Pdf granulosa cell tumors are rare neoplasms characterized by long natural history and favorable prognosis. Metastases from adult granulosa cell tumor of the ovary indicates a malignant primary. Over these years, more and more sex cordstromal tumors have been reported. Although previous cases of recurrence have been described, it is extremely unusual for these tumours to recur after thirty years.
Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Pdf hepatic metastases of granulosa cell tumour of the ovary. The molecular mechanism of ovarian granulosa cell tumors. 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. Patients may present with nonspecific symptoms such as abdominal pain, distention, or bloating. Doubling time accelerated with subcultures, and was 23. Presentation of two patients with malignant granulosa cell tumors. Granulosa cell tumor of the ovary, cea immunostain.
Granulosa cell tumours of the ovary are unique, hormo nally active, oestrogensecreting tumours accounting for 1 to 2% of all the ovarian malignancies. Characteristics and treatment results of recurrence in. 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. Granulosa cell tumor gct is the most common sex cordstromal tumor that stem from granulosa cells.
Molecular pathogenesis of granulosa cell tumors of the ovary. I am now to get some other tests and am waiting for an appointment. 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 return. I would like to hear and talk to other people who have this same type of cancer. 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. We describe a case of recurrence of granulosa cell tumour after 30 years, presenting as small. Gct accounts for 2% to 5% of all ovarian cancers and can be divided into two subtypes according to the differences of the age of patients, clinical and histopathologic features. 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. This seems to be a very rare type of ovarian cancer. 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. 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. The granulosa cell tumor of the ovary foundation gctf exists to raise funds for granulosa cell tumor of the ovary gct, ovarian cancer research. Clinical and pathological predictive factors in women with adulttype granulosa cell tumor of the ovary.
The granulosa or granulosatheca cell tumor is a hormone producing neoplasia that can suppress follicular development in. These tumours have preponderance for local spread and extremely late recurrence. Below are the links to the authors original submitted files for images. Get a printable copy pdf file of the complete article 1. Adult granulosa cell tumor agct is a sex cord stromal tumor scst which constitutes 25% of all ovarian cancers. Granulosa cell tumor of the ovary genetic and rare diseases. Recently i have be diagnosed with granulosa cell tumor of the ovary. Presentation of two patients with malignant granulosa cell.
Dec 01, 2012 well for being such a rare form of ovc, there seems to be a lot of us here. 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. Their natural history however is indolent with a very favorable longterm prognosis. Granulosa cell tumors of the ovary comprise a distinct subset of ovarian cancers that account for approximately 5% of all ovarian malignancies 6. Granulosatheca cell tumor pregnancy anabolic steroid administration developmental abnormality chromosomal abnormality ovarian tumors are typically unilateral.
In one patient, a combination of bleomycin, etoposide, and cisplatin was effective after initial surgery for malignant gct. Granulosa cell tumors gcts of the ovary account for 2 to 5 of ovarian malignancies. Ovarian adult granulosa cell tumor, accounting for 95% of ovarian granulosa cell tumor, mainly occurs in perimenopausal and postmenopausal women and exhibits unpredictably late recurrence features 36. Immunohistochemistry of ovarian granulosa cell tumours. Granulosa cell tumor of the ovary radiology reference. Ultrasound exam mistaked the diagnosis of ovarian mass in patient 5 and the granulosa cell tumor of the right ovary was diagnosed on the histological. Granulosa cell tumor is restricted at the ovary without having intracystic papillary protrusions. Granulosa cell tumor of the ovary requires regular, longterm.
