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Objectives: Iatrogenic ureteral injury is a severe surgical complication, with a highest incidence of 1.5% in gynecological surgeries. The purpose of this report is to document our initial experience with using methylene blue (MB) to label the ureter in gynecological laparoscopic surgeries and to explore its effectiveness and safety. This is also a novel description of simultaneously visualizing ureteral MB fluorescence and sentinel lymph nodes (SLN's) Indocyanine Green (ICG) fluorescence using the same camera. Methods: This study included patients undergoing gynecological laparoscopic surgeries, with the same surgeon performing all cases. During the early stages of each surgery, rapid intravenous infusion of MB was administered. For cases requiring SLN imaging, we also injected ICG solution into the cervix. Assessment of the included cases was conducted both intraoperatively and postoperatively. The group that had MB fluorescence (Group A) was compared to a control group that did not have it (Group B). Results: A total of 25 patients (Group A) received MB during surgery, demonstrating 45 ureters clearly, with an imaging success rate of 90%. Continuous and clearer fluorescence imaging was achieved in cases with ureteral hydronephrosis. In most patients, ureteral fluorescence was visible 15-20â min after intravenous infusion of MB, and 64% still exhibited fluorescence at the end of the surgery. In patients who had both ICG and MB, dual fluorescence imaging was achieved clearly. Among the included cases, there were no iatrogenic ureteral injuries (0%), which we observed to be lower than in patients who did not receive MB (1.3%). The rate of adverse events was similar in both groups. Conclusion: Using MB fluorescence is an effective and safe method of visualizing the ureters during gynecological surgeries, and can diminish iatrogenic ureteral injury without increased associated adverse events. It therefore may offer promising prospects for clinical application.
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BACKGROUND: Hereditary breast and ovarian cancer syndrome (HBOC) predisposes women to an increased risk mainly of breast and tubo-ovarian cancer. The aim of the study is to investigate whether being diagnosed with HBOC syndrome is itself a risk factor for sexual dysfunction. METHODS: An ad hoc questionnaire, including baseline demographic and clinical data, and the Sexual Function Questionnaire 28 (SFQ28) were administered to HBOC female carriers (study group) and to a control group. RESULTS: After propensity score matching (1:1), we enrolled 202 women, 101 in the study group and 101 in the control group. In a multivariate analysis, we finally found that menopausal status was the only risk factor for a significant low score in the domains Desire (HR 0.66; CI95% 0.47-0.93; p = 0.017), Arousal (Lubrication) (HR 0.52; CI95% 0.34-0.80; p = 0.003), Arousal (Cognitive) (HR 0.64; CI95% 0.44-0.95; p = 0.027), and Orgasm (HR 0.33; CI95% (0.16-0.70; p = 0.004), independent of risk-reducing surgery for gynecological malignancy. Psycho-oncology support is a protective factor for the Enjoyment domain (HR 1.38; CI95% 1.05-1.81; p = 0.022). CONCLUSIONS: HBOC syndrome itself does not affect SFQ28 domains, while menopausal status significantly influences sexual health, with potential mitigating effects of psycho-oncological support.
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Endometrioid ovarian adenocarcinoma is a common subtype of epithelial ovarian cancer that can arise on a background of endometriosis. Maximal cytoreductive effort with an aim to remove all macroscopic disease (achieve R0) is the single independent prognostic factor for survival. Complex multidisciplinary surgeries may be required in order to achieve this.
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Key Clinical Message: A surgical MDT approach to high-complexity surgeries can allow maximal resection in order to achieve disease control and excellent functional outcomes, as demonstrated here for a case of hidradenitis suppurativa in a patient with Crohn's disease. Abstract: Hidradenitis suppurativa is an autoimmune disease characterized by abscess and fistula formation with purulent discharge in intertriginous zones, and is associated with inflammatory bowel disease. We present the case of a patient with severe ongoing hidradenitis suppurativa causing osteomyelitis and affecting the perineum, on a background of Crohn's disease previously treated with panprotocolectomy and permanent ileostomy. The hidradenitis suppurativa was having a severe impact on the patient's quality of life, and she had failed to respond to conservative management. The patient opted for a radical two-step procedure: first her coccyx and sacrum were removed. The second step was a radical bilateral anterior vulvectomy and posterior vaginectomy, with preservation of the uterine body and cervix. An anterolateral thigh flap was used to reconstruct the perineum. This complex procedure required the expertise of multiple surgical specialties, including plastic, general, spinal, and gynecological oncology surgeons to achieve maximal disease resection, minimizing the risk of recurrence.
