granulosa cell tumours ( gcts ) though accounting for approximately 70% of malignant sex - cord stromal tumors are rare , comprising only 25% of all ovarian neoplasms [ 13 ] . these tumours arise from granulosa cells that are hormonally active stromal elements in close association with ovarian oocytes , which are responsible for the production of estradiol . the exact etiology of this malignancy remains unknown , with no identification of specific defined risk factors . the typical clinical scenario of a gct is an older postmenopausal woman with menstrual abnormalities who is found to have a singular pelvic mass that is curable by en - bloc resection . while such typical cases do exist , characteristics of gcts in clinical practice do not always fit within the confines of these parameters . as such , seeing past this  myth  to recognize and identify uncommon  realities  is necessary for the accurate diagnosis and management of gcts . the aim of this study is to discuss the  myths and realities  of gcts through a series of indexed cases in the context of relevant evidence - based literature . further , a comprehensive fourteen - year ( 19972011 ) search using the laboratory information system ( lis ) identified additional patients with gcts . patient demographics including age , sex , and tumour site were collected for analysis . a literature search using the national library of medicine interface pubmed was conducted using the search terms  granulosa cell  limited to the english language . this study was conducted with ethics approval from the university of saskatchewan biomedical research ethics review committee . ultrasonography confirmed the mass to be a 2  cm lesion , and an orchiectomy was carried out . on histological examination , the tumour was solid with regions of cystic spaces ( figures 1(a ) and 1(b ) ) . amphophilic cytoplasm with indistinct cell borders and an extensive fibrocollagenous stroma were identified ( figure 1(c ) ) . the lesional cells were positive to inhibin ( figure 1(d ) ) , confirming the sex - cord stromal nature of this neoplasm . twenty years post - operatively he is doing well , with no evidence of recurrence .   seven days after birth , a female neonate developed increased abdominal distension and constipation . a large mass was palpable on abdominal exam that prompted ultrasonography , which detected a 10  cm multiseptated cystic , mass . this was further confirmed by computed tomography ( ct ) scan , which showed the mass to be surrounded by a large volume of fluid ( figure 2(a ) ) . hematological investigations showed high levels of estradiol ( 310 ) , prolactin ( 34.9 ) , and thyroid - stimulating hormone ( tsh , 11.05 ) with normal levels of luteinizing hormone ( lh ) , follicle - stimulating hormone ( fsh ) , -fetoprotein , and -human chorionic gonadotropin ( -hcg ) . the young girl was taken to the operating room for a laparotomy , which revealed a large cystic mass of the left ovary with torsion ( figure 2(b ) ) . an oophorectomy with preservation of the left fallopian tube was carried out . on follow - up abdominal and pelvic ct , no evidence of residual disease was detected , and estradiol and tsh levels normalized . eighteen years post - operatively she is doing well , with no evidence of recurrence .   on histological examination , a multicystic lesion lined by granulosa cells was identified ( figure 2(c ) ) , without the typical theca internal layer lining the cysts . solid proliferations of granulosa - like cells were recognized in the ovarian stroma , and focal areas with a solid insular pattern and nongrooved nuclei were present ( figure 2(d ) ) . histological features were between those common to adult gct ( agct ) and juvenile gct ( jgct ) . an almost exclusive macrofollicular pattern typical of agct was contrasted by small areas of the solid , insular pattern as seen in jgct . the unusual facet in this case is the clinical presentation of an unsuspected gct in a seven - day - old neonate .   