Five Years of Multidisciplinary Care in Hereditary Cancer: Our Experience in a Spanish University Hospital.

OBJECTIVE: To analyse the evolution of a multidisciplinary heredofamilial cancer unit (HFCU) in a university hospital. METHODS: This was a retrospective analysis of the activity of our HFCU in its first 5 years of existence. RESULTS: Between July 2010 and July 2015, 1,518 patients from 1,318 families attended our HFCU. Genetic testing was offered to 862 patients. Of those, 833 (96.6%) accepted testing, with available results for 636 (76.4%). Pathogenic mutations in BRCA1 and BRCA2 were found in 175 patients. Lynch syndrome and adenomatous polyposis were the most frequent syndromes diagnosed (151/175, 86.3%) among 17 different syndromes studied. Of the 404 patients without a previous genetic diagnosis in the family, 62 (15.3%) were found to have mutations in disease-causing genes. Prophylactic surgery and follow-up (33.7%) or follow-up only (66.3%) was proposed for mutation carriers according to international guidelines and patients' preferences. CONCLUSION: We have a high mutation detection rate, genetic test acceptance, and compliance with risk reduction strategies. However, there is room for improvement, especially in genetic testing timing, considering that an increase in the indications for genetic testing is expected.

Intraoperative electron-beam radiation therapy with or without external-beam radiotherapy in the management of paraaortic lymph-node oligometastases from gynecological malignancies.

PURPOSE: To analyze long-term outcomes and prognostic factors in patients with paraaortic lymph-node oligometastases (LNO) from gynecological malignancies treated in a multimodal protocol. METHODS: Patients with a histological diagnosis of LNO gynecological cancer [uterine cervix (n = 14, 40 %), endometrial (n = 18, 51 %), ovarian (n = 3, 9 %)] who underwent surgery with radical intent and intraoperative radiotherapy (IORT), median dose 12.5 Gy) were considered eligible for participation in this study. Additionally, 51 % received external-beam radiotherapy (EBRT). RESULTS: From 1997 to 2012, a total of 35 patients from a single institution were analyzed. With a median follow-up time of 55 months (range 2-148), 5-year loco-regional control (LRC), disease-free survival (DFS) and overall survival (OS) were 79, 44 and 49 %, respectively. On multivariate analysis, no EBRT treatment to the LNO (p = 0.03), and time interval from primary tumor diagnosis to LNO <24 months (p = 0.04) remained significantly associated with locoregional recurrence (LRR). We found on multivariate analysis that only R1 margin status (p = 0.01) was significantly associated with OS. CONCLUSION: From the current series of patients with gynecological LNO, it emerges the fact that EBRT promotes local control. Future prospective studies might be designed according to the predicted risk of LRR focusing on different subgroups.

The use of radiotherapy for early breast cancer in woman at different ages.

Age is an important feature at the time of early breast cancer diagnosis. Radiotherapy is a mandatory component of treatment for breast-conserving strategies in early disease stages. Breast radiotherapy has rapidly evolved in the last 20 years. A tendency to less treatment volume (partial-breast irradiation) and less treatment time (hypofractionation) is consolidated in modern radiation oncology practice. Age and risk for local recurrence guide the decision-making process to electro-optimal treatment. Radiotherapy technological versatility offers multiple options for individualized (risk-age adapted) recommendations.

External-beam radiation therapy after surgical resection and intraoperative electron-beam radiation therapy for oligorecurrent gynecological cancer. Long-term outcome.

PURPOSE: The goal of the present study was to analyze prognostic factors in patients treated with external-beam radiation therapy (EBRT), surgical resection and intraoperative electron-beam radiotherapy (IOERT) for oligorecurrent gynecological cancer (ORGC). PATIENTS AND METHODS: From January 1995 to December 2012, 61 patients with ORGC [uterine cervix (52 %), endometrial (30 %), ovarian (15 %), vagina (3 %)] underwent IOERT (12.5 Gy, range 10-15 Gy), and surgical resection to the pelvic (57 %) and paraaortic (43 %) recurrence tumor bed. In addition, 29 patients (48 %) also received EBRT (range 30.6-50.4 Gy). Survival outcomes were estimated using the Kaplan-Meier method, and risk factors were identified by univariate and multivariate analyses. RESULTS: Median follow-up time for the entire cohort of patients was 42 months (range 2-169 months). The 10-year rates for overall survival (OS) and locoregional control (LRC) were 17 and 65 %, respectively. On multivariate analysis, no tumor fragmentation (HR 0.22; p = 0.03), time interval from primary tumor diagnosis to locoregional recurrence (LRR) < 24 months (HR 4.02; p = 0.02) and no EBRT at the time of pelvic recurrence (HR 3.95; p = 0.02) retained significance with regard to LRR. Time interval from primary tumor to LRR < 24 months (HR 2.32; p = 0.02) and no EBRT at the time of pelvic recurrence (HR 3.77; p = 0.04) showed a significant association with OS after adjustment for other covariates. CONCLUSION: External-beam radiation therapy at the time of pelvic recurrence, time interval for relapse ≥ 24 months and not multi-involved fragmented resection specimens are associated with improved LRC in patients with ORGC. As suggested from the present analysis a significant group of ORGC patients could potentially benefit from multimodality rescue treatment.

Intraoperative electron beam radiotherapy and extended surgical resection for gynecological pelvic recurrent malignancies with and without external beam radiation therapy: long-term outcomes.

