Granulosa cell tumors are divided into 2 types, adult and juvenile, and both types represent a distinct group of the socalled sex cordstromal tumors of the gonads. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for granulosa cell tumor of the. Her treatment included surgery to remove the uterus and ovaries. Granulosa cell tumor gct is a rare ovarian tumor originating from sex cord stromal cells 1. The embryological origin of granulosa cells remains controversial. Athanassiou 1st department of medical oncology, metaxas cancer hospital, piraeus, greece. In this study, gcts not only the only tumor that found but also present with high incidence in. Inhibin b is secreted by granulosa cell tumours and could be a useful marker for the disease. Granulosa cell tumor is restricted at the ovary without having intracystic papillary protrusions. Rapidly growing juvenile granulosa cell tumor of the ovary. Granulosathecacell tumor of the ovary in children jama. Recurrences usually occur in median at 5 years after the. Patients may present with vaginal bleeding caused by endometrial hyperplasia or uterine cancer as a result of prolonged exposure to tumor derived estrogen. Although the usefulness of ffdg petct is well demonstrated in the staging and followup of.
In 30 consecutive patients with a stage iiii granulosa cell tumor, inhibin a and b concentrations were measured in pre and posttreatment serum samples. Apr 30, 2010 granulosa cell tumor is a slowgrowing cancer that starts in the granulosa cells that surround the eggs within the ovary. However, high umbilical cord serum concentrations brenner tumors, lipoid cell tumors of the ovary, sclerosing stromalcell tumors, and adrenal tumors. The adult form is the most common tracing a very typical clinical pathological profile with a slow progression and recurrence that occurs long.
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. What hiring strategies have worked for you in this covid19 pandemic era. Ct and mri findings of sex cordstromal tumor of the ovary. Granulosa cell tumor of the ovary requires regular, long.
Adult granulosa cell tumor gct of the ovary is oftentimes a hormonally active, stromal cell neoplasm that is distinguished by its ability to secrete sex steroids such as estrogen. The objective of this study was to determine the clinicopathologic prognostic factors in adult granulosa cell tumors of the ovary. 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 granulosa cells in six tumors demonstrated immunoreactivity for vimentin, five. The molecular mechanism of ovarian granulosa cell tumors journal. Taira y, hirakawa m, nagayama c, ikemiyagi k, touma t, tokashiki m. If you are facebook, there is a page for the granulosa cell tumor foundation in new zeland.
Short report cisplatincontaining regimen in advanced or recurrent granulosa cell tumours of the ovary d. Clinical and pathologic prognostic factors in adult. Cytology of granulosa cell tumor of the ovary american. Gct exhibit a molecular profile that is consistent with fshresponsive granulosa cells of. Immunohistochemistry of ovarian granulosa cell tumours. Granulosa cell tumor of the ovary may create a diagnostic challenge in cytologic preparations. 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. Granulosa cell tumor of the ovary genetic and rare. Granulosa cell tumours are tumours that arise from granulosa cells. Granulosa cell tumors gct of the ovary are low grade tumor with a potential ability of late pelvic recurrences and distant metastases. Ovarian granulosa cell tumor gct is a malignant tumor originating from the sexcord stromal cells of the ovary.
Medroxyprogesterone acetate mpa in advanced granulosa. Adult granulosa cell tumor agct is a sex cord stromal tumor scst which constitutes 25% of all ovarian. Ovarian gct accounts for less than 5% of ovarian malignancies 1. Pathological examination returned to a left ovarian tumoral process evoking a tumor of the sex cords. They are estrogen secreting tumors and present as large, complex, ovarian masses. Granulosa cell tumors of the ovary with precocious puberty. Granulosa cell tumors gcts of the ovary account for 2 to 5 of ovarian malignancies. Granulosa cell tumor of the ovary genetic and rare diseases. We present two patients with malignant ovarian adult gct.
Gct exhibit a molecular profile that is consistent with fshresponsive granulosa cells of the late preovulatoy follicle 1, 2. In the other, an aromatase inhibitor was effective for recurrent malignant gct. Adulttype granulosa cell tumors of the ovary agcts are rare and recurrence is difficult to treat. The immunohistochemical study diagnosed an adult granulosa tumor. Granulosa cell tumors of the ovary are rare neoplasms that originate. While granulosa cell tumors are the most common type of ovarian tumor, producing precocious puberty, they only account for about 10% of all the reported cases. The patient was found to have a stage ic granulosa cell tumor of the ovary despite undergoing annual gynecologic examinations. The records of all patients were analyzed to determine clinical presentation, treatment, survival, and prognostic factors. Their natural history however is indolent with a very favorable longterm prognosis. Gcts of the ovary belong to the group of ovarian sexcord stromal tumors. Aug 25, 2009 granulosa cell tumors gcts of the ovary are relatively uncommon neoplasms, representing approximately 5 to 10% of all ovarian malignancies 1, 2.
