Sclerosing Stromal Tumour in Young Women: Clinicopathologic and Pathology S Ection Immunohistochemical Spectrum
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Original Article DOI: 10.7860/JCDR/2013/6031.3373 Sclerosing Stromal Tumour in ection S Young Women: Clinicopathologic and Pathology Immunohistochemical Spectrum ECMEL ISIK KAYGUSUZ1, SUNA CESUR2, HANDAN CETINER3, HULYA YAVUZ4, NERMIN KOC5 ABSTRACT muscle actin, desmine, progesterone receptor, calretinin, inhibin Aim: Sclerosing stromal tumor is a benign tumor of ovary. We were positive in all the cases; S-100, cytokeratin, cytokeratin7, aimed to review the clinical findings and immunohistochemical estrogen receptor were negative in all the cases; CD-10 was results of SSTs through the 7 diagnosed cases in our hospital. positive in 2 cases; C-kit was positive in 5 cases; melan-A was positive in 4 cases. Material and Methods: As immunohistochemical, blocks were applied with estrogen receptor , progesterone receptor, inhibin, Conclusions: The significance of these tumors is that it is calretinin, melan-A, CD10, smooth muscle actin, desmine, necessary to distinguish the histopathology in the frozen section vimentin, CD34, S-100, C-kit, cytokeratin , cytokeratin7. in order to protect the other adnexa because of the characteristics to be observed at early ages (2nd and 3rd decades). Results: Macroscopically, while 5 tumors had solid appearance, 2 tumors were composed of solid and cystic areas. All the Our findings support the conclusion that sclerosing stromal tumors were in shape of ovarian masses with good limits. tumors are benign–character tumors that stem from over stroma Microscopically, two types of cells were observed as fusiform and are hormonally active tumors because of the detected clinical fibroblast-like cells and theca-like cells with vacuolised and immunohistochemical results, although no hormonal effect cytoplasm. Immunohistochemical results: vimentin, smooth that could be supported with laboratory tests was observed. Key words: Sclerosing, Ovary, Immunohistochemistry INTRODUCTION reviewed. A block which represented the SST diagnosis best was Sclerosing stromal tumours (SSTs), which were defined by selected from each case and an immunohistochemical method Chalvardjan and Scully [1] in 1973 for the first time, are rare, was performed. The primary antibodies that were used were those stromal and benign tumours of ovary. SSTs constitute 6% of the for oestrogen receptor (ER), progesterone receptor (PR), inhibin, tumours that are derived from the stroma of ovary and more than calretinin, melanA, CD10, smooth muscle actin (SMA), desmine, 80% of such tumours are observed in young adult women in the vimentin, CD34, S–100, C–kit, cytokeratin (CK) and cytokeratin7 2nd and 3rd decades of life [2–5]. Sclerosing stromal tumours are (CK7). usually hormonally inactive, but it has been reported that some Immunoreactive cells were evaluated according to their staining cases are related to pregnancy, androgenic symptoms and densities and the percentage of positive cells (weak, 1+; moderate, endometrial carcinomas. The most frequent presenting complaint 2+; strong, 3+). A positive control was used for each primary is menstrual irregularity and pelvic pain. Macroscopically, they are antibody. usually observed as solid and typically unilateral tumours [6-9]. The sharpest histological finding is the pseudo-lobular pattern that RESULTS is formed by the cellular nodules that are separated from each other Clinical Findings by hypocellular, oedematous and collagenous stroma [10]. The The ages of the patients varied between 18 and 25 years (mean hemangiopericytomatous pattern–like dilated vascular structures age- 20 years). Clinically, menstrual irregularities were detected in are the characteristics of cellular areas, and sometimes, they can 2 patients, abdominopelvic pain was detected in 2 patients, and be associated with angiomatous lesions [11]. In microscopic pregnancies were detected in 3 patients. No virilisation was observed. examinations, the luteinized theca–like cells with vacuolized cytoplasm Although SSTs are usually hormonally inactive, most of our cases and fusiform fibroblast–like cells point out in hypercellular areas. had occurred together with pregnancies and menstrual irregularities. In this study, 7 SST cases who were aged between 18–25 All the tumours were unilateral. Five tumours were located in the years, who were diagnosed in our hospital, were examined right ovary and 2 tumours were located in the left ovary. morphologically, clinically and immunohistochemically (IHC) and CA125 tumour markers were within normal limits. All the cases were reviewed together with the literature data. were processed with frozen sections, 4 cases were underwent laparoscopic oopherectomies, and the other 3 patients underwent METHODS laparotomical adnexal mass excisions. Patients were followed for Seven cases who were aged between 17–25 years with a a period of 1 to 5 years (mean age–4 years) post–operatively. diagnosis of SST were selected from the files of our hospital Clinical findings and surgical procedures have been shown in between 2001 and 2011. The operational materials of all the [Table/Fig-1]. cases were examined. The clinical and macroscopic data of the Macroscopic Findings cases were obtained from our archival records and all the archival The sizes of the tumours were between 6 cm and 12 cm (mean preparations which were stained with hematoxylin–eosine were size–8.2 cm). All the tumours were observed within good margins. 