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      A rare occurrence of a steroid cell tumor of the pelvic mesentery: a case report

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      1 , , 2 , 1
      Journal of Medical Case Reports
      BioMed Central

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          Abstract

          Introduction

          Steroid cell tumors are microscopically characterized by abundant eosinophilic or vacuolated cytoplasm that is often positive for fat stains. These tumors could be of ovarian or ectopic adrenal origin. We present a rare case of a steroid cell tumor arising from the pelvic mesentery.

          Case presentation

          A 31-year-old Asian woman was undergoing treatment for infertility and virilizing symptoms. She underwent laparoscopy followed by laprotomy for a suspected ovarian cyst/pelvic mass. During the laprotomy, a mass of 9 × 7 cm was detected in the pelvic mesentery.

          Microscopically, the tumor showed large cells arranged predominantly in sheets with abundant granular cytoplasm and large vesicular nuclei with prominent nucleoli. The tumor was seen infiltrating surrounding adipose tissue. Immunohistochemically, the tumor cells showed strong positivity for kertain, inhibin, vimentine, melan-A, neuron-specific enolase, chromogranin, and S-100 protein and focal positivity to epithelial membrane antigen. An MIB1 index showed 4% nuclear positivity. The tumor cells were negative for calretinin, desmin, and muscle actin. Considering the clinical findings, histomorphology, and immunohistochemistry, we made the diagnosis of extraovarian and extra-adrenal steroid cell tumor (not otherwise specified) of the pelvic mesentery.

          Conclusions

          We report an extremely rare case of an extraovarian and extra-adrenal steroid cell tumor of the pelvic mesentery. The tumor was a cause of virilizing symptoms and infertility in the patient. Surgical removal of the tumor reverted the symptoms of virilization, and the patient subsequently conceived. Steroid cell tumors should be considered in differential diagnosis among women presenting with infertility and symptoms of virilization.

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          Most cited references13

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          Ovarian steroid cell tumors (not otherwise specified). A clinicopathological analysis of 63 cases.

          The clinical and pathological features of 63 steroid cell tumors, not otherwise specified, were reviewed. The patients ranged in age from 2 1/2 to 80 years. The most common initial manifestation was virilization (41%); four patients had estrogenic manifestations, and four had hypercortisolemia with Cushing's syndrome. The tumors, 6% of which were bilateral, ranged from 1.2 to 45 cm in greatest dimension. Microscopic examination revealed two types of cells, which had overlapping features: those with abundant eosinophilic cytoplasm and those with vacuolated cytoplasm. Fat stains were positive in 75% of the 16 cases in which they were performed. Follow-up data ranging from 1 to 19 years (average 5.2 years) in duration were available for 50 patients. In 24 cases, the tumor was designated probably benign (no evidence of spread beyond the ovary within 3 or more years postoperatively). In 18 patients, the tumor was clinically malignant. The best pathological correlates of malignant behavior were: the presence of two or more mitotic figures per 10 high power fields (92% malignant); necrosis (86% malignant); a diameter of 7 cm or greater (78% malignant); hemorrhage (77% malignant); and grade 2 or 3 nuclear atypia (64% malignant).
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            Identification of the most sensitive and robust immunohistochemical markers in different categories of ovarian sex cord-stromal tumors.