Cytomorphology and clinicopathologic correlation of the. Recently i have be diagnosed with granulosa cell tumor of the ovary. From the department of obstetrics and gynecology, university of wisconsin n the 45 cases of malignancy of the ovary proved by microscopic examination at the wisconsin general hospital during the past eight years, five have been granulosa cell tumors. I have been told that a total hysterectomy should follow. In addition, gct is a vascular tumor that may occasionally. Testicular granulosa cell tumor of the adult type is a very rare tumor, and to date, only a small number of cases have been reported. Adult granulosa cell tumor gct is a rare ovarian malignancy having good prognosis in comparison with other epithelial tumors. Although jgct have different histologic features compared to agct, the two types have similar imaging features because they have similar gross.
Granulosa cell tumor of the adult type archives of. Discussion granulosa cell tumors of the ovary are uncommon tumors with an indolent course. Pdf granulosa cell tumors are rare neoplasms characterized by long natural. The aim of this study was to explore the clinicopathological characteristics of recurrent adulttype granulosa cell tumor of the ovary agcot and evaluated the treatment results to define the prognostic parameters for survival after recurrence. The agct is more common type, conversely, less than 5% of tumors are the jgct and occur in mainly premenarchal girls and in women younger than 30 years. Characteristics and treatment results of recurrence in adult. It represents the commonest malignant sex cordstromal tumour and the. The new molecular biology of granulosa cell tumors of the. Granulosa cell tumor gct of the ovary is a rare tumor accounting for 2%5% of ovarian malignancies. Diagnosed and treated early, its not likely to return. Unlike epithelial ovarian tumors, they occur in young women and are usually detected at an early stage. In contrast to ovarian carcinomas, granulosa cell tumors may exhibit dotlike paranuclear. Composite mucinous and granulosa cell tumor of the ovary. Granulosa cell tumors of the ovary are a type of sex cord stromal ovarian tumor.
The imaging system provides some classic features, which can assist to diagnose the granulosa cell tumor. My 47yearold wife was diagnosed with a rare granulosa cell tumor gct of the ovary. May 21, 2012 the patient was found to have a stage ic granulosa cell tumor of the ovary despite undergoing annual gynecologic examinations. Although jgct have different histologic features compared to agct, the two types have similar imaging features because they have similar. Clinical and pathologic prognostic factors in adult granulosa. Granulosa cell tumour an overview sciencedirect topics. Within recent years granulosa theca cell tumors have attracted much interest, primarily because of their hormonal function. Granulosa cell tumors of the ovary comprise a distinct subset of ovarian cancers that account for approximately 5% of all ovarian malignancies 6. Pdf download for granulosa cell tumor of the ovary. While granulosacell tumors are the most common type of ovarian tumor, producing precocious puberty, they only account for about 10% of. Molecular pathogenesis of granulosa cell tumors of the ovary. A composite mucinous and granulosa cell tumor of the ovary in a 76.
However, there is sparse literature on the cytopathologic features of metastatic granulosa cell tumors mgct. Juvenile granulosa cell tumor in a nine year old sciencedirect. Aromatase inhibitor therapy as effective rescue in a patient with tamoxifenrefractory metastatic granulosa cell tumor of the ovary wolfgang lamm. Cisplatincontaining regimen in advanced or recurrent. Dec 01, 2012 well for being such a rare form of ovc, there seems to be a lot of us here. In patients with a granulosa cell tumor of the ovary, the value of serum inhibin a and b concentrations for the assessment of disease status was investigated. I would like to hear and talk to other people who have this same type of cancer. Characteristics and treatment results of recurrence in.
Adult granulosa cell tumors of the ovary are rare neoplasms, accounting for less than 5% of all ovarian malignancies. Extraovarian granulosa cell tumor gct is a very uncommon tumor, assumed to arise from the ectopic gonadal tissue along the embryonal route of the genital ridge. Here, the authors observe in agct a novel recurrent. This tumor is classified into juvenile gct and adult gct, and the majority of the cases are the adult type. Scullyluteinized adult granulosa cell tumors of the ovary. The granulosa cell tumor of the ovary foundation gctf exists to raise funds for granulosa cell tumor of the ovary gct, ovarian cancer research. We are not able to provide medical advice or cancer specialist referrals. Our experience with needle aspiration material, ascitic fluid, and peritoneal washings indicates that in fluids, despite some similarity between the tumor cells and reactive mesothelial cells, a correct diagnosis can be achieved by careful examination. Granulosa cell tumor of the ovary is a rare type of ovarian cancer that accounts for approximately 2% of all ovarian tumors. In addition to the tumor stage, residual disease, patient age, tumor size.
