Rare Gynecologic Cancers Research Articles

Claudin and p53 expression in vulvar lichen sclerosus and squamous-cell carcinoma

AimsVulvar squamous-cell carcinoma (SCC) is a rare gynaecological cancer. Vulvar SCC has been shown to develop from vulvar intraepithelial neoplasias, which are related to lichen sclerosus (LS). Most studies to...

Metabolic syndrome and rare gynecological cancers in the Metabolic syndrome and Cancer project (Me-Can)

BackgroundRisk factors for rare gynecological cancers are largely unknown. Initial research has indicated that the metabolic syndrome (MetS) or individual components could play a role. Materials and methodsThe Metabolic syndrome and Cancer project cohort includes 288834 women. During an average follow-up of 11 years, 82 vulvar, 26 vaginal and 43 other rare gynecological cancers were identified. Hazard ratios (HRs) were estimated fitting Cox proportional hazards regression models for tertiles and standardized z-scores [with a mean of 0 and a standard deviation (SD) of 1] of body mass index (BMI), blood pressure, glucose, cholesterol, triglycerides and MetS. Risk estimates were corrected for random error in the measurement of metabolic factors. ResultsThe MetS was associated with increased risk of vulvar [HR 1.78, 95% confidence interval (CI) 1.30–2.41) and vaginal cancer (HR 1.87, 95% CI 1.07–3.25). Among separate MetS components, 1 SD increase in BMI was associated with overall risk (HR 1.43, 95% CI 1.23–1.66), vulvar (HR 1.36, 95% CI 1.11–1.69) and vaginal cancer (HR 1.79, 95% CI 1.30–2.46). Blood glucose and triglyceride concentrations were associated with increased risk of vulvar cancer (HR 1.98, 95% CI 1.10–3.58 and HR 2.09, 95% CI 1.39–3.15, respectively). ConclusionThe results from this first prospective study on rare gynecological cancers suggest that the MetS and its individual components may play a role in the development of these tumors.

Chemoradiation for advanced primary vulval cancer.

Vulval cancer is a rare gynaecological cancer. There is no standard approach for treating locally advanced primary vulval cancer (FIGO stage III and IV). Combined treatment modalities have been developed using radiotherapy, chemotherapy and surgery. The advantages and disadvantages of such treatment is not well evaluated. To evaluate the effectiveness and safety of neoadjuvant and primary chemoradiation for women with locally advanced primary vulval cancer compared to other primary modalities of treatment such as primary surgery or primary radiation. We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library 2009, Issue 3), Cochrane Gynaecological Cancer Group Trials Register, MEDLINE and EMBASE (to July 2009). We also searched registers of clinical trials, abstracts of scientific meetings, reference lists of included studies and contacted experts in the field. Randomised controlled trials (RCTs) or non-randomised studies that included multivariate analyses of chemoradiation in women with locally advanced, primary squamous cell carcinoma of the vulva. Two review authors independently abstracted data and assessed risk of bias. An adjusted hazard ratio (HR) for overall survival was calculated for one non-randomised study and risk ratios (RRs) were used in an RCT to compare five-year death rates and adverse events in women who received neoadjuvant, primary chemoradiation or primary surgery. Adverse events were also reported more extensively in a further non-randomised study. All results were displayed in single study analyses. One RCT and two non-randomised studies that allowed for multivariate analyses met the inclusion criteria and included a total of 141 women.One RCT found that neoadjuvant chemoradiation did not appear to offer longer survival compared to primary surgery in advanced vulval tumours (RR = 1.29, 95% confidence interval (CI) 0.87 to 1.91). There was also no statistically significant difference in survival between primary chemoradiation and primary surgery in a study that included 63 women (pooled adjusted HR= 1.09, 95% CI 0.37 to 3.17) and in another study that only included 12 eligible women and compared the same interventions (HR was non-informative when statistical adjustment was made).Adverse events were extensively reported in only one study, which found no statistically significant difference in risk of adverse events between primary chemoradiation and primary surgery due to the very small numbers in each group. In the RCT there was no observed statistically significant difference between neoadjuvant chemoradiation and primary surgery. Adverse events were not reported in the largest study of 63 women. Quality of life (QoL) was not reported in any of the included studies. All studies were at high risk of bias. Women with advanced vulval tumours showed no significant difference in overall survival or treatment-related adverse events when chemoradiation (primary or neoadjuvant) was compared with primary surgery.The retrospective studies had a high risk of bias as the entry criteria for primary chemoradiation was based on inoperability or tumour requiring exenteration.The radiochemotherapy regimens varied widely. There was no data on QoL.There is no standard terminology for 'operable and inoperable vulval cancer', and for 'primary and neoadjuvant chemoradiation'. Stratification according to unresectability of the primary tumour and/or lymph nodes is needed, for good quality comparison.

