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 Table of Contents 
CASE REPORT
Year : 2021  |  Volume : 12  |  Issue : 1  |  Page : 82-84  

Steroid cell tumor of the ovary presenting with ascites: A rare neoplasm in a postmenopausal woman


1 Department of Cytology and Gynecological Pathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Obstetrics and Gynecology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
3 Department of Radiotherapy, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission30-May-2020
Date of Decision03-Jul-2020
Date of Acceptance26-Nov-2020
Date of Web Publication17-Apr-2021

Correspondence Address:
Parikshaa Gupta
Department of Cytology and Gynecological Pathology, Research 'A' Block, PGIMER, Sector 12, Chandigarh - 160 012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jmh.JMH_114_20

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   Abstract 


Steroid cell tumors of the ovary are rare sex-cord stromal tumors, accounting for approximately 0.1% of all ovarian neoplasms. Majority of these tumors are benign, occur in pre-menopausal women and are associated with hyperandrogenism. However, around one-third of cases are malignant and do not present with hormonal manifestations. A 48-year-old post-menopausal woman presented with complaints of gradually increasing progressive abdominal distension over the past 3 months. She had a history of weight gain but denied any symptoms of virilization. On examination, abdominal distension associated with ascites was noted. Serum CA125 level was raised. Contrast-enhanced computed tomography revealed a solid right adnexal mass. Based on the clinical impression of epithelial ovarian malignancy, the patient underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy and infracolic omentectomy. Histopathological examination revealed steroid cell tumor of the not otherwise specified type in the right ovary with the capsular breach. However, all other organs, including the omentum were free of tumor. The index case is unique for its presentation in a post-menopausal woman, association with ascites, elevated CA125 levels and lack of any virilization manifestations. Establishing an early and accurate tissue diagnosis is essential so that appropriate surgical management can be done to prevent the development of metastases in potentially malignant cases.

Keywords: Histopathology, immunohistochemistry, ovarian tumor, sex-cord stromal tumors, steroid cell tumor, virilization


How to cite this article:
Velamala P, Gupta P, Sikka P, Kumar D, Rajwanshi A. Steroid cell tumor of the ovary presenting with ascites: A rare neoplasm in a postmenopausal woman. J Mid-life Health 2021;12:82-4

How to cite this URL:
Velamala P, Gupta P, Sikka P, Kumar D, Rajwanshi A. Steroid cell tumor of the ovary presenting with ascites: A rare neoplasm in a postmenopausal woman. J Mid-life Health [serial online] 2021 [cited 2021 May 12];12:82-4. Available from: https://www.jmidlifehealth.org/text.asp?2021/12/1/82/313977




   Introduction Top


Steroid cell tumors of the ovary are rare sex-cord stromal tumors (SCST), accounting for approximately 0.1% of all ovarian neoplasms. These include three subtypes: stromal leuteomas, Leydig cell tumors, and steroid cell tumors, not otherwise specified (SCT-NOS).[1] Steroid cell tumor, NOS type is the more common, accounting for around 50%–60% of cases. As the name suggests, these tumors produce steroids and hence are associated with hyperandrogenism, leading to symptoms such as hair loss, hirsutism, temporal balding, and menstrual irregularities due to virilizing properties of the secreted hormones.[2] Owing to the hormonal symptoms, these patients are often diagnosed at an early stage, compared to ovarian epithelial neoplasms. However, around 20%–25% of patients lack these endocrine manifestations and hence may remain undiagnosed clinically for long periods.[3]


   Case Report Top


A 48-year-old post menopausal woman, presented with a history of progressively increasing abdominal distension and weight gain over the past 3 months. There was no associated abdominal pain, alteration of bowel/bladder habits, or vaginal discharge/bleeding. Her menstrual and obstetric history was not significant. She denied the intake of hormones or drugs and had not undergone any surgical intervention in the past.

