Retrospective Analysis of Patients with Gynaecological Uterine Sarcomas in a Tertiary Hospital.

Uterine sarcomas are rare and heterogeneous malignancies accounting for 1% to 3% of all gynaecological tumours. There are many histological subtypes recognised, including leiomyosarcomas, endometrial stromal sarcoma, and uterine carcinosarcoma, although the latest has been recently discarded in this group. Despite its low incidence, these types of cancer currently entail multiple challenges, either in diagnostics or clinical management, with a poor prognosis associated. The present work aimed to complete a comparative analysis of the different histological subtypes based on the clinicopathological characteristics of our population, the therapeutic characteristics, and associated prognosis in 161 patients treated in our centre during the period between 1985 and 2020. Moreover, a systematic review grouped a total of 2211 patients with a diagnosis of uterine sarcoma from 19 articles published in 16 countries from 2002 to 2021 was performed, all with retrospective analyses. Our results showed that apart from uterine carcinosarcoma, leiomyosarcoma is the most frequent subtype of uterine sarcoma, with unique clinical, demographic, and survival parameters. To our knowledge, this is the first systematic review conducted in this field and, thus, it shows the difficulties of collecting a significant number of patients per year, a valid reason why multicentre or national registries are recommended to allow a more exhaustive analysis of this pathology.

Maternal mortality trends in Spain during the 2000-2018 period: the role of maternal origin.

BACKGROUND: The available literature indicates that there are significant differences in maternal mortality according to maternal origin in high income countries. The aim of this study was to examine the trend in the maternal mortality rate and its most common causes in Spain in recent years and to analyse its relationship with maternal origin. METHODS: This was a cross-sectional study of all live births as well as those resulting in maternal death in Spain during the period between 2000 and 2018. A descriptive analysis of the maternal mortality rate by cause, region of birth, maternal age, marital status, human development index and continent of maternal origin was performed. The risk of maternal death was calculated using univariate and multivariate logistic regression analyses, with adjustment for certain variables included in the descriptive analysis. RESULTS: There was a total of 293 maternal deaths and 8,439,324 live births during the study period. The most common cause of maternal death was hypertensive disorders of pregnancy. The average maternal death rate was 3.47 per 100,000 live births. The risk of suffering from this complication was higher for immigrant women from less developed countries. The adjusted effect of maternal HDI score over maternal mortality was OR = 0.976; 95% CI 0.95 - 0.99; p = 0.048; therefore, a decrease of 0.01 in the maternal human development index score significantly increased the risk of this complication by 2.4%. CONCLUSIONS: The results of this study indicate that there are inequalities in maternal mortality according to maternal origin in Spain. The human development index of the country of maternal origin could be a useful tool when estimating the risk of this complication, taking into account the origin of the pregnant woman.

Salpingooforectomía bilateral profiláctica en pacientes con BRCA1 o BRCA2 / Prophylactic bilateral salpingo-oophorectomy in patients with BRCA1 or BRCA2

Resumen OBJETIVO: Determinar la prevalencia, factores clínicos o epidemiológicos de cáncer oculto en pacientes BRCA1 o BRCA2 operadas para salpingooforectomía bilateral profiláctica. Evaluar las complicaciones quirúrgicas en las cirugías laparoscópicas. MATERIALES Y MÉTODOS: Estudio observacional, retrospectivo, llevado a cabo en el Hospital Gregorio Marañón entre 2012 y 2018. Se revisaron las salpingooforectomías bilaterales profilácticas practicadas a pacientes con mutaciones confirmadas BRCA1 o 2, no diagnosticadas previamente de cáncer de ovario o trompa. Las principales variables analizadas fueron: antecedentes familiares, edad, índice de masa corporal, hábito tabáquico, mutación genética, cirugías abdominales previas, cáncer de mama, fecha de la cirugía, tiempo quirúrgico, tipo de cirugía, técnica de entrada a la cavidad abdominal y complicaciones quirúrgicas. El análisis estadístico se efectuó con SPSS 17.0. RESULTADOS: Se estudiaron 59 pacientes. La prevalencia de cáncer oculto fue 5 de 59. La edad media de las pacientes con diagnóstico de cáncer oculto en el momento de la cirugía fue 47.8 años. Se encontró antecedente de cáncer de mama en 43 de las 59 pacientes; en este subgrupo se encontró cáncer oculto en 2 pacientes. En el subgrupo sin antecedente de cáncer de mama, la frecuencia fue 3 de 16. No se encontraron diferencias estadísticamente significativas entre ambos grupos (p = 0.118). Todas las pacientes a quienes se diagnosticó cáncer oculto, salvo una, eran fumadoras. La tasa de complicaciones intraoperatorias fue 2 de 51 y 1 de 51 las postoperatorias CONCLUSIONES: Las pacientes con BRCA1 o BRCA2 son un grupo de alto riesgo oncológico que requiere seguimiento y asesoramiento específicos en unidades especializadas de hospitales de tercer nivel de atención.

