Cystic Granulosa Cell Tumor Research Articles

AMH producing purely cystic virilizing adult granulosa cell tumor in 17 years old girl: a case report and review of literatures

BackgroundAndrogen-producing granulosa cell tumor in adolescent girl is rare condition and clinical characteristics are not fully elucidated.Case presentationSeventeen years old girl complained of secondary amenorrhea was referred to our out-patient consultation. Markedly elevated serum testosterone, LH, and AMH levels were noted. Mild hirsutism and clitoromegaly were presented. Transabdominal ultrasonography and MRI revealed cystic mass occupied pelvic cavity probably originated from left ovary. Right ovary showed polycystic appearance. Laparoscopic left ovarian cystectomy was performed. After the surgery, her menstruation resumed along with normalized hormonal parameters, and clinical hyperandrogenism were improved. Since the scarcity of cellular lining of inner cyst wall, definitive pathological diagnosis was difficult. After the consultation with gynecological pathologist, the tumor was diagnosed as sex cord stromal tumor, highly suspicious for adult granulosa cell tumor. Residual left salpingo-oophorectomy was performed by additional laparoscopic surgery. Her serum testosterone and AMH levels were remained low with regular menstrual cycles and no evidence of recurrence.ConclusionsAndrogen-producing cystic granulosa cell tumor is rare gynecological disorders, which need both gynecologic oncological and endocrinological approach. Its clinical manifestations may bring some clues to the pathogenesis of ovulatory dysfunctions, such as polycystic ovary syndrome.

Cystic Granulosa Cell Tumors of the Ovary: An Analysis of 80 Cases of an Often Diagnostically Challenging Entity.

Granulosa cell tumors (GCTs) of both adult (AGCT) and juvenile (JGCT) types can rarely be completely or dominantly cystic, creating diagnostic difficulty because the cyst lining epithelium is often denuded. To describe clinical, gross, microscopic, immunohistochemical, and molecular features of cystic GCTs with an emphasis on their differential diagnosis. We report 80 cystic GCTs (24 AGCTs and 56 JGCTs) in patients from ages 3 to 83 years (average ages, 35 years for AGCT and 22 years for JGCT). Nineteen of 43 patients with known clinical information (3 AGCT and 16 JGCT) had androgenic manifestations. All tumors were greater than 8 cm (average, 17 cm) with minimal to absent gross solid component. Denudation of cells lining the cysts was prominent. Invagination of the epithelium into the cyst walls was a key diagnostic feature, was present as cords, trabeculae, solid nests, and small and large follicles, and was identified in most tumors (17 AGCTs and 45 JGCTs). Cytologic atypia was essentially absent in AGCTs, whereas 14 JGCTs showed moderate to severe atypia of bizarre type. A theca cell component was present in all tumors and was extensive in 54. A FOXL2 hotspot mutation was identified in 1 of 4 AGCTs tested. Despite extensive denudation, the finding of typical architectural patterns and cytologic features as well as, in some cases, androgenic manifestations helps differentiate cystic GCTs from follicle cysts, the most common and challenging differential diagnosis, as well as other cystic neoplasms that may enter the differential diagnosis. FOXL2 sequencing may show a false-negative result in cystic AGCT because of the limited number of cells present within the tumor sample.

Clinical Characterization of Patients with Ovarian Mass Combined with Dysplasia of Secondary Sexual Characteristics

