Ovarian Steroid Cell Tumor Research Articles

Ovarian steroid cell tumor with biallelic adenomatous polyposis coli inactivation in a patient with familial adenomatous polyposis

Familial adenomatous polyposis (FAP) is an autosomal dominant cancer predisposition syndrome that accounts for approximately 0.5-1% of all colorectal cancer cases. It is caused by germline mutations in the gene encoding the adenomatous polyposis coli (APC) tumor suppressor. Somatic APC inactivation due to mutation or loss of heterozygosity (LOH) promotes the development of adenomatous polyps by stabilizing the transcriptional coactivator β-catenin. Although colorectal cancer is by far the most common malignancy seen in FAP patients, the widespread use of prophylactic colectomy in these patients has increased the clinical importance of extracolonic tumors that are part of the neoplastic spectrum in FAP. Many of these tumors exhibit LOH or somatic APC mutation, strongly supporting a causative role of APC inactivation in their pathogenesis. Here we describe a 47-year-old female FAP patient with clinical manifestations of virilization who was found to have an ovarian steroid cell tumor, a rare neoplasm not known to be associated with FAP. Immunohistochemical analysis of the ovarian tumor demonstrated strong nuclear β-catenin staining consistent with somatic APC inactivation, and molecular analysis confirmed biallelic APC inactivation in the tumor. Our findings provide the first evidence that ovarian steroid cell tumors may be an extracolonic manifestation of FAP and implicate β-catenin activation as an oncogenic mechanism in ovarian steroid cell tumorigenesis.

Ovarian Steroid Cell Tumour: Correlation of Histopathology with Clinicopathologic Features

Ovarian steroid cell tumours (not otherwise specified) are rare neoplasms of the ovary and are classified under lipid cell tumours. Their diagnosis can be considered as one of exclusion. Histopathologically, the tumour should carefully be evaluated for microscopic features of malignancy, but it is essential for the clinician and the pathologist to remember that in these tumours, pathologically benign histomorphology does not exclude the possibility of clinically malignant behaviour. Our case study focuses on the comparative findings in a postmenopausal female diagnosed with an ovarian steroid tumour (not otherwise specified). A careful correlation between clinical and surgical evaluation and microscopic analysis is necessary, as is a regular followup.

Ovarian Steroid Cell Tumor, NOS Presenting with Massive Ascites and Elevated CA-125

Steroid cell tumors, not otherwise specified, are rare and usually benign. Ovarian sex cord stromal tumors of the ovary presenting with virulization in middle age women (average age: 43) are reported. Rarely, these tumors occur in postmenopausal women and, even more seldom, show a malignant clinical behavior. Although excess androgen secretion with virtualization is usually the primary presenting symptoms, rarely patients with this tumor present primarily with ascites. An unusual case was reported of a postmenopausal woman with a malignant steroid cell tumor, NOS, who was diagnosed with sudden onset of massive ascites as the primary clinical presentation. Aspects of clinical and pathologic findings as well as management pathways are introduced.

Refractory Hypertension and Isosexual Pseudoprecocious Puberty Associated with Renin-Secreting Ovarian Steroid Cell Tumor in a Girl

Steroid cell tumor, not otherwise specified (NOS), are rare ovarian tumor, in addition, it is more rare in children. The majority of these tumors produce several steroid hormones, particularly testosterone. Estrogen also secreted by steroid cell tumor, NOS, but it is uncommon. Furthermore, hypertension is an infrequent sign in steroid cell tumor, NOS. An 8.5-yr-old girl with hypertension and frequent vaginal spotting visited at our clinic. On laboratory evaluation, secondary hypertension due to an elevated plasma renin level and isosexual pseudoprecocious puberty was diagnosed. Right solid ovarian mass was detected in radiologic tests. She underwent a right ooporectomy and it revealed renin and progesterone receptor positive steroid cell tumor, NOS. After operation, her blood pressure returned to normal level and vaginal bleeding disappeared. Even though this case is very rare, when hypertension coincides with virilization or feminization, a renin-secreting ovarian steroid cell tumor, NOS, should be considered.