Abstract OBJECTIVE: To determine the prevalence of occult cancer in BRCA1 and/or BRCA2 patients undergoing prophylactic bilateral salpingo-oophorectomy. To determine associated clinical or epidemiological factors. To evaluate surgical complications in surgeries performed via laparoscopy. MATERIAL AND METHODS: Retrospective observational study conducted at the Gregorio Marañón hospital between 2012 and 2018. Review of prophylactic bilateral salpingo-oophorectomies performed in patients with confirmed BRCA1 and/or 2 mutations and not previously diagnosed with ovarian and/or fallopian cancer. Main variables: family history, age, body mass index, smoking habit, genetic mutation, previous abdominal surgeries, breast cancer, surgery date, surgical time, type of surgery, technique of the entrance to the abdominal cavity, surgical complications. The statistical analysis was performed using SPSS 17.0. RESULTS: 59 patients were included. The prevalence of occult cancer was 5/59. The average age (at the time of surgery) of patients diagnosed with occult cancer was 47.8 years. 43/59 had a history of breast cancer; in this group occult cancer was found in two patients. In the group with no history of breast cancer, frequency of occult cancer was 3/16. No statistically significant differences were found between both groups (p = 0.118). All patients diagnosed with occult cancer, except one, were smokers. The rate of intraoperative complications was 2/51 and 1/51 postoperative. CONCLUSIONS: Patients with BRCA1 and/or BRCA2 mutations are a group of high cancer risk that require specific monitoring and advice in specialized units of third level hospitals.

Gynecological management of patients diagnosed with fanconi anemia / Manejo ginecológico de la paciente diagnosticada de anemia de fanconi

We present the gynecological and clinical management of women diagnosed with Fanconi anemia at our hospital, which is a reference center in Spain. Fanconi anemia is considered a rare disease. It is an autosomal recessive chromosomal instability syndrome in which more than 20 genes are affected. The disease involves progressive bone marrow failure, various congenital abnormalities, and an increased predisposition to cancer; hence the importance of a gynecological management protocol

Presentamos el manejo clínico ginecológico que llevamos a cabo en nuestro hospital de las pacientes mujeres diagnosticadas con anemia de Fanconi; siendo nuestro hospital uno de los hospitales de referencia en nuestro país. La anemia de Fanconi, considerada como una de las enfermedades raras, es una enfermedad autosómica recesiva, con inestabilidad cromosómica. En la actualidad se han descrito más de 20 genes afectados. Clínicamente hay una insuficiencia medular progresiva, diversas anomalías congénitas e incremento a la predisposición a producir cáncer; de allí la importancia de protocolizar la actuación de manejo de estas pacientes desde el punto de vista ginecológico

Implicaciones de la biopsia selectiva de ganglio centinela en cadena mamaria interna sobre la radioterapia adyuvante en mujeres con cáncer de mama sin invasión axilar / Role of internal mammary sentinel lymph node biopsy in adjuvant radiotherapy in women with breast cancer without axillary involvement

Objetivo: Determinar la implicación de la biopsia selectiva de ganglio centinela (BSGC) a nivel de la cadena mamaria interna (CMI) en la radioterapia (RT) adyuvante cuando no se realiza BSGC a nivel axilar en pacientes con cáncer de mama. Pacientes y métodos: Se realizó un estudio analítico retrospectivo seleccionando pacientes con cáncer de mama en estadios precoces diagnosticado entre enero de 2008 y diciembre de 2016. Se incluyeron únicamente aquellas pacientes con indicación de BSGC y migración exclusiva del radiotrazador a CMI. Se recogieron las características clínicas de las pacientes y anatomopatológicas del tumor, el tratamiento quirúrgico, el tratamiento adyuvante, la recurrencia locorregional y la supervivencia global. Resultados: Se diagnosticaron un total de 2.622 carcinomas infiltrantes de mama. Se documentó drenaje exclusivo a CMI en 23 pacientes (2,2%). En el 65,2% (15/23) se localizó el ganglio centinela en CMI y se extrajeron una media de 1,61 ganglios. Se administró RT adyuvante sobre CMI en el 75% de las pacientes que recibieron RT sobre cadenas ganglionares. Se administró quimioterapia en el 52,2% (12/23) y hormonoterapia en el 87% (20/23) de las pacientes. Se encontró asociación estadísticamente significativa entre la localización del ganglio centinela y la RT sobre la CMI (p<0,003) y también entre el resultado del ganglio centinela y la RT a este nivel (p<0,006). Una de las pacientes presentó una recidiva mamaria y otra tuvo metástasis óseas. La mediana de seguimiento fue de 60 meses. No se notificó ningún fallecimiento. Conclusiones: Tanto el éxito en la localización del ganglio como el resultado de la biopsia en CMI modifican de manera significativa la RT a este nivel

Objective: To determine the role of internal mammary sentinel lymph node (IM-SLN) biopsy in adjuvant radiotherapy in female patients with early-stage breast cancer when axillary lymph node biopsy is not performed. Patients and methods: Retrospective cohort study of female patients with early-stage breast cancer diagnosed between January 2008 and December 2016. Only patients with an indication for SLN biopsy and migration of the radiotracer into the internal mammary nodes were included. Data were recorded and analysed on the patients' clinical features, tumour histology, surgical treatment, adjuvant treatment, locoregional recurrence and overall survival. Results: A total of 2,622 invasive breast carcinomas were diagnosed. Twenty-three (2.2%) patients had exclusive drainage to the internal mammary nodes. IM-SLN was detected in 65.2% (15/23) of patients, with a mean of 1.61 nodes harvested per patient. Seventy-five per cent of patients receiving adjuvant radiation therapy received it on the internal mammary chain. Chemotherapy and endocrine therapy were administered to 52.2% (12/23) and 87% (20/23) of patients, respectively. A statistically significant association was found between IM-SLN identification and radiotherapy to the internal mammary chain (P<.003), as well as between IM-SLN involvement and radiotherapy to the internal mammary chain (P<.006). One patient developed breast cancer recurrence and another patient developed bone metastases. The median follow-up was 60 months. No deaths were reported. Conclusions: Both IM-SLN identification and involvement modify the rate of radiotherapy to the internal mammary chain in patients with early-stage breast cancer