Objective To investigate the clinical characteristics of preadolescent and adolescent female patients with ovarian mass combined with dysplasia of secondary sexual characteristics. Methods This study retrospectively analyzed 18 cases of ovarian mass combined with dysplasia of secondary sexual characteristics aged 0-19 years admitted to Peking Union Medical College Hospital from January 2012 to November 2019.By analyzing the clinical manifestations,surgical methods,postoperative pathology,therapies and prognosis of the cases,we summarized the diagnosis and treatment ideas. Results Among the 18 cases,7(7/18,38.9%)developed secondary sex signs before puberty,including 5 cases showing precocity(including 2 cases of juvenile granulosa cell tumor,1 case of gonadoblastoma,1 case of ovarian follicular cyst,and 1 case of 46,XY simple gonadal dysplasia combined with dysgerminoma)and 2 cases presenting masculine manifestations(1 case of steroid cell tumor and 1 case of sclerosing stromal tumor).The rest 11(11/18,61.1%)cases showed abnormal development of secondary sexual characteristics during puberty,including 8 cases with masculine manifestations or abnormal menstruation after menarche(7 cases with sex cord stromal cell tumor and 1 case with cystic granulosa cell tumor),2 cases with primary amenorrhea(1 case with androgen insensitivity syndrome combined with testicular sertoli cell tumor and 1 case with endometriosis cyst combined with reproductive tract malformation),and 1 case diagnosed as 46,XX gonadal dysplasia with serous cystadenoma and no secondary sexual development during puberty. Conclusions Sex hormone levels should be actively tested in the case of prepubertal secondary sexual characteristics appearing early,pubertal secondary sexual characteristics being abnormal(underdevelopment),and/or menstrual abnormalities.Imaging examination should be performed to exclude ovarian organic lesions,and chromosome karyotype analysis should be performed if necessary.The diagnosis of ovarian mass in preadolescent and adolescent females with related symptoms should first be alerted to cord stromal cell tumor.It is recommended to rule out the possibility of combined reproductive tract malformation in the adolescent patients with primary amenorrhea.Chromosome examination should be conducted to rule out the possibility of gonadal dysplasia in the adolescent patients with primary amenorrhea and/or no development of secondary sexual characteristics.

A RETROSPECTIVE SURVEY OF NEOPLASIA IN MANAGED GIRAFFES (GIRAFFA CAMELOPARDALIS).

Giraffes (Giraffa camelopardalis) are commonly managed in zoos and conservation programs worldwide, but the current understanding of the occurrence and progression of neoplastic disease in this species is limited by the scarcity of published reports. This study collated documented cases of neoplasia on the basis of gross and histologic evaluation of ante- and postmortem samples. In total, 30 giraffes from 22 institutions across the United States were included. Subspecies was not reported in all cases, but those identified included Masai (Giraffa camelopardalis tippelskirchi), Rothschild (Giraffa camelopardalis rothschildi), and reticulated subspecies (Giraffe camelopardalis reticulata). Thirteen animals died natural deaths, 15 were euthanized, and 2 were alive at the time of this article. A total of 38 tumors were reported and classified as 18 different diagnoses, including leiomyoma (7), adenoma (4), luteoma (4), lymphoma (4), pheochromocytoma (3), squamous cell carcinoma (3), adenocarcinoma (2), ameloblastic fibroma (1), carcinomatosis of undetermined cell lineage (1), cavernous hemangioma (1), cystic granulosa cell tumor (1), dysgerminoma (1), fibrosarcoma (1), leukemia (1), lipoma (1), pituitary nerve sheath tumor (1), rhabdomyosarcoma (1), and teratoma (1). Multiple concurrent neoplastic lesions were documented in six cases. Mesenchymal tumors (18) were the majority of neoplasms. The most prevalent location, regardless of tumor type, was the female reproductive tract (14). Twenty-four neoplastic lesions were incidental findings at necropsy, whereas eight neoplasms were considered to be the primary cause of death. The findings reported here identify multiple neoplastic lesions in giraffes and could provide insight to the future management of this species.

Abnormal Elevation of Anti-Mullerian Hormone and Androgen Levels Presenting as Granulosa Cell Tumor.

We report a rare subtype of adult cystic granulosa cell tumor (AGCT) characterized by elevated anti-Mullerian hormone and hyperandrogenism. A 35-year-old woman with primary infertility, hyperandrogenism, and irregular menses who was previously diagnosed with polycystic ovarian syndrome was diagnosed with AGCT based on histopathological examination and FOXL2 genetic test after laparoscopy. Due to fertility aspirations, she underwent controlled ovarian stimulation followed by embryo cryopreservation before salpingo-oophorectomy, and two embryos were frozen-thawed and transferred after surgery. A healthy female infant was delivered at 40 weeks’ gestation. Cystic granulosa cell tumors should be considered a differential diagnosis in patients with persistent ovarian cysts and hyperandrogenism. Younger patients with AGCT with fertility goals should consider active assisted reproduction measures to preserve fertility before treatment for AGCT.

