[A woman with a vulvar swelling, a rare manifestation of the Klippel-Trenaunay-Weber syndrome]. / Een vrouw met een vulvaire zwelling, een zeldzame uiting van het Klippel-Trenaunay-Weber-syndroom.

A woman with a vulvar swelling, a rare manifestation of the Klippel-Trenaunay-Weber syndrome. - A 32-year-old woman presented with a vulvar swelling. The swelling had always been there but had increased after her last pregnancy, 5 years earlier. Because of progressive complaints, such as pain and a feeling of pressure, she now wished surgical correction. The swelling turned out to be varicosis of the right labium majus associated with the Klippel-Trenaunay-Weber syndrome. The vulvar swelling was removed by electrocoagulation. The pathological diagnosis was 'venous haemangioma'. The Klippel-Trenaunay-Weber syndrome is a congenital skin condition in which vascular angiomas, varicosis and trophic changes in the soft tissue and skeleton can arise. Vulvar involvement is rare. The abnormality can be removed surgically, for example by electrocoagulation. There is a chance of recurrence.

Adnexal masses in pregnancy.

With the widespread use of routine abdominal ultrasound examination during pregnancy, adnexal masses are observed with increasing frequency. Most patients are clinically asymptomatic at the time of presentation, and most of the adnexal masses detected during early pregnancy disappear during the first 16 weeks of pregnancy. Ovarian tumors are estimated to occur in about 1 in 1,000 pregnancies and of these 3% are malignant. Here we present an overview about frequency, diagnostic procedures and pathological characteristics of these ovarian tumors. Moreover, current modalities for treatment during pregnancy are summarized. Surgical treatment of the adnexal masses has to be performed with adequate staging and debulking equal to the treatment of non-pregnant women. However, whereas during organogenesis abortion has to be considered prior to chemotherapy, later in pregnancy surgical debulking as complete as possible, followed by taxol-platinum chemotherapy is indicated. If the fetus is not viable at the time of primary surgery, neoadjuvant chemotherapy and complementation of surgery after delivery of the baby should be performed. It should be stressed that chemotherapy for ovarian cancer applied during pregnancy appears to be safe. However, no studies have evaluated the long-term consequences for children exposed to intra-uterine chemotherapy. Aspiration of cysts should be avoided, as the correlation between the histological evaluation of an ovarian malignancy and the cytological evaluation of aspirates is poor. Moreover, spillage of malignant cysts is hazardous for the patient.