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Objective: identification and description of clinical and morphological characteristics of rare forms of stromal cell tumors of the ovaries.Materials and methods. Retrospectively processed over 10 thousand case histories of patients with various ovarian tumors who were examined and treated at the N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia and the National Center for Oncology and Hematology of the Ministry of Health of Kyrgyz Republic. In 473 patients were diagnosed stromal cell tumors, among them 3 cases of rare forms of tumors of the stroma of the sex cord were identified: stromal tumor from Sertoli cells, malignant luteoma of the ovary, lipid‑cell tumor from Leydig cells. All patients underwent a planned morphological study of surgical material and immune-histochemical study.Results. The detailed data of clinical observations of rare types of tumors, which was presented in the literature by isolated cases, are presented, the description of which in the literature is presented by isolated cases. The possible effect of clinical and morphological characteristics on the survival of patients with rare forms of stromal cell tumors of the ovaries were analyzed.Conclusions. The necrobiotic changes in the tumor, the degree of malignancy, and the stage of the disease are potential predictors of poor prognosis in patients with rare forms stromal cell tumors of the ovaries which require confirmation in a larger sample of patients.

Ovarian adrenal rest tumors (OART) are tumors that develop in females with congenital adrenal hyperplasia (CAH). In contrast to their counterpart in testicles, they are exceptional and few cases have been reported in the literature. In this report, we present clinicopathological findings of a female patient with CAH due to 21-hydroxylase deficiency who was incidentally diagnosed with OART with a review of the literature. The 14-year-old patient, who was raised as a boy, developed a virilizing syndrome with high testosterone levels that were attributed to non adherence to her replacement corticosteroid therapy. She consulted for sex reassignment surgery. Pelvic ultrasound was normal. She underwent hysterectomy and bilateral adnexectomy. No abnormalities were noticed during the operation. Grossly, both ovaries were variegated with well circumscribed and lobulated, brownish-yellow nodules. Histologically, the nodules were composed of nests of large polygonal cells with centrally located nuclei and prominent nucleoli. There was mild atypia and no crystals of Reinke. Thus, the findings of the histopathological examination were consistent with bilateral OART. Histological differential diagnosis of OART can be challenging particularly with leydig cell tumor, stromal luteoma and steroid cell tumors, not otherwise specified. OART must be considered in women with CAH and persistent virilizing symptoms despite negative imaging results.

A luteoma is a benign ovarian mass that can have malignant characteristics on sonography. Pelvic sonography is used to help distinguish benign from malignant masses. The workup of adnexal masses during pregnancy may involve the use of both transabdominal and transvaginal sonography as well as Doppler imaging, magnetic resonance imaging, and serum tumor markers to identify the risk of malignancy. This study presents a case of a benign ovarian luteoma of pregnancy detected during sonography. The mass was removed during the first trimester due to malignant sonographic features. Counseling and treatment regarding ovarian masses should consist of a multidisciplinary team made up of physicians in maternal-fetal medicine, radiology, neonatology, and sonographers.

Los tumores de células esteroides de ovario se clasifican en luteoma estromal, tumor de células de Leydig y tumor de células esteroideas sin otra especificación, según su origen embrionario. El tumor ovárico de células esteroideas sin otra especificación es un tumor benigno raro, pero con potencial maligno; representa menos del 0,1% de todos los tumores de ovario. Deben ser considerados como causa de virilización en mujeres adultas por la producción de testosterona. Solo un feto femenino corre riesgo de virilización. Al igual que otros tumores del estroma ovárico, los tumores deben ser tratados quirúrgicamente. La cirugía está indicada en casos de agrandamiento ovárico unilateral sólido, debido a un 50% de probabilidad de malignidad. En el embarazo, los tumores ováricos de células esteroideas sin otra especificación son excepcionalmente raros y deben ser diferenciados del luteoma del embarazo y otras neoplasias malignas del ovario. Con mayor frecuencia pueden complicarse con rotura y/o torsión. Se presenta un caso de tumor ovárico de células esteroideas sin otra especificación durante el embarazo.

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.

Background This is the first report about a vaginal leiomyoma concomitant with an ovarian luteoma in a bitch. Case presentation A 11-year-old intact female Labrador retriever was referred because of anuria, constipation and protrusion of a vaginal mass through the vulvar commissure. The bitch had high serum progesterone concentration (4.94 ng/ml). Because of the possibility of progesterone responsiveness causing further increase of the vaginal mass and since the bitch was a poor surgical candidate a 10 mg/kg aglepristone treatment was started SC on referral day 1. A computerized tomography showed a 12.7 × 6.5 × 8.3 cm mass causing urethral and rectal compression, ureteral dilation and hydronephrosis. A vaginal leiomyoma was diagnosed on histology. As serum progesterone concentration kept increasing despite aglepristone treatment, a 0.02 ng/mL twice daily IM alfaprostol treatment was started on day 18. As neither treatment showed remission of clinical signs or luteolysis, ovariohysterectomy was performed on referral day 35. Multiple corpora lutea were found on both ovaries. On histology a luteoma was diagnosed on the left ovary. P4 levels were undetectable 7 days after surgery. Recovery was uneventful and 12 weeks after surgery tomography showed a reduction of 86.7% of the vaginal mass. The bitch has been in good health and able to urinate without any complication ever since. Conclusions This case demonstrates the importance of identifying progesterone related conditions as well as the importance of judiciously using a combined medical and surgical approach.

