Prognostic factors in patients with uterine sarcoma: the SARCUT study.

OBJECTIVE: Uterine sarcomas are a rare and heterogeneous group of malignancies that include different histological sub-types. The aim of this study was to identify and evaluate the impact of the different prognostic factors on overall survival and disease-free survival of patients with uterine sarcoma. METHODS: This international multicenter retrospective study included 683 patients diagnosed with uterine sarcoma at 46 different institutions between January 2001 and December 2007. RESULTS: The 5-year overall survival for leiomyosarcoma, endometrial stromal sarcoma, undifferentiated sarcoma, and adenosarcoma was 65.3%, 78.3%, 52.4%, and 89.5%, respectively, and the 5-year disease-free survival was 54.3%, 68.1%, 40.3%, and 85.3%, respectively. The 10-year overall survival for leiomyosarcoma, endometrial stromal sarcoma, undifferentiated sarcoma and adenosarcoma was 52.6%, 64.8%, 52.4%, and 79.5%, respectively, and the 10-year disease-free survival was 44.7%, 53.3%, 40.3%, and 77.5%, respectively. The most significant factor associated with overall survival in all types of sarcoma except for adenosarcoma was the presence of residual disease after primary treatment. In adenosarcoma, disease stage at diagnosis was the most important factor (hazard ratio 17.7; 95% CI 2.86 to 109.93). CONCLUSION: Incomplete cytoreduction, tumor persistence, advanced stage, extra-uterine and tumor margin involvement, and the presence of necrosis were relevant prognostic factors significantly affecting overall survival in uterine sarcoma. The presence of lymph vascular space involvement and administration of adjuvant chemotherapy were significantly associated with a higher risk of relapse.

Fertility sparing treatment in patients with endometrial cancer (FERT-ENC): a multicentric retrospective study from the Spanish Investigational Network Gynecologic Oncology Group (SPAIN-GOG).

OBJECTIVE: The primary objective was to evaluate the response rate of conservative treatment for endometrial cancer, and the secondary objective was to assess oncological, fertility and obstetric outcomes in patients who underwent fertility preservation treatment. MATERIAL AND METHODS: This multicentre, observational, retrospective study evaluated endometrial cancer patients who underwent fertility-sparing treatment in Spanish centres between January 2010 and January 2020. Seventy-three patients with stage IA endometrioid adenocarcinoma of the uterus were included in the study. RESULTS: The levonorgestrel intrauterine device (LNG-IUD) was the most common fertility-sparing treatment (53.4%), followed by megestrol acetate (20.5%) and medroxyprogesterone acetate (16.4%). During the 24-month follow-up period, the rate of complete response to fertility-sparing management was 74% (n = 54), and 8.2% (n = 6) of patients presented a partial response. Additionally, 13 (17.8%) patients presented with persistent disease and six (8.2%) relapsed after response. The LNG-IUD was associated with a higher complete response rate than the other methods (87.2 vs. 58.8%; p = 0.01). Surgical treatment (at least hysterectomy) was performed in 44 (60.3%) patients as the end of fertility-sparing treatment. Four (5.5%) patients presented relapse after surgery, associated with final FIGO stage III (p = 0.036), myometrial invasion > 50% (p = 0.018) and final tumour grade 2-3 (p = 0.018). The mean follow-up period was 57.8 (range 6-159) months. The 5-year relapse-free survival and overall survival rates were 92.6% [95% CI (81.3, 97.2)] and 93.5% [95% CI (80.7, 97.9)], respectively. During follow-up, three patients (4.1%) died of the disease after completion of surgical treatment. Up to 50.7% of patients included in the study attempted to get pregnant. Of these, the rate of pregnancy was 81.1% (n = 30/37), and reproductive techniques were used for this purpose in 78.4% of cases. CONCLUSIONS: Fertility-sparing management presented a high response rate in patients with endometrial cancer. LNG-IUD was associated with a better response rate compared to the other treatment options. Moreover, in patients using this management method, pregnancy could be achieved using reproductive techniques.

Prognostic factors for recurrence and survival in uncommon variants of vulvar cancer.