1932 Journal of Clinical and Diagnostic Research. 2013 Sept, Vol-7(9): 1932-1935 www.jcdr.net Ecmel Isik Kaygusuz et al., Sclerosing Stromal Tumor of the Ovary S. Age Clinic CA–125 Side Tumour Gross Surgery A diffuse positivity was detected in all tumours with vimentin. No. size (cm) appearance Both SMA and desmin stained strongly in fibroblast–like cells, but 1 21 pregnancy Normal right 8.5 Solid laparoscopy no staining was observed in the areas where the theca-like cells 2 19 pregnancy Normal left 6 Solid laparoscopy were dominant. 3 19 Menstruel Normal left 6 Solid laparoscopy Inhibin [Table/Fig-2] and Calretinin [Table/Fig-3] were positive in irregularity theca-like vacuolized cells. 4 25 Menstruel Normal right 9 Solid laparotomy While PR was focally positive in all the cases , ER was not positive irregularity in any cases. 5 25 Abdominal- Normal right 10 Solid laparotomy discomfort C–kit was weakly positive in 5 cases, melan-A [Table/Fig-4] was 6 19 pregnancy Normal right 6 Solid–cystic laparoscopy weakly positive in 4 cases and CD–10 was weakly positive in 3 cases in focal areas. 7 18 Abdominal- Normal right 12 Solid–cystic laparotomy discomfort No staining was observed in our cases with CK and CK–7. [Table/Fig-1]: Clinical findings and surgical procedures The immunohistochemical profile of our cases has been shown in Five tumours were in solid structures on the incision surface, and 2 [Table/Fig-5]. tumours were composed of solid and cystic areas. The diameters of these cystic areas varied between 0.1 and 8 cm, and there DISCUSSION was clear liquid in the cysts. The solid components were white to Ovarian neoplasms are rare in adolescents and they are mainly yellowish–pink in nodular areas. tumours of germ cell origin. SSTs of the ovary are of sex–cord stromal origin, that occur in young women with a mean age of 28 years. This Microscopic Findings type was defined by Chalvaridjan and Scully (1973) for the first time All the tumours were surrounded by fibrous capsules and they were and it is often observed in the 2nd and 3rd decades of life [1]. To the best located within good margins. The irregular distribution of tumour of our knowledge, although this tumour has been described before in cells resulted in cellular pseudolobules that were separated by adolescents, only one paediatric case of a bilateral SST (in a 10 year hypocellular areas which had resulted from oedema and hyalinization. old premenarchal girl) has been described in the clinical literature [12]. Two different types of cells were exhibited in the cellular areas. One An overview on all case reports on SSTs which had been reported type was fibroblast–like fusiform cells, and the other was luteinized between 2003–2013, has been shown in [Table/Fig-6]. the ca–like cells with a vacuolized cytoplasm. In some cases, the nuclei in these cells were eccentrically located in some places and Sclerosing stromal tumours are rarely seen together with they reminded of signet ring cells. No mitosis was detected. Nuclei pregnancies; only 8 pregnant patients have been reported in had a vesicular appearance, and sometimes nuclei were evident. literature so far. According to the literature, our 3 pregnant patients are the reported 9th, 10th and 11th patients. Key words: Sclerosing, Ovary, Immunohistochemistry Small–medium sized and thin–walled ectatic blood vessels were detected, especially in the cellular component. These vessels had Clinically, there was menstrual irregularity in 2 cases and a hemangiopericytomatous pattern and some were shaped like a abdominopelvic pain in 2 cases, and 3 cases were pregnant. boomerang. Necrosis was observed only in 2 cases and there was These findings made us think that these tumours were hormonally hyalinization and calcification in 2 cases. active. In the literature, premenarchal, pregnancy women, virilization, cliteromegaly, amenorrhe were presented. Immunohistochemical Findings Macroscopically and microscopically, characteristic histopatholog- [Table/Fig-2]: Inhibin positive cells [Table/Fig-3]: Calretinine positive cells [Table/Fig-4]: Melan-A positive cells (P/I) 1 2 3 4 5 6 7 ical findings of the SSTs of ovary were observed in all the cases. Vimentin 90/3+ 80/3+ 70/3+ 80/3+ 70/3+ 90/3+ 80/3+ The hormonal activities of these tumours have been presented SMA 80/3+ 70/3+ 90/3+ 90/3+ 70/3+ 70/3+ 90/3+ before. Lam and Geittman [13] proved that these tumours synthe- sized dehydroepiandrosterone and that when steroidogenesis oc- Desmin 45/3+ 60/3+ 40/3+ 50/3+ 45/3+ 55/3+ 40/3+ curred, it was usually oestrogenic, which caused irregular menses, Inhibin 30/3+ 10/2+ 10/2+ 30/3+ 40/3+ 30/2+ 30/3+ amenorrhoea, and infertility.