Teaching Case

There is a history of a mass in the right labia for 10 years, which had recently increased in size with superficial cutaneous ulcerations. The mass was soft on palpation with focal areas of indurations. A provisional clinical diagnosis of a malignant neoplastic lesion like liposarcoma was made and the patient was referred for MRI for further evaluation.

MRI showed a large well-defined soft tissue mass lesion in the right labia with multiple flow voids within. The lesion was T1 hypointense (Fig. 1) and T2 hyperintense (Fig. 2), showing no suppression of signals on fat saturated images (Fig. 3). It measured 20x22cm extending from the right labial fold up to the mid-thigh. A fairly well-defined T1 hyperintense, T2 hypointense focal lesion was noted in the centre suggestive of a haematoma (Fig. 1). It showed contrast opacification on T1FS post.contrast study (Fig. 4). There was no obvious mass lesion in the pelvis compressing the iliac veins. A radiograph of the mass showed phleboliths within (Fig. 5). An ultrasound scan of the patient showed a large soft tissue lesion involving the right labia with multiple enlarged tortuous venous channels involving almost the entire soft tissue mass lesion (Fig. 6). These dilated veins were compressible with a probe and showed colour flow within (Fig. 7).

Varicose veins are common in pregnancy, mostly seen in the vulvar and peri-vulvar areas. They are found in 4% in women and are most commonly seen during pregnancy. Pelvic congestion syndrome also causes secondary varices and chronic pain in the pelvis, which was originally described by Gooch in 1831 [1]. Symptoms commonly encountered with varicoceles are pelvic discomfort, vulvar pressure, pruritus, a sensation of prolapse and patient anxiety [2]. Hence it is important to rule out other conditions like inguinal hernia or bartholin duct cyst, which are the close differentials [3]. It is important to be familiar with the causes and its appearance as it allows the surgeon to treat it correctly. Sometimes they may suddenly appear by the middle of the first trimester of pregnancy, and then also increase in size in subsequent pregnancies. In non-pregnant women it is commonly seen as a part of “nut cracker syndrome”, where the left renal vein is compressed between the aorta and the superior mesenteric artery. A contrast CT with angiography can be useful for this. Venous reflux into the left renal vein also causes pelvic congestion syndrome leading to varices [2]. Pelvic CT or MRI can be done to look for the dilated veins in the pelvis. Deficient valves in the pelvic veins cause free and profuse circulation, which in turn increases the risk of varices in pregnancy, mostly after 26 weeks of gestation. Other causes include hormones like prostaglandins A1, A2, E1, and E2 [4]. However in non-pregnant women, the most common cause for varices is local venous insufficiency and venous incompetence. The role of genetic factors should be kept in mind, which predisposes these venous changes. In our patient, since pelvic imaging did not reveal any mass or any obvious cause for labial varices, venous insufficiency or hormonal causes could have been the possible aetiology behind the varices.
Treatment doesn’t include any standard approach. It is treated according to the symptom and origin of reflux. Earlier management included hysterectomy and/or ligation of the ovarian veins, for pelvic congestion with varices. Laparoscopic ligation was later introduced for ovarian veins. Recently they are treated commonly with embolisation. This is done by the interventional radiologist. The success rate is good with embolisation of about 70-85% and also causes less discomfort for patients [5].
Since the mass was diagnosed as labial variceal mass on imaging which was likely harmless to the patient, unlike the initial clinical diagnosis of liposarcoma, the patient opted for conservative management with local care of the ulcers and periodical observation.