Tumor de ovario de los cordones sexuales con túbulos anulares: reporte de caso y revisión de la literatura / Ovarian sex cord tumor with annular tubules: case report and review of the literature

RESUMEN Objetivos: Reportar un caso de Tumor de Ovario de los Cordones Sexuales con Túbulos Anulares (TCSTA), hacer una revisión de la literatura acerca del diagnóstico, tratamiento y pronóstico de esta condición. Materiales y métodos: Se informa el caso de una mujer que consultó al Instituto Nacional de Cancerología, Bogotá (Colombia), con diagnóstico final de TCSTA avanzado, quien recibió tratamiento quirúrgico y quimioterapia con evolución satisfactoria a los 6 meses. Se realizó una búsqueda de la literatura en las bases de datos Medline vía PubMed, LILACS y Scielo, que incluía reportes y series de caso de mujeres con diagnóstico de TCSTA, publicados desde 1990, sin incluir rango de edad. Se extrajo información sobre el diagnóstico, tratamiento y pronóstico reportado. Se realizó resumen narrativo de los hallazgos. Resultados: Se incluyeron 14 publicaciones que incluían 26 pacientes. La edad media al diagnóstico fue de 22,5 años. Los síntomas principales fueron alteraciones de la menstruación y dolor pélvico. La tomografía computarizada fue la tecnología de imágenes más frecuentemente utilizada. El tratamiento fue quirúrgico, siempre usado, acompañado de quimioterapia (29 %); 2 casos recibieron radioterapia. Hubo recaída en el 20 % de los casos. La mortalidad fue del 12,5 %, toda en el primer año. Conclusiones: La información sobre la utilidad diagnóstica de las imágenes, marcadores tumorales y estudios de histoquímica es escasa, como también los datos sobre el pronóstico de la entidad. El tratamiento quirúrgico es el de elección, teniendo en cuenta el deseo de fertilidad de la paciente y el estadio tumoral. Se requieren más estudios que documenten de manera más detallada el manejo de esta condición.

ABSTRACT Objectives: To report a case of ovarian sex cord tumor with annular tubules (SCTAT) and conduct a literature review on diagnosis, treatment and prognosis of this condition. Material and methods: Case report of a woman with a final diagnosis of advanced SCTAT seen at the National Cancer Institute in Bogota (Colombia) who received surgical treatment and chemotherapy with a satisfactory course after 6 months. A literature search was conducted in the Medline via PubMed, LILACS and Scielo databases, including case reports and series of women diagnosed with SCTAT published since 1990, not using age ranges. Information about diagnosis, treatment and reported prognosis was retrieved. A narrative summary of the findings was prepared. Results: Fourteen publications with 26 patients were included. Mean age at diagnosis was 22.5 years. The main symptoms were menstruation abnormalities and pelvic pain. Computed tomography (CT) was the imaging technology most frequently used. Surgical treatment was used in all cases, together with chemotherapy in 29 %; 2 patients received radiotherapy. Recurrence occurred in 20 % of cases. Mortality was 12.5 %, with all deaths occurring within the first year. Conclusions: There is a paucity of information about the diagnostic utility of imaging, tumor markers and histochemical studies, as well as prognosis of this disease condition. Surgery is the treatment of choice, taking into consideration the patient's wishes regarding fertility, as well as the stage of the tumor. Further studies are needed to provide more detailed information about this condition.