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It is currently very difficult to compare different management strategies for complex obstetric surgery, such as hysterectomy for severe Placenta Accreta Spectrum (PAS), as there is no widely accepted consensus for the classification of maternal surgical morbidity. Many studies focus on the amount of blood products transfused or admission to intensive care units (ICU). However, these are dependent on local policies and available resources. It also gives an incomplete representation of the entire 'patient journey' after they leave the operating room. Subsequent repeat procedures for lower urinary track damage is arguably worse from the woman's perspective than a short stay on an intensive care unit (ICU) for observation. We suggest a version of the Clavien-Dindo morbidity classification specific to obstetrics. Then employ it to build a quantitative morbidity score which aims to reflect the whole 'patient experience' including the post-operative pathway. We then demonstrate the utility of this system in a cohort of women with Placenta Accreta Spectrum (PAS). The Clavien-Dindo classification was modified to reflect obstetric procedures and a quantitative morbidity measure, the Soleymani and Collins Obstetric Morbidity Score (SaCOMS), was developed based on this. Both were then validated using a survey-based consultation of a panel of experts in PAS and retrospectively applied to a cohort of 54 women who underwent caesarean hysterectomy for PAS. Clinicians with expertise in PAS believe that the Modified Obstetric Clavien-Dindo classification system and the novel SaCOMS tool can improve assessment of maternal morbidity, and better reflect the 'patient experience'. Application of the classification system to a single-centre PAS cohort suggested that surgery by gynecologic-oncology surgeons may be associated with decreased incidence and cumulative morbidity outcomes for women with PAS, especially those with the most severe presentation. This study presents a clinically useful obstetric-specific classification system for surgical morbidity. SaCOMS also provides a quantitative reflection of the full patient- journey experienced as a result of surgical complications enabling a more patient-centered representation of morbidity.
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Histerectomía , Placenta Accreta , Humanos , Femenino , Placenta Accreta/cirugía , Embarazo , Histerectomía/efectos adversos , Adulto , Cesárea/efectos adversos , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/diagnóstico , Índice de Severidad de la EnfermedadRESUMEN
INTRODUCTION: Vulval cancer is a rare gynaecological malignancy. In this study, we present a tertiary centre case analysis to examine the recurrence patterns and survival outcomes of vulval squamous cell carcinoma (SCC). METHODS: This is a retrospective cohort study of women who received treatment at Oxford University Hospitals between February 2010 and July 2022 for primary vulval SCC. RESULTS: We included 98 cases. The median age at diagnosis was 68 years. Human Papillomavirus (HPV) infection and lichen sclerosis were observed in 21 and 50 cases, respectively. Surgical excision was the primary treatment. Recurrence within 2 years was more common with advanced stage (p = 0.047, RR = 2.26) and extracapsular lymph node spread (p = 0.013, RR = 2.88). Local recurrence was not associated with a specific cut-off value for tumour-free margin. Poor survival outcomes were observed with higher grade (p = 0.01), advanced FIGO stage (p < 0.001), HPV-independent cancer (p = 0.048), lymph node involvement (p < 0.001, HR = 7.14), extracapsular spread (p < 0.001, HR = 7.93), lymphovascular space invasion (p = 0.002, HR = 3.17), tumour diameter wider than 23 mm (p = 0.029, HR = 2.53) and depth of invasion more than 6 mm (p = 0.006, HR = 3.62). Perineural invasion is associated with shorter disease-free survival. Five-year cancer-specific survival rates for stages I, III, and IV were 90.2%, 40.8%, and 14.3%, respectively.