Sclerosing stromal tumors of the ovary are very rare. We report the case of a 29-year-old pregnant woman who presented with a history of preterm labor and gestational hypertension. A huge mass measuring 30 cm in greatest dimension was recognized on the right ovary during cesarean section. Signet ring cell infiltration was seen during intraoperative frozen section consultation. She underwent a right salpingo-oophorectomy, and the permanent diagnosis was sclerosing stromal tumor infiltrated by signet ring cells. Pregnancy-related changes were seen in tumor morphology. Signet ring cells were immunoreactive with antibodies MUC5ac, MUC2, and EMA. Thus, stromal cells were immunoreactive with antibodies inhibin and calretinin. Endoscopic biopsy of the stomach showed a signet ring cell carcinoma, from which the metastasis had originated. This is the first reported case in the literature, and is notable for its challenging differential diagnosis, which included a pregnancy luteoma and primary signet ring stromal tumor of the ovary.

A white, lobular mass was found in the right ovary of a pregnant Risso's dolphin (Grampus griseus) at necropsy. The mass was unilateral and occupied most of the pre-existing ovarian tissue. Histologically, the mass was composed of diffuse sheets of polyhedral cells with abundant eosinophilic cytoplasm and oval nuclei, separated by fibrous connective tissue. Only a few ovarian follicles were observed at the periphery of the mass. Immunohistochemically, the large eosinophilic cells were positive for vimentin and negative for pan-cytokeratins. Based on the histopathological features, the present case was diagnosed as luteoma. In human medicine, luteoma of pregnancy, a tumor-like proliferative lesion occurring in pregnant women, is well described. In veterinary medicine, luteoma associated with pregnancy has never been described. The present study would provide useful information for understanding the characteristics of luteoma in animals.

Introduction: Steroid cell tumors (SCTs) constitute less than 0.1% of all ovarian tumors. They are divided into 3 categories according to cell of origin: Stromal Luteoma arising from stromal cells of the ovary, Leydig cell tumor arising from Leydig cells, and SCT not otherwise specified (NOS) when the origin of the tumor is not defined. Case Presentation: Herein is presented a case of SCT diagnosed one month after a caesarian section delivery of a female fetus with ambiguous genitalia. The patient was admitted to the emergency department with the findings of acute abdomen, and surgery was performed under emergency conditions. The patient had virilization and hoarsening of the voice before surgery. Intraoperatively, a 21-cm ovarian mass was detected and resected with unilateral salpingo-oophorectomy. Conclusion: A histopathological examination of the tumor showed a tumor with cystic degeneration, necrosis, hemorrhage, and tumoral embolism. The pathological examination revealed ovarian SCT. Virilization was resolved immediately after the surgery. In women with virilization who give birth to a fetus with ambiguous genitalia, SCTs should be kept in mind.

A rare case of an androgen-producing stromal luteoma of the ovary in a postmenopausal woman, diagnosed by means of selective venous blood sampling

Background: Steroid-cell tumors of the ovary are very rare, especially during pregnancy, and they must be distinguished from luteoma of pregnancy. Case: An 18-year-old female, gravida 3, para 1-0-1-1, at 38 weeks' of gestation, had an adnexal mass that was discovered incidentally during a Caesarean section. The tumor was excised and her male infant was normal. Results: Histologic workup revealed the tumor to be a steroid-cell tumor, which is exceedingly rare in pregnancy. Conclusions: Ovarian steroid-cell tumors, which are malignant one-third of the time, are difficult to distinguish from luteoma of pregnancy. (J GYNECOL SURG 32:351)

The extensive use of sonography for antenatal screening has led to an increased frequency of incidental adnexal mass diagnosis during pregnancy. Therefore, it is of utmost importance that the physician is familiar with the diagnosis and management of this entity. In this chapter, the methods of diagnosis of an adnexal mass in pregnancy and the approaches available for management are reviewed.

Ovarian sex cord-stromal tumors are infrequent and represent approximately 7% of all primary ovarian tumors. This histopathologic ovarian tumor group differs considerably from the more prevalent epithelial ovarian tumors. Although sex cord-stromal tumors present in a broad age group, the majority tend to present as a low-grade disease that usually follows a nonaggressive clinical course in younger patients. Furthermore, because the constituent cells of these tumors are engaged in ovarian steroid hormone production (e.g., androgens, estrogens, and corticoids), sex cord-stromal tumors are commonly associated with various hormone-mediated syndromes and exhibit a wide spectrum of clinical features ranging from hyperandrogenic virilizing states to hyperestrogenic manifestations. The World Health Organization sex cord-stromal tumor classification has recently been revised, and currently these tumors have been regrouped into the following clinicopathologic entities: pure stromal tumors, pure sex cord tumors, and mixed sex cord-stromal tumors. Moreover, some entities considered in the former classification (e.g., stromal luteoma, stromal tumor with minor sex cord elements, and gynandroblastoma) are no longer considered separate tumors in the current classification. Herein, we discuss and revise the ultrasonography, computed tomography, and magnetic resonance imaging characteristics of the different histopathologic types and clinicopathologic features of sex cord-stromal tumors to allow radiologists to narrow the differential diagnosis when facing ovarian tumors.

