LGCmain
Indian Journal of Pathology and Microbiology
Home About us Instructions Submission Subscribe Advertise Contact e-Alerts Ahead Of Print Login 
Users Online: 1911
Print this page  Email this page Bookmark this page Small font sizeDefault font sizeIncrease font size


 
CASE REPORT Table of Contents   
Year : 2009  |  Volume : 52  |  Issue : 1  |  Page : 97-99
Bilateral sertoli-leydig cell tumor of the ovary: A rare case report


1 Departments of Pathology, Obstetrics, Jawahar Lal Nehru Medical College, Aligarh Muslim University, Aligarh, India
2 Department of Gynecology, Jawahar Lal Nehru Medical College, Aligarh Muslim University, Aligarh, India

Click here for correspondence address and email
 

   Abstract 

Sertoli leydig cell tumors also known as arrhenoblastoma, are a rare member of the sex cord-stromal tumor group of ovarian and testicular cancers, comprising less than 1% of all ovarian tumors, which occur in young adults and are almost always unilateral. We hereby report a case of a 17-year-old female presenting with a short history of irregular menses and an abdominal lump, which was histologically proven to be a bilateral sertoli leydig cell tumor of the ovary, an exceptionally rare entity in itself.

Keywords: Bilateral, sertoli leydig cell tumor

How to cite this article:
Alam K, Maheshwari V, Rashid S, Bhargava S. Bilateral sertoli-leydig cell tumor of the ovary: A rare case report. Indian J Pathol Microbiol 2009;52:97-9

How to cite this URL:
Alam K, Maheshwari V, Rashid S, Bhargava S. Bilateral sertoli-leydig cell tumor of the ovary: A rare case report. Indian J Pathol Microbiol [serial online] 2009 [cited 2020 Apr 8];52:97-9. Available from: http://www.ijpmonline.org/text.asp?2009/52/1/97/44981



   Introduction Top


Sertoli leydig cell tumors are rare sex cord-stromal neoplasms that account for less than 1% of ovarian tumors, occurring most commonly in young adults. [1],[2] A majority of the patients present with clinical features of virilization due to excessive secretion of testosterone by the tumor and more rarely estrogenization. However, 50% of the patients may have no endocrine symptomatology and may present with abdominal pain and lump only. [3],[4] Almost all the cases are unilateral, bilaterality being an exceptionally rare feature of these rare ovarian tumors. [4],[5],[6],[7] We report here an unusual case of a bilateral sertoli leydig cell ovarian tumor in a 17-year-old female presenting with irregular menses and abdominal lump.


   Case Report Top


A 17-year-old unmarried female presented in the gynecology department with a 4 month history of irregular menstrual cycle and abdominal distension. Her age at menarche was 12 years old. On abdomen examination, a lump was felt in the right side of the abdomen seemingly arising from the pelvis. There were no clinical features suggesting virilization, namely hirsuitism, hoarseness of voice, acne, clitoromegaly, breast atrophy, temporal hair recession, or increase in musculature. An ultrasonography revealed the presence of bilateral solid ovarian masses with a small cyst. A laparotomy revealed ovarian tumors on both sides. A bilateral salpingo-oopherectomy was performed. Also, an intraoperative peritoneal wash was sent for cytological examination.

The peritoneal washings showed sheets of atypical looking cells that were suggestive of malignancy and a probable diagnosis of metastatic adenocarcinoma was given [Figure 1].

Grossly, the right ovary measured 25x23x8 cm with a smooth external surface and on cut section showed yellowish areas with a small cystic space. The left ovary measured 10x8x3 cm with a similar gross appearance [Figure 2].

Microscopically, the ovaries were replaced by a nodular tumor composed of dual population of cells [Figure 3a]. These consisted of cords of immature round to oval  Sertoli cells More Details with darkly staining nuclei and islands of pale leydig cells with abundant vacuolated cytoplasm and round nuclei containing prominent nucleoli [Figure 3b]. Mitotic figures were less than 5 per 10 HPF. A focal retiform pattern was also seen [Figure 3c]. Few cystic spaces were seen [Figure 3d]. Periodic Acid Schiff staining was performed, which was positive in cords of sertoli cells [Figure 4]. The overall morphology was suggestive of sertoli leydig cell tumor of intermediate differentiation (Meyer's Type II), confined to the ovary, hence, Stage 1c according to the staging classification of the International Federation of Gynecology and Obstetrics.


   Discussion Top


Sertoli leydig cell tumors are rare sex cord stromal neoplasms accounting for less than 1% of ovarian tumors [2] and the majority present at an average age of 25 years. [1] The tumor is almost always unilateral unlike in our patient in which it was bilateral, which is extremely rare. A total of 40% of the cases present with androgen excess and more rarely estrogenization and 50% have a lump in the abdomen. Our patient was a 17-year-old female with oligomenorrhea and an abdominal lump.