a 76-year - old woman presented with a growth at the umbilicus and bowel obstruction . ct scan showed a complex mass arising from the anterior bladder wall / dome of the bladder ( figure 3(a ) ) . with the clinical suspicion of a urachal carcinoma , the patient was taken to the operating room where she underwent a partial cystectomy and anterior abdominal wall mesh reconstruction . the mass was smooth and solid cystic ( figure 3(b ) ) , with extension from the bladder 's dome to the umbilicus . at microscopic examination , sheets of spindle cells in focal retiform - like areas with uniform grooved nuclei were identified ( figure 3(c ) ) . immunohistochemical analysis found the mass to be positive to vimentin , with focal positivity to inhibin a ( figure 3(d ) ) and cytokeratin . an incidental pelvic mass was detected in a 64-year woman with a past history of total abdominal hysterectomy and bilateral salpingooophrectomy for a gct 16 years prior . a ct scan confirmed the presence of a large heterogenous solid - cystic pelvic mass ( figure 4(a ) ) . the preoperative diagnosis of this mass included abscess , gastrointestinal stromal tumor , and extracolonic mass . she underwent laparotomy with pelvic washings for cytology and en - bloc resection of the left ovarian mass . on gross examination , necrosis reminiscent of colonic adenocarcinoma admixed with diffuse , microfollicular , and cords of neoplastic cells were observed ( figure 4(b ) ) . deeper sections confirmed the presence of the glandular neoplasm originating from a diverticular outpouching of the overlying colonic mucosa , confirming the presence of colonic adenocarcinoma arising in a diverticulum of the large bowel . this was further supported by immunohistochemistry that confirmed the two components of this collision tumour with inhibin positivity in the gct component ( figure 4(c ) ) and cytokeratin positivity in the adenocarcinoma component ( figure 2(d ) ) .    a 45-year - old woman presented to the emergency room with a two - year history of menorrhagia and acute dyspnea . an enhanced ct scan revealed a giant mass ( estimated size , 36  cm craniocaudal ) filling the pelvis and abdomen and causing displacement of the bowel loops . the patient developed imminent renal failure and her hemoglobin levels continued to drop despite receiving 9 units of blood . it was suspected that her mass was haemorrhaging , and she was taken to the operating room . a 3540  cm , multilobulated , haemorrhagic mass was found in her right ovary , and a total abdominal hysterectomy with bilateral oophorectomy , with en - bloc mass resection and partial omentectomy were carried out . microscopic examination showed the presence of sheets of neoplastic cells interspersed with rupture and acute hemorrhage ( figure 5(a ) ) . the ovarian tumour showed granulosa cells arranged in solid sheets , trabeculae , tubules , and microfollicles that included call - exner bodies ( figures 5(b ) and 5(c ) ) . immunohistochemical analysis found the neoplastic cells to stain positive for vimentin , inhibin ( figure 5(d ) ) , cd99 , and afp . the unique presentation of this gct is an acute abdomen due to tumor rupture with hemorrhage . surgical review a 14-year ( 19972011 ) surgical pathology review yielded 37 cases of gct , with an overall prevalence of 0.0041% ( 37/902 100 ) . of these cases , 36 were females and one male ( index case 4 ) . within the female population , patient 's age ranged from 7 days to 85 years , with a mean of 52 years ( median 48 years ) . locations of gcts included 17 cases in the right ovary ( 47.2% ) , 15 in the left ovary ( 41.7% ) , and one ( 2.8% ) in each of the bowel , abdominal wall , and omentum . only four ( 10.8% ) concurrent pathologies included leiomyoma of the uterus ( 21% ) , benign cysts ( 14% ) , adenomyosis ( 7% ) , coexisting ovarian adenocarcinoma ( 3% ) , and endometrial carcinoma ( 3% ) . a 14-year ( 19972011 ) surgical pathology review yielded 37 cases of gct , with an overall prevalence of 0.0041% ( 37/902 100 ) . of these cases , 36 were females and one male ( index case 4 ) . within the female population , patient 's age ranged from 7 days to 85 years , with a mean of 52 years ( median 48 years ) . locations of gcts included 17 cases in the right ovary ( 47.2% ) , 15 in the left ovary ( 41.7% ) , and one ( 2.8% ) in each of the bowel , abdominal wall , and omentum . only four ( 10.8% ) concurrent pathologies included leiomyoma of the uterus ( 21% ) , benign cysts ( 14% ) , adenomyosis ( 7% ) , coexisting ovarian adenocarcinoma ( 3% ) , and endometrial carcinoma ( 3% ) . granulosa cell tumours ( gcts ) were first described in 1855 by rokitansky as chronicled in chew et al . gcts are rare sex - cord stromal tumors that are thought to arise from the normal proliferating granulosa cells of the late preovulatory follicle as they share many morphological and biochemical features with these cells . additionally , theca cells , interstitial cells , and stromal fibroblasts have also been delegated as cells of origin for gct . . recently , there is growing evidence to suggest that granulosa stem cells ( gscs ) do exist . identification of normal and neoplastic gscs and the factors that regulate their behavior will determine the treatment of all ovarian cancers including gcts in the future . however , currently , little is known regarding the molecular and genetic changes associated with gcts . they have been associated with a variety of genetic abnormalities such as trisomy 12/14 , monosomy 22 , and > 90% of adult gct 's have a missense somatic c134w mutation in the foxl2 gene . in men , the typical clinical scenario of a gct is usually a middle - aged female presenting with a pelvic mass who is cured by en - bloc resection of the mass . these characteristics however are not generalizable to all gcts as these lesions can present in many different facets and have a tendency to behave unpredictably , thus complicating both diagnosis and therapeutic management . accurate recognition of the true  realities  of multifaceted gct is therefore vital for the precise diagnosis and management of these lesions . some of the existing myths of gcts will now be contrasted with their true realities in the context of evidence - based literature . this cancer can be divided into three types based on the cell of origin ( germ , epithelial , and stromal ) with each conferring different histopathological features and clinical outcomes . stromal tumours are further classified based on the tissue types involved as sertoli , leydig , theca , and granulosa . granulosa cell tumours ( gcts ) account for 1 - 2% of all ovarian tumours   and arise from the granulosa cells that normally surround the oocytes and line the developing follicle . these include a ) these neoplasms are derived from the mesenchyme of the developing genital ridge and b ) these neoplasms arise from precursors within the mesonephric and coelomic epithelium . the presence of extraovarian gct 's as seen in our case 3 supports the latter theory . to date though chromosomal anomalies and/or autocrine and endocrine signalling abnormalities are proposed aetiologies , the current etiology postulated is one of multifactorial origin . these lesions are considered a low - grade type malignancy , with 7090% of neoplasms being diagnosed at stage 1 . the high detection rate at an early stage may be due to the endocrine symptoms that often present early in the functioning tumors . low staging at diagnosis confers an excellent prognosis , with 5-year survival rates reported between 7595% ( stage 1 ) ; however , these rates drop to 5575% and 2250% for stages ii and iii / iv respectively . this may be partially due to limited treatment options for advanced and recurrent disease . although predominantly occurring in the granulosa cells of the female ovary , gcts are also reported to arise within the male testis , as seen in our index case 1 . testicular sex - cord stromal tumours are rare , comprising only 4% of all testicular tumours . juvenile gct ( jgct ) is far more common than adult gct ( agct ) within the testicle with no preferred laterality within the testis [ 9 , 10 ] . approximately half of testicular jgcts are diagnosed within the first month of life , and over 95% within the first year as seen in our case 1 . the differential diagnosis of testicular jgct includes yolk sac tumour , undifferentiated sex - cord stromal tumour , gynandroblastoma , and gonadoblastoma . typically , males present with a painless indolent testicular swelling . due to estrogen hypersecretion , patients may be impotent , and 25% have gynaecomastia [ 9 , 13 ] . an intra - abdominal mass of an undescended testis and/or a testicular torsion may additionally be present . jgcts in undescended testis are benign and do not reach an adequate size to cause pressure on other organs . though patients with lymph node metastases usually have a longer survival period , the presence of distant metastases is usually associated with a dismal prognosis . histologically , testicular gct resembles ovarian , presenting as a solid , cystic mass with microfollicular , gyriform , insular , and trabecular patterns . cells are typically immunopositive for vimentin , inhibin , smooth muscle actin , cd99 , and s-100 [ 9 , 18 ] . genetically , chromosomal abnormalities such as an atypical y chromosome and mosaicism may be present . most patients with gcts are perimenopausal or early postmenopausal , with a median age of diagnosis between 5054 years . nevertheless , as our surgical review demonstrates , gcts may arise in neonates ( index case 2 ) or in patients over the age of 80 ( series ) . gcts in neonates , as in index 2 , are a rare occurrence , with