PURPOSE: To analyze the prognostic factors of recurrence and overall survival in rare histotypes of vulvar cancer. METHODS: An international multicenter retrospective study including patients diagnosed with vulvar cancer was performed. One hundred centers participated in the study and 2453 vulvar cancer cases were enrolled from January 2001 until December 2005. After exclusion of squamous vulvar cancer, Paget´s disease and vulvar melanoma 112 tumors were analyzed for the present study. RESULTS: The mean age at diagnosis was 64.9 ± 17.2 years. 99 (88.4%) patients had a single lesion, in 25 (22.3%) cases the vulvar tumor involved the midline, and only 13 (11.5%) patients had clinically positive inguinal lymph nodes. The mean size of the lesion was 33.8 ± 33.9 mm. Regarding the surgical treatment, 2 (1.8%) patients underwent skinning vulvectomy, 63 (56.3%) local excision, 41 (36.6%) vulvectomy, 3 (2.7%) exenteration and 3 (2.7%) did not receive any surgical treatment. The mean free surgical margin was 8.2 ± 9 mm and 7 (6.2%) patients presented positive inguinal nodes. Radiotherapy was administered in 22 (19.6%) patients and it was performed postoperatively in all cases; 14 (12.5%) patients received adjuvant chemotherapy. The mean overall follow-up time was 44.1 ± 35.7 months. The risk factors associated with overall survival were chemotherapy and radiotherapy, tumor size and stromal invasion (p < 0.05). The only independent factor significantly associated with global recurrence and absence of metastasis was radiotherapy (p = 0.02 and p = 0.002, respectively). CONCLUSION: Postoperative radiotherapy seems to be the only independent factor associated with recurrence and overall survival in uncommon types of vulvar cancer.

Prognostic factors in patients with vulvar cancer: the VULCAN study.

OBJECTIVE: This study aimed to analyze the prognostic factors for overall and progression-free survival in patients with vulvar cancer. METHODS: This international, multicenter, retrospective study included 2453 patients diagnosed with vulvar cancer at 100 different institutions. Inclusion criteria were institutional review board approval from each collaborating center, pathologic diagnosis of invasive carcinoma of the vulva, and primary treatment performed at the participating center. Patients with intraepithelial neoplasia or primary treatment at non-participating centers were excluded. Global survival analysis and squamous cell histology subanalysis was performed. RESULTS: After excluding patients due to incomplete data entry, 1727 patients treated for vulvar cancer between January 2001 and December 2005 were registered for analysis (1535 squamous, 42 melanomas, 38 Paget's disease and 112 other histologic types). Melanomas had the worse prognosis (p=0.02). In squamous vulvar tumors, independent factors for increase in local recurrence of vulvar cancer were: no prior radiotherapy (p<0.001) or chemotherapy (p=0.006), and for distant recurrence were the number of positive inguinal nodes (p=0.025), and not having undergone lymphadenectomy (p=0.03) or radiotherapy (p<0.001), with a HR of 1.1 (95% CI 1.2 to 1.21), 2.9 (95% CI 1.4 to 6.1), and 3.1 (95% CI 1.7 to 5.7), respectively. Number of positive nodes (p=0.008), FIGO stage (p<0.001), adjuvant chemotherapy (p=0.001), tumor resection margins (p=0.045), and stromal invasion >5 mm (p=0.001) were correlated with poor overall survival, and large case volume (≥9 vs <9 cases per year) correlated with more favorable overall survival (p=0.05). CONCLUSIONS: Advanced patient age, number of positive inguinal lymph nodes, and lack of adjuvant treatment are significantly associated with a higher risk of relapse in patients with squamous cell vulvar cancer. Case volume per treating institution, FIGO stage, and stromal invasion appear to impact overall survival significantly. Future prospective trials are warranted to establish these prognostic factors for vulvar cancer.

Prognostic value of lymph node ratio and number of positive inguinal nodes in patients with vulvar cancer.

