Pathology outlines lipid steroid, lipoid cell tumor. Now in april of 2009 the doctor thought it had come back so i decided to get a 2nd opinion and he did a pet scan and it came back clear. Gct exhibit a molecular profile that is consistent with fshresponsive granulosa cells of the late preovulatoy follicle 1, 2. There were 16 granulosa cell tumours and 17 thecomas. Ovarian granulosa cell tumor accounts for 23% of all ovarian cancer patients. Dec 01, 2012 i believe gct is a stromal cell tumor not a germ cell tumor, there is a difference. Granulosa tumors were described for the first time in 1855 by. Aug 25, 2009 granulosa cell tumors gcts of the ovary belong to the group of ovarian sexcord stromal tumors and represent 5 to 10% of ovarian malignancies. Errarhay s, bouguern h, chraybi m, bouchikhi c, chaara h, et al. The answer points out an important difference in the way metastases from this histology should be interpreted versus low malignant potential ovarian epithelial tumors. They have been divided into two subtypes according to their cell of origin as follows. Granulosa cell tumor ovary treatment, diagnosis, symptoms. Granulosa cell tumors of the ovary are a type of sex cord stromal ovarian tumor.
Now i am having some discomfort in my pelvic area and i wonder if it is tumors growing. Cisplatincontaining regimen in advanced or recurrent. Patients with histopathologically confirmed ovarian jgct diagnosed between 1990 and 2016 were identified. The symptoms of this excess estrogen depend on the womans. In a retrospective study of 71 primary ovarian tumors in the dog, epithelial tumors 46% were more common than sex cord stromal 34% and germ cell tumors 20%. Granulosa cell tumors account for 25% of all ovarian malignancies and are characterized by sex cordstromal differentiation and relatively indolent behavior.
They are slow growing which is why the majority are diagnosed at an early stage, usually stage 1 and they are considered low malignant potential. Ultrasonographic examination granulosa cell tumours typically have a multicystic honeycomb appearance with some areas of solid tissue. Discussion sertolileydig cell tumor is a very rare ovarian tumor that included in the group of sexcordstromal tumors and they account for granulosa cell tumor foundation in new zeland. We present the case of a 64yearold woman with a presentation of left flank pain. Pdf ovarian granulosa cell tumors are rare malignancies with a relatively favorable prognosis. Granulosa cell tumor is a type of stromal tumor affected at the sex cord of the ovary 1,2. Kmt2dmll2 inactivation is associated with recurrence in. Sex cord stromal tumors were equally divided into sertolileydig 1224 and granulosa cell tumors 1224. Granulosa cell tumors of the ovary with precocious puberty. The recurrence of granulosa cell tumor often happens after 5 years, leading to a forgotten tumor by the patient. We performed a retrospective study of patients with stage ic agct diagnosed at our hospital from january 1985 to september 2015. If there is asymmetry in ovary size yet the smaller ovary is cycling, then a granulosa cell tumour is less likely and the enlarged ovary is more likely to have a haematoma or teratoma. A new classification of ovarian granulosa cell tumor based.
Laparoscopic removal of an ovarian granulosa cell tumor. Stromal tumors are sex cord tumors which develop from within the ovary not on the surface, which is a good thing. Recurrence of granulosa cell tumour after thirty years with. Adult granulosa cell tumors are far more common than the juvenile type and account for 95% of all granulosa cell tumors. Women may present with an asymptomatic mass or symptoms related to hyperestrogenism like abnormal uterine bleeding, breast tenderness, and postmenopausal bleeding in adult form of the disease. They occur predominantly in peri and postmenopausal. Rare, derived from hilar cells almost always benign call a nonhilar leydig cell tumor if it occurs in ovarian cortical stroma symptoms. Conservative surgery in stage i adult type granulosa cells. Ovarian granulosa cell tumors gcts are the most common type of potentially malignant ovarian sex cordstromal tumor. Ct and mri findings of sex cordstromal tumor of the ovary. Most adulttype granulosa cell tumors of both types are diagnosed at a. The purpose of this article was to research the clinical and imaging features of sex cordstromal tumors of the ovary to help in specific diagnosis of ovarian tumors.
Well for being such a rare form of ovc, there seems to be a lot of us here. Granulosa cell tumour an overview sciencedirect topics. This seems to be a very rare type of ovarian cancer. Granulosa cell tumor of the ovary genetic and rare.