Objective To describe a pregnancy that was complicated by the virilization of the mother and two 46XX infants. Methods We outline the clinical presentation and diagnosis of the virilization of a mother and her twins, reviewing pertinent literature. Results We report the case of a 40-year-old Caucasian female who conceived a trichorionic triplet pregnancy through in vitro fertilization (IVF) but underwent cytoreduction at 13 weeks of gestation, leaving a diamniotic dichorionic twin pregnancy. At 16 weeks of gestation the mother experienced increasing acne, facial hair, and deepening of her voice. Due to preeclampsia, the twins were delivered via caesarean section at 33 weeks of gestation. The infants had male-appearing external genitalia (Prader score IV-V) but no palpable gonads. Congenital adrenal hyperplasia was ruled out for both twins and they were both found to have a uterus and a 46XX karyotype. Maternal testosterone level was elevated at birth (1,981 ng/dL), but the infants had normal levels. Maternal testosterone levels returned to normal after delivery, consistent with a luteoma of pregnancy, although imaging was negative for a mass. Conclusion This is the second reported case of complete virilization associated with a luteoma of pregnancy. Whether or not IVF and related procedures increase the risk for a luteoma and whether or not fetal reduction procedures disrupt placental aromatases and increase the risk of virilization in the face of elevated androgen levels are questions that require further research. (Endocr Pract. 2014;20:e18-e23)

The objective of this study was to investigate the incidence of ovarian tumours in camels and to identify their common types. A total of 500 non-pregnant dromedary camels’ ovaries aged between 6–15 years old were collected from Giza abattoirs during the period of January 2016 to January 2018 and were examined for the presence of any neoplasm. Tissue specimens were taken from all of the collected ovaries for histopathological examination. A total of 34 (6.8%) camels were found with neoplastic lesions included; papillary cystadenoma (0.6%), fibroadenoma (0.2%), granulosa cell tumour (1.2%), luteoma (0.8%), thecoma and luteinised thecoma (0.4%), fibrothecoma (0.4%), teratoma (2%), fibroma (1.6%), cavernous haemangioma (0.4%) and mixed tumours (0.6%). In conclusion, teratoma was the most common type of ovarian tumour followed by granulosa cell tumour, luteoma, fibroma, cavernous haemangioma, fibrothecoma, thecoma and luteinised thecoma, while fibroadenoma was the least observed neoplasm.

In the present study, a rare case of ovarian luteoma is described in a 5-year-old German Shepherd dog. Histopathologically, the tissue sections composed of multiple lobules of polyhedral cells with abundant, finely vacuolated cytoplasm, centrally located nuclei surrounded by fine well- vascularized connective tissue. Neoplastic cells showed strong cytoplasmic positivity for inhibin-α and vimentin and moderate for calretinin. Based on histopathological and im- munohistochemical findings, the tumor was diagnosed as ovarian luteoma.

We report the case of a 61-year-old Filipino woman with postmenopausal virilization presenting with androgenic alopecia, progressive hirsutism and deepening voice over 5 years due to elevated testosterone levels. Ultrasound and computed tomography imaging suggested a left adnexal mass. Histology obtained following total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAHBSO) demonstrated stromal luteoma of the left ovary. On immunohistochemistry, the tumor was positive for inhibin. Postoperatively, there was note of rapid normalization of testosterone level.

Androgen excess (hyperandrogenism) is a common endocrine disorder affecting women of reproductive age. The potential causes of androgen excess in women include polycystic ovary syndrome, congenital adrenal hyperplasia (CAH), adrenal tumors, and racial disparity among many others. During pregnancy, luteoma, placental aromatase deficiency, and fetal CAH are additional causes of gestational hyperandrogenism. The present report reviews the various phenotypes of hyperandrogenism during pregnancy and its origin, pathophysiology, and the effect of hyperandrogenism on the fetal developmental trajectory and offspring consequences.

Luteoma of pregnancy is a rare, benign condition characterized by a tumor-like mass of the ovary that emerges during pregnancy and regresses spontaneously after delivery. It is usually asymptomatic and the diagnosis is generally incidental. Luteoma arises from the proliferation of luteinised cell under the influence ofβ-hCG and can be hormonally active, with production of androgens resulting in maternal and fetal hirsutism and virilisation. We report a case of a 25-year-old primigravida who presented at 28 weeks of gestation with virilisation symptoms. Serum androgen levels were seven-hundred-fold higher than normal. A diagnosis of pregnancy luteoma was made at the time of caesarean section. The ovarian mass, serum androgen levels, and the condition of the patient improved after delivery.

