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

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.

Carcinoma endometroide con áreas semejantes a tumor estromal de cordones sexuales / Endometrioid carcinoma with similar areas of sex cord stromal tumor

Conocido antiguamente como el "asesino silencioso", el cáncer de ovario muestra un espectro de síntomas inespecíficos que, una vez reconocidos, facilitan un diagnóstico temprano y prolongan la supervivencia de la paciente. Se presenta el caso de una paciente de 57 años referida de consulta privada por sangrado post menopaúsico con US transvaginal que revela datos de malignidad ovárica, laboratorios de rutina y marcadores tumorales normales...

Tumor de células esteroideas de ovario: reporte de un caso y revisión de la literatura / Steroid cell tumor of the ovary: a case report and literature review

El tumor de células esteroideas de ovario es una neoplasia poco frecuente que se origina del estroma o de los cordones sexuales y es potencialmente maligno. La mayoría de estos tumores producen hormonas esteroides siendo la testosterona la que se eleva frecuentemente. Se presenta el caso de una paciente de 30 años que presentó signos de virilización asociado a masa pélvica, ascitis e incremento de la testosterona. Fue operada y sometida a estadiaje de ovario. El resultado del estudio anatomopatológico fue tumor de células esteroideas. Una revisión de los aspectos clínicos, diagnósticos y terapéuticos de estos tumores, es presentada.

Steroid cell tumor of the ovary is a rare malignancy originating from the stromal or sex cord and is potentially malignant. Most of these tumors produce steroid hormones testosterone being which arises frequently. We report a case of a patient 30 who showed signs of virilization associated with pelvic mass, ascites and increased testosterone. She was operated and subjected to ovarian cancer staging. The result of the pathology was steroid cell tumor. A review of clinical aspects, diagnostic, and treatment of these tumors, is presented.

Bilateral sex cord tumor with annular tubules of ovary without Peutz-Jeghers syndrome: a case report.

Ovarian sex cord tumor with annular tubules is a very rare tumor .Afourteen year old Nepali girl presented with abdominal distension and lower abdominal pain.On laparotomy bilateral ovarian masses were found. It was totally solid on the right side and was partially solid and partially cystic on the left side. The patient had no other symptoms and features of Peutz-Jeghers syndrome. Microscopic examination showed characteristic findings of sex cord tumor with annular tubules on both sides. The patient was doing well 2 months after surgery. The case is presented with review of literature because of the rare clinical presentation.

Mazabraud's Syndrome Coexisting with a Uterine Tumor Resembling an Ovarian Sex Cord Tumor (UTROSCT): a Case Report

The association of intramuscular myxoma and fibrous dysplasia is a rare disease known as Mazabraud's syndrome. We present a case of Mazabraud's syndrome coexisting with a uterine tumor and resembling an ovarian sex cord tumor (UTROSCT). This uterine tumor showed a high mitotic index and cytological atypia. To the best of our knowledge, the coexistence of the two different entities has not been reported in the literature.

Tumor de los cordones sexuales con túbulos anulares del ovario no asociado a síndrome de Peutz-Jeghers: reporte de un caso / Ovarian sex cord-stromal tumor with annular tubules non-associated to Peutz-Jeghers syndrome: a case report

Se presenta el raro caso de una mujer de 20 años con un tumor del estroma gonadal específico del ovario, variedad tumor de los cordones sexuales, con túbulos anulares no asociado a síndrome de Peutz-Jeghers. Se revisa la literatura sobre el manejo de esta entidad.

Ovarian sex cord tumour with annular tubules diagnosed by fine needle aspiration cytology--a case report.

Sex cord tumor with annular tubules (SCTAT) is a rare ovarian neoplasm with distinctive morphological features whose cytological features have rarely been described. We report a case of SCTAT diagnosed on fine needle aspiration cytology (FNAC) with a brief review of literature. A 35 year old female presented with recurrent abdominal mass. Fine needle aspiration was done under ultra sound guidance. Cytology smears showed three dimensional tubular structures, metachomatic hyaline masses and uniform neoplastic cells dispersed and in cohesive clusters. Cell block study showed the characteristic simple and complex tubules leading to the diagnosis. The cytological findings of a rare ovarian neoplasm are described, which is only the third such report in English literature. The cytologic features of ovarian SCTAT are as distinctive as its histologicfeatures. Cell block study brings out the distinctive architecture of the tumor and helps in differentiating it from other closely related tumors.

Tumor maligno del estroma gonadal de Sértoli-Leydig: comunicación de un caso clínico y revisión de la literatura / Sertoli-Leydig tumor of the gonadal stroma

Los tumores malignos del estroma gonadal representan 0.2 por ciento de todos los tumores del testículo y son casi exclusivos del adulto. Son relativamente refractarios a la radioterapia y a la quimioterapia, y la sobrevida media de la enfermedad metastásica es de dos años. Se presenta un caso clínico de tumor a células de Sértoli-Leydig en un hombre de 45 años que consultó por esterilidad y se hace una revisión de la literatura