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Carcinoma de Células Escamosas , Metástasis Linfática , Recurrencia Local de Neoplasia , Estadificación de Neoplasias , Centros de Atención Terciaria , Neoplasias de la Vulva , Humanos , Femenino , Neoplasias de la Vulva/patología , Neoplasias de la Vulva/cirugía , Neoplasias de la Vulva/mortalidad , Carcinoma de Células Escamosas/patología , Carcinoma de Células Escamosas/mortalidad , Carcinoma de Células Escamosas/cirugía , Estudios Retrospectivos , Anciano , Persona de Mediana Edad , Pronóstico , Tasa de Supervivencia , Infecciones por Papillomavirus/complicaciones , Anciano de 80 o más Años , Adulto , Clasificación del Tumor , Márgenes de Escisión , Invasividad NeoplásicaRESUMEN
Endometriosis may contribute to Mullerian adenosarcoma development but makes diagnosis challenging given similar symptoms. Survival benefit has not been definitively shown for chemotherapy, hormonal therapy, or radiotherapy, consolidating surgery as the mainstay treatment. Local excision may be a treatment option for patients with confined tumors wishing to preserve their fertility.
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Background: Lymph node metastasis in vulvar cancer is a critical prognostic factor associated with higher recurrence and decreased survival. A survival benefit is reported with adjuvant radiotherapy but with potential significant morbidity. We aim to clarify whether there is high-quality evidence to support the use of adjuvant radiotherapy in this setting. Objectives: The aim of the study was to assess the effectiveness and safety of adjuvant radiotherapy to locoregional metastatic nodal areas. Search Methods: We conducted a comprehensive and systematic literature search of MEDLINE, Embase, Cochrane Central Register of Controlled Trials, Google Scholar, ClinicalTrials.gov, and the National Cancer Institute. We considered only randomized controlled trials (RCTs). Main Results: We identified 1,760 records and finally retrieved only one eligible RCT (114 participants with positive inguinofemoral lymph nodes). All women had undergone radical vulvectomy and bilateral inguinal lymphadenectomy and had been randomized to adjuvant radiotherapy or to intraoperative ipsilateral pelvic lymphadenectomy without adjuvant radiotherapy. At 6 years, the overall survival (OS) was 51% versus 41% in favor of radiotherapy (HR 0.61; 95% CI 0.30-1.3) without significance and with very low certainty of evidence. At 6 year, the cumulative incidence of cancer-related deaths was 29% versus 51% in favor of adjuvant radiotherapy (HR 0.49; 95% CI 0.28-0.87). Recurrence-free survival at 6 years was 59% after adjuvant radiotherapy versus 48% after pelvic lymphadenectomy (HR 0.39; 95% CI 0.17-0.88). Three (5.3%) versus 13 (24.1%) groin recurrences were noted, respectively, in the adjuvant radiotherapy and pelvic lymphadenectomy groups. There was no significant difference in acute toxicities for pelvic lymphadenectomy compared to radiotherapy. In women with positive pelvic lymph nodes (20%), the OS at 6 year was 36% compared with 13% in favor of adjuvant radiotherapy. Late cutaneous toxicity rate appeared to be greater after radiotherapy (19% vs. 15%) but with less chronic lymphedema (16% vs. 22%). Conclusion: There is only very low-quality evidence on administering adjuvant radiotherapy for inguinal lymph node metastases. Although the identified study was a multicenter RCT, there was a reasonable imprecision and inconsistency because of small study numbers, wide confidence intervals in the data, and early trial closure, resulting in downgrading of the evidence.