Hyperreactio Luteinalis (Multiple Luteinized Follicle Cysts): A Report of 10 Cases.

Hyperreactio luteinalis is a rare entity arising in pregnancy and in the setting of gestational trophoblastic diseases (ie choriocarcinoma, molar pregnancy) that presents with, typically, bilateral ovarian enlargement due to numerous follicle cysts. While the phenomenon is benign and spontaneously regresses following delivery or treatment, a specimen may be seen in pathology when oophorectomy or cystectomy is performed to exclude malignancy or to manage acute complications such as torsion. Such resections may exhibit overlapping microscopic features with cystic granulosa cell tumors. We thus reviewed 10 cases of hyperreactio luteinalis in the setting of pregnancy, the largest pathologic cohort to date, to highlight notable features of this disorder. Patients ranged from 22 to 30 yr old. Most patients (n=6) presented at time of cesarean section with incidentally discovered ovarian masses. Three patients presented in the postpartum period, and 1 underwent surgery at 28 wk gestation due to the finding of a unilateral ovarian mass. The ovaries ranged from 8.5 to 29 cm and were multicystic and bilateral in 8 of the cases. Histologic examination demonstrated multiple, variably sized cystic follicles lined by a granulosa cell layer of varying thickness and theca cells with marked eosinophilic cytoplasm. Stromal edema was often prominent, with theca cells occasionally noted in nests, cords, and as single cells in foci of edema. Mitoses were generally seen more often in the granulosa cell layer (mean=2.6 per high power fields) compared with the theca cell layer (mean=1 per 10 high power fields). This series documents the key features of hyperreactio luteinalis that differentiate it from the other benign mass forming lesions encountered in pregnancy, most notably large solitary follicle cyst of pregnancy and puerperium, as well as cystic granulosa cell tumors, especially the juvenile variant, which may also present during pregnancy. Of particular use in differentiating them from juvenile granulosa cell tumor is the absence of pale or vacuolated cytoplasm and solid growth of granulosa cells in cases of hyperreactio luteinalis.

Lesions of the sheep reproductive system found in a slaughterhouse in the state of Bahia, Brazil

ABSTRACT: The present study aimed to evaluate and diagnose pathologies of the reproductive system of slaughtered sheep in slaughterhouses in the state of Bahia since there are few data on the subject as well as it is important to note the diagnosis of these diseases to apply appropriate control and prophylaxis measures. The research was carried out in slaughterhouses in the state of Bahia according to the “Serviço de Inspeção Federal” and “Serviço de Inspeção Estadual” for sheep slaughter. From July 2018 to February 2019, visits and monitoring of 1,072 slaughtered sheep were carried out. The animals came from 22 municipalities in the state of Bahia, aged from six to 18 months. During slaughter, the sheep reproductive systems were sectioned for evisceration and lesion collection. Additionally, epidemiological surveys related to origin, age, and race were obtained. For bacteriological examination, collections were performed with sterile scalpel slides and swabs in Stuart medium sterile tubes and refrigerated in a thermal box. For histopathological analysis, fragments were fixed in 10% formaldehyde and routinely processed for histology, stained with Hematoxylin and Eosin (HE). A study of the sheep’s main pathological changes in slaughterhouses in the state of Bahia was carried out, in which 1.072 genital systems were evaluated, and 211 alterations were identified. The most affected reproductive organs were the ovaries (65.3%), followed by the uterus (29.4%) and uterine tubes (5.3%). In the ovaries, the most frequent lesion was a follicular cyst (34.1%); in the fallopian tubes, the cysts represented 3.3% of the lesions, and in the womb, endometritis was observed in 9% of the animals. Other pathologies identified in the ovaries were: luteinized cyst (2.3%); cystic granulosa cell tumor (0.5%); benign lesion (0.5%); agenesis unilateral (0.5%), in addition to other changes of little clinical significance, such as corpus luteum cysts (11.8%) and paraovarian cysts (15.6%). In the fallopian tubes, hydrosalpinx was observed (1%), as well as adenoma (0.5%), agenesis (0.5%), and cysts (3.3%). Uterine lesions included hydrometra (2.3%); Cystic Endometrial Hyperplasia (CEH), abscess, and pyometra (1.4% each); adenomyosis, womb sera petechiae, and total segmental aplasia (1% each); two pregnant uterus, in early pregnancy, presented pyometra (0.9%), however, no change was observed in the fetuses; and uterine polyp (0.5%). Ectopic pregnancy with fetal maceration (0.5%) was observed; vaginitis occurred in 0.5% of the animals, and endometrial melanosis in 8.5%. The high incidence of follicular cysts and endometritis are characterized as diseases that reduce the reproductive efficiency in herds, causing infertility and economic losses in production.

