Menopausia quirúrgica en pacientes con mutación BRCA, el papel de la terapia hormonal / Surgical Menopause in Patients with BRCA Mutation, the Role of Hormone Therapy

Introducción: las mujeres con mutación BRCA1/2 (mBRCA) tienen un riesgo aumentado de desarrollar cáncer de mama (CM) y ovario (CO). La salpingo-oforectomía bilateral (SOB) se asocia con la reducción del riesgo del 80% para CO y un 50% para CM. Se recomienda realizarla entre los 35 y 40 años. Como consecuencia se produce una menopausia prematura, con un impacto negativo sobre la calidad de vida por la presencia de síntomas climatéricos, aumento del riesgo de enfermedad cardiovascular, osteoporosis y riesgo de alteración cognitiva. La terapia hormonal (THM) es el tratamiento más eficaz para la prevención de estos síntomas. Estado del arte: distintos estudios han demostrado un mayor riesgo de CM en mujeres posmenopáusicas que reciben THM en particular con terapia combinada, estrógeno + progesterona (E+P). Según el metanálisis de Marchetti y cols., en las mujeres portadoras de mBRCA que recibieron THM, no hubo diferencias en el riesgo de CM comparando E solo con E+P. En el estudio de Kotsopoulos, incluso se encontró un posible efecto protector en aquellas que usaron E solo. Otro estudio en portadoras sanas demostró que, en las mujeres menores de 45 años al momento de la SOB, la THM no afectó las tasas de CM. Sin embargo, en las mujeres mayores de 45 años, las tasas de CM fueron más altas. Como el esquema de E+P se asocia con un mayor riesgo relativo (RR) de CM, las dosis de progestágenos utilizados se deberían limitar, eligiendo derivados naturales de progesterona, de uso intermitente para disminuir la exposición sistémica. Según diferentes guías internacionales, a las portadoras de mBRCA sanas que se someten a una SOB se les debe ofrecer THM hasta la edad promedio de la menopausia. Conclusión: la menopausia prematura disminuye la expectativa de vida; es por ello que una de las herramientas para mejorar y prevenir el deterioro de la calidad de vida es la THM. El uso de THM a corto plazo parece seguro para las mujeres portadoras de mBRCA que se someten a una SOB antes de los 45 años, al no contrarrestar la reducción del riesgo de CM obtenida gracias a la cirugía. (AU)

Introduction: women with BRCA1/2 (mBRCA) mutation have an increased risk of developing breast (BC) and ovarian (OC) cancer. Bilateral salpingo-oophorectomy (BSO) is associated with an 80% risk reduction for OC and 50% for BC. The recommended age for this procedure is 35 to 40 years. The consequence is premature menopause, which hurts the quality of life due to the presence of climacteric symptoms, increased risk of cardiovascular disease, osteoporosis, and a higher risk of cognitive impairment. Hormone therapy (MHT) is the most effective treatment for preventing these symptoms. State of the art: different studies have shown an increased risk of BC in postmenopausal women receiving MHT, particularly with combined therapy, estrogen + progesterone (E+P). According to the meta-analysis by Marchetti et al., in women carrying mBRCA who received MHT, there was no difference in the risk of BC compared to E alone with E+P. In the Kostopoulos study, there was also a possible protective effect in those who used E alone. Another study in healthy carriers showed that in women younger than 45 years at the time of BSO, MHT did not affect BC rates. However, in women older than 45 years, BC rates were higher. As the E+P scheme is associated with a higher RR of BC, the doses of progestogens should be limited, choosing natural progesterone byproducts of intermittent use to decrease systemic exposure. According to various international guidelines, healthy mBRCA carriers undergoing BSO should be offered MHT until the average age of menopause. Conclusion: premature menopause decreases life expectancy, which is why one of the tools to improve and prevent deterioration of quality of life is MHT. Short-term use of MHT appears safe for women with mBRCA who undergo BSO before age 45 as it does not counteract the reduction in the risk of MC obtained by surgery. (AU)

