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.

Tumor de ovario borderline: concordancia entre biopsia contemporánea y biopsia definitiva / Borderline tumor of the ovary: accuracy of intraoperative frozen section and definitive biopsy

OBJETIVO: Los tumores de ovario borderline (BOT) son un grupo de lesiones neoplásicas de origen epitelial del ovario que presentan características de tumores malignos, pero sin invasión del estroma, y se caracterizan por tener un buen pronóstico. El objetivo del estudio es determinar la concordancia diagnóstica entre biopsia contemporánea y definitiva de los BOT en nuestro centro hospitalario. MÉTODO: Se realizó un estudio analítico retrospectivo de corte transversal de las biopsias contemporáneas y definitivas de BOT en la base de datos de anatomía patológica del Hospital Padre Hurtado, entre los años 2010 y 2019. El análisis estadístico de concordancia se realizó mediante test de kappa. RESULTADOS: Se revisaron 4546 informes de biopsias entre los años 2010 y 2019. Se pesquisaron 163 tumores malignos de ovario, de los cuales 69 (42,33%) correspondieron a BOT. De estos, 39 fueron serosos (56,2%), 28 mucinosos (40,57%) y 2 (2,8%) de tipo endometrioide. El resultado de concordancia diagnóstica de BOT seroso es moderada, del 75,71% con un índice de kappa de 0,5143 (p = 0,000), y el de BOT mucinoso es débil, del 65,71% con un índice de kappa de 0,2398 (p = 0,0222). CONCLUSIONES: Los BOT corresponden a un gran porcentaje dentro de los tumores malignos del ovario, siendo el subtipo seroso el más común. La concordancia entre biopsia contemporánea y definitiva es de débil a moderada.

OBJECTIVE: Borderline ovarian tumors (BOT) are a group of neoplastic lesions of epithelial origin of the ovary that present characteristics of malignant tumors but without stromal invasion and are characterized by having a good prognosis. The objective of the study is to determine the concordance between frozen section and definitive biopsy of BOT in our hospital center. METHOD: A retrospective, cross-sectional analytical study of the frozen section and definitive BOT biopsies was performed in the pathological anatomy database of the Padre Hurtado Hospital during the years 2010 and 2019. The statistical and concordance analysis was performed using kappa tests. RESULTS: 4546 biopsy reports were reviewed during 2010 and 2018. A total of 163 malignant ovarian tumors were investigated, of which 69 (42%) corresponded to BOT. Of these, the most common subtypes were 39 (56.2%) serous, 28 (40.57%) mucinous and 2 (2.8%) endometroid. The concordance results of serous BOT is moderate, 75.71% with a kappa index of 0.5143 (p = 0.000), and mucinous BOT have fair concordance, 65.71% with a kappa index of 0.2398 (p = 0.0222). CONCLUSIONS: BOT correspond to a large percentage of malignant tumors of the ovary, with the serous subtype being the most common. The concordance between contemporary and definitive biopsy is between fair and moderate.

Encefalitis por anticuerpos contra el receptor N-metil-D-aspartato secundaria a teratoma ovárico en una paciente pediátrica / Encephalitis due to antibodies against the N-methyl-D-aspartate receptor secondary to ovarian teratoma in a pediatric patient

La encefalitis por anticuerpos contra el receptor N-metilD-aspartato es un proceso inmunomediado en el que autoanticuerpos se dirigen contra la subunidad GluN1 del receptor de glutamato del sistema nervioso central. Se caracteriza por la aparición aguda o subaguda de síntomas psiquiátricos, como confusión, pérdida de la memoria a corto plazo, cambios de conducta, catatonía, seguidos por manifestaciones neurológicas, tales como convulsiones, alteraciones del movimiento, disfunciones autonómicas, coma y depresión respiratoria. Es grave y potencialmente mortal. Su asociación con teratoma de ovario como síndrome paraneoplásico fue descrita en mujeres jóvenes. En la población pediátrica, es mucho menos frecuente y se reporta en comunicaciones de 1 o 2 pacientes y en series de pocos casos. Se presenta una paciente de 13 años con encefalitis paraneoplásica por anticuerpos contra el receptor N-metil-Daspartato, secundaria a un teratoma ovárico maduro.

The encephalitis due to antibodies against the N-methylD-aspartate receptor is a process immune-mediated in which antibodies are directed against the GluN1 subunit of the glutamate receptor in the central nervous system. It is characterized by an acute or subacute onset of psychiatric symptoms such as confusion, short-term memory loss, behavioral changes, catatonia followed by neurological manifestations such as seizures, movement disturbances, autonomic dysfunctions, coma, and respiratory depression. It is serious and life threatening. Its association with ovarian teratoma as a paraneoplastic syndrome was described in youngwomen. In the pediatric population it is much less frequent and is reported in publications of one or two patients and in series of few cases. We present a 13-year-old patient with encephalitis paraneoplastic due to antibodies against the N-methyl-Daspartate receptor, secondary to a mature ovarian teratoma.

