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Recurrent gynandroblastoma of ovary-A case report: a molecular and immunohistochemical analysis.

Gynandroblastoma is a rare ovarian tumor that is composed of both Sertoli cells and granulosa cells. Only 23 cases have been reported in the literature, and recurrence has never been described. We report the first case of a recurrent gynandroblastoma along with its molecular analysis and immunohistochemical studies. A 49-year-old Gravida 0 woman with a 10-year prior diagnosis of ovarian-mixed stromal tissue tumor (well-differentiated Sertoli cell and granulosa cell tumor) and staging laparotomy, presented now with a retroperitoneal mass and an elevated inhibin level. CT scan was suspicious for recurrence. The patient had no prior adjuvant therapy. The histomorphological features of the recurrent tumor had both Sertoli cell and granulosa cell tumor. The molecular analysis of both primary and recurrent tumor showed minor genetic instability in the 17q12.2 gene locus with no dedifferentiation or progression, which is consistent with a low-grade tumor. The immunohistochemical staining profile showed positivity for CD99, inhibin, calretinin, and vimentin; focal positivity for cytokeratin AE1/AE3 and negative for EMA and melan-A. All the previously mentioned immunostainings support the diagnosis. We report the first case of a recurrent gynandroblastoma 10 years after initial presentation along with its molecular analysis and immunohistochemical studies.

Adult↗

Gynandroblastoma: its ultrastructure.

Gynandroblastoma is a rare, sex-cord stromal tumor of the ovary that shows morphologic evidence of female and male differentiation. Such a tumor produced masculinization in a 24-year-old woman, whose symptoms disappeared following removal of the tumor. By electron microscopy, the granulosa cell nests displayed Call-Exner (CE) bodies of the hyaline type composed of multiple layers of basal lamina resembling CE bodies of the normal graafian follicle. In contrast, CE bodies of a classic granulosa theca cell tumor were of the spongiform type, consisting of a space limited by a single basal lamina containing altered granulosa cells and cell processes. Both types of CE bodies are believed to arise following secretion by and/or degeneration of granulosa cells, the variation in morphology between the two resulting from differences in amounts of basal lamina deposited. The tubular components of the tumor resembled more closely the rete ovarii than did Sertoli cells, and its proposed that such structures be called by the alternate and less specific term "androblastoma." The identity of Leydig cells was established by demonstrated of intracytoplasmic Reinke crystals. Despite a difference in architectural pattern, there was a close ultrastructural resemblance between the different sex-cord components of the gynandroblastoma.

Adult↗

Gynandroblastoma with elements resembling juvenile granulosa cell tumor.

Gynandroblastoma is an extremely rare sex cord-stromal tumor that exhibits significant ovarian and testicular differentiation. In most previously reported tumors, adult granulosa cell tumor has formed the ovarian-type component and Sertoli or Sertoli-Leydig cell tumor (SLCT) has formed the testicular-type component. In contrast, the ovarian-type element in the case reported here resembled juvenile granulosa cell tumor (JGCT). The testicular-type elements accounted for 20% of the tumor and resembled intermediate-grade SLCT. The Sertoli cells had strong cytoplasmic staining for cytokeratin CAM 5.2 and positive nuclear staining with estrogen and progesterone receptor, whereas the JGCT-like areas were negative for these antibodies. Ultrastructurally, the JGCT-like areas consisted of groups of cells that were invested by a basal lamina and had low nuclear-cytoplasmic ratios, cytoplasmic lipid droplets, and simple junctional complexes. The Sertoli cells in the SLCT-like areas had long, tight junctions and well-formed desmosomes. Gynandroblastoma usually presents clinically as an abdominal mass, often associated with either virilizing or feminizing manifestations. The prognosis is favorable and similar to that of the individual tumor components, but clinical follow-up in the small number of cases has been limited.

Adolescent↗

Gynandroblastoma of the ovary.

A gynandroblastoma of the ovary in a 17-year-old girl is reported. Primary amenorrhoea, hirsuties and slight clitoral enlargement were associated with the tumour and high levels of circulating testosterone were found. This is the first gynandroblastoma in which Reinke crystalloids have been identified in the Leydig cell component. The criteria for the diagnosis of this tumour and its histogenesis are discussed.

17-Hydroxycorticosteroids↗

Gynandroblastoma of the ovary. A case report.

An ovarian gynandroblastoma was found in a 59-year-old woman. The tumor consisting of mixed Sertoli-Leydig cells and granulosa-theca cells demonstrated no malignant features. Bisexual cells in gynandroblastoma derived from a common gonadal mesenchyme precursor or indifferent cell. The excision of appendages with tumor or hysterectomy was sufficient.

Female↗

Gynandroblastoma in pregnancy: case report and review of literature.

Gynandroblastoma is an extremely rare tumor, composed of sex cord and stromal cells of both ovarian (granulosa-theca) and testicular (Sertoli-Leydig) types. We believe that its occurrence during pregnancy has not been previously reported. The patient was a 32-year-old woman who during her pregnancy was noted to have a progressively enlarging, unilocular left ovarian cyst. Beginning at 18 weeks gestation, the fetus required multiple platelet transfusions for severe alloimmune thrombocytopenia. A viable baby girl was delivered by cesarean section at 39 weeks gestation. At that time, an ovarian cystectomy also was performed. When the histology of the tissue subsequently became known, a left salpingo-oophorectomy was performed for gynandroblastoma. One year later, at the time of laparoscopic sterilization, the examination of the pelvis was normal.