The prognosis of sertoli leydig cell tumors is usually good and correlates with the stage and degree of differentiation. [1],[3] Our case was Meyer's Type II with intermediate differentiation which accounts for 54% of sertoli leydig cell tumors and was Stage Ic at presentation in keeping with reported series in which more than 90% of sertoli leydig cell tumors are Stage 1 at presentation. [2] In one series, 11% of intermediate differentiated and 59% of poorly differentiated tumors behaved in a clinically malignant fashion. [3] Presentation with Stage II or higher disease is also associated with a poor outcome. However, tumors without any apparent poor prognostic factors may behave in an aggressive fashion. [8] In a series by Zaloudek and Norris, 4 out of 20 poorly differentiated tumors, 1 out of 44 tumors of intermediate differentiation and none of the 7 well-differentiated tumors were malignant. A total of 25% of the Stage I tumors of intermediate differentiation with a retiform pattern were malignant as opposed to 10% of those with no retiform component. [9]

Recurrence of malignant sertoli leydig cell tumors is relatively early and the recurrent tumor is usually confined to the pelvis and abdomen but distant metastasis to the lung, scalp and supraclavicular lymph nodes have been reported. [3]

A bilateral salpigo-oopherectomy was done in our patient. However, adjuvant therapy may be advisable for Stage I tumors, which are poorly differentiated, have mesenchymal elements, or are ruptured tumors of intermediate differentiation. [3] Because in this patient, the tumor is Stage 1 and differentiation appears to be intermediate, the prognosis is expected to be good. However, the patient is being closely monitored.

Cytological diagnosis, especially intraoperative peritoneal washings of sex cord-stromal ovarian tumors, which constitute less than 1% of ovarian malignancies, should always be made with caution. A histopathological examination is mandatory in such cases for a definite diagnosis, more so in a young patient presenting with a lump in the abdomen with irregular menses and the absence of virilizing symptoms.


   Acknowledgement Top


Dr. Mehar Aziz MD; DNB (Pathology), Senior Professor, Department of Pathology, JNMC, AMU, Aligarh, India.

 
   References Top

1.Ayhan A, Tuncer ZS, Hakverdi AU, Yuce K. Sertoli leydig cell tumors of the ovary: A clinicopathologic study of 10 cases. Eur J Gynaecol Oncol 1995;17:75-8.  Back to cited text no. 1    
2.Choong CS, Fuller PJ, Chu S, Jeske Y, Bowling F, Brown R, et al . Sertoli leydig cell tumor of the ovary: A rare cause of precocious puberty in a 12 month old infant. J Clin Endocrinolmetab 2002;87:49-56.  Back to cited text no. 2    
3.Young RH, Scully RE. Ovarian sertoli leydig cell tumors: A clinicopathological analysis of 207 cases. Am J Surg Pathol 1985;9:543-69.   Back to cited text no. 3  [PUBMED]  
4.Oliva E, Alvarez T, Young RH. Sertoli cell tumors of the ovary: A clinicopathologic and immunohistochemical study of 54 cases. Am J Surg Pathol 2005;29:143-56.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Crum CP. The female genital tract. In: Kumar V, Abbas AK, Fausto N, eds. Robbins and Cotran Pathologic Basis of Disease. 7th ed. Philadelphia: WB Saunders; 2004. p. 1059-118.  Back to cited text no. 5    
6.Rosai J. Rosai and Ackerman's Surgical Pathology. 9th ed, vol. 2. St Louis Missouri: Mosby Company; 2004.  Back to cited text no. 6    
7.Young RH, Clement PB, Scully RE. Sex cord-stromal, steroid cell and germ cell tumors of the ovary. In: Mills SE, editor. Sternberg's Diagnostic Surgical Pathology. 4th ed. Philadelphia: Lippincot Williams and Wilkins; 2004. p. 2579-615.  Back to cited text no. 7    
8.Mooney EE, Nogales FF, Bergeron C, Tavassoli FA. Retiform sertoli leydig cell tumors: Clinical, morphological and immunohistochemical findings. Histopathology 2002;41:110-7.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]
9.Zaloudek C, Norris HJ. Sertoli leydig cell tumors of the ovary: A clinicopathologic study of 64 intermediate and poorly differentiated neoplasms. Am J Surg Pathol 1984;8:405-18.  Back to cited text no. 9  [PUBMED]  

Top
Correspondence Address:
Veena Maheshwari
2/82, Arya Nagar, Avantika Phase 2, Ramghat Road, Aligarh 202 001, UP
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.44981

Rights and Permissions


    Figures

  [Figure 1], [Figure 2], [Figure 3a], [Figure 3b], [Figure 3c], [Figure 3d], [Figure 4]

This article has been cited by
1 Bilateral, metachronic ovarian Sertoli–Leydig cell tumour in an 11-year-old patient: A case report
C. Gómez-Peñaloza,A. Cañavera-Constantino,G. Aristi-Urista
Revista Médica del Hospital General de México. 2018; 81(3): 139
[Pubmed] | [DOI]
2 Bilateral Sertoli-Leydig Cell Tumor in a Primigravida: A Rare Case
Ruchita Tyagi,Parimal Agrawal,Raje Nijhawan,GRV Prasad
Rare Tumors. 2014; 6(2): 60
[Pubmed] | [DOI]



 

Top
 
  Search
 
  
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
    Introduction
    Case Report
    Discussion
    Acknowledgement
    References
    Article Figures

 Article Access Statistics
    Viewed5091    
    Printed193    
    Emailed6    
    PDF Downloaded303    
    Comments [Add]    
    Cited by others 2    

Recommend this journal