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OBJECTIVES: Enhanced recovery after surgery (ERAS) protocols provide well-known benefits in the immediate recovery with a shorter length of stay (LOS) and also in gynecological surgery. However, the impact of ERAS has not been clearly showed yet regarding long-term consequences and health-related quality of life (HRQL). The aim of this study was to investigate the impact of ERAS on HRQL after hysterectomy for endometrial cancer. DESIGN: An observational retrospective study with propensity score matching (PSM) was performed. PARTICIPANTS: We administered the SF-36 validated questionnaire to women underwent hysterectomy and lymph nodal staging before and after introducing ERAS protocol, getting, respectively, a standard practice (SP) and ERAS group. SETTINGS: The study was conducted at the academic hospital. METHODS: We collected demographic, clinical, surgical and postoperative data and performed a PSM of the baseline confounders. We administered the questionnaire 4 weeks after the surgery. The SF-36 measures HRQL using eight scales: physical functioning (PF), role physical (RLP), bodily pain (BP), general health (GH), vitality (Vt), social functioning (SF), role emotional (RLE) and mental health (MH). RESULTS: After PSM, we enrolled a total of 154 patients, 77 in each group (SP and ERA). The two groups were similar in terms of age, BMI, anesthetic risk, Charlson comorbidity index (CCI), and surgical technique (minimally invasive vs. open access). Median LOS was shorter for ERAS group (5 vs. 3 days; p = 0.02), while no significant differences were registered in the rates of postoperative complications (16.9% vs. 17.4%; p = 0.66). Response rates to SF-36 questionnaire were 89% and 92%, respectively, in SP and ERAS group. At multivariate analyzes, the mean scores of SF-36 questionnaire, registered at 28 days weeks after surgery (range 26-32 days), were significantly higher in ERAS group for PF (73.3 vs. 91.6; p < 0.00), RLP (median 58.3 vs. 81.2; p = 0.02), and SF (37.5 vs. 58.3; p = 0.01) domains, when compared to SP patients. LIMITATIONS: Further follow-up was not possible due to the anonymized data derived from clinical audit. CONCLUSIONS: ERAS significantly increases the HRQL of women who underwent surgery for endometrial cancer. HRQL assessment should be routinely implemented in the ERAS protocol.
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Neoplasias Endometriales , Recuperación Mejorada Después de la Cirugía , Histerectomía , Calidad de Vida , Humanos , Femenino , Neoplasias Endometriales/cirugía , Neoplasias Endometriales/psicología , Persona de Mediana Edad , Estudios Retrospectivos , Anciano , Encuestas y Cuestionarios , Puntaje de Propensión , Tiempo de Internación/estadística & datos numéricosRESUMEN
Chronic, severe Crohn's disease in a young female patient can result in surgical complexity. The rarity of the presentation of intractable pelvic abscesses within this etiology with additional considerations given to fertility concerns and hence requirement for input from a multi-disciplinary team makes this a vital case in building a consensus for evidence-based surgical management. A 29-year-old nulliparous woman was referred to our tertiary centre for surgical management of Crohn's disease with known tubo-ovarian abscess and abdominoperineal and abdominal wall sinuses. Her previous surgical history included 4 midline laparotomies, subtotal colectomy and proctectomy with stoma formation. The patient underwent egg collection to preserve fertility. This was followed by midline laparotomy and abdominoperineal resection, which involved a retrograde radical modified hysterectomy using the Hudson technique, alongside excision of the perineal sinus, with reconstruction of the perineal defect using an internal pudendal artery perforator gluteal fold flap, and in addition to excision and drainage of the abdominal wall abscess. Involvement was sought from gynecological oncology, colorectal, urology, plastics, stoma, fertility, microbiology, and gastroenterology teams, which enabled successful preservation of end organ function and improvement in patient psychological well-being. This case is a paradigm of surgical challenge, requiring expert gynecological oncology techniques including a retroperitoneal approach, nerve and vessel sparing considerations alongside colorectal and urological procedures. Moreover, we believe that our blueprint for effective multi-disciplinary practice will inform the future management of gynecological surgery. Therefore this report aims to contribute towards the optimum management of the gynecological sequelae of Crohn's disease.
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Neoplasias Colorrectales , Enfermedad de Crohn , Humanos , Femenino , Adulto , Enfermedad de Crohn/complicaciones , Enfermedad de Crohn/cirugía , Absceso/etiología , Absceso/cirugía , Pelvis , Grupo de Atención al PacienteRESUMEN
Large loop excision of the transformation zone is an extremely common procedure routinely carried out in a gynaecology or colposcopy outpatient setting under local anaesthetic. Here, we present a rare case resulting in emergency hysterectomy. A healthy para 3, who had been diagnosed with microscopic cancer of the cervix, attended colposcopy for repeat excision. The colposcopy revealed a normal cervix, and diathermy loop excision was performed. During the procedure, heavy bleeding from the anterior cutting edge was noted. Despite the best attempts to manage the haemorrhage conservatively in outpatients, the bleeding persisted, and the patient was transferred to theatres. Examination under anaesthesia revealed an injury to the descending branch of the uterine artery, and emergency hysterectomy was performed. Immediate recognition of an extremely rare complication, fast decision-making and a cross-disciplinary approach led to a satisfactory outcome.