Primary ovarian leiomyosarcoma in a postmenopausal woman diagnosed using immunohistochemistry / Philippine Journal of Obstetrics and Gynecology

@#Primary ovarian leiomyosarcoma is a very rare tumor which is most commonly seen in postmenopausal women. Primary ovarian leiomyosarcoma has a very poor prognosis, with less than 20% of patients being alive at 5 years. The case is a 51‑year‑old female who presented with a lower abdominal mass secondary to an ovarian new growth. An exploratory laparotomy, peritoneal fluid cytology, unilateral salpingo‑oophorectomy with malignant frozen section of affected ovary, which revealed malignant tumor, proceeded with surgical staging total abdominal hysterectomy contralateral salpingo‑oophorectomy, then proceeded to complete surgical staging with infracolic omentectomy, Jackson‑Pratt drain insertion was performed. Microscopic and immunohistochemical findings established the diagnosis of primary ovarian leiomyosarcoma. Surgery is the cornerstone of treatment, while the role of chemotherapy and radiotherapy is still not clear because substantial data are lacking. The prognosis of primary pure ovarian leiomyosarcomas is extremely poor and there is no established treatment modality for this rare type of tumor.

Tumores ováricos en pediatría: revisión de los casos en un servicio de pediatría durante 10 años / Pediatric ovarian tumors: a 10-year review in a pediatric service

Los tumores ováricos, a diferencia de lo que sucede en la edad adulta, son infrecuentes en la población pediátrica. Predomina la estirpe germinal, con altas tasas de supervivencia. El objetivo de este estudio es presentar la epidemiología, clínica, diagnóstico y tratamiento de las pacientes de 0-15 años con diagnóstico, entre 2007 y 2017, de tumor ovárico en nuestro centro. Fueron 8 los casos encontrados de 171 tumores diagnosticados (el 4,7 %), con edad media de presentación de 12,5 años. Predominaban, al momento del debut, alteraciones menstruales, dolor abdominal y aumento de perímetro abdominal. Fueron de tipo germinal 6/8, y el teratoma maduro fue el más frecuente. Todas se diagnosticaron con ecografía abdominal, y se confirmó el diagnóstico en 7/8 con resonancia magnética. Se intervinieron todos los casos; predominó la salpingo-ooforectomía, y una paciente precisó quimioterapia adyuvante. La supervivencia libre de enfermedad fue del 100 %.

Unlike adults, ovarian tumors are infrequent in the pediatric population, predominating the germ line at this age, with high survival rates. The objective is to present the epidemiological, clinical, diagnosis and therapeutic characteristics of 0 to 15-year-old patients diagnosed with ovarian tumor in our center between 2007 and 2017.Eight cases out of 171 diagnosed tumors (4.7 %) were found, with a mean age of presentation of 12.5 years. At the moment of diagnosis, menstrual disturbances, abdominal pain and an increase in abdominal circumference predominated. Six out of eight were germ cell tumors, being the mature teratoma the most frequent one. All cases were diagnosed with abdominal ultrasound scan, confirmed in 7/8 cases with magnetic resonance imaging. All cases underwent surgery, predominating salpingo-oophorectomy with one patient requiring adjuvant chemotherapy. Disease-free survival was 100 %.

Hormone therapy after risk-reducing surgery in patients with BRCA1/BRCA2 mutation: evaluation of potential benefits and safety

SUMMARY Women with mutations in the BRCA 1 and 2 genes are at increased risk for ovarian and breast cancer and therefore candidates for risk-reducing surgery, including salpingo-oophorectomy and mastectomy. Risk-reducing salpingo-oophorectomy (RRSO) is considered the most effective prophylactic measure for ovarian cancer prevention in this group of patients. This procedure involves loss of ovarian function and induced menopause. Estrogen therapy is the most effective treatment for controlling vasomotor symptoms and improving the quality of life of climacteric women. However, the potential hormonal stimulation of these tumors and the risk of breast cancer are a concern regarding the safety of hormone replacement therapy (HRT) in this population. This article aims to review the current evidence regarding the potential benefits and safety of HRT after RRSO.