Cistoadenoma volumoso associado a teratoma ovariano maduro, com evolução para pseudomixoma peritoneal: relato de caso / Giant cystadenoma associated with mature ovarian teratoma with progression to pseudomyxoma peritonei: a case report

Massas anexiais representam grande desafio diagnóstico, devido a seu caráter geralmente assintomático¹. Traz-se caso peculiar de paciente que evoluiu com sintomas exuberantes de rápida evolução, situação característica de cistoadenomas, tumores benignos de origem epitelial²,³. Realizada laparotomia, com achados macroscópicos que revelaram conteúdo mucinoso e presença de pelos e neovascularização em seu interior. O anatomopatológico confirmou o diagnóstico de cistoadenoma associado a teratoma ovariano, sendo assim considerado um tumor de colisão ovariana.

Adnexal masses represent a major diagnostic challenge due to its usually asymptomatic characteritcs¹. We present a peculiar case of a patient who evolved with exuberant symptoms of rapid evolution, characteristic of cystadenomas, benign tumors of epithelial origin²,³. Laparotomy was performed, with macroscopic findings that revealed mucinous content and the presence of hairs and neovascularization inside. Pathology confirmed the diagnosis of cystadenoma associated with ovarian teratoma, thus being considered an ovarian collision tumor.

Teratoma ovariano como causa de dor abdominal em criança na idade pré-escolar / Ovarian teratoma as a cause of abdominal pain in preschool children

Introdução: Os tumores ovarianos, raros na infância, apresentam maior incidência entre 8 e 9 anos, tendo pico aos 19 anos. A sintomatologia principal manifesta-se por dor abdominal, massa palpável, febre, constipação e mais raramente polaciúria e disúria. Os teratomas ovarianos maduros embora sejam neoplasias majoritariamente benignas, se malconduzidas, podem evoluir para emergências cirúrgicas. Objetivo: Analisar o teratoma ovariano maduro como possível etiologia de dor abdominal crônica em pacientes pediátricos. Relato de Caso: Criança, 2 anos e 9 meses, levada ao atendimento médico com relato de dor abdominal crônica, 3 meses de evolução, com períodos de agudização, de forte intensidade, associada à disúria e polaciúria conduzida como principal suspeita infecção do trato urinário (ITU). A partir da clínica compatível, associado a leucocitúrias, mesmo não havendo crescimento bacteriano nas culturas, devido à recorrência do quadro, seguiu-se a propedêutica adequada de investigação de ITU de repetição, através do rastreio de má formações das vias urinárias. O ultrassom abdominal total com dinâmica do trato urinário constatou presença de volumosa formação expansiva originada provavelmente em ovário esquerdo. Estendida a pesquisa diagnóstica foi realizada tomografia computadorizada do abdome e pelve com achados sugestivos de teratoma ovariano esquerdo, exercendo comportamento expansivo local com desvio e compressão de estruturas anatômicas circunvizinhas. Com a hipótese diagnóstica evidenciada foi encaminhada à cirurgia pediátrica e oncologia para tratamento adequado, com realização de ooforectomia e seguimento clínico. Conclusão: Importância da propedêutica adequada para casos de dor abdominal crônica em pacientes pediátricos devido à extensão de possíveis diagnósticos diferenciais.

Introduction: Ovarian tumors, rare in childhood, have a higher incidence between 8 and 9 years, peaking at 19 years. The main symptomatology is manifested by abdominal pain, palpable mass, fever, constipation, and more rarely polaciuria and dysuria. Mature ovarian teratomas although they are mostly benign neoplasms, if poorly conducted, they can progress to surgical emergencies. Objective: To analyze mature ovarian teratoma as a possible etiology of chronic abdominal pain in pediatric patients. Case Report: Child, 2 years and 9 months, taken to medical care with report of chronic abdominal pain, three months of evolution, with periods of sharpness, of strong intensity, associated with dysuria and polaciuria and primary suspected urinary tract infection (UTI). From the compatible clinic, associated with leukocytes, though there is no bacterial growth in the cultures, due to the recurrence of the condition, appropriate propaedeutic investigation of repeat UTI was followed by screening for urinary pathway malformations. Total abdominal ultrasound with urinary tract dynamics was requested, which that found the presence of a large expansive formation probably originating in the left ovary. Extended diagnostic research, the computed tomography of the abdomen and pelvis was performed with findings suggestive of left ovarian teratoma exerting local expansive behavior with deviation and compression of surrounding anatomical structures. With the diagnostic hypothesis evidenced, she was referred to pediatric surgery and oncology for appropriate treatment with oophorectomy and clinical follow-up. Conclusion: Importance of adequate propaedeutics for cases of chronic abdominal pain in pediatric patients due to the extent of possible differential diagnoses.