Preoperative differentiation of benign and malignant non-epithelial ovarian tumors: clinical features and tumor markers / Diferenciação pré-operatória de tumores ovarianos não epiteliais benignos e malignos: características clínicas e marcadores tumorais

Abstract Objective To evaluate the role of clinical features and preoperativemeasurement of cancer antigen 125 (CA125), human epididymis protein(HE4), and carcinoembryonic antigen (CEA) serum levels in women with benign and malignant non-epithelial ovarian tumors. Methods One hundred and nineteen consecutive women with germ cell, sex cordstromal, and ovarian leiomyomas were included in this study. The preoperative levels of biomarkers were measured, and then surgery and histopathological analysis were performed. Information about the treatment and disease recurrence were obtained from the medical files of patients. Results Our sample included 71 women with germ cell tumors (64 benign and 7 malignant), 46 with sex cord-stromal tumors (32 benign and 14 malignant), and 2 with ovarian leiomyomas. Among benign germ cell tumors, 63 were mature teratomas, and, amongmalignant, fourwere immatureteratomas. Themost common tumors in the sex cordstromal group were fibromas (benign) and granulosa cell tumor (malignant). The biomarker serum levels were not different among benign andmalignant non-epithelial ovarian tumors. Fertility-sparing surgeries were performed in 5 (71.4%) women with malignant germ cell tumor. Eleven (78.6%) patients with malignant sex cord-stromal tumors were treated with fertility-sparing surgeries. Five women (71.4%) with germ cell tumors and only 1 (7.1%) with sex cord-stromal tumor were treated with chemotherapy. One woman with germ cell tumor recurred and died of the disease and one woman with sex cord-stromal tumor recurred. Conclusion Non-epithelial ovarian tumors were benign in the majority of cases, and the malignant caseswere diagnosed at initial stages with good prognosis. Themeasurements of CA125, HE4, and CEA serum levels were not useful in the preoperative diagnosis of these tumors.

Resumo Objetivo Avaliar o papel das características clínicas e a medida pré-operatória dos níveis séricos de CA125, HE4, e CEA em mulheres com tumores de ovário não epiteliais benignos e malignos. Métodos Cento e dezenovemulheres consecutivas comtumores ovarianos de células germinativas, do cordão sexual-estroma, e miomas ovarianos foram incluídas neste estudo. Os níveis pré-operatórios dos biomarcadores foram medidos, a cirurgia e a análise histopatológica foram realizadas. Informações sobre tratamento e recorrência da doença foram obtidas dos prontuários médicos das pacientes. Resultados Nossa amostra incluiu 71 mulheres com tumores de células germinativas (64 benignos e 7 malignos), 46 com tumores do cordão sexual-estroma (32 benignos e 14 malignos), e 2 com leiomiomas ovarianos. Entre os tumores benignos de células germinativas, 63 eram teratomas maduros, e, entre os malignos, quatro eram teratomas imaturos. Os tumores mais comuns do grupo do cordão sexual-estroma foram fibromas (benignos) e tumores de células da granulosa (malignos). Os níveis séricos dos biomarcadores não diferiram entre os tumores de ovário não epiteliais benignos e malignos. A cirurgia preservadora de fertilidade foi realizada em 5 (71,4%) mulheres com tumores malignos de células germinativas. Onze (78,6%) mulheres com tumores do cordão sexual-estromamalignos foram tratadas comcirurgia preservadora de fertilidade. Cinco (71,4%)mulheres com células germinativas e apenas 1 (7,1%) com tumor do cordão sexual-estroma foram tratadas com quimioterapia. Uma mulher com tumor de células germinativas recidivou e morreu da doença. Uma mulher com tumor do cordão sexual-estroma recidivou. Conclusão Os tumores de ovário não epiteliais foram benignos namaioria dos casos e os malignos foram diagnosticados em estágios iniciais, com bom prognóstico. A medida dos níveis séricos de CA125, HE4, e CEA não foram úteis no diagnóstico préoperatório desses tumores.