RESUMO Mulheres portadoras de mutações nos genes BRCA 1 e 2 possuem risco aumentado para cânceres de ovário e mama e, portanto, são candidatas às cirurgias redutoras de risco, incluindo a salpingo-ooforectomia e a mastectomia. A salpingo-ooforectomia redutora de risco (SORR) é considerada a medida profilática mais efetiva para prevenção do câncer de ovário nesse grupo de pacientes. Esse procedimento implica a perda da função ovariana e menopausa induzida. A estrogenioterapia é o tratamento mais efetivo para o controle de sintomas vasomotores e melhora da qualidade de vida de mulheres no climatério. No entanto, a potencial estimulação hormonal desses tumores e o risco de câncer de mama constituem uma preocupação com a segurança da terapia hormonal (TH) nesta população. Este artigo tem como objetivo uma revisão das evidências atuais quanto aos benefícios potenciais e segurança da TH após SORR.

Bilateral lower thoracic erector spinae plane block in open abdominal gynecologic oncology surgery: a cases series / Bloqueio bilateral do plano eretor da espinha torácica inferior em cirurgia oncológica ginecológica aberta por via abdominal: série de casos

Abstract Objective and background: Erector spinae plane block is a novel analgesic truncal block that has been popularized due to its ease of performance and perceived safety. Erector spinae plane block has been postulated to target the ventral rami and rami communicates of spinal nerves, thus providing somatic and visceral analgesia. In this case series, we describe our experience of bilateral erector spinae plane block placed at the low thoracic level in open gynecologic oncology surgery in three patients. Method: Under ultrasound guidance, erector spinae plane blocks were done, preoperatively, at the 8th thoracic transverse process bilaterally. Numeric rating scale for pain and opioid consumption of the first 48 postoperative hours were recorded. Results: Pain scores ranged from 0 to 4 among the three patients and 48 h opioid consumption in oral morphine equivalents of 4, 6 and 18 mg. No adverse events were recorded up to patient discharge from the hospital. Conclusions: Erector spinae plane block provided effective analgesia in our case series. While its true mechanism of action remains obscure, the available case reports show encouraging analgesic results with no adverse events recorded. Formal prospective randomized trials are underway to provide further evidence on its efficacy, failure rate and safety.

Resumo Justificativa e objetivo: O bloqueio do plano do músculo eretor da espinha é um novo bloqueio troncular analgésico popularizado devido à sua facilidade de aplicação e segurança percebida. O bloqueio do plano do músculo eretor da espinha foi postulado para atingir os ramos ventrais e os ramos comunicantes dos nervos espinhais, proporcionando analgesia somática e visceral. Nesta casuística, descrevemos nossa experiência com o bloqueio do plano do músculo eretor da espinha bilateral depositado no nível torácico inferior em cirurgia oncológica ginecológica aberta em três pacientes. Método: Os bloqueios do plano do músculo eretor da espinha guiados por ultrassom foram administrados no pré-operatório, entre o 8° e o 10° processo transverso do tórax bilateralmente. Os valores de uma escala de classificação numérica para dor e consumo de opioides nas primeiras 48 horas de pós-operatório foram registrados. Resultados: Os escores de dor variaram de 0-4 entre as três pacientes e o consumo de opioide em 48 horas equivaleu à morfina oral (4, 6 e 18 mg). Nenhum evento adverso foi registrado até a alta hospitalar das pacientes. Conclusões: O bloqueio do plano do músculo eretor da espinha proporcionou analgesia efetiva em nossa casuística. Embora o mecanismo de ação verdadeiro permaneça obscuro, os relatos de casos disponíveis mostram resultados analgésicos encorajadores, sem eventos adversos registrados. Ensaios prospectivos randômicos formais estão em andamento para fornecer mais evidências sobre sua eficácia, taxa de falha e segurança.