Ovarian metastasis from colorectal adenocarcinoma / Metástase ovariana de adenocarcinoma colorretal

Introduction: Ovarian metastases of gastrointestinal origin, also called Krukenberg tumors, have a guarded prognosis. Physicians need to look for alternatives in diagnosis and treatment for this clinical condition in order to improve the outcome of the patients. Objectives: To report the experience of the authors in the treatment of these patients, and to perform a review of the literature on the epidemiology, clinical presentation, diagnosis, treatment, and prognosis for ovarian metastases from colorectal cancer. Methods: We collected clinical information regarding the patients treated for ovarian metastasis from colorectal adenocarcinoma at our coloproctology service, and performed a search on the PubMed database using the terms colorectal cancer, ovarian metastasis, Krukenberg tumor and surgery. Conclusion: Large abdominal tumors are the most frequent presentation of ovarian metastasis from colorectal cancer. The diagnosis is based on a histopathological analysis, levels of carcinoembryonic antigen (CEA) and cancer antigen 125 (CA-125), and immunohistochemical studies for the cytokeratin 20 (CK20), caudal-type homeobox 2 (CDX2) and vilina markers. Citoreductive surgical procedures are the most promising approach to treatment, with the highest impact on overall survival. The prognosis is negatively influenced by the extent of the metastasis, by citoreductive surgical procedures with persistence of macro- or microscopic foci of the disease, and by low scores on the general well-being index of the patient. (AU)

Introdução: As metástases ovarianas de tumores gastrointestinais, também chamadas de tumores de Krukenberg, são neoplasias de prognóstico reservado. Exigem conhecimento de alternativas diagnósticas e terapêuticas para garantir melhora da sobrevida das pacientes. Objetivos: Relatar a experiência dos autores no tratamento dessas pacientes, e fazer uma revisão da literatura sobre a epidemiologia, apresentação clínica, diagnóstico, tratamento e prognóstico das metástases ovarianas do câncer colorretal. Métodos: Foi realizada uma coleta de informações clínicas de pacientes tratados por metástases ovarianas de adenocarcinoma colorretal em nosso serviço de coloproctologia, em conjunto com uma pesquisa na base de dados PubMed com os termos colorectal cancer, ovarian metastasis, Krukenberg tumor, e surgery. Conclusão: Volumosas massas abdominais constituem a principal apresentação clínica da doença. As alternativas diagnósticas incluem a avaliação histopatológica, a identificação dos níveis de antígeno cárcino-embriônico (ACE) e de antígeno de câncer 125 (CA-125), e exame imunoistoquímico de espécimes cirúrgicos para os marcadores citoqueratina 20 (CK20), homeobox 2 do tipo caudal (CDX2), e vilina. O tratamento citorredutor completo demonstrou o maior impacto na sobrevida dos pacientes. O prognóstico é influenciado negativamente pela extensão da doença metastática, por cirurgia citorredutiva com persistência focos microscópicos ou macroscópicos da doença, e baixo escore de índice de bem-estar geral do paciente. (AU)

Hipercalcemia en paciente pediátrica con disgerminoma ovárico: a propósito de un caso / Hypercalcemia in a pediatric patient with ovarian dysgerminoma: A case report

La hipercalcemia asociada a tumores malignos es una entidad poco frecuente en pediatría (0,5-1,3 % de los cánceres pediátricos). Es causada por diferentes mecanismos fisiopatológicos y los síntomas de presentación suelen ser inespecíficos, pero potencialmente graves. Presentamos un caso clínico de una paciente de 12 años con diagnóstico de disgerminoma ovárico bilateral. La enfermedad se presentó con hipercalcemia grave, tratada con hiperhidratación asociada a diuréticos de asa, bifosfonatos y, por último, la resección quirúrgica del tumor, que permitió la resolución definitiva del cuadro. A pesar de tratarse de un trastorno hidroeléctrico poco habitual en pediatría, dada la potencial gravedad de la hipercalcemia, es importante la detección y el tratamiento tempranos, con el fin de evitar complicaciones en el corto y el largo plazo

Hypercalcemia associated with malignant tumors is a rare entity in pediatrics (0.5-1.3 % of pediatric cancers). It responds to different pathophysiological mechanisms and the typical symptoms shown are usually nonspecific, but potentially serious. We present a clinical case of a 12-year-old patient with a bilateral ovarian dysgerminoma who was diagnosed with severe hypercalcemia at the onset, which required hyperhydration associated with loop diuretics, bisphosphonates and, eventually, the tumor resection surgery that allowed the final resolution of the clinical picture.Despite being a rare hydroelectric disorder in pediatrics, given the potential severity of hypercalcemia, early detection and treatment are important in order to avoid potential short- and long-term complications.