OBJECTIVE: To estimate the prognostic significance of lymph node ratio and number of positive nodes in vulvar cancer patients. METHODS: This international multicenter retrospective study included patients diagnosed with vulvar cancer treated with inguinal lymphadenectomy. Lymph node ratio (LNR) is the ratio of the number of positive lymph nodes (LN) to the number of removed LN. Patients were stratified into risk groups according to LNR. LNR was correlated with clinical-pathological parameters. Survival analyses were performed. RESULTS: This analysis included 745 patients. In total, 292 (39.2%) patients had positive inguinal LN. The mean (SD) number of resected and positive LN was 14.1 (7.6) and 3.0 (2.9), respectively. High LNR was associated with larger tumor size and higher tumor grade. Patients with LNRs 0% (N0), >0<20%, and >20% had 5-year overall survival (OS) rates of 90.9%, 70.7%, and 61.8%, respectively (P<0.001). LNR was associated with both local and distant recurrence-free survival (P<0.001). Patients with 0, 1, 2, 3 or >3 positive lymph nodes had 5-year OS rates of 90.9%, 70.8%, 67.8%, 70.8% and 63.4% respectively (P<0.001). In multivariate analysis, LNR (P=0.01) and FIGO stage (P<0.001), were associated with OS, whereas the number of positive nodes (P=0.8), age (P=0.2), and tumor grade (P=0.7), were not. In high-risk patients, adjuvant radiotherapy was associated with improved survival. CONCLUSIONS: LNR provides useful prognostic information in vulvar cancer patients with inguinal LN resection in vulvar cancer. LNR allows for more accurate prognostic stratification of patients than number of positive nodes. LNR seems useful to select appropriate candidates for adjuvant radiation.

European Nationality Influence in the Management of Squamous Cell Vulvar Cancer.

AIMS: Vulvar cancer is a rare disease. In western European countries, the incidence is 1.2 cases per 100,000 women/year. The aim of this study was to analyze the management and disease-free survival (DFS) of vulvar cancer among European countries. METHODS: An international multicenter retrospective study including patients diagnosed of squamous cell vulvar cancer was performed. Countries analyzed included Austria, Germany, Greece, Italy, Latvia, Lithuania, Poland, Portugal, Spain and Turkey. One hundred different centers were enrolled including 1,505 squamous cell tumors treated from January 2001 until December 2005. RESULTS: The mean free surgical margin was 9.93 mm, which was different between countries (p = 0.076); it ranged from 5.79 mm (95% CI 4.44-7.13) in Germany to 33.39 mm (95% CI 22.21-44.58) in Lithuania. The global 5-year overall survival rate was 61.4%, and the global 5-year DFS rate was 32%. DFS times were different between countries as well as the treatments carried out. The country with the shortest DFS time had 15.9 ± 3.1 months compared to the one with the longest time with 66.0 ± 17.4 months. CONCLUSION: Wide differences in treatments and DFS times have been observed between countries. Homogeneous management for squamous cell vulvar cancer is needed to obtain the best survival rates for all patients.

Prognostic Factors of Recurrence and Survival in Vulvar Melanoma: Subgroup Analysis of the VULvar CANcer Study.

OBJECTIVE: The aim of this study was to analyze the prognostic factors related to the recurrence rate and overall survival of vulval melanoma patients by means of a subgroup analysis of the VULvar CANcer study. METHODS: The international multicenter VULvar CANcer study involved 100 international centers, which contributed 2453 vulvar cancer cases. Of the 1727 patients finally included in the study, 42 were suffering from vulvar melanoma (2.4%). RESULTS: The mean follow-up for vulval melanoma patients was 44.1±35.7 months. Recurrence rate was 50%, and the mean recurrence-free survival was 43.5±6.6 months. For local recurrences, the mean recurrence-free interval was 63.3±8.6 months; for metastasis, 33.5±3.5 months. The 5-year recurrence-free survival rate was 28.6%. The mean overall survival for vulvar melanomas was 45.9±4 months and the 5-year overall survival rate was 78.6%. The only factor with prognostic significance regarding local recurrence of vulvar melanoma was tumor size (P = 0.003). American Joint Committee on Cancer staging was the only prognostic factor associated with metastatic disease at recurrence (P < 0.001). Finally, age of patient was significantly associated with overall survival (P < 0.001). CONCLUSIONS: Tumor size and American Joint Committee on Cancer stage were independent prognostic factors associated with local and distant recurrence, respectively. Patients' age was the only independent prognostic factor associated with overall survival.

Real Implication of Fertility-Sparing Surgery for Ovarian Cancer: Reproductive Outcomes.