Clinical features and management of women with borderline ovarian tumors in a single center in Brazil / Características clínicas e manejo de mulheres com tumores borderline de ovário em um hospital no Brasil

Abstract Objective The aim of the present study was to describe and analyze data of 57 women with borderline ovarian tumors (BOTs) regarding histological characteristics, clinical features and treatment management at the Department of Obstetrics and Gynecology of the Universidade Estadual de Campinas (Unicamp, in the Portuguese acronym). Methods The present retrospective study analyzed data obtained from clinical and histopathological reports of women with BOTs treated in a single cancer center between 2010 and 2018. Results A total of 57 women were included, with a mean age of 48.42 years old (15.43- 80.77), of which 30 (52.63%) were postmenopausal, and 18 (31.58%) were < 40 years old. All of the women underwent surgery. A total of 37 women (64.91%) were submitted to complete surgical staging for BOT, and none (0/57) were submitted to pelvic or paraortic lymphadenectomy. Chemotherapy was administered for two patients who recurred. The final histological diagnoses were: serous in 20 (35.09%) cases, mucinous in 26 (45.61%), seromucinous in 10 (17.54%), and endometrioid in 1 (1.75%) case. Intraoperative analyses of frozen sections were obtained in 42 (73.68%) women, of which 28 (66.67%) matched with the final diagnosis. The mean follow-up was of 42.79 months (range: 2.03-104.87 months). Regard ingthe current status of the women, 45(78.95%) are alive without disease, 2(3.51%) arealive with disease, 9 (15.79%) had their last follow-up visit > 1 year beforethe performanceof the present study but arealive, and 1 patient(1.75%) died of another cause. Conclusion Women in the present study were treated according to the current guidelines and only two patients recurred.

Resumo Objetivo O objetivo do presente estudo foi descrever uma série de 57 mulheres com tumores borderline de ovário (TBO) em relação às características histológicas, clínicas, e ao manejo do tratamento realizado no Departamento de Obstetrícia e Ginecologia da Universidade Estadual de Campinas (Unicamp). Métodos O presente estudo retrospectivo analisou dados obtidos dos registros clínicos e histopatológicos de mulheres com TBO tratadas em um único centro oncológico de 2010 a 2018. Resultados Um total de 57 mulheres foram incluídas, com uma média de idade de 48,42 anos (15,43-80,77), das quais 30 (52,63%) eram menopausadas, e 18 (31,58%) tinham < 40 anos. Todas as mulheres foram operadas. Um total de 37 mulheres (64,91%) foram submetidas a cirurgia de estadiamento completo para TBO, e nenhuma foi submetida a linfadenectomia pélvica ou paraórtica. O tratamento com quimioterapia foi administrado em duas pacientes que recidivaram. Os diagnósticos histológicos finais foram: seroso em 20 mulheres (35,09%), mucinoso em 26 (45,61%), seromucinoso em 10 (17,54%) e endometrióide em 1 (1,75%). A avaliação histológica intraoperatória foi realizada em 42 (73,68%) das mulheres, das quais 28 (66,67%) foram compatíveis com os diagnósticos finais. O tempo médio de seguimento foi de 42,79 meses (variando de 2,03 a 104,87 meses). Em relação ao status atual das mulheres, 45 (78.95%) estão vivas sem doença, 2 (3,51%) estão vivas com doença, 9 (15.79%) tiveram a última consulta de seguimento há > 1 ano antes da realização do presente estudo, mas estão vivas, e 1 paciente faleceu por outra causa. Conclusão As mulheres do presente estudo foram tratadas de acordo com as recomendações atuais e apenas duas mulheres apresentaram recorrência.