Identification of 5-methylcytosine-related signature for predicting prognosis in ovarian cancer

BACKGROUND: Ovarian cancer is one of the most common malignancies often resulting in a poor prognosis. 5-methylcytosine (m5C) is a common epigenetic modification with roles in eukaryotes. However, the expression and function of m5C regulatory factors in ovarian cancer remained unclear. RESULTS: Two molecular subtypes with different prognostic and clinicopathological features were identified based on m5C regulatory factors. Meanwhile, functional annotation showed that in the two subtypes, 452 differentially expressed genes were significantly related to the malignant progression of ovarian cancer. Subsequently, four m5C genes were screened to construct a risk marker predictive of overall survival and indicative of clinicopathological features of ovarian cancer, also the robustness of the risk marker was verified in external dataset and internal validation set. multifactorial cox regression analysis and nomogram demonstrated that risk score was an independent prognostic factor for ovarian cancer prognosis. CONCLUSIONS: In conclusion, our results revealed that m5C-related genes play a critical role in tumor progression in ovarian cancer. Further detection of m5C methylation could provide a novel targeted therapy for treating ovarian cancer.

Hereditary Breast and Ovarian Cancer Screening Syndrome Profile in Women Diagnosed with Breast Cancer from Paraná State Southwest / Perfil do rastreamento da síndrome hereditária de câncer de mama e ovário em mulheres diagnosticadas com câncer de mama na região sudoeste do Paraná

Abstract Objective This study evaluated the risk of the hereditary breast and ovarian cancer (HBOC) syndrome in patients with breast cancer by using the Family History Screening 7 (FHS-7) tool, a validated low-cost questionnaire with high sensitivity able to screen the HBOC risk in the population. Methods Women diagnosed with breast cancer (n=101) assisted by the Unified Health System at the 8th Regional Health Municipal Office of the state of Paraná answered the FHS-7, and the results were analyzed using IBM SPSS Statistics for Windows, Version 25.0. software (IBM Corp., Armonk, NY, USA). Results The risk of HBOC was 19.80% (n=20). Patients at risk exhibited aggressive tumor characteristics, such as high-grade tumors (30%), presence of angiolymphatic emboli (35%), and premenopausal at diagnosis (50%). Significant associations between the prevalence of high-grade tumors were observed inwomen younger than 50 years at diagnosis with HBOC (p=0.003). Conclusion Our findings suggest a possible family inheritance associated with worse clinical features in women with breast cancer in this population, indicating that HBOC investigation can be initially performed with low-cost instruments such as FHS-7.

Resumo Objetivo Este estudo avaliou o risco da síndrome hereditária de câncer de mama e ovário (HBOC, na sigla em inglês) em pacientes com câncer de mama utilizando a ferramenta Familial History Screening 7 (FHS-7), um questionário validado de baixo custo e com alta sensibilidade capaz de rastrear o risco de HBOC na população. Métodos Mulheres diagnosticadas com câncer de mama (n=101) assistidas pelo Sistema Único de Saúde da 8ª Regional de Saúde do estado do Paraná responderam ao questionário FHS-7, e os resultados foram analisados pelo software IBM SPSS for Windows, Version 25.0. (IBM Corp., Armonk, NY, EUA). Resultados A ocorrência do risco de HBOC foi de 19,80% (n=20). Pacientes em risco exibiram características agressivas do tumor como tumores de alto grau (30%), presença de êmbolos angiolinfáticos (35%) e pré-menopausa ao diagnóstico (50%). Associações significantes foram observadas entre a prevalência de tumores de alto grau e diagnóstico abaixo de 50 anos no grupo HBOC (p=0.003). Conclusão Nossos achados sugerem uma possível herança familiar associada a piores características clínicas em mulheres com câncer de mama nessa população, indicando que a investigação de HBOC pode ser realizada, inicialmente, com instrumentos de baixo custo, como o FHS-7.

Sistema IOTA ADNEX: ¿Confiable como predictor en pacientes con blastoma anexial? / ADNEX IOTA SYSTEM: Reliable as a predictor in patients with adnexal blastoma?

Objetivos: Evaluar el valor predictivo del sistema IOTA ADNEX®, en pacientes con diagnóstico de blastoma anexial. Objetivo secundario: Evaluar otras características sugestivas de malignidad no incluidas en el sistema IOTA ADNEX®. Materiales y métodos: Estudio observacional, retrospectivo, descriptivo. Se incluyeron 42 pacientes con diagnóstico ecográfico inicial de blastoma anexial que se atendieron y fueron operadas en nuestro servicio en el periodo 2013 - 2018, divididas en 2 grupos: Grupo A (21 pacientes con diagnóstico posterior de cáncer de ovario) y Grupo B (Pacientes con diagnóstico benigno postoperatorio). Se evaluó el valor predictivo del sistema IOTA ADNEX®, para dichas pacientes y se comparó los resultados del Grupo A vs Grupo B. Resultados: El aumento de Ca125, se encontró fuertemente asociado al cáncer de ovario. La diferencia entre Grupo A y B fue estadísticamente significativo p<0,0001. Encontramos una asociación entre el GRUPO A, con la predicción de cáncer de ovario, siendo esta diferencia estadísticamente significativa p<0,0001. Conclusión: De acuerdo a nuestros resultados el sistema ADNEX®, podría predecir tanto el riesgo de malignidad como de benignidad de un blastoma anexial de manera fidedigna. Dicho sistema presenta como ventaja el objetivar la interpretación de los estudios y su fácil implementación en todos los ámbitos. La adecuada caracterización e intervención pre quirúrgica permite la planificación del tratamiento mejorando el pronóstico de las pacientes (AU)