Granulosa cell tumours are tumours that arise from granulosa cells. It is an uncommon primary malignant tumor of the ovary and represents 2% to 5% of all ovarian cancers. There is also a private facebook group of almost 600 women diagnosed with gct called gct survivor sisters that you can request to join. Apr 30, 2010 granulosa cell tumor is a slowgrowing cancer that starts in the granulosa cells that surround the eggs within the ovary. Recurrence of granulosa cell tumour after thirty years. Aug 30, 2018 villella j, herrmann fr, kaul s, lele s, marchetti d, natiella j. It was suggested that mesonephric cells already closely associated with the oogonia proliferated throughout development to form the granulosa cell layer. Based on the information provided, this case of adult granulosa cell tumor is malignant and reportable. Radiation oncologyovarygranulosa cell tumor wikibooks. Granulosa cell tumor of the ovary, inhibin immunostain. The adult form is the most common tracing a very typical clinical pathological profile with a slow progression and recurrence that occurs long. This tumor is classified into juvenile gct and adult gct, and the majority of the cases are the adult type. Granulosa cell tumors of the ovary are a type of sex cord stromal ovarian tumor.
Pdf download for granulosa cell tumor of the ovary. Discussion granulosa cell tumors of the ovary are uncommon tumors with an indolent course. Diagnosed and treated early, its not likely to return. Granulosa cell tumor of the ovary, inhibin immunostain pathological and histological images courtesy of ed uthman at flickr. Adultonset ovarian granulosa cell tumor is a rare malignancy accounting for may 16, 2012 ovarian granulosa cell tumor gct is a malignant tumor originating from the sexcord stromal cells of the ovary.
The tumor was examined histologically and found to be a granulosatheca cell tumor. These tumours characteristically have a low malignant potential, and a tendency for local spread and high recurrence rates, sometimes many years after the original diagnosis 2, 3. Granulosa cell tumors are usually benign steroidproducing tumors that may grow very large. Granulosa cell tumor of the ovary is a rare type of ovarian cancer that accounts for approximately 2% of all ovarian tumors. In the other, an aromatase inhibitor was effective for recurrent malignant gct. The embryological origin of granulosa cells remains controversial. Granulosa cell tumor of the ovary, inhibin immunostain 5631091.
Often they are initially found during a routine reproductive examination of the mare, when one ovary is discovered to be excessively larger than the other. They are estrogen secreting tumors and present as large, complex, ovarian masses. Granulosa cell tumors constitute less than 5 % of all ovarian tumors. Ovarian granulosa cell tumor gct is a malignant tumor originating from the sexcord stromal cells of the ovary. Full text full text is available as a scanned copy of the original print version. Granulosa theca cell tumor pregnancy anabolic steroid administration developmental abnormality chromosomal abnormality ovarian tumors are typically unilateral. Adult granulosa cell tumor gct of the ovary is oftentimes a hormonally active, stromal cell neoplasm that is distinguished by its ability to. May 16, 2012 ovarian granulosa cell tumor gct is a malignant tumor originating from the sexcord stromal cells of the ovary. May 11, 2004 granulosa cell tumours are uncommon sex cordstromal tumours of the ovary, and comprise approximately 3% of all ovarian malignancies. They are characterized by a prolonged history and a tendency to late recurrences. In vitro studies of the tumor tissue revealed a significantly high capacity to effect the conversion of testosteronec14 to radio active estrogens.
The immunohistochemical study diagnosed an adult granulosa tumor. Granulosa cell tumor of the ovary, cea immunostain 5631596448. Granulosa cell tumors gcts are rare neoplasms with a relatively favorable prognosis. Granulosa tumors tgo are a rare entity of malignant ovarian tumors 5%. The records of all patients were analyzed to determine clinical presentation, treatment, survival, and prognostic factors. Granulosa cell tumor is a slowgrowing cancer that starts in the granulosa cells that surround the eggs within the ovary. I immediatelly started to be very suspicious, weepy, frightful and extremely impatient.
Granulosa cell tumors of the ovary gct are derived from the sex cords and the ovarian stroma. The tumor was examined histologically and found to be a granulosa theca cell tumor. Granulosa cell tumors of the ovary gct comprise a distinct subset of. A retrospective analysis of 26 patients of granulosa cell tumor of ovary from 2002 to 2011 was carried out. Granulosa cell tumours of the ovary are rare, comprising around 3% of ovarian tumours. Gct exhibit a molecular profile that is consistent with fshresponsive granulosa cells of the late preovulatoy follicle 1, 2. Hi, i just had a complete laparoscopicvaginal assisted hysterectomy. Pathological examination returned to a left ovarian tumoral process evoking a tumor of the sex cords. 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. We present two patients with malignant ovarian adult gct. They are the most common type of malignant sex cord stromal tumor scst, comprising 25% of all ovarian malignancies 1.
