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ORIGINAL ARTICLE Table of Contents   
Year : 2010  |  Volume : 53  |  Issue : 4  |  Page : 658-660
The morphologic and immunohistochemical spectrum of 16 cases of sclerosing stromal tumor of the ovary


Aga Khan Medical University, Karachi, Pakistan

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Date of Web Publication27-Oct-2010
 

   Abstract 

Background: Sex-cord-stromal tumors of the ovary are rare neoplasms and they differ from the common surface epithelial tumors in clinical and imaging features. Patients and Methods: This study was done to evaluate the morphologic and immunohistochemical pattern of 16 cases of sclerosing stromal tumor of the ovary diagnosed at a tertiary care hospital. All patients having sclerosing stromal tumor of the ovary were included with the help of medical records using the SNOMED coding system over 10 year period i.e. 1998 to 2008. Result: Sixteen cases of the stromal tumor of the ovary were diagnosed over this 10 year period; the age range was 23 to 40 years (median age 28). In 7 out of 16 cases, serum CA 125 level was done which was raised as per the available medical records. All of these cases showed unilateral. The gross appearence of all the tumors was similar and all had unilateral involvement. Mucin was negative in all of these cases.

Keywords: Neoplasms, ovary, stromal tumors

How to cite this article:
Qureshi A, Raza A, Kayani N. The morphologic and immunohistochemical spectrum of 16 cases of sclerosing stromal tumor of the ovary. Indian J Pathol Microbiol 2010;53:658-60

How to cite this URL:
Qureshi A, Raza A, Kayani N. The morphologic and immunohistochemical spectrum of 16 cases of sclerosing stromal tumor of the ovary. Indian J Pathol Microbiol [serial online] 2010 [cited 2020 Nov 24];53:658-60. Available from: https://www.ijpmonline.org/text.asp?2010/53/4/658/72017



   Introduction Top


Sex cord-stromal tumors of the ovary are rare, making up approximately 8% of all ovarian neoplasms. These tumors arise from two groups of cells in the ovary: stromal cells and primitive sex cord cells. Stromal cells contain fibroblasts, theca cells, and Leydig cells, and primitive sex cords include granulosa cells in the normal ovary, Sertoli cells in the testis, and Sertoli cells in ovarian tumors. [1] They differ from more common epithelial neoplasms in clinical and radiologic aspects. Understanding the clinical and imaging features of sex cord-stromal tumor of the ovary is helpful in specific diagnosis of ovarian tumors.

Sex cord-stromal tumors of the ovary affect all age groups and account for most of the hormonally active ovarian tumors that show estrogenic effects or virilization. Unlike patients with common epithelial tumors, of which 75% are considered to be at stage III or IV at diagnosis, approximately 70% of patients with these tumors are classified as having stage I lesions at presentation. [2],[3] Consequently, sex cord-stromal tumors are primarily treated surgically and generally have a good prognosis. In addition, these tumors may have characteristic imaging features.


   Patients and Methods Top


All patients of sclerosing stromal tumor of the ovary were included with the help of medical records using the SNOMED coding system over 10 year period i.e. 1998 to 2008.


   Results Top


The gross, histological, and immunohistochemical features were as follows;

Grossly there was unilateral involvement in all 16 cases; mostly these tumors were well demarcated with predominantly a solid white mass with intervening yellow areas and cyst formation [Figure 1], gross photograph.
Figure 1 :Gross photograph

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Low power examination showed pseudo lobules separated by stroma that varies from densely hyalinized to markedly edematous. Two cell types were identified with in the nodules spindle cells producing collagen and round to oval cells with small dark nuclei [Figure 2].
Figure 2 :Tumor cells with spindle cell morphology (H and E, ×200)

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Immunohistochemical stains inhibin [Figure 3] and calretinin [Figure 4] were diffusely positive.
Figure 3 :Inhibin immunohistochemical stain showing diffuse positivity in tumor cells (IHC, ×200)

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Figure 4 :Calretinin immunohistochemical stain showing diffuse positivity in tumor cells (IHC, ×200)

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Immunohistochemical features of all cases are described in [Table 1].
Table 1 :Histochemical and immunohistochemical stains


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   Discussion Top


Ovarian sex cord stromal tumors are classified as granulosa cell tumors, Sertoli's stromal tumors, steroid cell tumors, thecomas, and fibromas. Sclerosing stromal tumors were first described as a distinct entity among ovarian sex cord stromal tumors by Chalvardjian and Scully in 1973. [1] They are known to have the following characteristic clinical features: first, they usually occur in a younger age group, whereas other types of stromal tumors are most common in the fifth and sixth decades, about 80% of SSTs are encountered during the second and third decades. [2],[3] To date, malignant SST has not been reported. [4],[5],[6] In our study, all cases were unilateral, and in several previously published reports, unilateral occurrence has been described. [7],[8] Hormonal activity has been reported in a few documented cases, though none of our patients underwent hormonal assay, they demonstrated no symptoms suggesting hormonal activity. [6]