ABSTRACT: AIM: To assess the predictive value of the IOTA ADNEX® system, in patients diagnosed with adnexal blastoma. Secondary aim: To evaluate other characteristics suggestive of malignancy not included in the IOTA ADNEX® system. Materials and methods: Observational, retrospective, descriptive study. We included 42 patients with initial ultrasound diagnosis of adnexal blastoma, who were treated and operated in our service 2013-2018 period, divided into 2 groups: Group A (21 patients with subsequent diagnosis of ovarían cancer) and Group B (Patients with benign postoperative diagnosis). The predictive value of the IOTA ADNEX® system was evaluated for these patients and the results of Group A vs Group B were compared. Results: Ca125 was found to be strongly associated with ovarian cancer. The difference between Group A and B was statistically significant p <0.0001. We found an association between GROUP A, with the prediction of ovarian cancer, this difference being statistically significant p <0.0001. Conclusion: According to our results, the ADNEX® system could predict both the risk of malignancy and benignity of an adnexal blastoma reliably. This system has the advantage of objectifying the interpretation of the studies and their easy implementation in all areas. Proper characterization and presurgical intervention allows treatment planning to improve the prognosis of patients (AU)

Tumor de células de la granulosa de tipo juvenil en ovario izquierdo / Juvenile granulosa - cells tumor in the left ovary

RESUMEN Se presentó el caso de una adolescente de 14 años, en el servicio de Cirugía Pediátrica del Hospital Pediátrico Docente "Pedro Agustín Pérez" de Guantánamo, que refirió aumento de volumen de todo el abdomen y dolor abdominal difuso. Al examinarla se constató una tumoración visible y palpable en hemiabdomen inferior. Los estudios complementarios imagenológicos mostraron una masa ecogénica, heterogénea que ocupaba hipogastrio, más lateralizada hacia la izquierda. Tras discusión colectiva multidisciplinaria se le realizó oforectomía izquierda y los estudios anatomopatológicos confirmaron la presencia de tumor de células de la granulosa de tipo juvenil en ovario izquierdo. Técnicas quirúrgicas que permitan preservar la capacidad reproductiva a niñas con neoplasias malignas, resultan usadas ahora con frecuencia y garantizan una mayor calidad de vida.

ABSTRACT A 14-year-old girl presented to the pediatric surgery department at the Pediatric Teaching Hospital "Pedro Agustín Pérez" in Guantanamo. She reported a difuse abdominal pain and distention. The examination revealed a visible and palpable tumor in the lower hemiabdomen. Complementary imaging studies showed an ecogenic and heterogeneous mass situated in the left hypogastrium. After a multidisciplinary team meeting, an ophthalmectomy was performed, and anatomopathological studies confirmed the presence of juvenile granulosa cells tumor on the left ovary. Surgical techniques that allow girls with malignant neoplasms to preserve their reproductive capacity are now frequently used and they guarantee higher life quality.

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.

Practical recommendations for the management of benign adnexal masses / Recomendações práticas para o tratamento de massas anexiais benignas

Abstract Objective To performa comprehensive review to provide practical recommendations regarding the diagnosis and treatment of benign adnexal masses, as well as information for appropriate consent, regarding possible loss of the ovarian reserve. Methods A comprehensive review of the literature was performed to identify the most relevant data about this subject. Results In total, 48 studies addressed the necessary aspects of the review, and we described their epidemiology, diagnoses, treatment options with detailed techniques, and perspectives regarding future fertility. Conclusions Adnexal masses are extremely common. The application of diagnosis algorithms is mandatory to exclude malignancy. A great number of cases can bemanaged with surveillance. Surgery, when necessary, should be performed with adequate techniques. However, even in the hands of experienced surgeons, there is a significant decrease in ovarian reserves, especially in cases of endometriomas. There is an evident necessity of studies that focus on the long-term impact on fertility.