Fanconi anaemia (FA) is a rare recessive disorder marked by developmental abnormalities, bone marrow failure, and a high risk for the development of leukaemia and solid tumours. The inactivation of FA genes, in particular FANCF, has also been documented in sporadic tumours in non-FA patients. To study whether there is a causal relationship between FA pathway defects and tumour development, we have generated a mouse model with a targeted disruption of the FA core complex gene Fancf. Fancf-deficient mouse embryonic fibroblasts displayed a phenotype typical for FA cells: they showed an aberrant response to DNA cross-linking agents as manifested by G(2) arrest, chromosomal aberrations, reduced survival, and an inability to monoubiquitinate FANCD2. Fancf homozygous mice were viable, born following a normal Mendelian distribution, and showed no growth retardation or developmental abnormalities. The gonads of Fancf mutant mice functioned abnormally, showing compromised follicle development and spermatogenesis as has been observed in other FA mouse models and in FA patients. In a cohort of Fancf-deficient mice, we observed decreased overall survival and increased tumour incidence. Notably, in seven female mice, six ovarian tumours developed: five granulosa cell tumours and one luteoma. One mouse had developed tumours in both ovaries. High-resolution array comparative genomic hybridization (aCGH) on these tumours suggests that the increased incidence of ovarian tumours correlates with the infertility in Fancf-deficient mice and the genomic instability characteristic of FA pathway deficiency. Copyright (C) 2011 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

A 6-year-old female nulliparous Dutch Rabbit ( Oryctolagus cuniculus ) was presented in shock status with a history of abdominal distention, inappetance, lethargy, polydipsia, diarrhea and weight loss since 2 weeks ago. There was no breeding history. Radiographic survey showed increased soft tissue opacity dorsal to the urinary bladder and ventral to the colon on the lateral view consistent with uteromegaly and severe intestinal distention resembling intussusceptions. Conservative treatment was failed and the animal died. In necropsy, ileocecal intussusception was noted as the main death factor. Thickened uterine endometrium in both horns was observed and the uterus was filled with mucoid fluid. Both ovaries were multilobulated and distended. On the other hand, a small cyst sized 1.5 × 2.5 cm was diagnosed on the liver surface. Several samples were obtained from lesions for histopathological evaluation. Histopathologically, dilated cystic glands of variable size lined by densely packed epithelium and hyperplasia of the endometrium resulting in irregular folds or papillar projection into the lumen were evident and the sections of ovaries revealed ovarian luteoma that composed of polyhedral cells with pale stained vacuolated cytoplasm, centrally located nuclei with distinct cytoplasmic borders and the cytoplasm of cells contain lipid droplet. The occurrence of biliary cyst adenoma was confirmed by microscopic examination of the mass that composed of multicystic space in different sizes lined by flattened to cuboidal biliary epithelium with papillary projections. These cysts were seperated by variable amount of connective tissue. The lack of postmortem investigations in aged rabbits reduced the incidence of neoplastic disease diagnosis in this species. Whereas uterine adenecarcinoma and lymphosarcoma were commonly reported in rabbits, luteoma and biliary cyst adenoma that are well described in this case report were uncommon in rabbit medicine.

Hyperandrogenic states in pregnancy are almost always the result of a condition that arises during pregnancy. The onset of virilization symptoms is often very fast. The mother is protected against hyperandrogenism by a high level of SHBG, by placental aromatase and a high level of progesterone. The fetus is protected from the mother’s hyperandrogenism partly by the placental aromatase, that transforms the androgens into estrogens, and partly by SHGB. Nevertheless there is a significant risk of virilization of the female fetus if the mother’s hyperandrogenic state is serious. The most frequent cause of hyperandrogenic states during pregnancy are pregnancy luteoma and hyperreactio luteinalis. Hormonal production is evident in a third of all luteomas, which corresponds to virilization in 25-35 % of mothers with luteoma. The female fetus is afflicted with virilization with two thirds of virilized mothers. Hyperreactio luteinalis is created in connection with a high level of hCG, e.g. during multi-fetus pregnancies. This condition most frequently arises in the third trimester, virilization of the mother occurs in a third of cases. Virilization of the fetus has not yet been described. The most serious cause of hyperandrogenism is represented by ovarian tumors, which are fortunately rare.