few reported cases in less than one year of age . a common misconception is that the two subclassifications of gcts , adult - type gct ( agct ) and juvenile - type gct ( jgct ) , refer to the age of development . though agct and jgct occur more often in adults and children , respectively , either form may present throughout the entire population . the majority ( 95% ) are agcts , more commonly seen in adults [ 22 , 23 ] . agct has been reported in children ; however , less than 1% of these lesions occur in prepubertal girls . upto 90% of jgcts are diagnosed in patients under the age of 30 with half of jgct cases seen in less than 10 years of age [ 22 , 27 ] and 10% occurring in infants less than one year . differences between agct and jgct are distinct ; therefore , accurate identification is critical to guide patient management . unlike agct , jgct is considered by many to be a relatively benign tumour and in infantile males is the common type of sex - cord stromal tumour of the testis . cells producing hormones such as estradiol are present in 70% of jgcts . as such , in young patients , clinical evidence of isosexual precocious pseudo - puberty including breast enlargement , pubic and axillary hair development , vaginal secretions , irregular uterine bleeding , advanced somatic / skeletal developments , and secondary sex characteristics may all be associated with jgct [ 2 , 5 ] . these patients often have elevated estradiol levels , though this is not a requirement for precocious puberty . the risk of precocious puberty is especially high in jgct patients under one year of age , but gct in this age range is rare . such symptoms are present in 8090% of patients under the age of 8 with jgct . on radiologic imaging , jgct is often indistinguishable from other ovarian neoplasms , and identification of histopathology features such as a nodular / diffuse cellular growth and/or macrofollicules with eosinophilic / basophilic fluid in their lumen is often required for correct diagnosis . as these are younger women , uterine sparing surgery with conservation of the contralateral tube and ovary is recommended at the outset for maintenance of fertility . advanced - stage disease is responsive to combination chemotherapy with platinum agents [ 5 , 6 ] . the overall prognosis for jgct is excellent , with reports of survival as high as 97% in one study with a 3.5-year followup . after - resection , gross examination of a jgct is similar to that of an agct . on average , microscopically , these tumours are solid / cellular , with follicle formation , edema , and loose stroma . hyperchromatic granulosa cells with rounded nuclei surrounded by eosinophilic or vacuolated cytoplasm and high mitotic rates are additionally seen [ 2 , 5 ] . unlike agct , few call - exner bodies are identified . estradiol is one of the first hormones to be secreted by gcts and is responsible for clinical manifestations . among females , pre - pubescent girls may experience isosexual precocious puberty as a result of increased estrogen levels caused by hyperestrogenism . though hyperestrogenism is the common form of endocrine abnormality in gcts , it is important to consider juvenile gct in females presenting with androgen excess and precocious puberty . the adult gct appears to be the most common type of gct associated with virilisation , suggesting a propensity for increased androgen secretion in agcts . by contrast , these elevated estrogen levels in adults can cause abnormal uterine bleeding , menstrual irregularities , menorrhagia , or amenorrhea [ 20 , 27 ] . patients with virilising gcts can present with hirsutism , clitoromegaly , increased abdominal size , amenorrhea , and deepening of voice . rare case reports document jgcts presenting with paraneoplastic syndrome of hypercalcemia   and meig 's syndrome of pleural effusion with ascites . adult patients may present with vaginal bleeding caused by endometrial hyperplasia or uterine cancer as a result of prolonged exposure to tumor - derived estrogen . in addition , gct is a vascular tumor that may occasionally rupture and result in abdominal pain , hemoperitoneum , and hypotension , mimicking an ectopic pregnancy in younger patients . tumor rupture is often attributed to hemorrhagic cysts in upto 1015% of the cases [ 2 , 27 ] . occasionally gcts and theca cell tumours have been found in ovaries which show no enlargement and are therefore clinically occult [ 35 , 36 ] . gcts are the most common estrogen - producing neoplasms in females and are found to produce estradiol in approximately 4060% of