Resumo Objetivo Realizar uma revisão abrangente para fornecer recomendações práticas sobre o diagnóstico e tratamento demassas anexiais benignas, bemcomo informações para um consentimento adequado com relação à possível perda da reserva ovariana. Métodos Uma revisão abrangente da literatura foi realizada para identificar os dados mais relevantes sobre o assunto. Resultados No total, 48 estudos abordaram os aspectos necessários da revisão, e descrevemos sua epidemiologia, diagnósticos, opções de tratamento com técnicas detalhadas, e perspectivas sobre fertilidade futura. Conclusões As massas anexiais são extremamente comuns. A aplicação de algoritmos de diagnóstico é obrigatória para excluiramalignidade. A maioria dos casos pode ser manejada conservadoramente. A cirurgia, quando necessária, deve ser realizada com técnicas adequadas. No entanto, mesmo nas mãos de cirurgiões experientes, há diminuição significativa da reserva ovariana, principalmente nos casos de endometriomas. Há uma evidente necessidade de estudos que enfoquemo impacto das massas anexiais benignas na fertilidade em longo prazo.

Quiste gigante de ovario / Giant cyst of ovary

RESUMEN Históricamente, se describen como tumores de gran tamaño aquellos que pesan más de 12 Kg, entre ellos los ginecológicos y los de ovario; sobre todo antes del advenimiento de la ecografía y en poblaciones de mala situación socioeconómica y nivel cultural, como en los países del continente africano. Se presentó un caso de quiste gigante del ovario, en una paciente de 45 años de edad, que fue atendida en el Servicio de Cirugía General del Hospital Municipal de Bocoio, provincia de Benguela, República de Angola, en el año 2015. Se presentó por un aumento de tamaño del abdomen de varios años de evolución Se le diagnosticó una masa quística dependiente de ovario, por ultrasonografía por no contar con otro medio de diagnostico imagenologico. Se confirmó a través de una laparotomía exploradora la presencia de una tumoración quística gigante del ovario de 20 kg de peso. Esta patología es poco frecuente en la actualidad debido al desarrollo de la Cirugía y de los medios de diagnósticos que permiten su detección precoz. El estudio anatomopatológico ulterior informó un cistoadenoma seroso de ovario izquierdo. La evolución de la paciente fue favorable. Se consideró un caso interesante por lo infrecuente, la poca existencia de reporte de estos casos en la literatura, sobre todo en Cuba, lo que contribuye a aportar conocimientos a la comunidad médica (AU).

ABSTRACT Through the history those tumors weighting more than 12 kg have been described as great size tumors, among them the gynecological and the ovarian ones, especially before the appearance of the ultrasonography, and among populations of bad socio-economic situation and low cultural level, like in the countries of the African continent. The authors presented the case of a giant ovarian cyst in a patient aged 45 years, who assisted in the Service of General Surgery of the Municipal Hospital of Bocolo, province of Benguela, Republic of Angola, in 1915. She arrived to the consultation due to an increase of the abdominal size of several years of evolution. By ultrasonography because there was no other mean of imaging diagnosis, the diagnosis was an ovary-dependent cystic mass. Using laparoscopy it was confirmed the presence of a giant cystic tumor of the ovary of 20 kg weight. This disease is few frequent nowadays due to the development of Surgery and diagnostic means allowing a precocious detection. The subsequent anatomopathologic study informed a serous cystadenoma of the left ovary. The patient had a satisfactory evolution. The case was considered interesting given its infrequency, the scarce existence of reports of cases like this in the literature, especially in Cuba, contributing with knowledge for the medical community (AU).

Diagnostic value of the neutrophil/lymphocyte ratio, platelet/lymphocyte ratio, and thrombocytosis in the preoperative investigation of ovarian masses / Valor diagnóstico das razões neutrófilo/linfócito, plaqueta/ linfócito, e trombocitose na investigação pré-operatória de massas ovarianas

Abstract Objective To evaluate the diagnostic accuracy of cancer antigen 125 (CA125) and complete blood count (CBC) parameters, such as the neutrophil to lymphocyte ratio (NLR), the platelet to lymphocyte ratio (PLR), and thrombocytosis in patients with ovarian masses. Methods The present is a retrospective study conducted at a single tertiary hospital from January 2010 to November 2016. We included consecutive women referred due to suspicious adnexal masses. The CBC and CA125 were measured in the serum of 528 women with ovarian masses before surgery or biopsy. We evaluated the diagnostic performance of the NLR, PLR, platelets (PLTs), CA125, and the associations between them. We tested the clinical utility of the CBC parameters and CA125 in the discrimination of ovarian masses through decision curve analysis (DCA). Results The best balance between sensitivity and specificity was obtained by the associations of CA125 or PLTs ≥ 350/nL, with 70.14% and 71.66%, CA125 or PLTs ≥ 400/ nL, with 67.30% and 81.79%, CA125 or PLR, with 76.3% and 64.87%, and CA125 or NLR, with 71.09% and 73.89% respectively. In the DCA, no isolated CBC parameter presented a higher clinical utility than CA125 alone. Conclusion We showed that no CBC parameter was superior to CA125 in the prediction of the malignancy of ovarian tumors in the preoperative scenario.