BACKGROUND: to prove the effectivity of fertility-sparing procedures in early-stage ovarian cancer by assessing pregnancy rates and obstetrical outcomes. METHODS: we performed a retrospective multicenter study among 55 Spanish hospitals, collecting patients from 18 to 40 years old with diagnosis of early-stage ovarian cancer, epithelial (EOC) or non-epithelial (non-EOC), from January 2010 to December 2019. Data on the use of assisted reproductive techniques, pregnancy attempts and obstetrical outcomes were collected. RESULTS: a total of 150 patients met inclusion criteria, 70 (46.6%) EOC and 80 (53.4%) non-EOC. Pregnancy attempts were reported in 51 (34%) patients, with 42 (28%) patients carrying the pregnancy to term. Among them, 30 (71.4%) underwent surgery alone and 12 (28.6%) had additional postoperative chemotherapy. A total of 32 (76.1% patients) had spontaneous pregnancies and 10 (23.9%) required in vitro fertilization. There was only one (2.4%) complication reported. Vaginal delivery was reported in twenty-nine (69%) patients and cesarean section in five (11.9%) patients. CONCLUSIONS: fertility-sparing management for ovarian cancer seems to be an option with proven good pregnancy rates and low complications. The selection of patients must consider strict criteria in order to maintain a good prognosis.

Oncological outcomes of intraperitoneal chemotherapy in advanced ovarian cancer: BRCA mutation role.

INTRODUCTION: The knowledge of BRCA status offers a chance to evaluate the role of the intraperitoneal route in patients selected by biomolecular profiles after primary cytoreduction surgery in advanced ovarian cancer. MATERIALS AND METHODS: We performed a retrospective, multicenter study to assess oncological outcomes depending on adjuvant treatment (intraperitoneal [IP] vs intravenous [IV]) and BRCA status (BRCA1/2 mutated vs. BRCA wild type [WT]). The primary endpoint was to determine progression-free survival. The secondary objectives were overall survival and toxicity. RESULTS: A total of 288 women from eight centers were included: 177 in the IP arm and 111 in the IV arm, grouped into four arms according to BRCA1/2 status. Significantly better PFS was observed in BRCA1/2-mutated patients with IP chemotherapy (HR: 0.35; 95% CI, 0.16-0.75, p = 0.007), which was not present in BRCA1/2-mutated patients with IV chemotherapy (HR: 0.65; 95% CI, 0.37-1.12, p = 0.14). Significantly better OS was also observed in IP chemotherapy (HR: 0.17; 95% CI, 0.06-043, p < 0.0001), but was not present in IV chemotherapy in relation with BRCA mutation (HR: 0.52; 95% CI, 0.22-1.27, p = 0.15). For BRCA WT patients, worse survival was observed regardless of the adjuvant route used. The IP route was more toxic compared to the IV route, but toxicity was equivalent at the long-term follow-up. CONCLUSION: This retrospective study suggests that BRCA status can help to offer an individualized, systematic treatment after optimal primary surgery for advanced ovarian cancer, but is limited by the small sample size. Prospective trials are essential to confirm these results.

Single-port laparoscopic extraperitoneal para-aortic lymphadenectomy.

OBJECTIVE: The aim of this study was to evaluate the feasibility and the safety of single-port extraperitoneal laparoscopic para-aortic lymphadenectomy for patients with gynecologic cancer. METHODS: From July 2012 to January 2013, a total of 7 patients with gynecologic cancer underwent a laparoscopic pelvic and para-aortic lymphadenectomy with a single-port device. An extraperitoneal approach was performed for para-aortic lymphadenectomy using only one 2.5-cm incision on the left side. In 6 patients, additionally, hysterectomy and pelvic lymphadenectomy with conventional laparoscopy were performed to complete the treatment. RESULTS: Aortic dissection was complete in all cases without complications. The median age of the patients was 63 years (range, 48-78 years), and the median patient body mass index was 31 kg/m(2) (range, 19-38 kg/m(2)). The median number of para-aortic nodes was 17 (range, 10-25); the median operative time was 204 minutes (range, 120-300 minutes). The median hospital stay was 4 days (range, 3-6 days). No patient encountered postoperative complications. CONCLUSIONS: This study demonstrates the feasibility of single-port laparoscopic extraperitoneal para-aortic lymphadenectomy.

[Congenital cystic adenomatoid malformation. Consequences of prenatal diagnosis]. / Enfermedad adenomatoidea quística pulmonar congénita. consecuencias de su diagnóstico prenatal.