Tumor de los cordones sexuales con túbulos anulares ováricos asociado a síndrome de Peutz-Jeghers: reporte de un caso en la adolescencia / Tumor of the sexual cords with ovarian annular tubules associated with Peutz-Jeghers syndrome in adolescence: a case report

Los tumores de los cordones sexuales y estromales son neoplasias poco frecuentes, que corresponden al 8 % de los tumores primarios del ovario. El tumor de los cordones sexuales con túbulos anulares del ovario es considerado un subtipo y es infrecuente. Puede presentarse de manera esporádica o asociado al síndrome de Peutz-Jeghers y tiene diferente comportamiento y características en cada situación.Se presenta el caso de una paciente adolescente con diagnóstico de tumor de los cordones sexuales con túbulos anulares del ovario asociado a síndrome de Peutz-Jeghers

Tumors of the sexual and stromal cords are rare neoplasms, corresponding to 8 % of primary ovarian tumors. The tumor of the sexual cords with annular tubules of the ovary is considered a subtype and is uncommon. It can occur sporadically or associated with Peutz-Jeghers Syndrome, having different behavior and characteristics in each situation.We present the case of an adolescent patient with a diagnosis of a tumor of the sexual cords with annular tubules of the ovary associated with Peutz-Jeghers Syndrome

Two Cases of Uterine Tumors Resembling Ovarian Sex-cord Tumors: Rare Case of Uterine Tumor / 이화의대지

Uterine tumors resembling ovarian sex-cord tumors (UTROSCT) are very rare tumors that occur mainly in the uterine fundus of women in reproductive age. These tumors can be classified into group 1 and group 2 by histological results. In group 1, epithelial-like differentiation is partially observed in the tumors. In group 2, sex-cord elements are predominant in uterine mural mass. We experienced UTROSCT group 1 in a 29-year-old woman who complained of severe abdominal pain that started one week after delivery and UTROSCT group 2 case in a 49-year-old woman who complained of dysfunctional uterine bleeding. We report two different types of UTROSCT cases that we experienced.

Intraoperative consultation for ovarian tumors / 영남의대학술지

The primary function of intraoperative frozen consultation is to provide an as accurate and prompt diagnosis as possible during surgery and to guide the surgeon in further management. However, the evaluation of frozen section (FS) is sometimes difficult because of suboptimal tissue quality and frozen artifacts compared with routinely processed tissue section. The pathologist responsible for the FS diagnosis requires experience and good judgment. Ovarian tumors are a heterogeneous group of tumors including primary surface epithelial tumors, germ cell tumors and sex cord-stromal tumors, secondary tumors, and other groups of tumors of uncertain histogenesis or nonspecific stroma. Intraoperative FS is a very important and reliable tool that guides the surgical management of ovarian tumors. In this review, the diagnostic key points for the pathologist and the implication of the FS diagnosis on the operator’s decisions are discussed.

Endometriosis y carcinoma de celulas claras. Reporte de caso / Endometriosis and clear cell carcinoma. Case report

RESUMEN La endometriosis corresponde a la presencia de glándulas endometriales o estroma en sitios distintos a la cavidad uterina. Afecta del 5 al 15% de las mujeres en edad reproductiva y se asocia a importante sintomatología. La teoría de la menstruación retrógrada propuesta por Sampson es la más aceptada para explicar su etiología. Varios estudios han asociado esta enfermedad a un riesgo aumentado de neoplasia, ésta entidad, denominada endometriosis asociada a malignidad, se localiza en sitios extra gonadales en un 20% de los casos, donde la pared abdominal no alcanza más de 30 casos reportados. Si bien no existe un tratamiento estándar, la mayoría de los autores han adaptado el protocolo de tratamiento para el cáncer de ovario asociado a endometriosis.

ABSTRACT Endometriosis corresponds to the presence of endometrial glands or stroma at sites other than the uterine cavity. It affects 5 to 15% of women of reproductive age and is associated with a significant symptomatology. The theory of retrograde menstruation proposed by Sampson is the most accepted to explain its etiology. Several studies have associated this disease with an increase in neoplasia, the entity, called endometriosis associated with malignancy, is located in extra gonadal sites in 20% of cases, where the abdominal wall does not reach more than 30 reported cases. Although there is no standard treatment, most authors have adapted the treatment for ovarian cancer associated with endometriosis.

