Pradesh, India
2 Department of Psychiatry, Lala Lajpat Rai Memorial Medical College, Meerut, Uttar Pradesh, India
3 Department of Surgery, Mayo Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
4 Department of Urology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
|Date of Web Publication||19-Mar-2015|
Dr. Monika Rathi
228/16, Saket Colony, North Civil Lines, Muzaffarnagar - 251 001, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
| Abstract|| |
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.
Keywords: Retiform variant, sertoli-leydig cell tumour, sex-cord stromal tumor
|How to cite this article:|
Rathi M, Budania SK, Khalid M, Mittal A. Bilateral retiform variant of sertoli leydig cell tumour of ovary: An uncommon tumor with review of literature. J Mid-life Health 2015;6:35-8
|How to cite this URL:|
Rathi M, Budania SK, Khalid M, Mittal A. Bilateral retiform variant of sertoli leydig cell tumour of ovary: An uncommon tumor with review of literature. J Mid-life Health [serial online] 2015 [cited 2023 Jan 31];6:35-8. Available from: https://www.jmidlifehealth.org/text.asp?2015/6/1/35/153625
| 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.  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 [Figure 2], [Figure 3], [Figure 4], [Figure 5] and [Figure 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 [Figure 7] and [Figure 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 2: Sertoli cells arranged in cords, sheets and aggregates (HE 100×)|
Click here to view
|Figure 7: Cytokeratin positivity in Sertoli component of sertoli leydig cell tumor of ovary (100×)|
Click here to view
|Figure 8: Vimentin positivity in Leydig component of sertoli leydig cell tumor of ovary (HE 100×)|
Click here to view
| Discussion|| |
SLCTs of the ovary comprise less than 0.1% of ovarian neoplasms.  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.  It is seen that 80% of the patients with ovarian SLCTs and virilising manifestations present with elevated serum levels of testosterone and androstenedione. , Ultrasound is considered as the best imaging technique for the initial assessment of mass. , However, SCLTs are unilateral and are confined to ovary at the time of diagnosis.  Most of the tumors are stage I at the time of diagnosis.  Only less than 5% patients present with extra-ovarian spread.  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. 
Grossly, they are predominantly solid but may show cystic areas also.  Well-differentiated SLCT have an average diameter of 5 cm whereas intermediate type and poorly differentiated types have an average diameter of 15 cm.  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.  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. 
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. 
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.  Post-operative chemotherapy, radiotherapy or a combination of both, may also be considered in the patients with above mentioned poor prognostic factors. 
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.  Similarly the 5 year survival rates for stage 1 tumor is 95% and that for stage III and Stage IV is zero percent. ,
| Acknowledgement|| |
We acknowledge Engineer Ayush for his technical help.
| References|| |
Zaloudek CF. Tumours of the female genital tract. In: Fletcher DM, editor. Diagnostic Histopathology of Tumours. 3 rd
ed. London: Churchill Livingstone Elsevier; 2007. p. 595-8.
Rosai J. Female reproductive system-ovary. In: Rosai J, editor. Rosai and Ackerman's Surgical Pathology. 10 th
ed. St. Louis: Elsevier Mosby; 2011. p. 1600-1.
Osborn RH, Yannone ME. Plasma androgens in the normal and androgenic female: A review. Obstet Gynecol Surv 1971;26:195-228.
Prunty FT. Hirsutism, virilism and apparent virilism and their gonadal relationship. II. J Endocrinol 1967;38:203-27.
Zanotti KM. The clinical manifestations and diagnosis of Sertoli-Leydig cell tumors of the ovary. CME J Gynecol Oncol 2002;7:129-33.
de Oliveira Franzin CM, Kraft ML, Faundes D, Zeferino LC, Alvarenga M, Marussi EF. Detection of ovarian Sertoli-Leydig cell tumors exclusively by color Doppler sonography. J Ultrasound Med 2006;25:1327-30.
Abu-Zaid A, Azzam A, Alghuneim LA, Metawee MT, Amin T, Al-Hussain TO. Poorly differentiated ovarian sertoli-leydig cell tumor in a 16-year-old single woman: A case report and literature review. Case Rep Obstet Gynecol 2013;2013:858501.
Lee KR, Tavassoli FA, Prat J, Dietel M, Gersel DJ, Karseladze AI, et al
. Tumours of the ovary and peritoneum. In: Fattaneh A, editor. Pathology and Genetics. Tumours of the Breast and Female Genital Organs. 3 rd
ed. Lyon: IARC Press; 2003. p. 113-7.
Young RH, Scully RE. Ovarian Sertoli-Leydig cell tumors. A clinicopathological analysis of 207 cases. Am J Surg Pathol 1985;9:543-69.
Zaloudek C, Norris HJ. Sertoli-Leydig tumors of the ovary. A clinicopathologic study of 64 intermediate and poorly differentiated neoplasms. Am J Surg Pathol 1984;8:405-18.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]