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Displasia del Cuello del Útero , Neoplasias del Cuello Uterino , Femenino , Embarazo , Humanos , Colposcopía/efectos adversos , Neoplasias del Cuello Uterino/cirugía , Displasia del Cuello del Útero/cirugía , Arteria Uterina/cirugía , Histerectomía/efectos adversos , Enfermedad IatrogénicaRESUMEN
The study of fallopian tube (FT) function in health and disease has been hampered by limited knowledge of FT stem cells and lack of in vitro models of stem cell renewal and differentiation. Using optimized organoid culture conditions to address these limitations, we find that FT stem cell renewal is highly dependent on WNT/ß-catenin signaling and engineer endogenous WNT/ß-catenin signaling reporter organoids to biomark, isolate, and characterize these cells. Using functional approaches, as well as bulk and single-cell transcriptomics analyses, we show that an endogenous hormonally regulated WNT7A-FZD5 signaling axis is critical for stem cell renewal and that WNT/ß-catenin pathway-activated cells form a distinct transcriptomic cluster of FT cells enriched in extracellular matrix (ECM) remodeling and integrin signaling pathways. Overall, we provide a deep characterization of FT stem cells and their molecular requirements for self-renewal, paving the way for mechanistic work investigating the role of stem cells in FT health and disease.
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Trompas Uterinas , beta Catenina , Femenino , Humanos , beta Catenina/metabolismo , Trompas Uterinas/metabolismo , Transcriptoma/genética , Células Madre/metabolismo , Vía de Señalización Wnt , Organoides/metabolismo , Proteínas Wnt/genética , Proteínas Wnt/metabolismo , Receptores Frizzled/metabolismoRESUMEN
Background: Mucinous ovarian carcinomas (MOCs) are rare ovarian tumours accounting for 3% of all epithelial ovarian carcinomas (EOCs). They are either expansile or infiltrative, based on the tumour's histological pattern of invasion. MOCs have a distinct molecular profile, natural history, chemo-sensitivity, and prognosis compared to other EOCs. The aim of this study was to describe patient and tumour characteristics, as well as survival outcomes of expansile and infiltrative primary MOCs. Methods: This was a retrospective cohort study conducted at a tertiary cancer centre. Patients had surgery for primary MOC between Jul 1, 2010 and Oct 28, 2022. All patients discussed at the Oxford multidisciplinary team (MDT) meeting with a diagnosis of MOC were included. We excluded patients with mucinous metastatic carcinoma (MMC), dual histological diagnoses, those who died before treatment was initiated, and patients with incomplete records. Results: A total of 47 patients were identified and 14 were excluded. Out of the remaining 33 MOCs, 23 (70.6%) were expansile and 10 (30.4%) were infiltrative. The median follow-up was 37 months (95% CI: 14.1-69.8). Patients with infiltrative tumours were older than those with expansile tumours (median age 62 vs. 55 years, P=0.049). Infiltrative tumours were diagnosed at a more advanced International Federation of Gynaecology and Obstetrics (FIGO) stage compared to expansile tumours: FIGO stage II/III 50% vs. 8.2% (P=0.002). We found paired-box gene 8 (PAX8) more frequently expressed in expansile tumours (75% vs. 37.5%, P=0.099). Adjuvant treatment was administered in 50% of patients with infiltrative disease, compared to only 13% of those with expansile disease (P=0.036). 80% of patients who have relapsed had received adjuvant chemotherapy, compared to 17.2% of patients without relapse (P=0.012). At 3 years, there was a statistically significant difference in progression-free survival (PFS) (94.7% vs. 65.6%, P=0.02) between the expansile and infiltrative groups, but no difference in overall survival (OS) (88.8% vs. 90%, P=0.875). Conclusions: Patients with infiltrative tumours were older, more likely to have bilateral tumours and more likely to have an advanced FIGO stage at diagnosis. Adjuvant treatment was more likely to be administered to patients with infiltrative tumours, however, this did not prevent relapse. PFS at 3 years was significantly higher in patients with expansile tumours. PAX8 was more frequently expressed by expansile tumours.