Ovarian Gynandroblastoma with a Juvenile Granulosa Cell Tumor Component in a Postmenopausal Woman: A Case Report and Literature Review

Gynandroblastoma is an extremely rare sex cord-stromal tumor with both female (granulosa cell tumor) and male (Sertoli-Leydig cell tumor) elements. Juvenile granulosa cell tumors are also very rare and are so named because they usually occur in children and adolescents. A 71-year-old woman with right upper quadrant abdominal pain visited our hospital. Pelvic computed tomography showed a large multilocular cystic mass, suspected to be of ovarian origin. We performed a total abdominal hysterectomy (total abdominal hysterectomy was performed) with bilateral salpingo-oophorectomy. A 13-cm multilocular cystic mass with serous fluid was observed in her right ovary. Upon microscopic examination, the solid component of the mass showed both Sertoli-Leydig cell and juvenile granulosa cell differentiation, which we diagnosed as gynandroblastoma. Gynandroblastoma with a juvenile granulosa cell tumor component is extremely rare and, until now, only six cases have been reported in the English literature. We report the first gynandroblastoma with a juvenile granulosa cell tumor component diagnosed in an elderly patient, along with a literature review.

Tumor de células de la granulosa: neoplasia estromal de los cordones sexuales: revisión de la literatura, a propósito de dos casos clínicos / Granulosa cell tumor: stromal neoplasm of the sexual cords: review of the literature and clinical cases

Los tumores de las células de la granulosa, hacen referencia a un conjunto de neoplasias derivadas del tejido estromal de los cordones sexuales, secretores de estrógenos, que corresponden al 1-5% de todos los tumores malignos del ovario, aunque pueden presentar localizaciones extraováricas. Son tumores raros e infrecuentes, cuya incidencia general varía de 0,4 a 1,7 casos por cada 100.000 mujeres al año. Se clasifican en dos grupos diferenciados en función de la edad de las pacientes, su historia natural y sus características patogénicas: tumores de células de la granulosa tipo adulto (TCGA) o tipo juvenil (TCGJ). El diagnóstico de esta rara patología se basa en cuatro pilares fundamentales, por un lado, las manifestaciones clínicas y la confirmación mediante pruebas de imagen, y, por otro lado, los marcadores tumorales y el estudio inmunohistoquímico de las muestras biológicas. En el manejo terapéutico de los estadios iniciales de la enfermedad (supervivencia a los 5 años superior al 90% en los estadios I), se recomienda el tratamiento quirúrgico mediante histerectomía con anexectomía bilateral en pacientes con TCGA y deseos genésicos cumplidos, reservando la anexectomía unilateral para los casos de TCGJ o para aquellas pacientes sin descendencia (aunque con posterioridad se recomienda completar el tratamiento). La quimioterapia sistémica postoperatoria se incluye cuando existe extensión extraovárica o recurrencia de la enfermedad. La tasa de recidiva (considerado uno de los principales factores de mal pronóstico), es alta en estadios avanzados, aunque la probabilidad de desarrollar metástasis a distancia es baja.

Tumors of the granulosa cells refer to a set of neoplasms derived from the stromal tissue of the sex cords, secretory of estrogens. They correspond to 1-5% of all malignancies of the ovary, though they may have extraovarian locations. These tumors are rare and uncommon, whose general incidence may vary from 0.4 to 1.7 cases per 100,000 women per year. They are classified into two groups depending on the age of the patients, their natural history and their pathogenic characteristics: adult type tumors of the granulosa cell (AGCT) or juvenile type (JGCT). The diagnosis of this rare pathology is based on the study of clinical manifestations and confirmation through image tests, and also on tumor markers tests and the immunohistochemical study of biological samples. In the therapeutic handling of the early stages of the disease (5-year survival greater than 90% in stage I), surgical treatment via hysterectomy with bilateral adnexectomy in patients with AGCT and met genesic perspectives is recommended; for the cases of JGCT or for those patients without progeny, preserving the unilateral adnexectomy is justified (although completion of the treatment is recommended at later stages). Postoperative systemic chemotherapy is practiced when there is extraovarian extension or recurrence of the disease. The rate of relapse (considered one of the main factors of poor prognosis), is high in advanced stages, although the probability of developing distant metastasis is low.