BACKGROUND: Advances in technology and the specialized training of gynecologists in ultrasound have led to an increase in fetal diagnoses. Congenital cystic adenomatoid malformation (CCAM) is of particular interest because of its difficulty in predicting the disease evolution. OBJECTIVE: To review the cases of prenatal diagnosis of CCAM during the last five years in our hospital, and to analyze their evolution as a consequence of its diagnosis. PATIENTS AND METHODS: Retrospective study that reviewed the cases of CCAM between 2005 and 2010 treated in our hospital. We evaluated gestational age, type of CCAM and evolution in at least the first 12 months. RESULTS: Twenty-one cases were diagnosed (1 for every 2,660 deliveries in our hospital of reference), 3 of them with CCAM type 1 (14.3%), 8 with type 2 (38.1%) and 10 with type 3 (47.6%). Two patients proceeded with a medical interruption of pregnancy; in 11 patients lesions were stable, in eight they disappeared and one fetus suffered severe mediastinal shift with little healthy lung, and died during the first postpartum week. Four of eight cases in which the image disappeared were considered free of disease after birth. Of the 19 cases in which pregnancy was not interrupt, 15 had mediastinal shift and 6 did not; in five of them (83.3%), the image disappeared and only one remained stable. The lesion disappeared in only three cases of the 13 who had mediastinal shift (p < 0.01). Lobectomies were necessary in 8 of 19 cases, four are considered free of the disease and seven are still in follow-up. CONCLUSION: Congenital cystic adenomatoid malformation is a condition in which the council is extremely complex, but most cases evolved favorably. Severe complications such as hydrops are described in up to 25% of all CCAM.

Influence of Clinical and Surgical Factors on Uterine Carcinosarcoma Survival.

BACKGROUND: The aim of this study was to assess the impact of prognostic factors on the survival of patients diagnosed with uterine carcinosarcoma. METHODS: A sub-analysis of the SARCUT study, a multicentric retrospective European study, was carried out. We selected 283 cases of diagnosed uterine carcinosarcoma for the present study. Prognosis factors influencing survival were analyzed. RESULTS: Significant prognostic factors for overall survival were: incomplete cytoreduction (HR = 4.02; 95%CI = 2.68-6.18), FIGO stages III and IV (HR = 3.21; 95%CI = 1.83-5.61), tumor persistence after any treatment (HR = 2.90; 95%CI = 1.97-4.27), presence of extrauterine disease (HR = 2.62; 95%CI = 1.75-3.92), a positive resection margin (HR = 1.56; 95%CI = 1.05-2.34), age (HR = 1.02; 95%CI = 1.00-1.05), and tumor size (HR = 1.01; 95%CI = 1.00-1.01). Significant prognostic factors for disease-free survival were: incomplete cytoreduction (HR = 3.00; 95%CI = 1.67-5.37), tumor persistence after any treatment (HR = 2.64; 95%CI = 1.81-3.86), FIGO stages III and IV (HR = 2.33; 95%CI = 1.59-3.41), presence of extrauterine disease (HR = 2.13; 95%CI = 1.44-3.17), administration of adjuvant chemotherapy (HR = 1.84; 95%CI = 1.27-2.67), a positive resection margin (HR = 1.65; 95%CI = 1.11-2.44), presence of LVSI (HR = 1.61; 95%CI = 1.02-2.55), and tumor size (HR = 1.00; 95%CI = 1.00-1.01). CONCLUSIONS: Incomplete cytoreduction, presence of tumor residual after treatment, advanced FIGO stage, extrauterine disease, and tumor size are significant prognostic factors decreasing disease-free survival and overall survival of patients with uterine carcinosarcoma.

Primary breast angiosarcoma in a young woman.

INTRODUCTION: Angiosarcomas of the breast are a rare subtype of sarcomas that frequently are diagnosed after radiation therapy for primary breast cancer. Primary angiosarcomas are rare entities accounting 0.05% of all malignant breast neoplasm. PRESENTATION OF CASE: We report a case of primary angiosarcoma of the breast in a 25 years woman, with no previous radiotherapy, treated with a total mastectomy followed by radio-chemotherapy. DISCUSSION: Total mastectomy appears to be the only treatment known that has proven to benefit these patients. Adjuvant treatment has not proven value up until today. The 5-year disease free survival for grade 1 tumors can be as high as 76%, and up to 15% for grade 3. CONCLUSION: Due to the rarity of these tumors there is no standard therapies approach.