In the 1970s, evidence emerged that the first cells to make contact with the oogonia were of mesonephric origin. Granulosa cell tumors gcts of the ovary arise from the cells that would typically give rise to specialized gonadal stroma surrounding the oocytes. The patient had left adnexectomy, peritoneal cytology. Recurrence of granulosa cell tumour after thirty years with. The answer points out an important difference in the way metastases from this histology should be interpreted versus low malignant potential ovarian epithelial tumors. However, high umbilical cord serum concentrations brenner tumors, lipoid cell tumors of the ovary, sclerosing stromalcell tumors, and adrenal tumors. The tumor shown at left is six inches in length, but larger examples are found. Granulosa cell tumors of the ovary cause higher than normal levels of estrogen in a womans body. I am now to get some other tests and am waiting for an. Adult granulosa cell tumor of left ovary of 92 year old mexican american woman. The morphology and immunoprofile of the cellular aggregates in the paratubal soft tissue are consistent with metastatic granulosa cell tumor.
Investigations revealed a secondary tumour in segment vi and vii of the liver. 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. This type of tumor is known as a sex cordstromal tumor and usually occurs in adults. The granulosa or granulosa theca cell tumor is a hormone producing neoplasia that can suppress follicular development in the contralateral ovary.
Although granulosa cells normally occur only in the ovary, granulosa cell tumours occur in both ovaries and testicles see ovarian cancer and. Granulosa cell tumor of the adult type archives of. Griffith et al41 incubated the tissue obtained from a luteinized granulosatheca cell tumor with progesteronec14 and. Pathological and histological images courtesy of ed uthman at flickr. Well for being such a rare form of ovc, there seems to be a lot of us here. For language access assistance, contact the ncats public information officer. Microscopic findings revealed a tumour with histological features identical to that removed eleven years before. Granulosa cell tumor of the ovary requires regular, long. The imaging system provides some classic features, which can assist to diagnose the granulosa cell tumor. I have been told that a total hysterectomy should follow. A case of metastatic granulosa cell tumour of the ovary is reported. A diagnosis of malignant ovarian tumor was made, and surgery was performed.
Gct exhibit a molecular profile that is consistent with fshresponsive granulosa cells of. Villella j, herrmann fr, kaul s, lele s, marchetti d, natiella j. The granulosa cells were isolated, the tumor was cultured and underwent over 100 passages in 25 months. Granulosa cell tumors of the ovary are rare neoplasms that originate. If you are facebook, there is a page for the granulosa cell tumor foundation in new zeland. We are not able to provide medical advice or cancer specialist referrals. The imaging features table 2 on page showed a tumor of the right ovary in 4 cases, a bilateral tumor in 1 case and a peritoneal recurrency tumor in the culdesac of douglas in one case. Md anderson, 1999 19491988 pmid 10094877 radiation treatment of advanced or recurrent granulosa cell tumor of the ovary. Pdf granulosa cell tumors constitute less than 5 % of all ovarian tumors. It is the most common type of sex cordstromal tumors. However, negative immunostains often provide the bonus of giving a better view into cytologic details of a tumor. Granulosa cell tumor of the ovary genetic and rare diseases nih. The left ovary showed enlargement in the ultrasound and when they tested it after removal, they confirmed that it was a granulosa cell tumor.
Chromosomal number varied considerably and showed hyperploidy. Granulosa cell tumor of the ovary journal of clinical oncology. Granulosa cell tumor ovary treatment, diagnosis, symptoms. Recurrences usually occur in median at 5 years after the. Granulosa cell tumor of the ovary genetic and rare. Granulosa cell tumor can be diagnosed by using radiological imaging tools. If you have problems viewing pdf files, download the latest version of adobe reader.
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