On examination, her abdomen was distended with the presence of ascites. She did not have increased facial hair or acne. Contrast-enhanced computed tomography revealed a solid right adnexal mass measuring 8.9 cm × 11.5 cm × 9.5 cm. CA125 levels were raised (472 U/mL), however, other tumor markers (carcinoembyonic antigen, alpha fetoprotein, lactate dehydrogenase and CA 19-9) were within the normal limits. Based on the clinical, biochemical and radiological findings, a provisional diagnosis of an epithelial ovarian malignancy was made.

The patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy and infracolic omentectomy. Intra-operatively, 500 ml of straw colored fluid was found in the peritoneal cavity; however, no tumor deposits were noted.

Grossly, the right ovarian mass measured 11 cm × 9.5 cm × 8 cm with the capsular breach. On cut-section, the tumor was relatively well-circumscribed, predominantly solid (98%) with occasional small cystic areas filled with thick-mucoidy secretions (2%). The solid areas had a lobulated appearance, were firm and yellowish [Figure 1]. Left ovary measured 2.3 cm × 1.8 cm × 1 cm, bilateral fallopian tubes measured 5cm each, uterus measured 6.5 cm × 6 cm × 5 cm, and were grossly within the normal limits. Microscopically, the tumor showed a lobulated appearance with tumor cells arranged predominantly in diffuse sheets. The lobules were separated by thin fibrovascular septae. The tumor cells depicted mild pleomorphism, were round to polygonal with distinct cell membranes, round-to-oval centrally placed nuclei, vesicular chromatin, prominent nucleoli, and abundant clear to vacuolated cytoplasm. The nuclear atypia was mild, being grade 2. The mitotic index was <2/10 high power fields. Tumor necrosis or Reinke crystals were not seen. The capsular breach was confirmed microscopically. Based on the histopathologic features, a diagnosis of steroid cell tumor, NOS was rendered. All other organs, including the omentum, were free of tumor. Immunohistochemistry for inhibin showed granular cytoplasmic positivity in the tumor cells [Figure 1].
Figure 1: (a) Outer surface of the right ovarian mass showing lobulated appearance and capsular breach by the tumor; (b) Cut section of the ovarian mass showing solid, yellowish, lobulated tumor; (c) Relatively circumscribed tumor with tumor cells arranged in sheets (H and E, ×10); (d) Section showing capsular breach by tumor cells (H and E, ×4); (e) Section showing sheets of polygonal tumor cells with well-defined cell membranes, central nuclei with prominent nucleoli and abundant amount of clear to vacuolated cytoplasm (H and E, ×40); (f) Immunohistochemistry for Inhibin showing granular cytoplasmic positivity in the tumor cells (Inhibin, ×20)

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   Discussion Top


SCSTs of the ovary are rare and account for 4%–6% of all ovarian tumors.[4] Ovarian steroid cell tumors (OSCTs) are much rarer, with a reported frequency of around 0.1% of all the ovarian tumors.[5] Most commonly, OSCT is seen in premenopausal women with a mean age of 43 years.[6] The most common clinical presentation is abdominal pain and virilizing symptoms.[7] Presentation in postmenopausal women is rare and establishing an early clinical diagnosis in such cases becomes challenging, especially in the absence of virilizing manifestations.

OSCTs are usually benign, solid, and unilateral.[8] Approximately 25% of cases of SCT-NOS type are asymptomatic or present without hormonal manifestations. In addition, malignant OSCTs are also hormonally inactive.[3] The presence of ascites has been rarely noted in these tumors.[8] The index case presented with ascites without any symptoms of virilization. In addition, she had elevated CA125 levels, which also is an infrequent finding in OSCTs.[8] The most plausible explanation for this could be the mechanical irritation of the mesothelium by associated ascites.