patients . this estradiol production is directly related to the release of testosterone secreted by the theca cells . however , not all gcts are hormonally active or have theca cells that secrete testosterone , and therefore diagnostic testing for these hormones lacks sensitivity and specificity . normal granulosa cells are responsible for production not only of estradiol but also peptide hormones including inhibin , activin , follistatin , and antimullerian hormones . gct patients usually present with elevated levels of inhibin , a negative feedback regulator of fsh secretion ; however , this hormone is not specific for these tumours [ 2 , 3 , 37 ] . mom et al . evaluated the sensitivities and specificities of serum inhibin levels in 30 women with granulosa cell tumors ( inhibin a was 67 and 100% and inhibin b was 89 and 100% , resp . ) . serum inhibin levels are currently available for diagnosis and clinical followup of women with granulosa cell tumors of the ovary   it can , therefore be used to monitor response to therapy or to detect recurrences . mullerian inhibitory substance ( mis ) which is produced in the developing follicles is often elevated in gcts , though it is not specific for diagnosis [ 2 , 3 ] . this hormone is produced exclusively by granulosa cells in postnatal females and both prenatally and postnatally by the sertoli cells in the male testis . this hormone functions in male fetuses to induce regression of the mullerian system . normally , mis is found in low levels in reproductive - aged females and functions as a paracrine inhibitory factor that decreases the response of the resting ovarian follicle to follicle - stimulating hormone ( fsh ) thus ensuring the emergence of a single dominant follicle . serum mis may be a marker of ovarian reserve and typically disappears from the serum after menopause or bilateral oophorectomy . however , in patients with gcts , levels have been shown to parallel the extent of disease . serum mis levels are not routinely available for clinical use in the context of gct diagnosis and followup . however , a commercial version of the ultrasensitive elisa assay has become available and may lead to wider clinical use of mis in the future . serum mis levels thus correlate well with tumor presence in patients with gcts and elevated levels are considered highly specific for gct in postmenopausal or oophorectomized women . it may also be elevated in women with sertoli - leydig cell tumors of the ovary , but is not typically produced by other gonadal or extragonadal tumors . this is in sharp contrast to inhibin and estradiol levels , both of which may be elevated in a variety of other extraovarian disorders . this marker may thus eventually be used for both diagnosis and follow - up evaluations of patients with gcts . preclinical research is also ongoing to evaluate the clinical use of targeting the mis receptor for therapy in cancers expressing this receptor . follicle regulatory protein is secreted by the granulosa cells and is elevated in some patients with gcts . however , the clinical significance of this marker is still undetermined [ 20 , 27 ] . gcts have been reported in coexistence with a number of pathologies including mucinous cystadenoma [ 7 , 41 , 42 ] , cystic teratoma , ovarian fibroma , ovarian angiosarcoma , adenosarcoma , cystadenosarcoma , sclerosing peritonitis , gastric signet - ring cell carcinoma , and cervical lipoleiomyoma . in the indexed case 4 gct was found to coexist with colonic adenocarcinoma as a unique collision tumor which has been discussed previously in detail . the presence of such coexiting pathologies may contribute to increased confusion and be a deterrent to the accurate clinical and pathological recognition of this uncommon neoplasm . uterine pathologies that have been reported to occur with gct include glandular hyperplasia , atypical adenomatous hyperplasia , adenocarcinoma insitu , and invasive carcinoma . endometrial hyperplasia is a common finding alongside gct , occurring in 2550% , which may occur due to estrogen produced from the gct stimulating the endometrium . a simultaneous uterine carcinoma can be found in 510% of patients with gct   and are often well differentiated and in an early stage . perhaps due to the diagnosis at an earlier stage , patients with synchronous endometrial and ovarian cancers have a better prognosis than patients with a single malignancy that is typically detected once it has become more extensive . the rarity of these lesions prevents randomized control trials to determine the specific best consensus