Follicle Cysts of the Ovary: A Report of 30 Cases of a Common Benign Lesion Emphasizing its Unusual Clinical and Pathologic Aspects.

The common ovarian follicle cyst is typically straightforward from both clinical and pathologic perspectives, but may have a variety of unusual features from both aspects at various stages of life. Lack of familiarity with these may lead to diagnostic quandaries, the most common of which is distinguishing between a follicle cyst and cystic granulosa cell tumor of either adult or juvenile type. We reviewed 30 cases of follicle cysts, all sent in consultation, to highlight unusual aspects of a common lesion. Patients ranged from 3 d to 47 yr old. Clinical presentations included precocious puberty, pelvic pain, or an incidentally discovered pelvic mass, including those occurring in neonates and in 2 adults with pituitary adenomas, one of which was diagnosed 3 yr after presentation with the ovarian cyst. Size ranged from 0.5 cm (deflated) to 18.5 cm, with 7 exceeding 8 cm in greatest dimension. Twelve cases demonstrated small satellite cystic follicles in the wall of the dominant cyst. The granulosa cell layer varied in thickness and mitotic activity (which ranged from 1 to 36 per 10 HPF), but uniformly displayed round nuclei that lacked nuclear grooves. Luteinization of the granulosa cell layer, theca layer, or both was seen across all clinical scenarios, with unluteinized cysts being most common in precocious puberty patients. This series documents that although typically smaller, a subset of follicle cysts are the same size as cystic granulosa cell tumors and the 2 entities may be grossly indistinguishable. Helpful clues to the diagnosis of follicle cyst are the lack of nuclear grooves (vs. adult granulosa cell tumor) and lack of invagination of granulosa cells into the cyst wall (vs. both forms of granulosa cell tumor). Mitoses in the granulosa cells are of no aid in the differential with either form of granulosa cell tumor as follicle cysts may exhibit brisk mitotic activity. Our series highlights some of the unusual clinical aspects, one relatively well known-an association with isosexual precocity, but 2 not as widely known, those occurring in neonates and those due to a pituitary adenoma, the latter sometimes not being discovered until a few years after presentation with a follicle cyst.

Ovarian Granulosa Cell Tumors: Cystic Presentation of Six Cases

The World Health Organization classifies ovarian granulosa cell tumors as sex cord stromal tumors pure sex cord and both, adult granulosa and juvenile granulosa cell tumors are described. Usually, they appear as solid or cystic solid tumors and less commonly the tumors are entirely cystic, which results in scarce literature published on this presentation. Juvenile type presents histologically a lobulated appearance Call-Exner bodies are infrequent, luteinization is common, and it is usual to observe follicles containing eosinophilic or basophilic secretions. Adult type typically shows a proliferation of uniform cells with vesicular nuclei that show typical grooves. It is important to take into account the entirely cystic presentation to avoid wrong diagnoses such as ovarian follicular cyst. From the pathological point of view there are no overwhelming data that could clearly differentiate a cystic granulosa cell tumor from a follicular cyst. However, some authors have described macroscopic and microscopic findings that should be taken into account to perform the differential diagnostic between these entities. Six cystic granulosa cell tumor cases from the period 1973–2017 from our Pathology Department were studied, and they included three adult granulosa cell tumors and three juvenile granulosa cell tumors. We analyzed clinical presentation and histological features like patterns, nuclear atypia, epithelium-stroma relationship, mitotic index, and presence of necrosis in order to establish whether there is a correlation among these parameters and clinical behavior.