Causa muy rara de pubertad precoz periférica en una niña: tumor de cordones sexuales con túbulos anulares / A very rare cause of peripheral precocious puberty in a girl: ovarian sex cord tumor with annular tubules

El tumor de los cordones sexuales con túbulos anulares es una neoplasia del estroma gonadal muy infrecuente. Representa el 0,05-0,6% de todos los tumores ováricos, según series. Se presenta un caso especialmente inusual, en una niña de 6 años, detectado a raíz de una pubertad precoz periférica isosexual. Su interés radica en que no se halló ninguna masa anexa al ovario, sino únicamente una asimetría gonadal, sin signos radiológicos de malignidad. Se realizó una salpingo-ooforectomía unilateral con linfadenectomía pélvica y paraaórtica ipsilateral, por vía laparoscópica, tras confirmarse la presencia de células tumorales en la biopsia intraoperatoria. La evolución posterior de la paciente fue favorable.

Sex cord tumor with annular tubules is an extremely uncommon gonadal stromal neoplasm. It represents 0.05-0.6% of all ovarian tumors, according to series. An unusual case is presented in a 6-year-old girl, detected as a result of an isosexual peripheral precocious puberty. The highlight of this case is that no mass attached to the ovary was found, but only a gonadal asymmetry without radiological signs of malignancy. After confirming the presence of tumoral cells by intraoperative biopsy, unilateral salpingo-oophorectomy with ipsilateral para-aortic and pelvic lymphadenectomy was performed. Afterwards, the evolution of the patient was favorable.

A case report of a young girl with mucinous borderline tumor of the ovary

Ovarian tumors are relatively rare in children and adolescent. The incidence of malignancies in these groups is 1% to 1.5%. The common histologic type is non-epithelial type such as germ cell tumors or sex cord-stromal tumors and only 10% to 17% of those are epithelial tumors. It is important to accurately diagnose in the early these rare tumors for proper staging and treatment to save the patient's life and fertility. We present a case of a 13-year-old girl with a giant ovarian mucinous borderline tumor.

Insights into granulosa cell tumors using spontaneous or genetically engineered mouse models / 대한생식의학회지

Granulosa cell tumors (GCTs) are rare sex cord-stromal tumors that have been studied for decades. However, their infrequency has delayed efforts to research their etiology. Recently, mutations in human GCTs have been discovered, which has led to further research aimed at determining the molecular mechanisms underlying the disease. Mouse models have been important tools for studying GCTs, and have provided means to develop and improve diagnostics and therapeutics. Thus far, several genetically modified mouse models, along with one spontaneous mouse model, have been reported. This review summarizes the phenotypes of these mouse models and their applicability in elucidating the mechanisms of granulosa cell tumor development.

Testicular malignant Leydig cell tumor: A case report / 中华男科学杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathological features of testicular malignant Leydig cell tumor (TMLCT) and improve the non-invasive diagnosis of the disease.</p><p><b>METHODS</b>We retrospectively analyzed the clinicopathological data on a case of TMLCT, detected the circulating tumor cells (CTC) in the peripheral venous blood, and reviewed the related literature.</p><p><b>RESULTS</b>The patient, a 47-year-old male, underwent radical orchidoepididymectomy under general anesthesia. Postoperative pathology confirmed the lesion to be TMLCT, which was mainly composed of Leydig cells and suspected with vessel carcinoma embolus. Immunohistochemistry showed the tumor cells to be positive for α-inhibin, Ki67, CD30, vimentin, EMA, and PLAP, but negative for CK, CK7, S100, CD10, SMA, Des, AFP, hCG, CEA, CK19, CD117, Oct-4, LCA, CD20, Pax-5, CD3, and CD43. Two CTCs were detected in the peripheral venous blood. The patient received 3 courses of chemotherapy for retroperitoneal multiple lymph nodes metastasis post-operatively. Subsequent CT imaging manifested no obvious reduction of the retroperitoneal lymph nodes and consequently the patient again underwent retroperitoneal lymphadenectomy and cryoablation. At 8 months after treatment, CT examination revealed notably enlarged retroperitoneal lymph nodes with the right adrenal gland evidently invaded.</p><p><b>CONCLUSION</b>TMLCT is an extremely rare sex-gonad stromal tumor with high malignancy and poor prognosis, and CTCs may be used for its early diagnosis and prognostic prediction.</p>