UNLABELLED Pregnancy luteomas are rare, benign, neoplasms of the ovary thought to be caused by the hormonal effects of pregnancy. They are usually asymptomatic and found incidentally during imaging or surgery. However, they may present with virilization of the mother or infant or cause complications due to a mass effect or hemorrhage secondary to torsion. Luteomas spontaneously regress postpartum. We present a case of a woman with the classic presentation of a pregnancy luteoma and the common challenges of subsequent management. Pregnancy luteomas present a diagnostic and management challenge in that they can mimic the presentation of malignant ovarian tumors. There have been fewer than 200 case reports of pregnancy luteomas and a relative paucity of data to provide guidance for clinical management. However, certain general principles emerged from a review of modern cases. Management of pregnancy luteomas depends on the clinical situation. Luteomas present most commonly in the second half of pregnancy, with a solid ovarian mass that is frequently bilateral, elevated testosterone levels and some aspects of virilization. With high clinical suspicion for pregnancy luteoma, clinical monitoring and postpartum radiologic follow-up may be an appropriate management strategy to avoid unnecessary surgery. However, in some cases with atypical presentation or with complications from the mass, surgical intervention may be necessary for diagnostic or management purposes. Patients who present in the first half of pregnancy generally have more severe symptoms and are more likely to require surgical intervention for management of mass effect. When there is a high clinical suspicion for pregnancy luteoma, conservative management is appropriate since these tumors will usually regress spontaneously. After completing this CME activity, obstetrician/gynecologists should be better able to evaluate clinical presentations of pregnancy luteomas, examine the complexities involved in diagnosing neoplasms suspicious for pregnancy luteoma, and counsel patients about appropriate management and treatment options. TARGET AUDIENCE Obstetricians & Gynecologists, Family Physicians, Pediatricians Learning Objectives: After completion of this article, the reader should be better able to evaluate the clinical presentations of pregnancy luteomas; examine the complexities involved in the diagnosis of neoplasms suspicious for pregnancy luteoma; and counsel patients on appropriate management and treatment for suspected luteomas.

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.

We report a rare case of benign sex cord-stromal tumor consisted largely of luteoma with minor portion of Sertoli cell tumor located at the position of the left ovary excision in an 11-year-old ovariectomized bitch. Granulosa cell component was lacking, and both luteal and Sertoli cell portions were entirely positive for inhibin alpha and neuron-specific enolase, whereas luteoma portion alone was positive for Wilms' tumor-1 (WT1), immunohistochemically. The results suggest that this tumor is a possible complication of incomplete ovarian excision at the time of ovariectomy and consisted of uncommon hybrid of luteal and Sertoli cells to be diagnosed as an unclassified sex cord-stromal tumor if applied in human cases. WT1-expression pattern suggested the signature of the difference in the phenotype of these cell types.

The presence of Y-chromosome material in patients with dysgenetic gonads increases the risk of gonadal tumors and/or nontumoral androgen-producing lesions. The patients' prognosis can vary, depending on their karyotype. The objective of this study was to investigate the presence of Y-chromosome mosaicism in Turner syndrome patients and its association with the development of gonadal tumors and/or nontumoral androgen-producing lesions. Eighty-seven Turner syndrome patients were studied. Genomic DNA was extracted from peripheral blood and genes SRY and TSPY and DYZ3 repeat of the Y chromosome were amplified by polymerase chain reaction. To the Y-positive patients, prophylactic gonadectomy was offered. The data disclosed hidden Y-chromosome mosaicism in 16 (18.5%) of the patients. SR Y sequence was detected in all of the 16 patients, and 4 (4.6%) of them presented DYZ3 repeat region and TSPY gene. Eleven of the patients with Y-positive sequences agreed to undergo the prophylactic surgery. In 2 cases, bilateral gonadoblastoma was found and, in another case, the histopathologic study of the gonads revealed hilus cell hyperplasia. In a further case, there were hilus cell hyperplasia and a stromal luteoma. In conclusion, a systematic search for hidden Y-chromosome mosaicism, especially SRY, in Turner syndrome patients is justified by the possibility of preventing gonadal lesions.

Luteoma of pregnancy is a rare, tumorlike ovarian mass that develops during pregnancy and regresses after delivery. Generally, these masses are discovered incidentally during cesarean delivery or tubal ligation. Some of these patients will develop hirsutism or virilization during late pregnancy with or without fetal masculinization due to circulating androgens. The imaging features of this entity have been only rarely reported. An incidentally discovered luteoma of pregnancy in a 23-year-old patient during routine obstetric ultrasound is described and the image features in ultrasound and magnetic resonance (MR) imaging are discussed and compared with other studies. The patient underwent surgery to extract this mass considering the imaging findings were suspicious for neoplasia and the size and location could have potentially caused dystocia. This type of mass can mimic ovarian neoplasia and a correlation with imaging and laboratory findings can avoid an unnecessary surgical procedure during pregnancy.

We present a patient, treated for 3 months with clomiphen citrate after 5 years of infertility. This treatment resulted in a twin pregnancy, one degenerated into a partial hydatidiform mole and the other into a very early embryo death. The karyotype was a mosaic one: 63% of metaphases showed triploidy - 69 XXX and 37% diploidy - 46 XX. Despite all medical advice, she returned 8 months later with a new pregnancy, which proved to be a new partial hydatidiform mole, this time a single one. Karyotype was, also, a triploidy - 69 XXX. The genetic map of both genitors was performed, showing no aberrations. Unfortunately, the patient came back, once again, 5 months later, with a new positive pregnancy test. Ultrasonography revealed a new very early embryo death, the histopathological analysis establishing to be a single 'pure' stop in evolution of the pregnancy. As all the three pregnancies obtained after treatment with clomiphene were abnormal, two being partial hydatidiform moles and one being a premature miscarriage, without any genetic aberrations of the genitors, it seems very possible that clomiphene, apart from improving fertility, also increases the risk of abnormal ovum appearance.