OBJECTIVE: To analyze prognostic factors in patients treated with intraoperative electrons containing resective surgical rescue of locally recurrent gynecological cancer (LRGC). METHODS: From January 1995 to December 2012, 35 patients with LRGC [uterine cervix (57%), endometrial (20%), ovarian (17%), vagina (6%)] underwent extended [multiorgan (54%), bone (9%), soft tissue (54%), vascular (14%)] surgery and intraoperative electron-beam radiation therapy [IOERT (10-15 Gy)] to the pelvic recurrence tumor bed. Sixteen (46%) patients also received external beam radiation therapy [EBRT (30.6-50.4 Gy)]. Survival outcomes were estimated using the Kaplan-Meier method, and risk factors were identified by univariate and multivariate analyses. RESULTS: Median follow-up time for the entire cohort of patients was 46 months (range, 3-169). Ten-year rates for locoregional control (LRC) and overall survival (OS) were 58 and 16%, respectively. On multivariate analysis non-EBRT at the time of pelvic re-recurrence [HR 4.15; p = 0.02], no tumor fragmentation [HR 0.13; p=0.05] and time interval from primary tumor to LRR < 24 months [HR 5.16; p=0.01], retained significance with regard to LRR. Non-EBRT at the time of pelvic re-recurrence [HR 4.18; p=0.02] and time interval from primary tumor to LRR < 24 months [HR 6.67; p=0.02] showed a significant association with OS after adjustment for other covariates. CONCLUSIONS: EBRT treatment integrated for rescue, time interval for relapse ≥ 24 months, and not multi-involved fragmented resection specimens are associated with improved LRC in patients with LRGC in the pelvis. Present results suggest that a significant group of patients may benefit from EBRT treatment integrated with extended surgery and IOERT.

[Breast cancer during pregnancy]. / Cáncer de mama durante el embarazo.

As women in western countries delay childbearing, it has been hypothesized that the incidence of breast cancer diagnosed during pregnancy will increase. Breast carcinoma during pregnancy(BCP) put the health of the mother in conflict with that of the fetus. The aim is to give optimal treatment to the mother to maximise the chances of survival, whilst minimising the risk of harm of the fetus. Few breast surgeons or oncologist develop expertise in this area owing the rarity of the association. We report the epidemiology, pathology, clinical picture, therapeutic management and fetal outcome of pregnant women with breast cancer treated in our institution.

Ran stimulates spindle assembly by altering microtubule dynamics and the balance of motor activities.

The guanosine tri-phosphatase Ran stimulates assembly of microtubule spindles. However, it is not known what aspects of the microtubule cytoskeleton are subject to regulation by Ran in mitosis. Here we show that Ran-GTP stimulates microtubule assembly by increasing the rescue frequency of microtubules three- to eightfold. In addition to changing microtubule dynamics, Ran-GTP also alters the balance of motor activities, partly as a result of an increase in the amount of motile Eg5, a plus-end-directed microtubule motor that is essential for spindle formation. Thus, Ran regulates multiple processes that are involved in spindle assembly.

Dependencia química en mujeres / Chemical dependence in women

El presente trabajo es un intento de analizar la fármaco dependencia en mujeres, diferenciándola de la adicción en varones. Se evalúan diversas variables características en relación a los aspectos sicológicos y sociales. Se analiza la situación mundial de la fármaco dependencia y el SIDA. Se discute sobre los tratamienos convencinales y el por qué resulta más difícil la rehabilitación ded la fármaco dependencia. Se plantean alternativas en el tratamiento. Se predsentan dos edstudios nacionales. El p;rimero de ello realizado en el Servicio de Gineco-Obstetricia del Hospital Guillermo Almenara en 100 gestantes para determinar la existencia de adicción a drogas. El segundo, es una encuesta realizada en alumnos de un colegio particular de Lima. con la finalidad de elaborar un Plan de Prevención de Drogas. Dicha encuesta, es en base al cuestionario de CONADIC de la Ciudad de México. Se hace un análisis de los resultados y se elaboran las conclusines

[Pelvic fibromatosis, as a cause of abdominal mass]. / Fibromatosis pélvica, como causa de masa abdominal.

Pelvic fibromatosis was found in a 26-year-old patient who was operated on for a possible uterine myoma. The histopathological diagnosis revealed an idiopathic retroperitoneal fibrosis. A second operation was performed in order to complete the exercise of the mass. Immunohistochemical techniques backed the initial diagnosis of pelvic fibromatosis.

Aproximación al diagnóstico y prevención de consumo de drogas en gestantes / Aproximation at diagnostic and prevention to drugs abuse in pregnancy

En este trabajo, se presenta el estudio de 100 casos de gestantes admitidas por emergencia del Servicio de Gineco-obstetricia del Hospital Nacional Guillermo Almenara Irigoyen y se elebora una ficha de registro para detectar el uso, abuso y adicción a alcohol y drogas.Se analizan los resultados obtenidos y se propone la elaboración de una cartilla que debe ser distribuida a nivel nacional para saber los datos de morbi-mortalidad reales de alcohol y drogas en gestantes y recién nacidos lo que permita efectuar una campaña real de prevención.

Melanotic adenocarcinoma of the anorectum.

A variety of tumors, besides those formed by melanocytes, may contain melanin pigment. Most of them have originated from the neural crest. Epithelial tumors with melanosomes are very unusual. We report the case of a 76-year-old woman with rectal adenocarcinoma that invaded the intestine and vagina extensively. At the anorectal site, melanin pigment was found in the cytoplasm of the malignant neoplastic epithelial cells, and melanosomes were ultrastructurally confirmed at various developmental stages. Melanocytes with cytoplasmic processes spreading between neoplastic cells were also present as the apparent source of the pigment contained in the neoplastic cells.