|Year : 2014 | Volume
| Issue : 2 | Page : 231-233
Sclerosing stromal tumor of the ovary: A case report
Navjot Kaur1, Neelam Sharma2, Vijay Kaushal2, Sarita Asotra2, Jagjit Singh Chahal3
1 Department of Pathology, Dr. Rajendra Prasad Government Medical College, Tanda, Himachal Pradesh, India
2 Department of Pathology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
3 Department of Biochemistry, Dr. Rajendra Prasad Government Medical College, Tanda, Himachal Pradesh, India
|Date of Web Publication||11-Nov-2014|
Department of Pathology, Dr. Rajendra Prasad Government Medical College, Tanda - 76 001, Himachal Pradesh
Source of Support: None, Conflict of Interest: None
Sclerosing stromal tumors are benign ovarian neoplasms of the sex cord-stromal category, occurring predominantly in the second and third decades of life. Herein, we report a 23-year-old female who presented with pelvic pain, irregular menses but normal hormonal status and was diagnosed as having a right ovarian tumor. A right oophorectomy was performed, and microscopic examination revealed a sclerosing stromal tumor of the right ovary. We stress the importance of being familiar with sclerosing stromal tumors when evaluating ovarian neoplasms in young women, in order to contribute to the appropriate clinical management, preventing extensive and unnecessary surgery, and preserving fertility.
Keywords: Ovary, sclerosing stromal tumor, sex-cord stromal tumor
|How to cite this article:|
Kaur N, Sharma N, Kaushal V, Asotra S, Chahal JS. Sclerosing stromal tumor of the ovary: A case report
. Arch Med Health Sci 2014;2:231-3
| Introduction|| |
Sclerosing stromal tumors (SSTs) of the ovary have been described as a pathologically and clinically distinct subgroup within the thecoma-fibroma spectrum of benign ovarian sex cord-stromal tumors. It is a rare ovarian disease with prevalence of 1.5-6% of ovarian stromal tumors occurring predominantly in young women. It is distinguished from other ovarian tumors by the production of collagen and a pseudolobular pattern with cellular areas separated by edematous and collagenous areas.  Most of the reported cases have been unilateral. All SSTs encountered to date have been benign and no recurrence after conservative surgery has been reported in the literature. Hence, a correct pre- or intraoperative diagnosis is important so that a conservative surgical approach can be undertaken. We are reporting a case of a 23-year-old female who developed an SST of the right ovary.
| Case Report|| |
A 23-year-old unmarried woman reported to the gynecology out-patient department (OPD) with the complaints of pelvic pain and irregular menses for the last 4 months. On per abdomen examination, a vague hypogastric mass was palpable. Ultrasonography revealed a right ovarian mass of size 4 cm × 4 cm with heterogeneous echotexture. Contrast enhanced computed tomography (CECT) showed a complex enhancing mass of size 4.7 cm × 4.4 cm × 3.8 cm in right ovary with minimal fluid in the pouch of douglas. Left ovary was normal in shape and echotexture. All routine hematological tests, biochemical tests, and x-ray chest were normal except for an elevated CA-125 level of 164 IU/ml. With a preoperative diagnosis of a right ovarian tumor, the patient underwent exploratory laparotomy. Intraoperatively, there was a right ovarian tumor, which was solid and well encapsulated. The other ovary and uterus were normal and there were no deposits on the bowel wall, omentum or other viscera. Peritoneal wash was done and sent for cytology, which showed only mesothelial cells and no evidence of cancer. A right oophorectomy was performed, and care was taken that the ovary is removed intact. The specimen was then sent for histopathological examination. Postoperative course was uneventful.
Grossly, the right ovarian mass measured 4.5 cm × 4.0 cm × 3.0 cm. The mass was lobulated, well demarcated, smooth and gray-white to yellow in color. On cut-section, the mass was solid, gray-white with yellow flecks along with focal mucinous and cystic change [Figure 1]. No necrosis or hemorrhage was observed. Normal ovarian tissue was not identified.
|Figure 1: Cut section of the ovarian mass showing solid, gray-white areas with yellow flecks along with focal mucinous and cystic change|
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Microscopic examination showed ill-defined pseudolobules of cellular areas and hypocellular, edematous or collagenous areas [Figure 2]; it had a prominent vasculature, prominent sclerosis around clusters of individual cells, and cellular heterogeneity of the vacuolated luteinized theca-like cells, and spindle-shaped fibroblast-like cells in the cellular areas.