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Mixed endometrial carcinoma (MEEC) refers to rare endometrial tumours that are composed of two or more distinct histotypes, at least one of which is serous or clear cell. The aim of this study was to evaluate the epidemiology, treatment outcomes and survival rates of patients with mixed endometrial carcinoma. The medical records of 34 patients diagnosed with MEEC between March 2010 and January 2020 were reviewed retrospectively. Clinicopathological variables and treatment strategies were assessed, and overall survival and disease-free survival rates were evaluated. The histology of endometrioid and serous component was found in 26 (76.5%) patients, followed by serous and clear-cell components (5/34, 14.5%) and mixed endometrioid serous and clear-cell components (3/34, 8.8%). The median age at diagnosis was 70 years (range 52-84), and the median follow-up time was 55 months. The 5-year disease-free survival and the 5-year overall survival were 50.4% and 52.4%, respectively. Advanced disease stage was identified as an independent predictor of inferior disease-free (<0.003) and overall survival (p < 0.001). Except for stage, none of the traditional prognostic factors was associated with disease recurrence or death from disease. MEECs represent rare high-risk endometrial carcinomas with significant diagnostic and treatment challenges. Undoubtedly, the implementation of a molecular analysis can offer further diagnostic and management insights.
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Key Clinical Message: Radical gynecology oncology surgeries are feasible in patients refusing blood transfusion, when performed with careful preoperative (with hemoglobin optimization and patients' counseling), intraoperative (with hemostasis and stepwise devascularization, hemodilution, and autologous cell salvage) and postoperative (considering iron infusion or erythropoietin) planning with a multidisciplinary team involvement. Abstract: We describe the case of a female Jehovah's Witness patient in her 60s undergoing pelvic exenteration, focusing on the preoperative, intraoperative, and postoperative measures that allowed an uncomplicated surgery without blood transfusion. Blood transfusions are common in the surgical management of gynecology oncology patients, up to 93% of patients undergoing pelvic exenteration may require blood products. However, increasingly more patients are cautious in receiving blood products, either for fear of potential risks or for religious believes. It is therefore vital to optimize the management of these patients in order to avoid blood transfusions. In this case, we summarize the management of a lady in her 60s who underwent laparotomy, pelvic exenteration, Bricker colicureterostomy, and end colostomy formation for recurrent endometrial carcinoma, despite previous total abdominal hysterectomy and bilateral salpingo-oophorectomy followed by brachytherapy, chemotherapy, and external beam radiotherapy for high-grade serous carcinoma. Preoperatively, an advance decision to refuse blood products was discussed to ascertain all the options that were suitable. As her preoperative hemoglobin was acceptable (127 g/L), no further intervention was required. Intraoperatively, blood loss was effectively minimized with meticulous hemostasis, stepwise pelvic devascularization, intraoperative hemodilution, and cell salvage. Despite these interventions, total blood loss was 1030 mL and postoperative hemoglobin was 113 g/L. Postoperative measures therefore included intravenous iron infusion, minimization of phlebotomy, and optimization of cardiopulmonary status. Erythropoietin was also considered, but was not necessary as patient responded to the previous measures well and was successfully discharged after an uncomplicated recovery. Only few cases of total pelvic exenteration have been described in the literature for Jehovah's Witness patients. However, our case shows that laparotomy and pelvic exenteration is feasible in patients refusing blood products, if performed under a multidisciplinary team and with careful preoperative, intraoperative, and postoperative planning, also in the setting of previous radical hysterectomy and co-adjuvant therapy.