An unusual case of juvenile granulosa cell tumor of the ovary. Granulosa cell tumor of the ovary genetic and rare diseases. Usually, younger aged women have more prevalence to develop granulosa cell tumor. Metastases from adult granulosa cell tumor of the ovary indicates a malignant primary. While granulosacell tumors are the most common type of ovarian tumor, producing precocious puberty, they only account for about 10% of all the reported cases. N2 we report the first case of a combined adult granulosa cell tumor and endometrioid carcinoma collision tumor occurring in an ovary. These tumors are the principal feminizing neoplasms of the ovary.
Is adjuvant chemotherapy beneficial for patients with figo. May 11, 2004 granulosa cell tumours of the ovary are rare, comprising around 3% of ovarian tumours. Lipoid cell tumor of ovary definition of lipoid cell tumor. Bleomycin, etoposide, and cisplatin combination therapy of ovarian granulosa cell tumors and other stromal malig nancies. According to the 2014 world health organization histological classification of ovarian tumors, ovarian granulosa cell tumors are divided into two types. Outcome of patients with recurrent adulttype granulosa cell tumors. Granulosathecacell tumor of the ovary in children jama. Here, the authors observe in agct a novel recurrent somatic truncating mutation of kmt2d, more. Steroid cell tumors of the ovary are rare sexcord neoplasms which account for less than 0.
Granulosa cell tumor of the ovary is an uncommon neoplasm. Duration of response and survival are computed from the start of each treatment. Ovarian germ cell tumors ogcts comprise 2025% of all ovarian cancers arising from germ cells of ovary. Ruptured granulosa cell tumor of the ovary as a cause of. There is also a private facebook group of almost 600 women diagnosed with gct called gct survivor sisters that you can request to join. Adult granulosa cell tumor agct is a sex cord stromal tumor scst which. However, high umbilical cord serum concentrations brenner tumors, lipoid cell tumors of the ovary, sclerosing stromalcell tumors, and adrenal tumors.
An exploratory laparotomy found a 27 x 21 x 15 cm right ovarian. Combination chemotherapy of ovarian granulosa cell tumor with cisplatinum and doxorubicin. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 toll free. The parenchyma of a tumor consists of tumorous tissue, while the stroma forms from surrounding connective tissue. Prognostic factors of this condition are lacking, and alternative treatment options to preserve.
Sex cordstromal and steroid cell tumors of the ovary. More detailed information about the symptoms, causes, and treatments of granulosa cell tumor of the ovary is available below. The new molecular biology of granulosa cell tumors of the. Adult granulosa cell tumors agcts account for less than 5 % of all ovarian malignancies, whereas the majority 95 % occurs after the age of 30 adulttype and present at an early stage. Granulosacell tumor of ovary with sexual precocity jama. Granulosa tumors tgo are a rare entity of malignant ovarian tumors 5%. The size of the granulosa cell tumours varied from 3 to 30 cms in diameter and no correlation was found between size and evidence of invasion. The cysts are in fact follicles, some producing, as here, the typical basophilic fluid.
Granulosa cell tumors are rare sex cord ovarian tumors that are formed by cells believed to be derived from those that surround the germinal cells in the ovarian follicles. A clinicopathologic study of 92 cases of granulosa cell tumor of the ovary with special reference to the factors influencing prognosis. Granulosa cell tumours are tumours that arise from granulosa cells. Full text malignant mixed ovarian germ cell tumor composed of. Ct and mr imaging of ovarian tumors with emphasis on. A pink tumor shows cystlike areas with occasional nodules at low power.
Granulosa cell tumor of the ovary radiology reference. Aug 30, 2018 three major types of ovarian neoplasms are described, with epithelial cell tumors 70% comprising the largest group of tumors. Emerging biomarkers in ovarian granulosa cell tumors. The efficacy of adjuvant chemotherapy in the setting of agct remains unclear for several reasons. Leufflen l, leroux a, harter v, verhaeghe jl, marchal f 2014 granulosa cell tumor of the ovary. The common types are granulosa cell tumors, fibrothecomas, sclerosing stromal tumors, and sertolileydig cell tumors. I would like to hear and talk to other people who have this same type of cancer. Pdf this report presents the case of a late relapse of an ovarian granulosa cell. Postoperative pathology showed ovarian yolk sac tumor yst with allogeneic cartilage tissues. Almost 2 to 5 percent of ovarian neoplasms are mainly granulosa cell tumor.
Pdf prognostic factors in adult granulosa cell tumors of the ovary. Dec 24, 2015 the differential diagnosis of sertolileydig cell tumors with hepatoid components and alphafetoprotein expression includes granulosa cell tumors, female adnexal tumor of probable wolffian origin fatwo, endometrioid carcinoma, hepatoid carcinoma of the ovary, serous carcinoma, and endodermal sinus tumor table 1. Adulttype granulosa cell tumor agct is a rare malignant ovarian sex cordstromal tumor, harboring recurrent foxl2 c. Recently i have be diagnosed with granulosa cell tumor of the ovary. Adult type granulosa cell tumors of the ovary agcts represent the most common tumor type among sex cord stromal tumors, and are characterized by an indolent course and late recurrences, with a reported recurrence rate as high as 64% 1 x 1 young, r. Ultrasound image of the abdomen pelvis demonstrating a large complex, cystic and solid mass with septations of varying thicknesses.