A Cystic Granulosa Cell Tumor of Ovary: An Incidental Finding in a 50 years Old Lady

A Cystic Granulosa Cell Tumor of Ovary: An Incidental Finding in a 50 years Old Lady

Unicystic granulosa cell tumor.

Granulosa cell tumors are the most frequent sex cord stromal tumor and account for approximately 5% of all primary ovarian tumors. About 5% occur before puberty and 40% occur in the menopausal age group; however, these tumors have an indolent biological behavior [1]. The clinical presentation in most cases is due to estradiol secretion but some may be nonfunctional or may present with virilizing symptoms. Unilocular and multilocular cystic granulosa cell tumors have been reported in the literature but are rare disease processes [2,3].

A Case of Juvenile Cystic Granulosa Cell Tumor of the Ovary

A Case of Juvenile Cystic Granulosa Cell Tumor of the Ovary

Granulosa cell tumors of unilocular cystic type

We reviewed the clinical, pathological and immunohistochemical features of six unilocular cystic granulosa cell tumors (UCGs) of the ovary. The mean age of the patients was 46 years and the presenting features included abdominal distension, pain, urinary frequency, dyspareunia and amenorrhea. The tumors were unilateral, thin walled and consisted of a single large cavity with a smooth internal lining. The mean tumor-diameter was 10 cm and in all instances the UCGs were of adult histological type. The granulosa cells in six tumors demonstrated immunoreactivity for vimentin, five neoplasms showed reactivity for both alpha-inhibin and progesterone-receptor protein and four showed reactivity for smooth muscle actin. All tumors were nonimmunoreactive with antibodies to epithelial membrane antigen, beta-inhibin, estrogen-receptor protein, testosterone and s100 antigen. Only one of the three patients tested preoperatively had an elevated serum inhibin concentration and this returned to normal six weeks post-operatively. We conclude that UCGs are distinguished by the infrequency of diagnostic serum tumor markers and clinically evident endocrine activity. Correct diagnosis is dependent on histological examination and the finding of a monotonous population of cells, a trabecular growth pattern, nuclear grooves, low mitotic activity and an immunohistochemical profile compatible with granulosa cell tumors.

Large cystic granulosa cell tumor: Case report

This is a case of a large granulosa cell tumor of the ovary. The patient was a 42-year-old, white woman, gravida 1, para 1. The family claimed that she had a "water tumor" in her abdomen. The patient was afraid of seeing a doctor. She died at home without any medical attention. During autopsy we removed approximately 20 gallons of turbid reddish brown fluid from the cystic tumor. The tumor's cystic wall and the fluid weighed approximately 77 kg (170 pounds). No metastasis was found in any other organs.

Ovarian Cysts in Newborn Infants-Reply

In Reply —Eggermont and colleagues propose the use of ultrasound-guided percutaneous aspiration of ovarian cysts in the neonatal period. The striking variety of location, size, and components prevents a universal therapeutic approach to the treatment of ovarian cysts. A conservative approach with sonographic reevaluation in uncomplicated cysts less than 5 cm in diameter seems indicated in the neonatal period in view of the natural tendency of these cysts to resolve spontaneously. 1 Misdiagnosis, however, is possible; cystadenomas and cystic granulosa cell tumors may mimic simple ovarian cysts. 2 Large cysts (10 to 12 cm) are more likely to undergo torsion or to rupture; hence, immediate operative intervention is preferred. Puncture of large ovarian follicular cysts guided by laparoscopy has been successfully performed. 3 Reappearance of the cyst, however, may ensue. Transabdominal puncture of ovarian cysts shares some of the drawbacks of conservative management (ie, misdiagnosis, recurrence). For large cysts, percutaneous aspiration is

Bilateral granulosa cell tumors of the ovaries in infancy

A case of bilateral cystic granulosa cell tumors of the ovaries in an 11-wk-old infant is described. The patient presented with a large abdominal mass, but without sexual precocity. Treatment consisted of bilateral salpingo-oophorectomy without subsequent chemotherapy or radiation, and 2 yr follow-up has shown no evidence of recurrence of this tumor.