Adult↗

Gynandroblastoma of the ovary: a case report with an immunohistochemical and ultrastructural study.

An ovarian gynandroblastoma in a 60-year-old woman is described. The cut-surface of the right ovary showed multiple macrofollicles separated by white fibrous tissues and multiple ill-defined yellowish nodules. The tumour consisted of substantial amount of a granulosa cell element and a Sertoli cell element with intermingled Leydig cells. Immunohistochemically, the tumour cells in both the granulosa cell and Sertoli cell elements were positive for cytokeratin CAM5.2. The granulosa cell element showed strong membrane staining of Ewing's sarcoma antigen 013 and the Sertoli cell element was locally positive. Vimentin was observed in both the Sertoli cell element and the granulosa cells. Both elements and the Leydig cells were uniformly negative for epithelial membrane antigen, muscle specific actin, CD31 and CD34. The tumour was aneuploid by flow cytometry. The patient was well with no evidence of tumour five months after surgery.

Female↗

Hormone studies in a gynandroblastoma.

Plasma gonadotropin, testosterone, androstenedione, and estradiol levels were determined before and after removal of a 37-pound gynandroblastoma from an 18-year-old virilized, mentally retarded female. Testosterone and androstenedione levels preoperatively were in the normal female range and fell dramatically after castration. Histologic examination showed active atypical granulosa and Sertoli elements. The hormonal data obtained do not account for the marked virilization observed.

Adolescent↗

Gynandroblastoma of ovary with juvenile granulosa cell component and heterologous intestinal type glands.

An ovarian gynandroblastoma in a 15-year-old girl is described. The predominant component was juvenile granulosa cell tumour. Areas of adult granulosa cell tumour and Sertoli cell elements were also present. Stromal theca and luteinised cells were identified. An additional histological finding was the presence of heterologous intestinal type glands. There was positive immunohistochemical staining of juvenile and adult granulosa cell areas with inhibin and MIC2 antibodies. Electronmicroscopy showed a close ultrastructural resemblance between tumour cells in granulosa and Sertoli cell areas, in spite of differences in architectural pattern, suggesting that both morphological components may derive from a single cell of origin. The tumour demonstrates a unique combination of elements which has not previously been described.

Adolescent↗

Gynandroblastoma of the ovary.

Gynandroblastoma is an extremely rare primary tumour of the ovary showing morphological evidence of both male and female differentiation. We describe the light and ultrastructural features of this tumour and review the present knowledge about its nature, function and behaviour.

Adolescent↗

Gynandroblastoma of the ovary having a typical morphological appearance: a case study.

A rare gynandroblastoma of the right ovary with a typical morphological appearance in a 65-year-old woman is reported. The tumor comprised both a granulosa cell element with a Call-Exner body and a Sertoli cell element. Pathologically, this case could be the most typical of all the world's established cases. Clinically, there were feminizing symptoms such as post-menopausal genital bleeding and endometrial cystic hyperplasia. Pre-operative serum hormonal assays indicated elevated levels of estrone, estradiol and testosterone, and low levels of gonadotropins. These returned to normal after surgery.

Aged↗

Granulosa cell tumor of the ovary with Sertoli-Leydig cell tumor components (gynandroblastoma).

A rare granulosa cell tumor with Sertoli-Leydig cell tumor components (gynandroblastoma) arising in the left ovary was reported in a 63-year-old female. Microscopically, the tumor was composed predominantly of granulosa cells arranged mainly in a diffuse solid pattern, but in some areas there were a trabecular pattern and thecofibromatous stromal components. Also, well-differentiated Sertoli-Leydig cell tumor elements were present as a minor component. The tumor produced, endocrinologically, a large amount of estradiol, some androstenedione and a small amount of testosterone. The possibility that estradiol in the present tumor was produced predominantly from androstenedione via estrone was suggested by the results of an in vitro biosynthetic study.

Androstenedione↗

Gynandroblastoma of the ovary.

A left ovarian gynandroblastoma in a 24-year-old woman was clinically manifest by prolonged (7 years) amenorrheas interrupted by metrorrhagies, with moderate hypertrichosis is presented. An important urinary elimination of estrogens (1.500 gamma/24 h) and of 17-ketosteroids (25 gamma/24 h) with normal 17-OH and gonadotrophins were evidenced. The microscopic examination of the operated tumor showed structures of a granulous tumor intermingled with arrhenoblastomatous structures. Restoration of the patient's sexual biological conditions.

Adult↗

[Gynandroblastoma of the ovary. Review of the literature].

Gynadroblastoma is an extremely rare tumour of the ovary containing both a granulosa cell and a Sertoli-Leydig cell tumour. We report a case of gynandroblastoma of the right ovary Stage IA (FIGO) in a 15-year-old girl. Microscopically, the tumour composed predominantly of granulosa cells with a minoritary component, although more than 10%, of Sertoli cells. The present knowledge about its nature, function and behaviour are reviewed.

Adolescent↗