Sclerosing Sromal Tumor of the Ovary in Postmenopausal Women: A Report of Two Cases

Sclerosing stromal tumor (SST) was first delineated as a distinct ovarian sex cord stromal tumor in 1973 by Chalvardjian and Scully. It is a benign neoplasm, distinguished from other ovarian stromal tumors by the production of collagen and a pseudolobular pattern, and it tends to occur in the second and third decades of life in diagnosed patients. We discovered two rare cases of SST in post-menopausal women which are the topic of this report. These case studies are accompanied by a brief review of the literature.

Ginandroblastoma multiloculado, negativo para CD99 en una niña de 12 años: caso clínico / A twelve-years-old girl with multiloculated gynandroblastoma CD99 negative. Case report

Los ginandroblastomas son tumores del ovario extremadamente raros, los cuales comparten componentes de células de la granulosa y de células de Sertoli/Leydig. Se describe un caso de una niña de 12 años, quien presenta hemorragia uterina anormal y sensación de masa intraabdominal de crecimiento progresivo asociado a menorragia, niveles de CA-125 en 60,4 UI/mL y estudios de extensión que reportan masa quística en ovario izquierdo, manejada con ooforectomía. El estudio anatomopatológico muestra un tumor multiloculado lleno de material seroso, abundantes cuerpos de Call-Exner y 45% de células de Sertoli/Leydig. La inmunohistoquímica reveló inmunorreactividad para inhibina, calretinina y pCK, mientras que los marcadores CD99 y AE1/AE3 fueron negativos. Se trata del primer reporte de caso sobre un ginandroblastoma multiloculado, negativo para CD99 en una niña de 12 años, estudio que plantea un abordaje sistemático para los tumores de las células de los cordones sexuales.

The ginandroblastoma is an extremely rare ovarian tumor which shows components of granulosa cells and Sertoli/Leydig cells. We describe a case of a twelve-years-old girl who presented abnormal uterine bleeding and progressively growing intra- abdominal mass associated with menorrhagia, CA-125 60.4 UI/mL and extension studies reporting cystic mass in the left ovary. She underwent oophorectomy. Pathological study shows a multilocular tumor filled with serous material. Many Call-Exner bodies were observed in the histopathological analysis, 45% of Sertoli/Leydig cells. Immunohistochemistry was reactive for inhibin, calretinin and pCK while AE1/AE3 and CD99 markers were negative. This is the first case report about a multiloculated gynandroblastoma, negative for CD99 in a 12-years-old girl. Thus, the study of this clinical case represents a systematic approach for tumors of the sex cord cells.

Hyperandrogenism produced by ovarian tumors in women at different life stages / Hyperandrogenism produced by ovarian tumors in women at different life stages.

OBJECTIVE: to evaluate the different presentations of hyperandrogenism produced by ovarian tumors in women at different life stages DESIGN: case report. SETTING: academic institutions. PATIENT(S): 3 patients at different life stages, with increased androgen levels. INTERVENTION(S): review of hospital records. MAIN OUTCOME MEASURE(S): clinical and biochemical features, treatment and follow-up. RESULT(S): a 10 year-old girl with Leydig cell tumor presented with hyperandrogenemia, virilization and changes in social behavior. Another patient, at reproductive age, with a tumor>10 cm, presented with signs of virilization and abdominal mass, whose pathologic analysis disclosed a carcinoid tumor of the ovary associated with stromal hyperplasia. The third patient was a postmenopausal woman with severe alopecia, who presented a steroid cell tumor, rare at that age. CONCLUSION(S): the evaluation of women with signs and symptoms of virilization should include a detailed clinical record, thorough physical examination and an appropriate hormonal dosage, especially when images are inconclusive.