Clinical, ultrasonographic, computed tomography and histopathological manifestations of ovarian steroid cell tumour, not otherwise specified: our experience of a rare case with female virilisation and review of the literature

Introduction. Ovarian steroid cell tumours, not otherwise specified (NOS) are rare sex cord-stromal tumours of the ovary. These tumours should be considered a cause of isosexual precocious puberty in children and virilisation in adults.Case. We report a case of 40-year-old woman with mental handicap who presented with 3 years of amenorrhea and progressive virilisation. Pelvic ultrasonography identified a 6.19 × 6.15 cm well-defined echogenic-multilobular mass arising from the left ovary. Fluid in the cul-de-sac was noted. Colour Doppler examination with endovaginal ultrasonography showed high vascularity of the tumour with low resistance to flow. A computed tomography (CT) scan of the upper and lower abdomen showed a lobular mass with diaphragms in the left adnexal structure and fluid in the cul-de-sac; no adrenal gland enlargement or additional tumour was detected. Laboratory analysis revealed increased levels of serum total testosterone. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed. Histological examination showed a benign steroid cell tumour, NOS without evidence of necrosis, haemorrhage or invasion. The immunohistochemical study showed that the tumour cells were positive for inhibin, CD 99, Melan A and vimentin and negative to CK AE1, CK AE3, progesterone and estrogen receptors.Conclusion. Careful medical history, physical examination, laboratory serum values and imaging studies are helpful in making the pre-operative diagnosis. Steroid cell tumours, NOS are usually benign, unilateral and characterised by the composition of two similar-appearing polygonal cell types. They differ from Leydig cell tumours in the lack of crystals of Reinke in their cytoplasm.

Tumor de celulas esteroideas: reporte de un caso y revisión de la literatura

Introducción: el tumor de células esteroideas sin otra especificación es una neoplasia poco frecuente de los cordones sexuales del ovario, caracterizada por presentarse en cualquier edad y producir diferentes manifestacionesendocrinasporel influjohormonal de las pacientes que lo padecen. Su potencial de malignidad es de 50%.Objetivo: informar el caso de una paciente de 50 años a la cual se le diagnosticó un tumor de células esteroideas sin otra especificación de comportamiento maligno por medio de un estudio histológico y de inmunohistoquímica. Asimismo, se pretende revisar la literatura disponible respecto a la presentación clínica, la genética, el diagnóstico y el tratamiento de este tipo de neoplasia.Metodología: se realizó una búsqueda electrónica en las bases de datos MEDLINE, Embase, HINARI, Ovid, SciELO y BIREME, a partir de la cual se incluyeron artículos de investigaciones originales y artículos de revisión publicados entre 1987 y 2008.Resultados: se encontraron 74 reportes de casos, para los cuales las manifestaciones clínicas eran variables e inespecíficas dadas según la producción de hormonas tales como la testosterona, los estrógenos o los progestágenos. Las pacientes también podían presentar dolor abdominal, ascitis severa, y síndrome de Cushing. De otro lado, el diagnóstico diferencial incluyó otros tumores esteroideos como: tecomas, luteinizantes, luteomas del embarazo, carcinoma primario de células claras, metastásico de células renales, tumores de células de Leydig y sus subtipos hiliares y no hiliares. Ante esto, el tratamiento requerido debió ser quirúrgico con tratamiento hormonal adyuvante con agonistas de receptores de la hormona liberadora de gonadotropina (GnRH).Conclusión: el tumor de células esteroideas es infrecuente y está asociado con síndromes endocrinos. El manejo no se ha estandarizado y debe individualizarse según la histología tumoral, el estadio quirúrgico y el deseo de fertilidad de la mujer. Asimismo, se requieren estudios para avanzar en el conocimiento de estos tumores.

Virilisation due to an ovarian steroid cell tumour in a child with Turner syndrome

Virilisation due to an ovarian steroid cell tumour in a child with Turner syndrome

Hirsutism with virilization in a postmenopausal woman due to a rare ovarian steroid cell tumor

Hirsutism with virilization in a postmenopausal woman due to a rare ovarian steroid cell tumor

P1012 An ovarian steroid cell tumor, not otherwise specified with pregnancy causing fetal 46,XX disorder of sex development