A definite diagnosis can be established only by histopathological examination. Microscopically, the tumor cells are arranged diffusely in sheets, are polygonal, having round central nuclei with vesicular chromatin, prominent nucleoli, and abundant pale to eosinophilic and vacuolated cytoplasm. Lipid stains like oil red-O highlight the cytoplasmic lipids droplets. On immunohistochemistry, these tumor cells are positive for sex-cord stromal markers like inhibin and calretinin. The differential diagnoses include thecoma, clear cell carcinoma, metastatic renal cell carcinoma and rarely pheochromocytoma. The salient features that can help in differentiating these histopathologic mimics are listed in [Table 1].
Table 1: Histopathologic differential diagnoses of steroid cell tumor

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Although majority are benign, around one-third can be clinically malignant.[9] Associated ascites, weight loss, and anorexia favor malignant behavior of the tumor. The index patient was post-menopausal and presented with abdominal distension without any hormonal symptoms. She had ascites, which favors malignant nature. The most predictive malignant features of OSCTs, as reported in the literature, are the presence of two or more mitotic figures per 10 high-power fields; necrosis; diameter of 7 cm or greater; hemorrhage; and grade 2 or 3 nuclear atypia.[10] In the index case, the tumor diameter was >7 cm and the nuclear atypia was grade 2. In addition, there was capsular breach; however, no peritoneal or pelvic tumor deposits.


   Conclusions Top


OSCT is an extremely rare neoplasm. Although majority are benign, one-third can be malignant. The index case is unique for its presentation in a post-menopausal woman, association with ascites, elevated CA125 levels and lack of any virilization manifestations. Establishing an early and accurate tissue diagnosis is important for prompt surgical management to prevent the development of metastases in such cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Young RH, Clement PB, Scully RE. Sex-cord, stromal, steroid cell and germ cell tumours of the ovary. In: Mills SE, Carter D, Greenson JK, Oberman HA, Reuter V, Stoler MH, editors. Sternbergs Diagnostic Surgical Pathology. 4th ed. Philadelphia, Pa, USA: Lippincott Williams & Wilkins; 2004. p. 2579-615.  Back to cited text no. 1
    
2.
Revathy M, Kanchana MP. Incidence of virilisation in sex cord stromal tumours of ovary, a 5-year experience in a tertiary care gynaecological centre. J Evol Med Dent Sci 2018;7:886-91.  Back to cited text no. 2
    
3.
Amneus MW, Natarajan S. Pathologic quiz case: A rare tumor of the ovary. Arch Pathol Lab Med 2003;127:890-2.  Back to cited text no. 3
    
4.
Scully RE. Classification of human ovarian tumors. Environ Health Perspect 1987;73:15-24.  Back to cited text no. 4
    
5.
Scully RE, Young RH, Clement PB. Tumors of the ovary, mal-developed gonads, fallopian tube, and broad ligament. In: Steroid Cell Tumors. Washington, DC, USA: Armed Forces Institute of Pathology; 1996. p. 227-38.  Back to cited text no. 5
    
6.
Öz M, Özgü E, Türker M, Erkaya S, Güngör T. Steroid cell tumor of the ovary in a pregnant woman whose androgenic symptoms were masked by pregnancy. Arch Gynecol Obstet 2014;290:131-4.  Back to cited text no. 6
    
7.
Jiang W, Tao X, Fang F, Zhang S, Xu C. Benign and malignant ovarian steroid cell tumors, not otherwise specified: Case studies, comparison, and review of the literature. J Ovarian Res 2013;6:53.  Back to cited text no. 7
    
8.
Outwater EK, Marchetto B, Wagner BJ. Virilizing tumors of the ovary: Imaging features. Ultrasound Obstet Gynecol 2000;15:365-71.  Back to cited text no. 8
    
9.
Veras E, Deavers MT, Silva EG, Malpica A. Ovarian steroid cell tumor, not otherwise specified: A clinicopathologic study. Zhonghua Bing Li Xue Za Zhi 2007;36:516-20.  Back to cited text no. 9
    
10.
Hayes MC, Scully RE. Ovarian steroid cell tumors (not otherwise specified): A clinicopathological analysis of 63 cases. Am J Surg Pathol 1987;11:835-45.  Back to cited text no. 10
    


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