Resumo Objetivo Avaliar a acurácia diagnóstica do antígeno de câncer 125 (cancer antigen 125, CA125, em inglês) e dos parâmetros do hemograma como as razões neutrófilo/linfócito (RNL), plaqueta/linfócito (RPL), e trombocitose em pacientes com massas ovarianas. Métodos Este é um estudo retrospectivo realizado em um hospital terciário no período de janeiro de 2010 a novembro de 2016. Foram incluídas de forma consecutiva mulheres encaminhadas por massas anexiais suspeitas. Foram dosados hemogramas e CA125 no soro de 528 mulheres com massas ovarianas antes da cirurgia ou biópsia. Foram avaliados os desempenhos diagnósticos da RNL, da RPL, das plaquetas (PLQs) e do CA125, considerando-os isoladamente e associados entre si. Testamos a utilidade clínica dos parâmetros do hemograma e do CA125 na discriminação das massas ovarianas por análise de curva de decisão (ACD). Resultados Os melhores equilíbrios entre sensibilidade e especificidade foram obtidos por meio das associações do CA125 ou PLQs ≥ 350/nL, com 70,14% e 71,66%, CA125 ou PLQs ≥ 400/nL, com 67,30% e 81,79%, CA125 ou RPL, com76,3% e 64,87%, e CA125 ou RNL, com 71,09% e 73,89%, respectivamente. Conclusão Na ACD, nenhum parâmetro do hemograma isolado se mostrou superior ao CA125 na predição de malignidade de tumores ovarianos no pré-operatório.

Carcinoma endometrioide sincrónico de ovario y endometrio / Ovarian and endometrial synchronous endometrioid carcinoma

ANTECEDENTES: existe una asociación demostrada entre endometriosis y algunas histologías del carcinoma epitelial de ovario. Por otra parte, se ha observado que hasta un 30% de las neoplasias de ovario se presentan de forma concomitante a neoplasias del endometrio. Para considerar la sincronicidad entre estos tumores, estos deben cumplir criterios anatomopatológicos estrictos como los descritos por scully. OBJETIVO: presentar un caso clínico de carcinoma endometrioide sincrónico de ovario y endometrio sobre focos de endometriosis, así como su diagnóstico y manejo. CASO CLÍNICO: paciente de 27 años que consulta por spotting intermenstrual. En la ecografía endocavitaria se observa un pólipo endometrial. Además, se describe un tumor anexial izquierdo de 42mm, trilobulado, con un polo sólido de 17×15mm. Se somete a una polipectomía histeroscópica y quistectomía ovárica laparoscópica. Asimismo, se reseca implante sospechoso en el fondo de saco posterior. El resultado anatomopatológico de las piezas quirúrgicas fue: pólipo endometrial con hiperplasia compleja con atipias y focos de adenocarcinoma endometrioide grado I; el tumor quístico ovárico izquierdo consistente con quiste endometriósico con focos de adenocarcinoma endometrioide. La lesión peritoneal corresponde a un implante de adenocarcinoma endometrioide grado I. El estudio de las características anatomopatológicas y la presencia del implante peritoneal sugieren el diagnóstico de un carcinoma endometrioide ovárico con origen en una lesión endometriósica sincrónico con un carcinoma endometrioide endometrial. CONCLUSIÓN: el diagnóstico diferencial entre la sincronicidad o diseminación de los tumores de ovario y endometrio de estirpe endometrioide supone un reto para el clínico y es fundamental para el correcto manejo de estas neoplasias.

BACKGROUND: there is a demonstrated association between endometriosis and some epithelial ovarian carcinoma histologies. On the other hand, it has been observed that up to 30% of ovarian neoplasms present concomitantly with endometrial neoplasms. To consider synchronicity between these neoplasms, they must meet strict pathological criteria such as those described by scully. OBJECTIVE: to introduce a case of an ovarian and endometrial synchronous endometrioid carcinoma implanted on endometriosis sites, as well as its diagnosis and management. CLINICAL CASE: a 27-year-old patient who consulted because of an intermenstrual spotting. The ultrasound image showed an endometrial polyp. Furthermore, a 42 mm left adnexal trilobal tumor with a 17×15mm solid pole was described. She underwent a hysteroscopic polypectomy and laparoscopic ovarian cystectomy. Likewise, resection of a suspicious implant in the posterior vaginal fornix was done. The pathological result of the surgical pieces was: endometrial polyp with complex hyperplasia with atypia and focal points of grade I endometrioid adenocarcinoma; the left ovarian cystectomy: endometriotic cyst with focal points of endometrioid adenocarcinoma. The peritoneal lesion corresponded to a grade I endometrioid adenocarcinoma implant. The study of the pathological characteristics and the presence of the peritoneal implant suggest the diagnosis of endometrioid ovarian carcinoma originated in a synchronous endometriotic lesion with endometrial endometrioid carcinoma. CONCLUSION: differential diagnosis between the synchronicity or spread of ovarian and endometrial endometrioid cell line carcinomas, is a great challenge and it is essential for the correct management of these neoplasms

Desmoplastic small round cell tumor of the ovary: A case report with a new modality of treatment and review of the literature