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BACKGROUND: Mesonephric adenocarcinoma (MA) of the vagina is a rare tumor that arises from mesonephric remnants (Wolffian) in the female genital tract. It is a neoplasm with no significant evidence about its diagnosis, treatment, follow-up and prognosis. METHODS: Systematic research of the literature was conducted in Scopus, PubMed/MEDLINE, ScienceDirect and the Cochrane Library, including observational prospective and retrospective studies, case series and case reports. We collected data regarding studies related to diagnosis and treatment options evaluating the following aspects: study design, population, treatment type, rate of surgical complications and fertility outcome. We further included a case report of laparoscopic management of MA with pictorial assays. RESULTS: Thirteen cases of MA of the vagina are available in the literature, including our case report. The median age at diagnosis was 52 years old; the majority of patients reported vaginal bleeding as a symptom (38%); and ultrasound, followed by a magnetic resonance and CT scan were the diagnostic tools most used. In 54% of the cases, a surgical biopsy was performed, and 92% of the patients underwent upfront surgery with an open access or vaginal resection except one case fully managed by minimally invasive surgery. Most of the patients (68%) received adjuvant treatment with chemotherapy or radiotherapy or a combination of them. The mean follow-up period was 6 years. CONCLUSIONS: Despite the rarity of this cancer and bizarre location, a minimally invasive approach seems feasible after multidisciplinary evaluation. According to the rarity of this tumor, any future case and follow-up data must be reported in the literature in order to enlarge the knowledge about it.
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Adamantinoma is a rare primary low-grade malignant tumour of the appendicular skeleton most commonly found in the tibia. It has an indolent course, with local recurrences and lung metastases occurring over a protracted duration. There have been several suggestions pertaining to a vascular origin in the literature, however, histogenesis remains unclear. Currently, guidelines are not available pertaining to clinical management. This paper presents an overview of the current literature regarding this unusual malignancy. It also explores disease etiology and acknowledges the benefits and challenges of investigations pertaining to diagnosis. It recognizes a paucity of recommendations regarding appropriate surveillance and follow up. This review aims to assist clinicians in the building of a consensus opinion for optimal adamantinoma case management under current circumstances where formal guidelines do not exist.
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Leiomyomas are common benign uterine smooth muscle tumours. Rarer subsets may demonstrate aggressive extrauterine growth which mimic metastatic disease. We discuss the case of a female patient in her 40s, with a long-standing atrophic right kidney, presenting with a 17 cm uterine mass demonstrating bilateral para-aortic and pelvic sidewall spread. Although biopsies favoured the diagnosis of a benign tumour, a leiomyosarcoma could not be excluded. The surgical complexity of the case was compounded by a tumour residing close to the only functioning kidney and engulfment of the inferior mesenteric artery. The surgical procedures indicated were a radical hysterectomy, the laterally extended endopelvic resection procedure to achieve clear margins in the pelvic sidewall and a left hemicolectomy. In the absence of formal guidelines, we present this challenging case to provide clarity into the histological assessment and surgical management of rare leiomyomas, as well as an overview of the current literature.
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Leiomiomatosis , Riñón Único , Neoplasias Uterinas , Humanos , Femenino , Leiomiomatosis/diagnóstico , Leiomiomatosis/cirugía , Leiomiomatosis/patología , Pelvis/patología , Abdomen/patología , Histerectomía/métodos , Neoplasias Uterinas/diagnóstico , Neoplasias Uterinas/cirugía , Neoplasias Uterinas/patologíaRESUMEN
High grade endometrioid endometrial cancer (HGEEC) is a heterogeneous group of tumors with unclear prognostic features. The aim of the present study is to evaluate the independent risk factors for recurrence and mortality and to describe the recurrence patterns of HGEEC. Ninety-six consecutive cases of HGEEC treated with primary surgery in a single Tertiary Center were retrospectively reviewed. Clinicopathological and treatment details were recorded, and all patients were closely followed up. Disease-free, overall and cancer-specific survival rates were 83.8%, 77.8% and 83.6%, respectively. Cervical stromal involvement was independently related to recurrence (HR = 25.67; 95%CI 2.95-223.30; p = 0.003) and cancer-related death (HR = 15.39; 95%CI 1.29-183.43; p = 0.031) after adjusting for other pathological and treatment variables. Recurrence rate was 16%, with 60% of these cases having lung metastases and only one case with single vaginal vault recurrence. 81.81% of the recurrences presented with symptoms and not a single recurrence was diagnosed in routine follow-up clinical examination. In conclusion, the recurrence pattern may suggest that patient-initiated follow-up (PIFU) could be considered a potential alternative to clinical-based follow-up for HGEEC survivors, especially for patients without cervical involvement and after two years from treatment. Additional caution is needed in patients with cervical stromal involvement.