Granulosa tumors tgo are a rare entity of malignant ovarian tumors 5%. These tumors are the principal feminizing neoplasms of the ovary. In one patient, a combination of bleomycin, etoposide, and cisplatin was effective after initial surgery for malignant gct. Granulosa cell tumors of the ovary account for less than 5% of all malignant ovarian tumors but represent the most common malignant sex cordstromal tumor and the most common clinically estrogenic ovarian tumor. Patients may present with nonspecific symptoms such as abdominal pain, distention, or bloating. Granulosa cell tumor of the ovary radiology reference. Successful treatment of adulttype granulosa cell tumor of the ovary by palliative radiotherapy. Histology following right salpingooophorectomy reported an earlystage figo ia granulosa cell tumour gct of the ovary. A 54 years old female patient presented with a mass and acute pain in abdomen. Functional neoplasms of the ovary are relatively infrequent compared to ovarian tumors in general, and only about 5% occur before puberty. This report presents a case of granulosa cell tumor of the ovary after the long. Md anderson, 1999 19491988 pmid 10094877 radiation treatment of advanced or recurrent granulosa cell tumor of the ovary.
Within recent years granulosathecacell tumors have attracted much interest, primarily because of their hormonal function. A new classification of ovarian granulosa cell tumor based. For language access assistance, contact the ncats public information officer. Granulosa cell tumor gct is a rare tumor in ovaries, accounts. Pediatric granulosa cell tumors of the ovary are rare functional neoplasms causing secondary precocious puberty. Granulosa cell tumors are the most common malignant sex cordstromal ovarian tumor. Sex cordstromal and steroid cell tumors of the ovary. The incidence of granulosa cell tumor in buffaloes was 18.
Radiation oncologyovarygranulosa cell tumor wikibooks. Granulosa cell tumors of unilocular cystic type sciencedirect. Rapidly growing juvenile granulosa cell tumor of the ovary arising in. A retrospective analysis of 26 patients of granulosa cell tumor of ovary from 2002 to 2011 was carried out. 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. Epidemiology sex cordstromal tumors represent approximately 8% of all ovarian tumors and are believed to arise from andor to contain combinations of the sex co. This type of tumor is known as a sex cordstromal tumor and usually occurs in adults. Granulosacell tumor of ovary with sexual precocity jama. Between 2000 and 2014, eight cases of mgct were diagnosed by fna. Paraffinembedded material of 47 ovarian tumours primarily diagnosed as granulosa cell tumours, including 2 cases of juvenile granulosa cell tumour, were studied immunohistochemically for the presence of intermediate filament proteins, epithelial membrane antigen and tumour markers.
This seems to be a very rare type of ovarian cancer. The patient had left adnexectomy, peritoneal cytology. Presentation of two patients with malignant granulosa cell. Juvenile granulosa cell tumor is an uncommon testicular tumor, most frequently seen in newborns and infants below the age of 2 years. Small, bland, cuboidal to polygonal cells in various patterns, including callexner bodies small folliclelike structures filled with acidophilic material, macrofollicular, trabecular, solid and insular patterns. In the 1970s, evidence emerged that the first cells to make contact with the oogonia were of mesonephric origin.
The aim of this study was to report the clinical and pathological characteristics of agct patients and to identify the prognostic factors. Clinical, pathologic, and followup data were collected. It was suggested that mesonephric cells already closely associated with the oogonia proliferated throughout development to form the granulosa cell layer. Ovarian granulosa cell tumors gcts are divided into adult gct agct and juvenile gct jgct. Granulosa cell tumors of the ovary gct are derived from the sex cords and the ovarian stroma. A retrospective analysis of ten cases of adult granulosa cell tumor of. At laparotomy this tumor proved to be a solid and cystic mass measuring 10 cm in greatest diameter. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Granulosa cell tumors of the ovary cause higher than normal levels of estrogen in a womans body. It is an uncommon primary malignant tumor of the ovary and represents 2% to 5% of all ovarian cancers. The new molecular biology of granulosa cell tumors of the ovary. These tumours are part of the sex cordgonadal stromal tumour or nonepithelial group of tumours. Granulosa cell tumor ovary treatment, diagnosis, symptoms. Dec 14, 2018 ovarian granulosa cell tumors gcts are divided into adult gct agct and juvenile gct jgct.
There is also a private facebook group of almost 600 women diagnosed with gct called gct survivor sisters that you can request to join. Nov 15, 2005 a composite mucinous and granulosa cell tumor of the ovary in a 76. The tumor was stage 1, but we are nervous about a recurrence. Granulosa cell tumor of the ovary with high fdg uptake. A new classification of ovarian granulosa cell tumor based on. Adult granulosa cell tumors agcts are the most common sex cordstromal tumors.
990 324 1249 795 1107 75 786 356 389 568 1353 285 186 660 1026 1352 1521 658 113 246 405 465 1575 1277 151 647 1243 1414 830 1335 872 555 88 1433 162 1270