practice guidelines for the management of gct . the mainstay treatment for gct is the same as for epithelial ovarian cancer , that is , surgical excision [ 6 , 20 , 27 ] . diagnostic laparoscopy has been described for the identification of tumour origin , extent , and resectability ; however , currently , laparoscopic resection is not advocated for gcts . as the incidence of bilateral disease is quite low , for women with reproductive function less than 40 years old and of reproductive age , fertility sparing surgery of unilateral salpingooophrectomy with endometrial biopsy is recommended , while women under 40 without reproductive function and those over 40 require a total abdominal hysterectomy ( tah ) as well as a bilateral salpingooophrectomy ( bso ) [ 3 , 20 , 27 ] . in patients with more advanced disease , tah and bso with complete tumour debulking are suggested . improved survival with palliative debulking hepatectomy for an unusual case of a grade i , stage i granulosa cell tumor that recurred 21 years following initial surgery has also been reported . peritoneal exploration , washing cytology , peritoneal biopsy , and partial omentectomy have been suggested as part of the staging procedure in all gct patients . careful examination of the contralateral ovary and tube , intra - abdominal organs , and peritoneum with sampling of the pelvic and para - aortic lymph nodes are recommended . aside from being a treatment option , surgery is also necessary for staging and accurate tissue diagnosis [ 3 , 27 ] . these parameters are important to determine , as poor prognostic features include a tumour size greater than 1015  cm , a high mitotic index , tumour rupture , and lymphatic invasion [ 3 , 6 , 31 , 50 ] . three forms of adjuvant therapy have been suggested to use in combination with surgery : hormonal therapy , radiotherapy , and chemotherapy . hormonal therapy is believed to act directly by affecting the tumour and/or indirectly by suppressing gonadotropins or endogenous steroids . aromatase inhibitors such as anastrozole and letrozole inhibit the conversion of androstenedione to estrone , and estradiol and testosterone to estradiol , reducing aromatization of androgens by upto 90% thereby enhancing the treatment of gct . gonadotropin - releasing hormone ( gnrh ) analogs like leuprolide have been used to decrease stimulation of granulosa cells through inhibition of ovarian steroidogenesis in recurrent gct . however , the fact that not all gcts respond to hormonal therapy despite nearly all gcts containing progesterone receptors indicates that multiple factors play a role in the hormonal regulation of the tumor cell . radiotherapy may be used as an adjuvant therapy or in the instance of recurrence , and is associated with an improved survival [ 27 , 29 , 52 ] . additionally , the use of palliative radiotherapy as an alternative strategy with potential disease control has been useful in symptomatic patients with localized or metastatic disease unqualified for surgery [ 2 , 20 ] . the use of chemotherapy has yielded encouraging results , associated with a longer disease - free survival . the chemotherapeutic agent cisplatin has the highest reported activity in the ovary , and when combined with doxorubicin , cyclophosphamide , bleomycin , vinblastine , or etoposide , an overall response rate of 6083% has been reported . the current standard recommended chemotherapeutic regimen for advanced , recurrent , or metastatic gct is bleomycin , etoposide , and cisplatin ( bep ) [ 3 , 20 , 27 ] . targeted identification of targets for novel therapeutic agents is also predicted in the future with increased knowledge about the molecular biology of both the normal and neoplastic gscs [ 2 , 6 , 52 ] . the cut surface is primarily solid , with haemorrhagic regions and a gray / white or yellow colour depending on the lipid content . these tumours are often filled with serous fluid or clotted blood and may be mistaken for mucinous cystadenoma or cystadenocarcinoma . gcts are sex - cord stromal neoplasms that on microscopic examination contain sex - cord - derived epithelial elements admixed with mesenchymal elements with a variety of combinations and degrees of differentiation . fibroblasts , granulosa , and theca cells make up a gct . depending on 4 variables including age at diagnosis , histology , therapy , and prognosis , cells are usually arranged around a central cavity named a call - exner body that has a microfollicular growth pattern similar to primordial follicles and contains eosinophilic materials as well as nuclear