A 27-year-old Caucasian (gravida 2 para 1) presented in week 16 of her twin pregnancy with worsening of hyperandrogenic symptoms. In week 17, she developed an acute abdomen due to a twisted, incarcerated right ovary, which was surgically removed. Histological analysis revealed a diffuse steroid cell hyperplasia. Postsurgery testosterone levels fell temporarily within normal limits, diminishing the hyperandrogenic symptoms. Over time androgen levels rose again slowly above normal values with clinical worsening of hirsutism. In the 32nd week of gestation, a cesarean section of two healthy female infants was necessary due to the development of preeclampsia. An ovarian biopsy revealed again the picture of hyperreactio luteinalis. Postpartum peripheral hormone levels fell within normal limits and the hyperandrogenic symptoms subsided.

Sex cord-stromal tumors (SCSTs) of the ovary are uncommon. Their behavior is unpredictable, often with late recurrence, making counseling, management, and prediction of prognosis challenging. A 65-year-old woman presenting with a SCST underwent a bilateral oophorectomy, the histology was unusual but likely to be a luteinized thecoma with suspicious features for invasion. Two years later following a gastrointestinal bleed, a metastasis within the small bowel mucosa was detected. This represents probable isolated hematogenous or lymphatic spread, which is highly unusual especially in the absence of concurrent peritoneal disease. This is the first reported case of a SCST recurring in small bowel mucosa, mimicking a primary colorectal tumor. This highlights the diverse nature and behavior of these tumors.

Background Virilization in female newborns typically results from congenital adrenal hyperplasia, requiring immediate diagnosis and treatment. We report a rare cause of virilization, maternal pregnancy luteoma, responsible for virilization of both a newborn and the mother. Luteomas are usually asymptomatic tumour-like ovarian lesions of pregnancy that secrete androgens in only 25% of cases. Many female infants born to masculinized mothers will also be virilized. Case A term infant born with ambiguous genitalia was transferred to a referral centre for investigation, diagnosis, and treatment. Assessment identified Prader II-III genitalia, an elevated serum testosterone level, a normal serum 17-hydroxyprogesterone level, and a normal female karyotype (46,XX). The mother had had virilization from the second trimester and was found to have an elevated serum testosterone level. Pelvic ultrasound assessment in the mother showed a complex right ovarian mass. Laparotomy was performed, and the mass was excised. Histopathology examination confirmed a luteoma. Conclusion High maternal serum testosterone levels due to a luteoma can result in virilization in the female newborn. This report emphasizes the need to consider possible underlying maternal pathology in evaluating a virilized female infant.

Steroid cell tumors account for less than 0.1% of all ovarian tumors. There are three steroid cell tumor subtypes: steroid cell tumor not otherwise specified (NOS), stromal luteoma and Leydig cell tumor. Steroid cell tumor, NOS, is the most common type and has malignant potential. We report the case of 39-year-old woman complaining of pelvic pain and amenorrhea. Physical examination revealed hirsutism. Transvaginal ultrasound showed an hyperechogenic homogenous cyst of 4 centimeters of the left ovary without signs of malignancy. The right adnexa was normal. MRI imaging showed a left ovarian mass of 34 mm × 30 mm with enhancement after intravenous gadolinium. A left salpingo-oophorectomy was carried out with peritoneal cytology by laparoscopy. Histology concluded to a steroid cell tumor not otherwise specified, which had a low malignant potential. After 2 months, we noticed the regression of hirsutism and the patient had normal menses. She’s currently pregnant at 6 months of gestation. A complete surgical staging will be achieved after childbearing.

Clinical Manifestation, Diagnosis and Histology of Ovarian Luteoma: Case Report of Female Virilisation and Review of the Literature Stromal luteoma has recently been called and classified in the steroid cell tumor subgroup by the World Health Organisation (WHO). It is a rare benign ovarian tumor, originated in the ovarian stromal-sex cords, and it has endocrine repercussion. Sex cord-stromal tumours include a heterogeneous group of neoplasms that represent 7% of the ovarian tumours; most of them produce steroid hormones with estrogenic and/or androgenic effects, thus allowing its early diagnosis. Stromal luteoma usually presents with hiperestrogenic manifestations. We present an exceptional case of stromal luteoma that produced androgens.