Sclerosing Stromal Tumor in an Elderly Postmenopausal Woman

Sclerosing stromal tumor (SST) of the ovary is a rare tumor derived from the sex cord stroma. This tumor was first described by Chalvaridjian and Scully in 1973. SST of the ovary is prevalence of 1.5% to 6% of ovarian stromal tumors. Patients are most commonly diagnosed in their 20s and 30s. There have been reports of SST postmenopausal women aged 65-, 67-, and 71 in the Republic of Korea; however, no report of this disease has been reported in women older than 80. In this study, we would like to report an 80-year-old postmenopausal woman who did not previously complain of any symptoms, and was finally diagnosed with SST. She was involved in a traffic accident, and huge pelvic mass was found during the evaluation of intra-abdominal hemorrhage. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed ; a final pathologic diagnosis reported SST.

A Case of Ovarian Steroid Cell Tumor, Not Otherwise Specified, Treated with Surgery and Gonadotropin Releasing Hormone Agonist

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. This report describes a case of an ovarian steroid cell tumor, NOS. A 35-year-old woman visited hospital with the complaint of metrorrhagia. Physical examination revealed increased pubic hair. Transvaginal ultrasound indentified a 4.9 x 3.4 cm, well-circumscribed and solid left ovarian tumor. After laparoscopic left oophorectomy, the tumor was revealed as an ovarian steroid cell tumor, NOS. During the laparoscopic surgery, tumor ruptured. Complete surgical staging was performed and no evidence of metastasis was found. Gonadotropin releasing hormone agonist was administered monthly for 6 months. The patient has had no evidence of recurrence for 43 months.

Tumor estromal esclerosante de ovário associado à síndrome de Meigs e gestação: relato de caso / Sclerosing stromal tumor of the ovary associated with a Meigs' syndrome and pregnancy: a case report

O tumor estromal esclerosante de ovário é uma neoplasia benigna extremamente rara, mais frequente em mulheres jovens e sem sintomas específicos na maioria dos casos. Menos de 150 casos foram descritos, dos quais 8 diagnosticados durante a gestação. Neste relato, documentamos a associação entre tumor estromal esclerosante de ovário, síndrome de Meigs e elevação dos níveis de CA-125 em gravidez a termo.

The sclerosing stromal tumor of the ovary is an extremely rare benign tumor more common in young women and without specific symptoms in most cases. Less than 150 cases have been described, of which 8 were diagnosed during pregnancy. In this report, we describe the association between sclerosing stromal tumor of the ovary, Meigs' syndrome and elevated levels of CA-125 in term pregnancy.

Tumor extragonadal de células de la granulosa / Extragonadal granulosa cell tumor

Los tumores extragonadales de la granulosa son extremadamente infrecuentes. Originados a lo largo del recorrido de las gónadas en formación durante su evolución embriológica, se presentan fundamentalmente en la mujer perimenopáusica. Son por lo general tumores de bajo grado de malignidad con recurrencia frecuente. Se estudió a una paciente de 51 años que evolucionó durante más de 10 años con muy pocos síntomas y un crecimiento lento hasta alcanzar 20 cm en el retroperitoneo. La intervención realizada fue satisfactoria(AU)

Extragonadal granulosa cell tumors are extremely infrequent. Originated along the path of the gonads in their formation during embryologic evolution, they are mainly present in the perimenopausal woman. Generally, they are tumors with a low degree of malignancy and frequent recurrence. A 51 year - old patient was studied who, for more than 10 years, presented few symptoms and a slow growth, reaching 20 cm in the retroperitoneum. The surgical procedure performed was satisfactory(AU)