Neoadjuvant chemotherapy followed by radical surgery and reconstruction of the vagina in a patient with stage II primary vaginal squamous carcinoma

Primary vaginal carcinoma is a rare gynecologic cancer. Radiotherapy is the standard treatment for patients affected by International Federation of Gynecology and Obstetrics stage II vaginal cancer. Neoadjuvant chemotherapy followed by radical surgery has been shown to be a valid therapeutic strategy in patients with cervical cancer; however, there is little information concerning the feasibility of neoadjuvant chemotherapy followed by radical surgery in patients with invasive vaginal carcinoma. We report the first case of stage II vaginal carcinoma with neoadjuvant chemotherapy followed by radical surgery combined with vaginal reconstruction using bilateral pudendal thigh fasciocutaneous flaps. The patient was free of disease with a satisfactory sexual life after 30 months.

Targeted therapies for rare gynaecological cancers

Some gynaecological cancers are uncommon, such as sex cord-stromal tumours, malignant germ-cell tumours, vulvar carcinoma, melanoma of the female genital tract, clear-cell carcinoma of the ovary and endometrium, neuroendocrine tumours of the cervix, and gestational trophoblastic neoplasia. All these cancers have different clinicopathological characteristics, suggesting different molecular biological pathogeneses. Despite aggressive treatment, some cancers recur or respond poorly to therapy. Comprehensive knowledge of the molecular biology of each cancer might help with development of novel treatments that maximise efficacy and minimise toxic effects. Targeted therapy is a new treatment strategy that has been investigated in various tumours in clinical and laboratory settings. Since these cancers are rare and large clinical trials are difficult to do, molecular biological techniques might allow rapid proof-of-principle experiments in few patients. Novel targeted agents either alone or in combination with other treatments offer promising therapeutic options.

Squamous Cell Carcinoma of the Vulva in Turner Syndrome

Turner syndrome (gonadal dysgenesis) is an important cause of short stature in girls and primary amenorrhoea, which is usually caused by loss of part or all the X-chromosome. It is well known that women with Turner syndrome are liable to develop various diseases such as autoimmune diseases like thyroiditis, and cardiovascular, urologic and bone structural diseases in addition to hypertension and diabetes 1 However the risk of cancer, with the exception being in the case of colonic and rectal cancers, does not seem to be elevated in Turner syndrome 1. Carcinoma of the vulva is a rare gynaecological cancer and in Turner syndrome it is extremely rare. We report a case of squamous cell carcinoma (SCC) of the vulva in a 61 year old Caucasian woman diagnosed with Turner syndrome at the age of 15. Radical vulvectomy with en-block inguinal femoral lymphadenectomy was carried out. Histopathological examination of the specimen confirmed complete excision of the tumour with absence of metastasis to the lymph nodes. Conclusion: This seems to be the first reported case of SCC of the vulva in a patient with Turner syndrome.