International Journal of Gynecology & ObstetricsVolume 107, Issue S2 p. S697-S697 Poster presentations P1012 An ovarian steroid cell tumor, not otherwise specified with pregnancy causing fetal 46,XX disorder of sex development F. Akimasa, F. AkimasaSearch for more papers by this authorH. Yasuyuki, H. YasuyukiSearch for more papers by this authorO. Masanobu, O. MasanobuSearch for more papers by this authorK. Norio, K. NorioSearch for more papers by this authorU. Koutarou, U. KoutarouSearch for more papers by this authorS. Kazuo, S. KazuoSearch for more papers by this author F. Akimasa, F. AkimasaSearch for more papers by this authorH. Yasuyuki, H. YasuyukiSearch for more papers by this authorO. Masanobu, O. MasanobuSearch for more papers by this authorK. Norio, K. NorioSearch for more papers by this authorU. Koutarou, U. KoutarouSearch for more papers by this authorS. Kazuo, S. KazuoSearch for more papers by this author First published: 20 November 2009 https://doi.org/10.1016/S0020-7292(09)62498-XAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article. Volume107, IssueS2Abstracts of XIX FIGO World Congress of Gynecology and ObstetricsOctober 2009Pages S697-S697 RelatedInformation

Ovarian steroid cell tumor: clinicopathologic study of 6 cases

类同醇细胞瘤是卵巢性索-间质肿瘤中少见的类型,仅占卵巢肿瘤的0.1%.此瘤好发于育龄妇女,多为卵巢实性肿物,术中常需进行冷冻切片病理诊断,该肿瘤冷冻与石蜡切片的正确诊断对临床选择恰当的手术方式有重要意义.我们对6例类固醇细胞瘤(其中1例合并子宫内膜腺癌)进行回顾性分析,探讨该组肿瘤的临床病理特征,为其诊断提供参考。

Virilizing ovarian steroid cell tumor in a 40 year old South Indian female: a case report

Abstract Virilism is the masculinization and enhancement of male secondary sexual characteristics in females. The etiology is usually of adrenal or ovarian origin. Here we report a case of virilizing Leydig cell type, steroid cell tumor of the left ovary, in a 40 year old female who presented with clinical signs and symptoms of virilization: deepening of voice, hirsutism (Ferriman-Gallwey score 26), clitoromegaly, and androgenic alopecia. On further evaluation, laboratory investigations revealed hyperandrogenism in the male range. Basal testosterone values were elevated. Folicle Stimulating Hormone and Luteinising Hormone levels were within normal limits. Dexamethasone suppression test did not alter cortisol or testosterone levels. An ovarian mass was confirmed radiologically. Following a total abdominal hysterectomy with bilateral salpingoophorectomy, histopathological studies confirmed a left sided steroid-cell ovarian tumor, Leydig cell type (stage T1N0M0), which proved to the etiology of virilization in this patient. Post-operatively her serum testosterone levels declined with near-complete reversal of symptoms over time.

Cushing’s syndrome caused by an ACTH-producing ovarian steroid cell tumor, NOS, in a prepubertal girl

Ectopic ACTH syndrome is a very rare cause of pediatric Cushing's syndrome. And if present, bronchial or thymic carcinoids predominate as causes. We hereby demonstrate a first case report of ACTH-producing ovarian steroid cell tumor, NOS, causing ectopic ACTH syndrome in a prepubertal girl.

Ovarian stromal luteoma in a patient with endometrial carcinoma

A 65-year-old patient with endometrial adenocarcinoma underwent hysterectomy. Pathological examination of the right ovary revealed a coexisting stromal luteoma. The possible relationship between the two neoplasms as well as the intriguing pathogenesis and controversial nomenclature of ovarian steroid cell tumors are discussed. The incidental discovery of a stromal luteoma in a total hysterectomy specimen removed for endometrial carcinoma raises interesting questions about the perceived innocuous nature of these rare lipid cell tumors.

A steroid-cell tumor of the ovary resulting in massive androgen excess early in the gonadol steroidogenic pathway

We describe the case of a 30-year-old woman with an ovarian steroid-cell tumor secreting markedly elevated levels of testosterone (28.3 nmol/l), dehydroepiandrosterone sulfate (19.7 μmol/l), androstenedione (>34.7 nmol/l) and 17-hydroxyprogesterone (100.5 nmol/l) into plasma. We could find no report within the literature of androgens at the levels described in our patient. We review the literature and discuss the possible endocrine etiology of the excess androgens observed in this case. We also discuss the differential diagnosis, investigation and interpretation of androgens of adrenal and ovarian origin.

A steroid-cell tumor of the ovary resulting in massive androgen excess early in the gonadol steroidogenic pathway

We describe the case of a 35-year-old woman with an ovarian steroid-cell tumor secreting markedly elevated levels of testosterone (28.3 nmol/l), dehydroepiandrosterone sulfate (19.7 μmol/l), androstenedione (>34.7 nmol/l) and 17-hydroxyprogesterone (100.5 nmol/l) into plasma. We could find no report within the literature of androgens at the levels described in this patient. Herein we review the literature and discuss the possible endocrine etiology of the excess androgens observed in this case. We also discuss the differential diagnosis, investigation and interpretation of androgens of adrenal and ovarian origin.