Tumores ováricos en niñas y adolescentes / Ovarían masses in infant-juvenile age

Introducción. El tratamiento quirúrgico adecuado para las pacientes pediátricas con lesiones ováricas es heterogéneo, y en las niñas es conveniente conservar los ovarios. El objetivo de este estudio es analizar los hallazgos relacionados con un grupo de pacientes a las que se operó por lesiones ováricas. Pacientes y métodos. Un estudio retrospectivo realizado durante 13 años con 56 pacientes menores de 17 años. Estas pacientes se dividieron en 3 grupos según el diagnóstico de patología ovárica: 25 tenían lesiones funcionales (quistes y torsión), 18 tenían lesiones ováricas epiteliales y 13 tenían tumores germinales. Se comparó a estos tres grupos en términos de menarquia, torsión, edad, duración, tamaño, dolor, tumor, vómitos, menstruación irregular, ubicación y tipo de operación. Resultados. Enestosgrupos,fueronmásfrecuentes los quistes foliculares, los cistoadenomas serosos y los teratomas. La media de edad de las pacientes fue de 12,18 ± 4,84 años. Los síntomas y signos más frecuentes fueron dolor (85,7%) e hinchazón (37,5%) en la región abdominopélvica. Se observó torsión en 21 pacientes (37,5%); la media del tamaño del tumor fue de 10,46 ± 6,55 cm. Se realizó una salpingoovariectomía (SO, por sus siglas en inglés) en 38 pacientes y una resección del quiste (CE, por sus siglas en inglés) en 18 pacientes. Se observaron más casos de torsión en las pacientes premenárquicas que en las menárquicas y en el grupo de lesiones funcionales. Se realizaron más resecciones quirúrgicas en el grupo de lesiones funcionales, y más SO en los grupos de lesiones epiteliales y tumores germinales. Conclusión. Se considera que la torsión y las patologías ováricas funcionales son frecuentes en la edad premenstrual, y las lesiones malignas son muy raras en todos los grupos etarios, por lo que se recomienda preservar la fertilidad en la cirugía.

Introduction. The appropraite surgical treatment to pediatric patients with ovarian lesions are heterogeneous and ovarian preservation is desirable in children. The aim of this study is to the discuss findings related to a set of patients who were operated on for ovarian lesions. Patients and methods. A retrospective study carried out in 13 years on 56 patients under the age of 17. These patients were divided into 3 groups according to ovarian pathologic diagnosis: 25 with functional (cyts and torsion), 18 with epithelial ovarian lesions and 13 with germ cell tumours. These three groups were compared in terms of menarche, torsion, age, duration, size, pain, mass, vomiting, irregular menstruation, location and operation type. Results. Follicle cysts, serous cyst adenomas and teratomas were the most common in these groups. The mean age of the patients was 12.18+4.84 years. The most common symptoms and signs were abdominal-pelvic pain (85.7%) and swelling(37.5%). Torsion was seen in 21 patients (37.5%), mean mass size was found to be 10.46+6.55 cm. A salpingo-oophorectomy (SO) was performed in 38 patients and cyst excision (CE) was performed in 18 patients. In premenarcheal cases, torsion was seen more in menarcheal cases and in the functional lesion group. CE was performed more often in the functional and t SO was performed often in the epithelial and germ cells groups. Conclusion. Torsion and functional ovarian pathologies are thought to be common in premenstrual ages and malign lesions are very rare in all age groups so we recommend ovarian protective surgery should be preferred.

Complete colonic obstruction secondary to endometriosis: A case report

This is the case of a 39 year old female who underwent an urgent laparotomy because of gut obstruction. Intraoperative impression was rectosigmoid carcinoma with frozen pelvis and assessed to be unresectable. hence a transverse colostomy was done. Although subsequent endoscopic and CEA evaluations gave the impression of intestinal endometriosis, malignancy could not be ruled out. Patient was started on Danazol and when it became ineffective, surgical control was contemplated. CT scan of the abdomen was done to assess for possible presence of abdominal malignancy and with a negative report, patient underwent abdominal subtotal hysterectomy, bilateral salpingo-oophorectomy, segmental resection of the rectosigmoid and appendectomy. Transverse colostomy was closed later. The objective of this paper was to report a case of complete rectosigmoid obstruction secondary to endometriosis, in order to demonstrate the difficulty in establishing an accurate diagnosis and the propensity of colonic endometriosis to mimic colon cancer.