Immunosuppression has been related to the incidence of tumor apparition, including endocrine tumors. The intrasplenic ovarian tumor (luteoma) is a typical benign endocrine tumor that develops under high gonadotropin stimulation and, from the immunological perspective, is located in a critical organ involved in immune response. To establish if immunosuppression could alter the development of this experimental tumor, the effects of cyclosporin A (CsA) and dexamethasone (Dex) were evaluated. After surgery, tumor-bearing and sham animals were kept without treatment for 4 weeks; thereafter, they were distributed into CsA (25 mg/kg), Dex (0.1 mg/kg), or vehicle (75:25 castor oll:ethanol) groups and were injected on alternate days for 50 days. Body weight was evaluated weekly. Animals were sacrificed after a jugular vein blood sample was obtained. Thymi were weighed. Tumors were measured and placed in formaline for histological studies. Serum luteinizing hormone (LH), follicle-stimulating hormone (FSH), prolactin (PRL), and estradiol were measured by radioimmunoassay. Hematological parameters were determined. CsA induced a significant decrease in survival rates both in tumor-bearing and sham animals (P < 0.01). Dex significantly impaired weight increase in both groups of animals. CsA induced a significant weight loss in sham animals, not observed in tumor-bearing animals. Dex induced thymus weight loss in both groups, whereas CsA induced thymus weight loss only in sham animals. Only Dex induced a decrease in lymphocyte number in both groups. CsA induced an increase in monocyte number only in sham animals. Treatments did not alter LH, FSH, or estradiol, whereas PRL was increased by CsA only in sham rats. Neither Dex nor CsA induced any significant variations in tumor volume, nor did they alter tumor histology. In addition, no visible metastases or alterations in other organs were observed. We conclude that, though immunological parameters were altered by the treatments, immunosuppressor drugs did not condition tumor development. In addition, tumors secrete one or more factor/s that counteract CsA effect.

OBJECTIVE: The aim of the present work was to study whether immunocytochemical parameters present in the normal ovary were altered after tumor development under high gonadotropin levels. METHODS: Ovarian tumors (luteoma): castrated female rats had an ovary grafted into the spleen; tumors were left to develop for 1, 2, 3 or 7 months. The presence of apoptotic cells (TUNEL method) and the expression of proliferating cell nuclear antigen (PCNA), gap junction protein (Cx43), steroidogenic acute regulatory protein (StAR), aromatase and synaptosome-associated protein of 25 kDa (SNAP-25) were determined by immunocytochemistry. Some of these findings were confirmed by RT-PCR (Cx43, StAR, SNAP-25). Inhibin subunit mRNAs were investigated by Northern blot. RESULTS: PCNA staining of tumors was mainly found in granulosa cells of transforming follicles and was absent from luteinized follicles. A nearly complete absence of apoptosis was observed. Cx43 was mainly found in follicles, while it was very weakly expressed or absent in luteinized follicles. StAR protein expression, indicating active steroidogenesis, was demonstrated only in luteinized follicles and in thecal cells, but was absent from granulosa cells. Aromatase immunoreactivity was very intense in granulosa and also present in luteal cells. Membrane-associated and cytoplasmic SNAP-25 immunostaining was determined in patches of endocrine cells in the follicles, as well as in the luteinized follicles. The expression of mRNAs for Cx43, StAR and SNAP-25 (RT-PCR) and inhibin subunits (Northern blots) were confirmed in 1-, 3- and 7-month-old tumors. CONCLUSIONS: These results indicated that luteoma most likely develop from unruptured follicles by hypertrophy and proliferation of follicular cells. Circulating gonadotropins seem to play a fundamental role in maintaining the expression of proteins typically expressed in normal ovary, while avoiding apoptosis in this tissue.

Granulosa cell tumors comprise approximately 10% of ovarian tumors and, although rare, are clinically important due to their potential for malignancy and recurrence. Although their morphological features have been carefully described, the global changes in gene expression associated with their formation remain undetermined. To initiate this characterization, we used a transgenic mouse model in which granulosa cell tumors occur with 100% penetrance in CF-1 mice that harbor a novel transgene encoding a chimeric LHβ subunit. When this transgene is expressed in other strains of mice, including (C57BL/6♀ × CF-1♂,Tg) F1 hybrids, luteomas develop even though levels of LH remain high. This dichotomous response permits a longitudinal comparison of global changes in transcriptomes uniquely associated with either granulosa cell tumors or luteomas. Herein we report numerous changes in the transcriptome, including a decrease in LH receptor mRNA and increases in several mRNAs that encode secreted proteins previously associated with granulosa cell tumors. Furthermore, we identified a constellation of mRNAs that encode proteins that may serve as new markers for this tumor phenotype. Additional experiments indicated that periodic treatment with human CG prevented formation of granulosa cell tumors in mice genetically predisposed to tumor development and, instead, led to the appearance of luteomas. More importantly, ovarian transcriptomes from the luteomas induced by ovulatory doses of human CG permitted refined confirmation of gene expression changes that were uniquely associated with either granulosa cell tumors in the permissive CF-1 genetic background or in luteomas in the F1 hybrids. Together, these dynamic changes in the ovarian transcriptome indict various signaling pathways potentially involved in mediating the actions of LH over time and, depending on genetic background, the formation of either a luteoma or a granulosa cell tumor.