Microscopically, SSTs are characterized by a number of distinctive features: a pseudolobular growth pattern, in which cellular areas are separated by edematous and collagenous hypocellular areas; collagenous sclerosis within the cellular areas; marked vascularity, with a "hemangiopericytomatous" pattern; and heterogeneity of the cell population. [9]

Immunohistochemical profile of our cases is vimentin, inhibin, calretinin and mic-2 positive and CK negative. Asma and desmin immunohistochemical stains were also positive. Same profile has been suggested by Kawauchi et al. [10] The differential diagnosis of SSTs should include thecoma/ fibroma, metastases, and malignant epithelial ovarian tumors. All these have distinctive morphological and immunohistochemical features. Ovarian metastases and malignant epithelial tumors usually occur in older patients, and dynamic magnetic resonance (MR) imaging does not usually reveal progressive centripetal enhancement. [11],[12]

We have described a series of ovarian tumors with distinct morphology that should be distinguished from a variety of ovarian tumors whose features may overlap to some degree. Because of limited follow up comment on behavior cannot be dogmatically made however according to whatever information we had, a benign behavior is suggested.


   Conclusion Top


Sclerosing stromal tumor of the ovary is a rare tumor and occurs in the younger age group. This tumor has typical gross, morphologic, and immunohistochemical features. It is important to recognize these rare tumors as there is a possibility that these may be misdiagnosed as aggressive neoplasms.

 
   References Top

1.Chalvardjian A, Scully RE. Sclerosing stromal tumor of the ovary. Cancer 1973;31:664-70.  Back to cited text no. 1
    
2.Irving JA, Young RH. Microcystic stromal tumor of the ovary: Report of 16 cases of a hitherto uncharacterized distinctive ovarian neoplasm. Am J Surg Pathol 2009;33:367-75.  Back to cited text no. 2
    
3.Charfi Darghouth L, Goutallier Ben Fadhel C, Lahmar A, Mestiri H, Khalfallah MT, M'Zabi Regaya S. Sclerosing stromal tumor of the ovary: A case report and literature review] Tunis Med 2007;5:430-2.   Back to cited text no. 3
    
4.Akyildiz EU, Ilvan S, Calay Z, Oktar H. Sclerosing stromal tumor of the ovary: Report of three cases. J BUON 2004;9:205-7.  Back to cited text no. 4
    
5.Pai RR, Shaktawat SS, Khadilkar UN, Lobo FD, Rao VS, Philipose R. Sclerosing stromal tumour of the ovary-a clinicopathologic spectrum. Indian J Pathol Microbiol 2005;48:370-2.  Back to cited text no. 5
    
6.Mathur SR, Dahiya S, Nayak A, Bhatla N, Singh MK. Sclerosing stromal tumour of ovary-a clinicopathological and immunohistochemical study of five cases. Indian J Pathol Microbiol 2004;47:333-5.  Back to cited text no. 6
    
7.Korczyiρski J, Gottwald L, Pasz-Walczak G, Kubiak R, Bieρkiewicz A. Sclerosing stromal tumor of the ovary in a 30-year-old woman. A case report and review of the literature. Ginekol Pol 2005;76:471-5.  Back to cited text no. 7
    
8.Bouraoui S, Ben Fadhl C, Bellil K, Guettiti-Tounsi H, Mekni A, Kchir N, et al. Sclerosing stromal tumor of the ovary. Report of 3 cases. Tunis Med 2004;82:1111-5.  Back to cited text no. 8
    
9.Kawauchi S, Tsuji T, Kaku T, Kamura T, Nakano H, Tsuneyoshi M. Sclerosing stromal tumor of the ovary: a clinicopathologic, immunohistochemical, ultrastructural, and cytogenetic analysis with special reference to its vasculature. Am J Surg Pathol 1998;22:83-92.  Back to cited text no. 9
    
10.Ihara N, Togashi K, Todo G, Nakai A, Kojima N, Ishigaki T, et al. Sclerosing stromal tumor of the ovary: MRI. J Comput Assist Tomogr 1999;23:555-7.   Back to cited text no. 10
    
11.Matsubayashi R, Matsuo Y, Doi J, Kudo S, Matsuguchi K, Sugimori H. Sclerosing stromal tumor of the ovary: radiologic findings. Eur Radiol 1999;9:1335-8.   Back to cited text no. 11
    
12.Torricelli P, Caruso Lombardi A, Boselli F, Rossi G. Sclerosing stromal tumor of the ovary: US, CT, and MRI findings. Abdom Imaging 2002;27:588-91.  Back to cited text no. 12
    

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Correspondence Address:
Asim Qureshi
Aga Khan Medical University, Karachi
Pakistan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.72017

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1]

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