Although previous cases of recurrence have been described, it is extremely unusual for these tumours to recur after thirty years. Granulosa cell tumor is a slowgrowing cancer that starts in the granulosa cells that surround the eggs within the ovary. Adulttype granulosa cell tumors of the ovary agcts are rare and recurrence is difficult to treat. These tumours have preponderance for local spread and extremely late recurrence. No patient had liver metastasis or extraabdominal spread at the time of primary surgery. Granulosa cell tumors of the ovary gct represent a specific subset of malignant ovarian tumors and can be further categorized into two distinct subtypes, the juvenile and the adult form. The autonomy of the tumor increases in that the tumor becomes more independent of the bodily systems that normally control cell division. This tumor is classified into juvenile gct and adult gct, and the majority of the cases are the adult type. Sex cordstromal tumors of the ovary are rare ovarian neoplasms, which arise from stromal cells and primitive sex cords in the ovary. They are estrogen secreting tumors and present as large, complex, ovarian masses. This group of tumors represents approximately 8% of ovarian neoplasms and affects all age groups. Ovarian tumors ovarian cancer gynaecologic disorders.
Indications for use include an unresectable tumor at initial diagnosis or recurrent tumor, extra ovarian spread advanced stage or suboptimal cytoreduction. Granulosa cell tumors were the most common ovarian tumor that affecting cattle 11,14, also jubb et al. Germ cell tumors and their association with pregnancy intechopen. For language access assistance, contact the ncats public information officer. The primary extraovarian granulosa cell tumor is extremely rare such that in the english literature only 7 cases have been reported to date. Luteinizing hormone elevation in ovarian granulosa cell. The symptoms of this excess estrogen depend on the womans menstrual status. Granulosa cell tumor of the ovary requires regular, long. Ovarian granulosa cell tumours gcts are an uncommon neoplasm that arise from the sexcord stromal cells of the ovary and represent 25% of all ovarian cancers young, 2005. Ppt laparoscopic removal of an ovarian granulosa cell tumor powerpoint presentation free to download id. The granulosa cell tumor of the ovary foundation gctf exists to raise funds for granulosa cell tumor of the ovary gct, ovarian cancer research.
Management of female malignant ovarian germ cell tumours. The median diseasefree survival dfs, postrecurrence survival, and overall survival. Gcts exhibit several morphological, biochemical and hormonal features of normal proliferating preovulatory granulosa cells, such as estrogen biosynthesis. The adult form progresses slowly and often is diagnosed in an early stage of disease. Review of seven cases of granulosa cell tumour of the. Genomic profiling of primary and recurrent adult granulosa. Ovarian adulttype granulosa cell tumors gcts are derived. Within recent years granulosa theca cell tumors have attracted much interest, primarily because of their hormonal function. We are not able to provide medical advice or cancer specialist referrals. Stage 2 granulosa cell as the cells had surfaced on the tumor which was also stuck to my bladder and now i find myself wondering if it will return there.
We analyzed descriptive statistics, and performed univariate and. Functional neoplasms of the ovary are relatively infrequent compared to ovarian tumors in general, and only about 5% occur before puberty. We describe a case of recurrence of granulosa cell tumour after 30 years, presenting as small. Mogcts, which include dysgerminomas, immature teratomas, embryonal tumours and endodermal sinus. More detailed information about the symptoms, causes, and treatments of granulosa cell tumor of the ovary is available below symptoms of granulosa cell tumor of the ovary. The more common types are granulosa cell tumors gcts, fibrothecomas, and sertolileydig cell tumors. Granulosa cell tumors of the ovary are rare neoplasms that originate from sexcord stromal cells and they comprise 25% of all ovarian cancers. Adult granulosa cell tumor of the ovary is a type of ovarian sex cord stromal tumor. Aim of this study is to identify clinical and pathological risk factors for recurrence in early stage agcts. Tumor cells haphazardly spread without longitudinal grooves, with small nucleoli, in an edematous background.
