Macromastia en la adolescencia: fibroadenoma gigante juvenil de la mama / Macromastia in adolescence: giant juvenile fibroadenoma of the breast

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Comparison of the anogenital distance and anthropometry of the perineum in patients with and without pelvic organ prolapse. / Comparación de la distancia anogenital y antropometría del periné en pacientes con y sin prolapso de órganos pélvicos.

OBJECTIVES: To determine whether there are differences in the anthropometric measures of the perineum for women with symptomatic pelvic organ prolapse who are candidates for surgery, with or without urinary incontinence, and for patients without pelvic floor dysfunction. The main objective was to measure the anogenital distance in its 2 variants: anoclitoral and anofourchette. The anogenital distance appears to be determined prenatally and is influenced by the intrauterine hormonal environment. The secondary objective was to measure the length of the genital hiatus, the perineal body and the distance between the 2 ischial tuberosities. MATERIAL AND METHODS: An observational case-control study was conducted with 58 patients. The cases (n=22) were patients with stages >II 2 in the Baden-Walker classification system. The controls were patients with normal pelvic floors. Measurements were performed with a digital calliper. The patients' tocogynecological history, lifestyle habits and risk factors were recorded. RESULTS: The case patients had a significantly shorter anogenital anofourchette distance than that of the control patients (P=.001), a significantly longer anogenital anoclitoral distance than the control patients (P=.0001) and a significantly longer genital hiatus length than the control patients (P=.02). CONCLUSIONS: This was an observational study with a small sample. We cannot determine whether the difference in these distances are caused by or are the result of this disease. Given that the anogenital distance appears to be determined prenatally, we question whether this changed distance could be a risk factor for developing pelvic floor dysfunction.

Prevalence of endometriosis in epithelial ovarian cancer. Analysis of the associated clinical features and study on molecular mechanisms involved in the possible causality.

PURPOSE OF INVESTIGATION: To determine the prevalence of endometriosis in patients with epithelial ovarian cancer and explore the differences between women with endometrioid and clear-cell histologic subtypes with and without associated endometriosis. MATERIALS AND METHODS: The medical charts of 496 patients with epithelial ovarian cancer at the Hospital Virgin de la Arrixaca (Murcia, Spain) between 1971 and 2010 were reviewed. RESULTS: Endometriosis was present in 27 (5.4%) of the 496 cases (p < 0001), and was associated with the endometrioid histotype in 13/45 cases (29%) and with the clear cell histotype in 7/22 (32%). The prevalence of an association with endometriosis according to histologic type was 28.8% (13/45) for endometrioid carcinoma and 31.8% (7/22) for clear-cell carcinoma. CONCLUSION: Both endometrioid and clear-cell ovarians tumours are associated with pelvic endometriosis. Patients with endometiosis associated ovarian cancer differ from non-endometiosis associated ovarian cancer in their clinical characteristics.

Síndrome de Williams-Beuren. La importancia del diagnóstico precoz y el manejo multidisciplinario. Revisión y presentación de 2 casos / Williams-Beuren syndrome. The importance of early diagnosis and multidisciplinary management. Review and report of 2 cases

El síndrome de Williams-Beuren (SWB) es una enfermedad rara, caracterizada por la presencia de cardiopatía, discapacidad intelectual, rasgos faciales característicos y alteraciones multiorgánicas. La base genética del síndrome es conocida y permite realizar un diagnóstico de certeza de forma precoz. El diagnóstico clínico de sospecha se lleva a cabo por la presencia de anomalías cardiacas características, como la estenosis aórtica supravalvular y/o la estenosis pulmonar, junto con discapacidad intelectual y/o facies peculiar. Los pacientes deben ser tratados de forma multidisciplinaria, dada la variedad de patologías asociadas y el impacto familiar que conlleva el diagnóstico precoz. Presentamos 2 casos de SWB diagnosticados durante el periodo de lactancia, con fenotipos y manifestaciones diferentes dentro del espectro clínico del síndrome (AU)