Prognostic factors for recurrence and survival among patients with invasive vulvar Paget disease included in the VULCAN study.

OBJECTIVE: To identify prognostic factors associated with recurrence and overall survival among patients with invasive vulvar Paget disease. METHODS: An analysis was conducted of patients with invasive vulvar Paget disease included in VULCAN, an international multicenter retrospective study of patients diagnosed with vulvar cancer between January 1, 2001, and December 31, 2005. Data regarding diagnosis, treatment, and follow-up were obtained from the patients' medical records. Univariate and multivariate analyses were performed. RESULTS: Among 1727 patients registered in the VULCAN database, 38 patients had invasive vulvar Paget disease. The mean follow-up time for these patients was 44.1±35.7 months, the overall recurrence rate (local lesions and distant metastases) was 58%, and the mean overall survival time was 58.5±0.5 months. Case load at the treating center inversely correlated with local recurrence (P=0.01). Tumor size and FIGO stage were associated with the presence of distant metastases (P<0.001 for both). Adjuvant therapy (radiotherapy or chemotherapy) was associated with a reduced risk of distant metastases and increased overall survival (P<0.001 for both). CONCLUSION: Local recurrences of invasive vulvar Paget disease were associated with the case load at the treating center. Distant recurrences were associated with tumor size and FIGO stage. It is possible that adjuvant radiotherapy or chemotherapy could offer patients benefits by increasing overall survival rates.

Prognosis and treatment-selection criteria for patients with high-grade stage IIIc-IV serous ovarian, fallopian tube or peritoneal cancer / Pronóstico y selección de tratamiento en pacientes con cáncer seroso de ovario, de trompas de Falopio o peritoneal de alto grado, estadio IIIc-IV

Objective: To determine the impact of implementing strict treatment-selection criteria on the overall outcome of women with high-grade serous advanced stage ovarian, fallopian tube, or primary peritoneal carcinoma. Material and methods: We included patients treated for high-grade serous advanced stage ovarian, fallopian tube, or primary peritoneal carcinoma at our Institution from January 2007 to March 2015. All other non-serous, low-grade histology tumors and secondary cytoreductions were excluded. strict treatment-selection criteria was used to decide on primary cytoreductive surgery versus neoad-juvant chemotherapy and type of adjuvant therapy. Collected data included patient and tumor characteristics, preoperative diagnostic procedures, surgical treatment, perioperative complications, and neoadjuvant and adjuvant chemotherapies. Appropriate statistical tests were used and survival analysis performed. Results: We identified 71 eligible patients. Mean age was 58.5 ± 11.8 years, 28.2% received neoadjuvant chemotherapy, and 77.5% had optimal cytoreductive surgery to < 1 cm residual disease. Major complications were observed in 16.9% of women, with no significant difference between neoadjuvant chemotherapy and primary cytoreductive surgery groups. With a median follow-up of 35.7 months, median overall survival was not achieved and 57.2% of patients were alive 54 months after surgery. A total of 24 out of 71 (33.8%) died of disease, 11 (45.8%) within two years after surgery. Median progression-free survival was 19.5 months (95% CI 14.8-24.3). Conclusions: Applying strict treatment-selection criteria for patients with high-grade serous advanced stage ovarian, fallopian tube, or primary peritoneal carcinoma ensures few surgical complications and excellent survival rates for the majority of these women