Abstract Objective Desmoplastic small round cell tumor (DSRCT) is a rare intraabdominal neoplasm that grows along serosal surfaces and is primarily found in young men. To Keywords date, only 16 cases of ovarian DSRCT have been previously reported in women in the English literature, and no large population-based studies on this topic exist. Case Report We report the case of a 19-year-old virgo with unremarkable past medical history, initially presented with abdominal fullness. After being treated with the optimal treatment modality (primary and secondary surgical debulking, unique chemotherapy, protocol and adjuvant radiotherapy), the patient has remained without tumor disease for 40 months. Conclusion Although the best therapy for patients with DSRCT has yet to be determined, combining complete surgical resection, adjuvant chemotherapy, and radiotherapy is required to prolong survival and to achieve proper quality of life.

Actinomicosis pélvica: a propósito de un caso que simula un tumor de recto / Pelvic actinomycosis: from a case that simulates rectal tumor

Resumen Introducción: La actinomicosis, es una infección crónica rara producida por bacterias del género Actinomyces sp. La afectación pélvica es una de sus formas más infrecuentes y en gran parte de los casos se relaciona al uso de un dispositivo intrauterino de larga data o a una cirugía previa. Como otras enfermedades raras, la infección es conocida como "la gran imitadora" por su variada forma de presentación y particular comportamiento pudiendo simular una neoplasia. El tratamiento es fundamentalmente médico y de buenos resultados. Caso Clínico: Damos a conocer el caso de una paciente que se presentó con un cuadro compatible con un tumor de recto, pero que resultó ser actinomicosis. El diagnóstico se realizó en base a la tinción de Gram, el cuadro clínico y el antecedente de un dispositivo intrauterino abandonado por más de 25 años. Fue corroborado posteriormente mediante anatomía patológica y tratada en forma exitosa con antibióticos por un periodo extendido. Conclusión: Si bien la actinomicosis es una patología infrecuente, debe ser considerada en el diagnóstico diferencial de los pacientes que se presentan con tumores de la pelvis. Un alto índice de sospecha y una actitud diagnóstica activa son fundamentales para un tratamiento oportuno, seguro y eficaz de esta enfermedad.

Introduction: Actinomycosis is a rare chronic infection caused by bacterias of the genus Actinomyces sp. Pelvic involvement is one of its most infrequent forms and in many cases it is related to the use of a longstanding intrauterine device or a previous surgery. Like other rare diseases, the infection is known as "the great imitator" because of its varied form of presentation and its particular behavior, which can simulate a neoplasm. The treatment is fundamentally medical with good results. Case Report: We present the case of a patient who presented with a rectal tumor but that turned out to be Actinomycosis. The diagnosis was made based on the Gram stain, the clinical presentation and the history of an intrauterine device left for more than 25 years. It was subsequently corroborated by pathological anatomy and successfully treated with antibiotics for an extended period. Conclusion: Although actinomycosis is an infrequent pathology, it should be considered in the differential diagnosis of patients who present with tumors of the pelvis. An active diagnostic attitude and a high index of suspicion are fundamental for the timely, safe and effective treatment of this disease.

Epidemiologia de cáncer de ovario colombia 2009- 2016 / Epidemiology of ovarian cancer colombia 2009- 2016

Resumen Introducción el cáncer es una de las enfermedades más temidas por la humanidad y el tumor maligno de ovario no es la excepción. Se caracteriza por su alta agresividad y por presentar síntomas inespecíficos, además de no contar, hasta el momento, con pruebas de tamizaje que permitan una detección precoz, convirtiéndose en uno de los cánceres femeninos con alta mortalidad ocupando el séptimo lugar a nivel mundial. Objetivo Medir la prevalencia, mortalidad y la letalidad asociadas al cáncer de ovario entre 2009 a 2016 en la población colombiana. Método se realizó un estudio descriptivo, transversal, ecológico. A partir de una base de datos en el RIPS de SISPRO y DANE se seleccionaron las mujeres con diagnóstico de tumor maligno de ovario. Resultados se hallaron 36.798 mujeres con diagnóstico de cáncer de ovario, la edad media fue de 63 años con una prevalencia de 31,66 por 100.000 mujeres, en los departamentos de Antioquia, Santander, y Bogotá. Se estimó una tasa de mortalidad de 3,9 por 100.000 mujeres, predominio en educación básica primaria, y régimen de seguridad social contributivo. La letalidad fue de 15,75%. Conclusiones En Colombia la prevalencia, mortalidad y letalidad entre 2009 a 2016 presentó una tendencia al incremento, predominio en casadas, bajo nivel educativo y menor acceso a los servicios de salud. En virtud de lo anteriormente expuesto, se abre la posibilidad de establecer prioridades sanitarias, diseño de futuras estrategias en prevención de la enfermedad en salud pública, detención precoz y con la consecuente disminución de la mortalidad.