debris . . a wide variety of growth patterns have been identified , and may be divided into two categories . the well - differentiated type includes microfollicular , macrofollicular , trabecular , insular , solid tubular , and gyriform architectural patterns . the moderately differentiated type includes a diffuse ,  sarcomatoid  growth pattern that is easily mistaken for a carcinoma or adenocarcinoma   as histological mimics . nuclear characteristics are a hallmark feature of agct , including a uniform , pale , and grooved these nuclear features may be used to differentiate agct with a diffuse pattern from poorly differentiated carcinoma , as carcinomatous nuclei are hyperchromatic and not grooved , and additionally do not demonstrate nuclear atypia and multiple mitotic figures to the same extent . immunohistochemical analysis can be used to confirm the diagnosis of gcts if the lesion 's morphology is non - predictive of histogenesis . a study by nofech - mozes et al . inhibin , calretinin , cd56 , and cd99 are part of the immunoprofile for both types of gct ; however , the lack of a single specific marker necessitates a panel of antibodies for the detection of these lesions . gct cells usually stain positive for inhibin , calretinin , cd99 , cd56 , vimentin , estrogen and progesterone receptors . other markers that can be positive leading to diagnostic confusion include cam5.2 , ae1/ae3 , cd10 , s100 , wt-1 , smooth muscle actin , and desmin . however , gct 's are usually negative for cytokeratin 7 and epithelial membrane antigen ( ema ) . the absence of staining with ema has diagnostic value in distinguishing gct from its multiplicity of histological look - alikes such as metastatic or primary carcinoma . gcts are unpredictable neoplasms that have the ability to extend locally or spread by lymphatics , especially to the para - aortic lymph nodes . distant metastatic sites of gct most commonly include the lung , liver , and brain . a quarter of gct patients will have recurrences , and the mean time to their detection is 510 years [ 3 , 27 ] . 1020% of patients may develop recurrences as late as twenty to forty years after the primary diagnosis [ 57 , 58 ] . one - third ( 33% ) of gcts recur in less than 5 years , half ( 50% ) between 59 years , and 17% ten or more years after the initial diagnosis [ 49 , 59 ] . splenic rupture from metastatic gct 29 years after the original curative resection has also been reported . as such , lifelong surveillance for these neoplasms is recommended . frequent sites of recurrence include the upper abdomen ( 5570% ) and the pelvis ( 3045% ) . this suggests that recurrences in early - stage patients may be attributed to preexisting diseased peritoneum during the initial surgery . in early - stage patients , risk factors for relapse include large tumour size , high mitotic index , and tumour rupture ; therefore , these features may indicate the need for postoperative adjuvant chemotherapy . additional postsurgical risk factors include advanced stage of presentation , lymphovascular space invasion , bilaterality , and ki67/p53 overexpression . higher stage disease is also related to aggressive tumour behaviour with recurrences .   the overall ten - year survival rates in patients with gct range between 60 to 90% . approximately 80% of females with advanced gct die due to the disease , which is partly related to the tendency for delayed recurrence . this unpredictability of the time interval for recurrent and/or metastatic disease indicates the requirement for a long - term clinical followup in all cases [ 3 , 27 ] . gct is best considered an unusual indolent neoplasm of low malignant potential with late recurrences that can arise in the ovaries and testicles in both the young and the old . the multifaceted clinical presentations coupled with the unpredictable biological behaviour with late relapses are diagnostic pitfalls necessitating a high degree of suspicion for accurate clinical and pathological diagnosis . surgery continues to be the primary cornerstone of initial treatment with chemotherapy and/or radiotherapy being reserved for advanced or recurrent disease states . lack of evidence - based predictive and prognostic factors continues to be a deterrent in accurately predicting the biological behaviour of individual gcts . however , long - term lifelong followup including physical / pelvic exam , abdominal / pelvic ct scan , and/or tumor markers as available is recommended in all patients with gcts as delayed tumor recurrences beyond 5 years are characteristic of this disease .