254: Cerebral Recurrence of the Fallopian Tube Cancer

Study ObjectiveTo present an unusual case of fallopian tube cancer, which recurred at the brain. Diagnosis was one by endoscopic biopsy.DesignFallopian tube adenocarcinoma is a rare gynecologic cancer, and central nervous system metastasis is an extremely rare event. The treatment of this disease is known be same as that of ovarian cancer with brain metastasis.SettingWe present a case of brain metastasis in a fallopian tube cancer patient.PatientsA 46-year-old Korean woman visited the OPD with severe headache. She had undergone a surgical treatment for FIGO stage IIIc fallopian tube adenocarcinoma and adjuvant chemotherapy 4 years ago. Brain MRI revealed a solitary lesion in the left thalamus to midbrain.InterventionThe mass was removed by endoscopic surgery. Pathologic review demonstrated metastatic adenocarcinoma, same finding of primary cancer. She was treated by radiation therapy and PC combined chemotherapy.Measurements and Main ResultsShe is being regular follow-up for 12 months after multimodal treatment. She is in the disease-free state and required regular follow-up.ConclusionA cerebral recurred fallopian tube adenocarcinoma case was treated by multimodal treatment, surgery, radiation and chemotherapy. Study ObjectiveTo present an unusual case of fallopian tube cancer, which recurred at the brain. Diagnosis was one by endoscopic biopsy. To present an unusual case of fallopian tube cancer, which recurred at the brain. Diagnosis was one by endoscopic biopsy. DesignFallopian tube adenocarcinoma is a rare gynecologic cancer, and central nervous system metastasis is an extremely rare event. The treatment of this disease is known be same as that of ovarian cancer with brain metastasis. Fallopian tube adenocarcinoma is a rare gynecologic cancer, and central nervous system metastasis is an extremely rare event. The treatment of this disease is known be same as that of ovarian cancer with brain metastasis. SettingWe present a case of brain metastasis in a fallopian tube cancer patient. We present a case of brain metastasis in a fallopian tube cancer patient. PatientsA 46-year-old Korean woman visited the OPD with severe headache. She had undergone a surgical treatment for FIGO stage IIIc fallopian tube adenocarcinoma and adjuvant chemotherapy 4 years ago. Brain MRI revealed a solitary lesion in the left thalamus to midbrain. A 46-year-old Korean woman visited the OPD with severe headache. She had undergone a surgical treatment for FIGO stage IIIc fallopian tube adenocarcinoma and adjuvant chemotherapy 4 years ago. Brain MRI revealed a solitary lesion in the left thalamus to midbrain. InterventionThe mass was removed by endoscopic surgery. Pathologic review demonstrated metastatic adenocarcinoma, same finding of primary cancer. She was treated by radiation therapy and PC combined chemotherapy. The mass was removed by endoscopic surgery. Pathologic review demonstrated metastatic adenocarcinoma, same finding of primary cancer. She was treated by radiation therapy and PC combined chemotherapy. Measurements and Main ResultsShe is being regular follow-up for 12 months after multimodal treatment. She is in the disease-free state and required regular follow-up. She is being regular follow-up for 12 months after multimodal treatment. She is in the disease-free state and required regular follow-up. ConclusionA cerebral recurred fallopian tube adenocarcinoma case was treated by multimodal treatment, surgery, radiation and chemotherapy. A cerebral recurred fallopian tube adenocarcinoma case was treated by multimodal treatment, surgery, radiation and chemotherapy.

Recent trends in the management of vulval cancer

The management of vulval cancer, a rare gynaecological cancer, should be undertaken in a cancer centre where appropriate expertise and care is delivered by a multidisciplinary team to maximise patient outcome. The optimal techniques for the management of early stage vulval cancer have been refined in recent years. Gynaecological oncologists have moved away from radical surgery to the vulva and groins to a less radical approach preserving vulval tissue and organ function. These surgical modifications in resection of the primary cancer have not compromised cure rates and have reduced morbidity. Improvements in imaging techniques and sentinel node detection may lead to a more conservative approach in the management of the groin nodes. Locally advanced or recurrent cancers in particular, require joint surgical and clinical oncology input by experienced clinicians. Rarer vulval tumours are usually managed in a similar manner to squamous cell cancer.

Patient Selection and Preoperative Evaluation for Radical Pelvic Surgery

Pelvic exenteration may be used for treating carcinomas of the cervix, vagina, vulva, and endometrium, some rare gynecologic cancers, and primary and recurrent rectosigmoid carcinomas. Patient selection is based on assessment of the patient’s attitude and mental condition, multisystem status, weight, age, and previous treatment history. In addition, a digital examination, an examination under anesthesia, imaging studies, and an exploratory laparotomy are performed to rule out the presence of metastases.