Female pseudohermaphroditism associated with luteoma of pregnancy (LP) is a rare condition characterized by varying degrees of masculinization of a female fetus. We describe a case, diagnosed at 13 weeks gestation. Transvaginal ultrasound at 5 weeks of gestation revealed a normal intrauterine gestational sac and an enlarged maternal right ovary. Re-examination at 13 weeks showed a fetus with male external genitalia. Cytogenetic investigation on amniotic fluid revealed a normal female karyotype 46,XX. Follow-up sonography confirmed the previous assignment of male external genitalia and a second amniocentesis was negative for the SRY gene. High levels of androgens were found in the maternal blood. A diagnosis of female pseudohermaphroditism associated with bilateral LP was made. A healthy girl was born by Caesarean section with complete masculinization of external genitalia (Prader V). Histology confirmed a bilateral LP. To the best of our knowledge this represents the first case of prenatal diagnosis of female pseudohermaphroditism associated with LP and demonstrates the feasibility of diagnosis by sonography from 13 weeks gestation. This is also the first case described of Prader V masculinization associated with LP.

Luteoma is a rare condition in pregnancy. In this article, we aimed to present a rare case of pregnancy luteoma detected during cesarean section and pathologically in the context of literature information.

Sex-cord ovarian tumours are a rare kind of ovarian neoplasm. Steroid cell tumours that account for 0.1% of all primary ovarian tumours are also a sub-group of sex-cord ovarian tumours. Steroid cel...

Os luteomas da gravidez são pseudotumores ovarianos diagnosticados pelo exame ultra-sonográfico ou durante realização de cesariana e laqueadura pós-parto. Determinam, na segunda metade da prenhez, sinais de virilização materna em um quarto dos casos, o mesmo ocorrendo com a metade dos fetos femininos destas gestantes virilizadas nos quais se observa hipertrofia clitoridiana ou fusão labial. As dosagens séricas maternas dos hormônios androgênicos durante a prenhez e do sangue umbilical por ocasião do parto revelam taxas significativamente aumentadas. No exame ultra-sonográfico apresentam-se como estruturas sólidas ou cístico-sólidas, que após o parto tendem a regredir com o ovário readquirindo as dimensões normais em poucas semanas. Os autores apresentam uma paciente que em duas gestações sucessivas apresentou virilização materna e fetal. Ao exame ultra-sonográfico foram evidenciadas imagem ovariana nodular e dosagens elevadas dos androgênios plasmáticos. Luteomas of pregnancy are ovarian pseudotumors diagnosed by ultrasound, during cesarean section or at postdelivery tubal ligation. Twenty-five per cent of the cases appear around the second half of pregnancy. Usually there are signs of maternal virilization and 50% are detected because female newborns show clitorimegaly and/or labial fusion. The concentrations of androgenic steroids in the maternal blood during pregnancy and in the cord blood at child-birth show significantly increased rates. The ultrasound shows solid or cystic-solid structures and few weeks after the delivery they decrease and the ovary size returns to normal. The authors report a case of a patient who exhibited virilization signs in two consecutive pregnancies as well as in the two female fetuses. At adnexal sonographic examination a solid tumoral image was found in both pregnancies. Serum androgen levels were increased.

Twenty cases of the distinctive tumor-like lesion of the ovary, pregnancy luteoma, are described, with emphasis on pathologic features. The masses occurred in patients from 15 to 44 years of age and were typically incidental findings at or near term. Four patients experienced androgenic manifestations. The luteoma was documented to be bilateral in only 4 cases, but the opposite ovary was usually not evaluated pathologically. They ranged up to 15 cm. Sectioning typically showed multiple nodules or a single discrete mass with a multinodular sectioned surface and a soft bulging appearance. Most lesions were brown, but a few were black or yellow at least focally. Foci of hemorrhage were common. The most common microscopic appearance was a diffuse growth, but it was punctuated in about three quarters of the cases by follicle-like spaces often containing colloid-like secretion. These spaces are much more characteristic of the pregnancy luteoma than of the lesion most often in the differential diagnosis, a steroid cell tumor, and accordingly may be of diagnostic aid. However, they may cause confusion, as follicle-like spaces are a nonspecific feature of a number of neoplasms involving the ovary and potentially in the differential diagnosis. Awareness of the clinical background and frequency of finding follicle-like spaces in pregnancy luteoma are important diagnostically, and standard immunohistochemical stains will aid should they be warranted.

We report a female newborn with characteristic signs of Antley-Bixler syndrome (ABS) such as midface hypoplasia, radiohumeral synostosis and multiple joint contractures. The newborn also presented ambiguous genitalia, stage Prader V, and congenital adrenal hyperplasia. The mother experienced midterm virilization due to a pregnancy luteoma. Her elevated androgen levels and virilization symptoms normalized post partum without treatment. The newborn had elevated serum testosterone and 17-OH-progesterone levels which remained elevated because of a 21-hydroxylase deficiency. The child's treatment in order of priority was: hydrocortisone substitution, craniofacial/skeletal anomaly management and surgical correction of the external genitalia. Mutations in the genes for fibroblast growth factor (FGF) 8 and receptors FGFR1, FGFR2, and FGFR3 were not detected. Conclusion A newborn girl with manifestations of the Antley-Bixler syndrome showed severe virilization probably caused by the association of a mild 21-hydroxylase deficiency and maternal hyperandrogenism due to a pregnancy luteoma. Abnormalities of androgen metabolism may be responsible for virilization reported in other cases of the Antley-Bixler syndrome.