Objetivo: determinar el impacto de la implementación de criterios estrictos de selección de tratamiento sobre el pronóstico de las mujeres con carcinoma seroso de ovario, trompa de Falopio o peritoneal primario en estadio avanzado y de alto grado. Material y métodos: entre enero de 2007 y marzo de 2015 se incluyeron pacientes tratadas por carcinoma ovárico seroso avanzado de alto grado, trompa de Falopio o carcinoma peritoneal primario en nuestro hospital. Se utilizaron criterios estrictos de selección de tratamiento para decidir sobre la cirugía citorreductora primaria versus quimioterapia neoadyuvante y el tipo de tratamiento adyuvante. Los datos recogidos incluyeron características del paciente y del tumor, procedimientos diagnósticos preoperatorios, tratamiento quirúrgico, complicaciones perioperatorias y quimioterapias neoadyuvantes y adyuvantes. Se utilizaron pruebas estadísticas adecuadas y se realizó un análisis de supervivencia. Resultados: se incluyeron 71 pacientes. La edad media fue de 58,5 ± 11,8 años, el 28,2% recibió quimioterapia neoadyuvante y el 77,5% tuvo una cirugía citorreductora óptima (< 1 cm de enfermedad residual). Se observaron complicaciones mayores en el 16,9% de las mujeres, sin diferencias significativas entre los grupos de quimioterapia neoadyuvante y de cirugía citorreductora primaria. Con una mediana de seguimiento de 35,7 meses, no se alcanzó la mediana de supervivencia global y el 57,2% de los pacientes estaban vivas 54 meses después de la cirugía. Un total de 24 de 71 (33.8%) murieron de enfermedad, 11 (45.8%) en los dos años después de la cirugía. La mediana de supervivencia libre de progresión fue de 19,5 meses (IC del 95%: 14,8-24,3). Conclusiones: la aplicación de criterios estrictos de selección de tratamiento para pacientes con carcinoma seroso ovárico, de trompa de Falopio o carcinoma peritoneal primario en estadio avanzado de alto grado asegura pocas complicaciones quirúrgicas y buenas tasas de supervivencia para la mayoría de estas pacientes

Prognostic factors associated with local recurrence in squamous cell carcinoma of the vulva.

OBJECTIVE: To analyze the prognostic factors related to the recurrence rate of vulvar cancer. METHODS: Retrospective study of 87 patients diagnosed of vulvar squamous cell carcinoma diagnosed at a tertiary hospital in Madrid between January 2000 and December 2010. RESULTS: The pathological mean tumor size was 35.1±22.8 mm, with stromal invasion of 7.7±6.6 mm. The mean free margin after surgery was 16.8±10.5 mm. Among all patients, 31 (35.6%) presented local recurrence (mean time 10 months; range, 1 to 114 months) and 7 (8%) had distant metastases (mean time, 5 months; range, 1 to 114 months). We found significant differences in the mean tumor size between patients who presented a relapse and those who did not (37.6±21.3 mm vs. 28.9±12.1 mm; p=0.05). Patients with free margins equal or less than 8 mm presented a relapse rate of 52.6% vs. 43.5% of those with free margin greater than 8 mm (p=0.50). However, with a cut-off of 15 mm, we observed a local recurrence rate of 55.6% vs. 34.5%, respectively (p=0.09). When the stromal invasion cut-off was >4 mm, local recurrence rate increased up to 52.9% compared to 37.5% when the stromal invasion was ≤4 mm (p=0.20). CONCLUSION: Tumor size, pathologic margin distance and stromal invasion seem to be the most important predictors of local vulvar recurrence. We consider the cut-off of 35 mm of tumor size, 15 mm tumor-free surgical margin and stromal invasion >4 mm, high risk predictors of local recurrence rate.

Vaginal laparoscopically assisted radical trachelectomy in cervical clear cell adenocarcinoma.

Adenocarcinoma of the cervix is a rare condition that has shown an increase in incidence, especially in the 20- to 34-year-old group. Adenocarcinoma represents about 5-10% of all tumours in this area, and, among these, the clear cell type accounts for 4-9%. This type of tumour affects mainly postmenopausal women but also occurs in young women with a history of prenatal exposure to diethylstilbestrol (DES). The prognosis for adenocarcinoma of the cervix is poor overall and worse for the clear cell variety. This article discusses a case of clear cell adenocarcinoma of the cervix, unrelated to intrauterine exposure to DES, in a woman of childbearing age who wished to preserve her fertility and was therefore treated by radical vaginal trachelectomy and pelvic lymphadenectomy.

