The genetic causes of the sequential occurrence of multiple primary malignancies in a young woman--5 years on.

The finding of three primary gynaecological malignancies in a young woman attending our unit was documented in 2001. We provide an update on this report as new events have prompted further discussion on the role of clinical guidelines in cancer management. The discovery of a genetic predisposition demonstrates the need for multidisciplinary input and heightened awareness in similar cases while the importance of treating each patient as an individual is emphasized.

The risk of malignancy index for ovarian tumours in Northeast Scotland--a population based study.

BACKGROUND AND AIMS: The Scottish Intercollegiate Guidelines Network (SIGN) recommends the use of the Risk of Malignancy Index (RMI) for ovarian tumours, a scoring system based on ultrasound findings, menopausal status and CA 125 level, in the pre-operative evaluation of pelvic masses. The aim of this study was to investigate the accuracy of this as a predictive method of discriminating benign from malignant disease. METHODS AND RESULTS: All women who underwent oophorectomy in 2004 at Aberdeen Royal Infirmary for suspected primary ovarian pathology were evaluated. The RMI was calculated and these results were compared with the final histopathology. The sensitivity of the RMI for diagnosing malignant ovarian disease was 94% (32/34) while the specificity was 70% (76/108). CONCLUSIONS: Compared to previous studies, the RMI score was highly sensitive in detecting malignant disease, although not as specific in excluding benign lesions, particularly cystadenomas and endometriomas. This can be improved by the refinement of imaging techniques as well as the use of laparoscopy in particular cases. The RMI score may also be especially valuable in directing referrals to a specialised centre.

The prognostic significance of micrometastases in node-negative squamous cell carcinoma of the vulva.

Nodal involvement is one of the most significant prognostic factors in squamous cell carcinoma (SCC) of the vulva. We conducted a retrospective analysis of 31 women with histologically node-negative SCC from a population-based cohort of Grampian women. Median follow-up was 42 months after radical vulvectomy with groin node dissection. In total, 13 women (42%) were found to have micrometastases on immunohistochemistry. The risk of recurrence was almost 20-fold higher in those with micrometastases compared to those without (hazard ratio=19.6 (95% CI 2.3-171).

Invasive adenocarcinoma of the vagina following surgery for adenocarcinoma in situ of the cervix--recurrence or implantation?

A 51-year-old woman underwent cervical conization for severe glandular abnormal cells. Histology noted adenocarcinoma in situ (AIS) with incomplete excision margins. Four months later, hysterectomy revealed no residual disease. Six months subsequently she developed invasive adenocarcinoma of the upper vagina. This report documents the unusual behavior of AIS and its management difficulties.

A reappraisal of the role of chlorambucil in patients with end stage ovarian cancer who have previously been treated with platinum regimens.

The role of chlorambucil in end stage platinum resistant epithelial ovarian cancer was evaluated in women with end stage ovarian cancer. They had received platinum based chemotherapy and all other intravenous chemotherapeutic options had been exhausted. Over a 15 year period, 30 patients were identified. The median age was 64.5 years (range 45-81). The median number of chlorambucil pulses was 4 (range 1-16). The median survival following the introduction of chlorambucil was 5.5 months (range 0.72-38.8). The 22 patients who survived for longer than three months were significantly younger than those who did not (p = 0.03). Apart from two patients who developed transient myelosupression there were no toxic side effects. Chlorambucil should be considered as a therapeutic option in end stage ovarian cancer. It is has minimal toxicity, and can be prescribed safely for long term use. In younger women, an increase in benefit may be anticipated.

Flap repair: an effective strategy for minimising sexual morbidity associated with the surgical management of vulval intra epithelial neoplasia.

OBJECTIVE: To assess the sexual function after local excision and flap repair for symptomatic vulval intraepithelial neoplasia (VIN). STUDY DESIGN: A retrospective analysis of five sexually active women who had persistent, symptomatic VIN diagnosed in a dedicated tertiary referral vulval clinic and treated with local excision and flap repair. Sexual function was assessed using a modified version of the Sabbatsberg Sexual Self-Rating Scoring (SRS) system. RESULTS: The mean age of the cohort was 32 years (range 30 to 51). Four patients had previously been unsuccessfully treated with local excision. Follow up ranged from 5 to 33 months. The SRS scores were 90, 90, 81, 72 and 25. Endogenous depression may explain this last score. There has been no recurrence of VIN. CONCLUSION: In sexually active women with symptomatic VIN III, a flap reconstruction should be considered in addition to local excision as the treatment of choice.

Flap repair: an effective strategy for minimising sexual morbidity associated with the surgical management of vulval intra epithelial neoplasia

Objective: To assess the sexual function after local excision and flap repair for symptomatic vulval intraepithelial neoplasia (Vin). Study Design: A retrospective analysis of five sexually active women who had persistent, symptomatic VIN diagnosed in a dedicated tertiary referral vulval clinic and treated with local excision and flap repair. Sexual function was assessed using a modified version of the Sabbatsberg Sexual Self-Rating Scoring (SRS) system. Results: The mean age of the cohort was 32 years (range 30 to 51). Four patients had previously been unsuccessfully treated with local excision. Follow up ranged from 5 to 33 months. The SRS scores were 90, 90, 81, 72 and 25. Endogenous depression may explain this last score. There has been no recurrence of VIN. Conclusion: In sexually active women with symptomatic VIN III, a flap reconstruction should be considered in addition to local excision as the treatment of choice (AU)

A subcutaneous uterus with unusual presenting features.

A 32 year old female, para 2 + 0 presented with a hard lump in the scar of a lower midline incision. She had had a myomectomy 2 years previously and subsequently noticed the lump 3 months later. Her only complaints were urinary frequency during menstruation and the suprapubic mass. Surgery was performed for what was initially thought to be a desmoid tumour. At surgery the uterus was found to be lying in the subcutaneous position with no peritoneal sac. The uterus was dissected free of the sheath and reduced into the pelvis, uneventfully. This rare occurrence of a subcutaneous non-gravid uterus in the absence of a hernial sac is reported and its clinical features and possible preventative measures are discussed.

Fetal outcome among obese parturients.

A controlled study of the fetal outcome among 300 obese gravidae revealed a perinatal loss ten times greater than among nonobese mothers. Diabetes mellitus, pre-eclampsia and advanced maternal age overwhelmingly contributed to this difference. However infants of obese grand multiparae were not at additional risk. Considering live infants the 1 min Apgar score was significantly lower in the study group and was confined to those mothers not at the extremes of maternal age, parity and gestational age. There was no difference in Apgar scores among infants delivered by cesarean section. However significantly more infants of obese mothers delivered vaginally were depressed. These results emphasize the need for stricter obstetric care in the elderly obese mother and those with pre-eclampsia and diabetes. The uncomplicated obese parturient appears to be at no additional risk in terms of fetal outcome.