Surgery is the primary treatment modality for granulosa cell tumors. Ovarian germcell tumors represent 1520% of all ovarian tumors. This tumor is classified into juvenile gct and adult gct, and the majority of the cases are the adult type. Prevalence almost 2 to 5 percent of ovarian neoplasms are mainly granulosa cell tumor. Management of female malignant ovarian germ cell tumours. Staging and histopathology helps in prediction of survival. Although fertilitysparing surgery for such patients is. Majority of the patients with granulosa cell tumors of the ovary present in early stage. Granulosa cell tumors comprise from 3 to 10% of all malignant ovarian tumors l3.
Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for granulosa cell tumor of the. The tumor does not produce any outward signs of its presence, and unlike granulosa theca cell tumors, the opposite ovary remains active. The role of systemic chemotherapy in the management of. Kmt2dmll2 inactivation is associated with recurrence in. Advanced stage and presence of residual disease were associated with inferior survival, but only prospective studies can ascertain their definite role. For language access assistance, contact the ncats public information officer. Ovarian granulosa cell tumor gct is a malignant tumor with slow progression. Mogcts, which include dysgerminomas, immature teratomas, embryonal tumours and endodermal sinus. Granulosa cell tumors gcts of the ovary arise from the cells that would. This type of tumor is known as a sex cordstromal tumor and usually occurs in adults. This relatively low incidence is one likely reason for the differing opinions on histopathology, treatment, and prognosis 4141.
Pdf ovarian granulosa cell tumors are rare malignancies with a relatively favorable prognosis. Granulosa cell tumor of the ovary genetic and rare diseases nih. The granulosa or granulosa theca cell tumor is a hormone producing neoplasia that can suppress follicular development in the contralateral ovary. Granulosa cell tumor of the ovary genetic and rare. Granulosa cell tumor of the ovary is a rare type of ovarian cancer that accounts for approximately 2% of all ovarian tumors. Granulosa cell tumors of the ovary cause higher than normal levels of estrogen in a womans body. The recurrence of granulosa cell tumor often happens after 5 years, leading to a forgotten tumor by the patient. Nonepithelial ovarian cancer esmo clinical practice guidelines. Granulosa cell tumors of the ovary gcts are rare malignancies arising from the ovarian sex cord stroma that account for 25% of the approximately 239,000 ovarian cancers diagnosed each. Malignant ovarian germ cell tumors mogcts in adolescents are rare.
Inhibin and androgens produced by the tumor suppresses production and secretion of follicle stimulating hormone fsh from the anterior pituitary. Pdf granulosa cell tumors of the equine ovary janet. Patients with granulosa cell tumors should be followed for the rest of their lives. The important prognostic factor is staging of the tumor. Ovarian stertoliledig cell tumor slct is a rare type of sex cordstromal tumor of the ovary. Pdf prognostic factors in adult granulosa cell tumors of the ovary. Clinicopathologic features of ovarian sertolileydig cell tumors. Pdf granulosa cell tumors of the equine ovary bill. Ovarian granulosa cell tumor gct is a malignant tumor originating from the sexcord stromal cells of the ovary. Granulosa cell tumor bep or vac x 34 cycles malignant as above teratoma embryonal as above bep x 34 cycles carcinoma debulk but bep x 34 cycles preserve fertility endodermal sinus tumor bep x 3 cycles if stage iiiv. Granulosa cell tumours of the ovary are unique, hormo nally active, oestrogensecreting tumours accounting for 1 to 2% of all the ovarian malignancies. Granulosa cell tumor of the ovary is a rare ovarian entity.
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