Cases reported "Granulosa Cell Tumor"

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1/6. Hepatic metastasis in granulosa cell tumor of the ovary.

    Hepatic metastases occur rarely in epithelial ovarian carcinoma and also appear to be unusual in malignant stromal tumors of the ovary (granulosa cell tumors). Recently two patients with extensive hepatic metastasis from this primary tumor, were treated. review of the experience at the City of hope National Medical Center provided three additional patients with a confirmed diagnosis of granulosa cell tumor of the ovary, one of whom is alive and disease-free. A review of clinical and pathologic data revealed that both of the other patients died of their disease and had hepatic metastases proven at autopsy. Hemorrhagic events complicating the clinical course of these patients were frequent. It is believed that the frequency of hepatic metastasis in granulosa cell tumor of the ovary may be higher than has been appreciated in the past, and that the cystic-hemorrhagic nature of these lesions contributes to the morbidity and mortality associated with granulosa cell tumors of the ovary.
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2/6. Juvenile granulosa cell tumor--another neoplasm associated with abnormal chromosomes and ambiguous genitalia. A report of three cases.

    Three infants, 3 months of age or younger with abnormal karyotypes and ambiguous genitalia, had gonadal juvenile granulosa cell tumors. Two of the patients had mixed gonadal dysgenesis and the third had an intersexual disorder of undetermined type. Two tumors arose in undescended testes, and the third in an undescended gonad of uncertain nature. The occurrence of this uncommon neoplasm in these infants indicates that it is another type of neoplasm that may develop in the gonad of a patient with an abnormal karyotype and ambiguous genitalia.
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3/6. Bilateral juvenile granulosa cell tumors in a 4-month-old dysmorphic infant. A clinical, histologic, and ultrastructural study.

    Juvenile granulosa cell tumors were encountered within bilateral cystic ovarian masses in a 4-month-old infant. The child was the product of a consanguinous pregnancy, and manifested poor growth, relative microcephaly, facial asymmetry, and a malformed left ear. There was no history of gestational drug or hormone ingestion, and no evidence of abnormal endocrine activity after birth, however, the serum alpha-fetoprotein level was mildly elevated. The tumors were well defined by sonography and there was no evidence of metastasis. Histologically, a dense proliferation of round to oval tumor cells showed considerable individual cell necrosis and frequent microcyst formation. There was no evidence of luteinization and only mild nuclear pleomorphism. Immunoperoxidase study failed to reveal alpha-fetoprotein. Ultrastructural study supported the granulosa cell nature of the tumor, but a few cells contained bundles of intracytoplasmic filaments. There has been no evidence of recurrent disease during a 16-month follow-up period, and serial alpha-fetoprotein determinations have remained in the reference range. Comparison with two previously reported bilateral juvenile granulosa cell tumors suggests that this tumor occurs in young infants, and is amenable to conservative therapy.
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4/6. The fine structure of a virilizing human granulosa-theca cell tumor. Observations on the nature of the hormone producing cell.

    The ultrastructure of a virilizing granulosa-theca cell tumor is reported. Although the histologic appearance is characteristic of a granulosa-theca tumor, several tumor cells contain crystalloids of Reinke which have not been associated with sex-cord-stromal tumors, but which are often present in ovarian hilus cells and leydig cells. The steroidogenic cell in this case displays many fine structural features common to mammalian steroidogenic cells, namely abundant smooth endoplasmic reticulum, a dispersed Golgi, mitochondria with tubular cristae, and lipid droplets. Other features of this cell, which contains crystalloids of Reinke, more closely resemble those of a theca-lutein cell than a granulose-lutein or hilus cell and support a stromal origin. However, this tumor illustrates the striking overlap in morphologic features and function among granulosa, theca, hilus, and leydig cells.
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5/6. Luteinized adult granulosa cell tumors of the ovary: a report of four cases.

    Four luteinized adult granulosa cell tumors of the ovary in women 22-76 (average 53) years of age are described. Two patients presented with postmenopausal bleeding, one with dysfunctional uterine bleeding and one with amenorrhea. The tumors were all stage Ia, ranged from 2 to 11 (average 5.1) cm in greatest dimension, and were typically solid, lobulated, and yellow. On microscopic examination the most striking feature was the abundant eosinophilic cytoplasm of most of the tumor cells, which resembled the granulosa cells of the corpus luteum. The tumor cells typically grew diffusely, but large nodules intersected by scanty fibrous septa were present in all the cases. Additional patterns present focally included cords or ribbons, nests, and thick trabeculae. The tumors had inconspicuous amounts of fibrothecomatous stroma; foci of stromal edema or myxoid change were present in three cases. The tumor cell nuclei were round, of moderate size, and pale, and many of them had nuclear grooves. There was no significant nuclear pleomorphism and the mitotic rate did not exceed 1 per 10 high power fields. Reticulum stains, available in three cases, confirmed the granulosa cell nature of the neoplasms by disclosing scanty intercellular fibrils in most areas. Immunostains, performed in two cases, showed positivity for vimentin in both of them and for cytokeratin, smooth-muscle actin, and S-100 in one. One tumor from a 75-year-old woman was associated with an ectopic decidual reaction in a periovarian adhesion. This patient had focal, severe atypical complex hyperplasia of the endometrium and focal secretory change in the endometrium uninvolved by the hyperplasia.(ABSTRACT TRUNCATED AT 250 WORDS)
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6/6. Juvenile granulosa cell tumor of the testis: a comparative immunohistochemical study with normal infantile gonads.

    This study concerns the nature of two different cell populations in a juvenile granulosa cell tumor (GCT) of the infantile testis. Immunohistochemical features of the tumor were compared with those of normal infantile testes (six cases) and ovaries (six cases). The testicular neoplasm showed follicles, cysts and solid nodules composed of an internal layer of polyhedral cells that expressed cytokeratin and vimentin. Most of the follicles and nodules were surrounded by an external layer of spindle cells that reacted to muscle-specific actin, vimentin, and focally to desmin. A neoplastic rather than reactive origin of the spindle cell population is favored by their concentric arrangement in a peritubular-like or theca-like fashion and by their immunohistochemical correlation with normal peritubular-myoid and theca external cells. Sertoli and granulosa cells of normal infantile gonads were positive for cytokeratin and vimentin; peritubular myoid and theca externa cells expressed muscle-specific actin, vimentin, and focally desmin. The occurrence of two well-differentiated components in the tumor favors its origin from the primitive specialized gonadal stromal cell that during neoplastic transformation develops bidirectional differentiation toward epithelial-like and smooth muscle-like lineages. The possibility that this tumor is composed of immature Sertoli and peritubular myoid cells is discussed.
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