Ovarian steroid cell tumor, not otherwise specified: a clinicopathologic study

To study the clinicopathologic features, diagnostic criteria, differential diagnosis and treatment options of ovarian steroid cell tumor, not otherwise specified (NOS). Light microscopy and immunohistochemical study was carried out in 8 cases of ovarian steroid cell tumor, NOS. The literature was reviewed. The 7 cases of benign ovarian steroid cell tumor, NOS were composed mainly of polygonal cells with granular eosinophilic cytoplasm and larger cells with vacuolated cytoplasm. They resembled the architecture of normal adrenal gland, with formation of cell nests and trabeculae. The single case of malignant ovarian steroid cell tumor had evidence of significant cellular pleomorphism, haemorrhage and coagulative tumor necrosis. The mitotic count measured about 7 per 10 high-power fields. Immunohistochemical study showed that the tumor cells expressed calretinin and alpha-inhibin. Differential diagnosis included oxyphilic granulosa cell tumor, thecoma, Sertoli cell tumor and clear cell carcinoma. The treatment options of benign ovarian steroid cell tumor, NOS was local excision or ipsilateral salpingo-oophorectomy, while the malignant counterpart should be treated with a combination of surgery and chemotherapy, including administration of GnRH agonist. Ovarian steroid cell tumor, NOS, is the most common type of ovarian steroid cell tumors. Most of which are associated with a benign clinical outcome. Immunohistochemistry is an important adjunct for diagnosis. The treatment options of ovarian steroid cell tumor, NOS depend on its malignant potential.

An ovarian steroid cell tumor with virilization and hypothyroidism: a case report

Steroid cell tumors are rare ovarian sex-cord stromal tumors with malignant potential. The majority of these tumors produce steroids with testosterone being the most common. The diagnosis of these rare tumors can be problematic especially in the case of a small nonpalpable ovarian tumor. A 41-year-old woman presented with the gradual onset of defeminization, mild hypothyroidism, and virilization. A physical examination revealed a relatively healthy woman with borderline hirsutism, clitoromegaly and adnexal fullness without a palpable tumor. Elevated serum levels of TSH and testosterone were found preoperatively. Five weeks after an oophorectomy, serum levels of TSH and testosterone returned to normal and menstruation returned. Nonpalpable virilizing ovarian steroid cell tumors may be difficult to diagnose. A careful study of the patient’s history, the development of defeminization followed by virilization, and a “characteristic” ultrasonogram, can be helpful for diagnosis. Hormonal studies including thyroid function should also be considered in an ovarian steroid cell tumor.

An Ovarian Steroid Cell Tumor Causing Virilization and Massive Ascites

Steroid cell tumors, not otherwise specified (NOS), are rare ovarian sex cord-stromal tumors with malignant potential. The majority of these tumors produce several steroids, particularly testosterone. Various virilizing symptoms such as hirsutism, temporal balding, and amenorrhea are common in these patients; however massive ascites is an infrequent symptom. A 52-year-old woman with the sudden onset of virilization and massive ascites presented for treatment at Severance Hospital. After clinical evaluation, the patient underwent an exploratory laparotomy and a complete surgical staging procedure. She recovered from the surgery uneventfully and was discharged from the hospital five days after surgery. We present here an unusual case of an ovarian steroid cell tumor, NOS, and a brief review of the literature regarding these types of tumors.

Vaginal Bleeding and Galactorrhea in a Child with Ovarian Steroid Cell Tumor

Vaginal Bleeding and Galactorrhea in a Child with Ovarian Steroid Cell Tumor

Steroid cell tumors, not otherwise specified (NOS), in an accessory ovary: a case report and literature review

Background Accessory ovaries are uncommon congenital abnormalities of the female reproductive tract. Steroid cell tumors at accessory ovaries are proportionately even rare. Case This 30-year-old woman had an accessory ovarian tumor attached to the infundibulum of the right fallopian tube which was solid and gross pathologically and microscopically showed the appearances of steroid cell tumor, NOS, a benign entity. Conclusion We report the first case of an accessory ovarian steroid cell tumor, NOS, which attached to the infundibulum of fallopian tube.