Williams-Beuren syndrome (WBS) is a rare disease characterized by heart disease, intellectual disability, characteristic facial features and systemic abnormalities. Its genetic background is well known, allowing early diagnosis. So far, clinical diagnosis is generally based on the presence of features such as cardiac supravalvular aortic stenosis and pulmonary artery stenosis in association with intellectual disability and/or unusual faces. Multidisciplinary management is essential for the wide variety of associated anomalies and the family impact that the early diagnosis and prognosis entails. Here we present 2 cases of WBS, diagnosed during the infant period, who showed different phenotypes and clinical findings within the WBS spectrum (AU)

Reproductive outcome after uterine-sparing surgery for endometrial stromal sarcoma.

► This is the second report of pregnancy following endometrial stromal sarcoma (ESS). ► The role of adnexectomy is controversial in stage I ESS. ► Adnexectomy does not appear to affect survival in stage I ESS.

Müllerian anomalies "without a classification": from the didelphys-unicollis uterus to the bicervical uterus with or without septate vagina.

OBJECTIVE: To point out possible discrepancies in the classic hypothesis of Müllerian development and to present cases that support an alternative hypothesis. DESIGN: Case reports. SETTING: University hospital. PATIENT(S): Six women with uterine malformation. INTERVENTION(S): Surgical, clinical, and outpatient evaluation of the anomalies. MAIN OUTCOME MEASURE(S): Assessment of the genital anomalies according to our embryologic investigations and embryologic-clinical classification and standard classifications for Müllerian malformations. RESULT(S): We have included examples of cases presenting with didelphys uterus with normal cervix, didelphys uterus with septate cervix, and bicornuate (or didelphys) uterus with septate cervix and vagina versus septate uterus with double cervix and vagina, septate bicervical uterus with isthmic communication, and normal simplex uterus with septate cervix and vagina. CONCLUSION(S): These case reports prove that the fusion and reabsorption processes in the development of the Müllerian ducts can be affected to different degrees in the superior-convergent and inferior-divergent portions, thus resulting in atypical or transitional cases without a classification. Therefore, for a more appropriate classification of the uterine malformations, even if the same degrees of fusion, resorption, and development defects are used, a separate classification should be made for each superior and inferior uterine segment.

Experimental contributions to the study of the embryology of the vagina.

BACKGROUND: Acién's hypothesis, deduced from patients with malformations of the female genital tract, especially those with renal agenesis and ipsilateral blind hemivagina, affirms the embryology of the human vagina as deriving from the Wolffian ducts and the Müllerian tubercle and could explain the embryological origin of all the female genital malformations reported. In this study, we investigated the hypothesis in rats. METHODS: Twenty-five pregnant rats were used to analyse female embryos (64) from day 15 (stage indifferent) to day 20 postcoitum (vagina completely formed). We performed transverse and longitudinal sections of embryos, haematoxylin-eosin tinction and immunohistochemical staining using markers specific to Wolffian derivatives. We also analysed the presence of these markers in the vagina of four adult rats. RESULTS: The Müller ducts converge until they fuse into one tube, but caudally they diverge and finally they fuse with the 'urogenital sinus bulbs' that are actually the distal portion of the Wolffian ducts according to the immunohistochemical marking with GZ1 and GZ2. The Müllerian tubercle is observed between those elements. Then, the immunohistochemical staining can be seen all along the completely formed vagina, which is also observed in the vagina of the adult rat. CONCLUSION: We prove the participation of Müller tubercle and Wolffian ducts in the formation of the vagina in rats, so we confirm experimentally Acién's hypothesis about the human vagina embryology.

Comparison between the meiotic activities in the male with hypogonadotropic hypogonadism and in the human fetal ovocytes.

In the testicles of males with postpuberal hypogonadotropic hypogonadism, we have detected a preleptotene phase similar to that described in the human ovary. The two characteristic stages of this phase, contraction and despiralization, were noted. In this paper the origin and the significance of preleptotene in relation to the hormonal levels are discussed.