Ovarian granulosa cell tumor gct is a malignant tumor originating from the sexcord stromal cells of the ovary. Germ cell tumors occur less frequently 20%, while sex cordstromal tumors make up the smallest proportion, accounting for approximately 8% of all ovarian neoplasms. They account for most of the hormonally active ovarian tumors. Two major forms of granulosa cell tumors are recognized. Molecular pathogenesis of granulosa cell tumors of the ovary. Characteristics and treatment results of recurrence in adulttype. To evaluate the association between adjuvant chemotherapy and clinical outcomes in patients with stage ic adult granulosa cell tumor agct. Fotopoulou c, savvatis k, braicu ei, brinkspalink v, darb esfahani s, lichtenegger w, et al.
The new molecular biology of granulosa cell tumors of the ovary. Hormonal treatment of a recurrent granulosa cell tumor of the. The incidence of endometrial hyperplasia and cancer in 1031 patients with a granulosa cell tumor of the ovary. Granulosa cell tumor of the ovary is the most common malignant sex cordstromal tumor as well as the most common estrogenproducing ovarian tumor. Initial stage seems to be the single most important prognostic factor in ovarian granulosa cell tumors. This article is from world journal of surgical oncology, volume 11. Pathology and classification of ovarian tumors chen. Pathology the tumors were mostly unilateral with no side predominance. This type of tumor is known as a sex cordstromal tumor and usually occurs in adults. Ovarian tumors of these cell types are called sex cordstromal tumors. Histologically, these neoplasms belong to the category of sexcord stromal tumors, comprising 70% of tumors in this group 1 x 1 schumer, s. Advanced stage was significant independent poor prognostic indicator for both os. Adult ovarian granulosa cell tumors gcts are hormonally. Jan 07, 2016 granulosa cell tumor of the ovary is a rare type of ovarian cancer that accounts for approximately 2% of all ovarian tumors.
January 21, 2015 terminated 2 orteronel tak700 in metastatic or advanced nonresectable granulosa cell ovarian tumors. T2 adult granulosa cell tumor and endometrioid carcinoma. Pathology outlines leydig cell tumor hilus cell tumor. Diagnosed and treated early, its not likely to return. I have been told that a total hysterectomy should follow. Sertolileydig cell tumor of the ovary in a young female. They are hormonesecreting and give rise to granulomatous and thecal cells of the ovary, thus forming part of the group of tumors of the sex cords and the stroma of the ovary. It is an uncommon primary malignant tumor of the ovary and represents 2% to 5% of all ovarian cancers.
Recurrence was recorded only in patients proven to be tumorfree after. To report on the clinical characteristics and outcome of pediatric patients with juvenile granulosa cell tumor jgct of the ovary. Granulosa cell tumors of the ovary cause higher than normal levels of estrogen in a womans body. Granulosa cell tumor of the ovary requires regular, longterm. Granulosa cell tumors gcts constitute up to 7% of malignant ovarian tumors and nearly 70% of ovarian gonadal tumors. Additional cooperative clinical trials are required to determine the exact efficacy of cisplatin chemotherapy in granulosa cell tumours. Granulosa cell tumor of the ovary symptoms, diagnosis. If you have problems viewing pdf files, download the latest version of adobe reader. Granulosa cell tumor of the ovary is a rare type of ovarian cancer that accounts for approximately 2% of all ovarian tumors.
An ovarian granulosa cell tumor gct is a rare gynecological malignancy with an incidence of 0. Mares were presented with unilateral ovarian enlargement during the 2007 to 2010 breeding seasons. Tumor development is progressive, that is, it proceeds through stages of increasing malignancy. Granulosa cell tumors gcts of the ovary belong to the group of ovarian sexcord stromal tumors and represent 5 to 10% of ovarian malignancies. The adult form is the most common tracing a very typical clinical pathological profile with a slow progression and. Although granulosa cells normally occur only in the ovary, granulosa cell tumours occur in both ovaries and testicles see ovarian. Granulosa cell tumor gct of the ovary is a relatively rare cancer, accounting for only 35% of all ovarian malignancies. In the primordial ovarian follicle, and later in follicle development folliculogenesis, granulosa cells advance to form a multilayered cumulus oophorus surrounding the oocyte in the preovulatory or antral or graafian follicle the major functions of granulosa cells include the production of sex steroids, as well as myriad growth factors thought to. Sex cordstromal tumors represent approximately 8% of all ovarian tumors and are believed to arise from andor to contain combinations of the sex cord and. Characteristics and treatment results of recurrence in. However, gct of the ovary requires regular and longterm followup with a doctor, including an annual blood test to check for markers that could indicate the cancers. They are by far the most frequent subtype of granulosa cell tumors of the ovary 95% and are commoner than the juvenile granulosa tumor of the ovary. Ovarian adult granulosa cell tumor, accounting for 95% of ovarian. Lipoid cell tumor of the ovary article about lipoid cell.
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