|Figure 2: Photomicrograph showing a pseudolobular pattern of cellular areas and hypocellular, edematous, or collagenous areas (H & E, ×100)|
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After an uneventful post-operative recovery, regular menstruation resumed. Regular follow-up showed no evidence of tumor relapse until 2 years after surgery.
| Discussion|| |
Sclerosing stromal tumor of the ovary as an entity is distinct from other sex cord-stromal tumors because of its unusual clinical presentation at an early age (80% occurring in second and third decades) and lack of hormonal manifestations. The most common presenting clinical symptoms include menstrual irregularity, pelvic pain, and non-specific symptoms related to the ovarian mass. , Our patient had irregular menses and pelvic pain.
Ultrasonography is a useful initial tool for differentiating between cystic and solid masses and determining the organ of origin. However, computed tomography and magnetic resonance imaging (MRI) are both more sensitive for delineating the nature of the mass and tumor extension. On MRI, a diagnosis of SST can be strongly suggested, when typical signal patterns such as hypointense nodules, hyperintense stroma, lobulation, strong enhancement with gadolinium, and a peripheral hypointense rim are present. 
The differential diagnosis of SSTs of the ovary includes other sex cord-stromal tumors such as fibromas and thecomas, which generally occur in the fifth or sixth decades of life. These may be differentiated from the SST on the basis of histopathology and immunohistochemical findings.  SSTs are characterized histopathologically by cellular pseudolobules, prominent interlobular fibrosis, marked vascularity, and a dual cell population: Collagen producing spindle cells and lipid-containing round or ovoid cells. The heterogeneity due to the variation in cellular size and shape are helpful features in the differential diagnosis of SST, and contrasts with the relative homogeneity of thecomas and fibromas. Also SSTs do not have hyalinized plaques, as do fibromas and thecomas.  Immunohistochemically, the cells of SST are positive for vimentin, smooth muscle actin, α-inhibin, and CD 99; and are negative for S-100 protein and epithelial markers.  Immunohistochemical analysis in our patient demonstrated positivity for inhibin [Figure 3] and vimentin and negativity for desmin, S-100 protein, and cytokeratin.
Vascular tumors are included in the differential diagnosis due to prominent vascularity, but inhibin positivity suggests the diagnosis of SST. On the other hand, massive ovarian edema may be confused with SST. But preserved ovarian tissue within the edematous stroma and absence of heterogeneity favors the diagnosis of massive ovarian edema. In addition, the edema of SST is zonal in contrast to that seen in massive ovarian edema or an edematous fibroma. Infrequently the vacuolated cells and the presence of signet-ring cells in association with edematous stroma may be mistaken for signet-ring cells of Krukenberg tumor of the ovary. But these malignant tumors occur typically in women in the sixth and seventh decades, are mostly bilateral and lack the pseudolobulated pattern of SSTs. Furthermore, signet-ring cells of Krukenberg tumors contain mucin rather than lipid, and they may exhibit mitotic activity and nuclear atypia. 
Our patient had elevated CA-125 levels. An elevated serum level of CA-125 is unusual, and it has been thought that it is the consequence of physical irritation and inflammation. Some cases of SST with raised CA-125 level have been reported, which reinforces the non-specificity of CA-125 as a marker of ovarian malignancy. ,
Due to the rarity of this particular ovarian neoplasm, it is not always possible to predict the presence of this tumor preoperatively, on the basis of clinical and sonographic findings and may even be misdiagnosed as aggressive neoplasms. Although a rare entity, one can encounter such cases in clinical practice, and this case highlights the limitations of clinical examination, imaging, and CA-125 levels and strongly supports that a definitive diagnosis of SST is made only by histopathological evaluation but at least a diagnosis of benign ovarian tumor is possible intraoperatively via frozen section analysis.
| References|| |
|1.||Peng HH, Chang TC, Hsueh S. Sclerosing stromal tumor of ovary. Chang Gung Med 2003;26:444-8. |
|2.||Park SM, Kim YN, Woo YJ, Choi HS, Lee JS, Heo SH, et al. A sclerosing stromal tumor of the ovary with masculinization in a premenarchal girl. Korean J Pediatr 2011;54:224-7. |
|3.||Akbulut M, Colakoglu N, Soysal ME, Duzcan SE. Sclerosing stromal tumor of the ovary: Report of a case and review of the literature. APJ 2004;1:84-9. |
|4.||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. |
[Figure 1], [Figure 2], [Figure 3]