Osteoporosis transitoria del embarazo: caso clínico / Transient osteoporosis of pregnancy: clinical case

La osteoporosis transitoria del embarazo es una entidad rara y autolimitada, de etiología desconocida. Se presenta el caso clínico de una mujer de 29 años que comenzó en semana 34 con un cuadro de dolor localizado en cadera derecha con impotencia funcional e inestabilidad en la bipedestación y dificultad para la marcha. Tras finalizar su gestación en semana 39 con un parto eutócico sin complicaciones, se incrementó el cuadro doloroso durante el puerperio precoz, con bloqueo de rotación interna y externa de miembro inferior derecho a la exploración. En la radiografía y en la resonancia magnética se evidenció una fractura intracapsular desplazada de fémur derecho, que precisó corrección quirúrgica. A pesar del tratamiento quirúrgico, el cuadro empeoró, localizándose posteriormente también en el miembro contralateral. Las pruebas de imagen fueron compatibles con una osteoporosis transitoria de ambas caderas. La clínica remitió paralelamente a la resolución radiológica del cuadro de forma definitiva a los 8 meses.

The transient osteoporosis of pregnancy is a rare and self-limited disease with an unknown etiology. We present the case of a 29 years old woman, who started at the 34th week of pregnancy with focalised pain on the right hip, with functional disability and instability in standing and gait difficulty. During the 39th week of pregnancy after giving birth without complication, the pain increased during the early puerperium. The medical examination revealed that the internal and external rotation of the inferior limb was blocked. X-ray and MRI showed a displaced intracapsular fracture in the right femur, which needed a surgical correction. Despite of the treatment, the pain worsened and focalised itself also on the left hip. Imaging tests were consistent with transient osteoporosis of both hips. The pain eased in parallel with the radiological resolution of the osteoporosis until its permanent resolution after 8 months.

Enfermedad de Steinert y embarazo: caso clínico / Steinert's disease and pregnancy: a case report

La enfermedad de Steinert es una enfermedad genética que se hereda con un patrón autosómico dominante, resultado de la expansión de la repetición de trinucleótidos CTG gen en el cromosoma 19, que codifica una proteína quinasa. Es una forma grave de distrofia muscular caracterizada por debilidad generalizada y degeneración muscular. El debut puede ocurrir en cualquier momento desde el nacimiento hasta la edad madura. Las complicaciones durante el embarazo son el aborto espontáneo, parto prematuro, el hidramnios, atonía uterina posparto, distocias intraparto y accidentes anestésicos. Presentamos el caso de una gestante de 37 años, asintomática con una anamnesis familiar sin patología de interés. Fue diagnosticada de diabetes gestacional y polihidramnios inespecífico en la semana 30 de embarazo. Acudió a nuestro centro en semana 35 de gestación por dinámica uterina y metrorragia. Se realizó una cesárea extrayéndose un feto masculino, con graves dificultades respiratorias y miotonía generalizada. Posteriormente, el neonato fue diagnosticado de enfermedad congénita de Steinert. El análisis genético de la madre reveló que ella padecía la misma enfermedad. El diagnóstico de enfermedad congénita de Steinert es muy difícil, sobretodo cuando los padres no son conscientes de la enfermedad. Nuestro objetivo es enfatizar en la importancia de una buena anamnesis y los marcadores que se pueden encontrar por ultrasonidos, como hidramnios, reducción del tono fetal y los movimientos activos, artrogriposis, micrognatia, que nos puede proporcionar al menos una sospecha prenatal.

Steinert's disease is a genetic condition, which is inherited in an autosomal dominant pattern, result from expansion of CTG trinucleotide repeat gene on chromosome 19, enconding a putative protein kinase. It is a severe form of muscular dystrophy marked by generalised weakness and muscular wasting. The onset can be any time from birth to middle age. The complications during pregnancy are miscarriage, premature labour, hydramnios, atonic postpartum hemorrhage, difficulties during delivery, anesthetic accidents. We report the case of a healthy 37 years old pregnant, with an ordinary family anamnesis. She was diagnosed of gestational diabetes and inespecific polyhydramnios during her 30 week of pregnancy. Due to labour contractions and metrorrhagia in the 35 week she came to our emergency department. She underwent a cesarean section delivery of a male baby, who suffered severe breathing difficulties and generalized myotonia. Afterwards, the baby was diagnosticated with Steinert's congenital disease. Following genetic analysis of the mother revealed that she also suffers Steinert's disease. The diagnosis of the congenital Steinert's disease is really difficult, when the parents are unaware of the disease. Our objective is to emphasize in the importance of a good anamnesis and the characters that can be found out by ultrasound like hydramnios, reduction of fetal tone and active movements, micrognathia, arthrogryposis, that can bring us at least to a prenatal suspicion.