            Different immunohistochemical sex cord-stromal markers have been previously studied in various types of ovarian sex cord-stromal tumors; however, the sensitivity for sex cord-stromal lineage may vary between markers, and some markers may not be as sensitive in some types of sex cord-stromal tumors compared with other tumors in this spectrum of neoplasms. The goals of this study were to determine which immunohistochemical markers are the most sensitive and immunohistochemically robust for sex cord-stromal lineage within a given type of ovarian sex cord-stromal tumor, and to establish whether there are substantial differences of expression of these markers between different types of sex cord-stromal tumors. Immunohistochemical stains for markers which have known variable specificity for sex cord-stromal lineage [inhibin, calretinin, MART-1/melan-A, CD99, steroidogenic factor 1 (SF-1, adrenal 4-binding protein), and WT1], were performed in 127 cases of 5 different types of ovarian sex cord-stromal tumors: adult granulosa cell tumor (n=32), Sertoli cell tumor (n=27), Sertoli-Leydig cell tumor (n=18), steroid cell tumor (n=25), and fibroma/fibrothecoma (n=25). All cases in each type of sex cord-stromal tumor expressed SF-1. Inhibin and calretinin were expressed in all groups of tumors but with a lesser frequency (56% to 100% and 36% to 100% of cases, respectively). All types of tumors except steroid cell tumor expressed WT1. Fibroma/fibrothecoma was the only type of tumor that did not express CD99. The only tumor groups that showed expression of MART-1 were Sertoli-Leydig cell tumor (restricted to the Leydig cell component) and steroid cell tumor (94% and 96% of cases, respectively). The type of sex cord-stromal tumor that was least frequently positive for several of the different markers studied was fibroma/fibrothecoma. Among all tumor groups combined, inhibin and WT1 were the 2 markers showing the most diffuse expression. Likewise, the single marker showing the most optimal combination of diffuse and strong staining (immunohistochemical composite score: possible range, 1 to 12) varied between tumors: adult granulosa cell tumor-inhibin (score 10.0); Sertoli cell tumor-WT1 (score 10.8); Sertoli-Leydig cell tumor (Sertoli cell component)-WT1 (score 10.4); steroid cell tumor-inhibin (score 11.2); and fibroma/fibrothecoma-WT1 (score 8.9). We conclude that most immunohistochemical sex cord-stromal markers have sufficient sensitivity for sex cord-stromal lineage. Although each of the different types of sex cord-stromal tumors has a slightly unique immunoprofile in terms of frequency and extent of expression, these differences are relatively minor for most types of tumors with certain exceptions (eg, WT1 is not diagnostically useful in steroid cell tumor; CD99 is not diagnostically useful in fibroma/fibrothecoma; the only sex cord-stromal tumor for which MART-1 is diagnostically useful is steroid cell tumor; inhibin and calretinin are less diagnostically useful in fibroma/fibrothecoma than in the other types of tumors, but expression in fibrothecoma was higher than in fibroma). SF-1 is the most sensitive sex cord-stromal marker among the most common types of sex cord-stromal tumors. Given the findings relating to sensitivity and extent of expression in this study, and known specificity in the literature, the most informative sex cord-stromal markers to be used for the distinction from nonsex cord-stromal tumors are inhibin, calretinin, SF-1, and WT1 (the exact number of markers to be used should be based on the degree of difficulty of the case and level of experience of the pathologist); however, the utility of immunohistochemistry for the diagnosis of fibroma/fibrothecoma is somewhat limited.
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              Calretinin, a more sensitive but less specific marker than alpha-inhibin for ovarian sex cord-stromal neoplasms: an immunohistochemical study of 215 cases.

              Although inhibin has been shown to be a sensitive marker for ovarian sex cord-stromal and fibrous neoplasms, it may be negative in some cases. Calretinin, a mesothelial marker, has shown promise as a marker for sex cord-stromal neoplasms. The aim of this study was to evaluate and compare calretinin and inhibin as immunohistochemical markers for sex cord-stromal and fibrous neoplasms. A total of 215 ovarian neoplasms were immunostained with commercially available antibodies to calretinin and inhibin. These tumors included 87 sex cord-stromal (39 granulosa cell, 13 Sertoli-Leydig, 4 Sertoli, 9 thecomas, 14 fibrothecomas, and 8 other stromal tumors), 37 fibrous (20 fibromas, 9 adenofibromas, and 8 fibrosarcomas), 65 epithelial, 22 germ cell, and 4 miscellaneous neoplasms. The staining was evaluated on a 0-4 scale based on percentage of neoplastic cells labeling: 0 = none; 1+ = 1-25%; 2+ = 26-50%; 3+ = 51-75%; 4+ = 76-100%. Calretinin reactivity was detected in 100% of sex cord-stromal and 90% of fibrous neoplasms, including 32 that were inhibin negative (2 granulosa cell tumors, 1 Sertoli-Leydig cell tumor, 1 thecoma, 3 fibrothecomas, 16 fibromas, 6 adenofibromas, and 3 fibrosarcomas). All four calretinin-negative fibrous neoplasms were inhibin negative. Calretinin staining was also detected in 22% of epithelial neoplasms but none of the germ cell and miscellaneous neoplasms tested. Inhibin staining was detected in 92% of sex cord-stromal neoplasms, 22% of fibrous neoplasms, 2% of epithelial neoplasms, and none of the germ cell and miscellaneous neoplasms tested. Calretinin has a 97% sensitivity and 85% specificity for sex cord-stromal and fibrous neoplasms, whereas inhibin has a 71% sensitivity and 99% specificity. This study shows that both calretinin and inhibin are useful in the diagnosis of ovarian sex cord-stromal and fibrous neoplasms. Calretinin is a more sensitive but less specific immunohistochemical marker than inhibin. Calretinin is particularly useful in the diagnosis of sex cord-stromal and fibrous neoplasms that are inhibin negative. The high frequency of calretinin in fibrous neoplasms suggests that a subgroup of these neoplasms may be derived from specialized gonadal stromal cells, perhaps thecal cells.
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                Author and article information

                Journal
                J Med Case Reports
                Journal of Medical Case Reports
                BioMed Central
                1752-1947
                2011
                18 October 2011
                : 5
                : 517
                Affiliations
                [1 ]Department of Pathology, Cancer Institute (WIA), Adyar, Chennai, Tamil Nadu, 600 020, India
                [2 ]Department of Medical Oncology, Cancer Institute (WIA), Adyar, Chennai, Tamil Nadu, 600 020, India
                Article
                1752-1947-5-517
                10.1186/1752-1947-5-517
                3212982
                22008415
                9b530947-9d2c-44ab-aaac-657408a9753d
                Copyright ©2011 Murhekar et al; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 23 December 2010
                : 18 October 2011
                Categories
                Case Report

                Medicine
                Medicine

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