Author(s)

Ginanni B, Marchetti M, Lauretti D, Sabato M, Ceccarelli A, Vallini V, Spirito N, Bulleri A, Caramella D, Bartolozzi C

Patient

female, 31 year(s)

A 31-year-old primigravida with abdominal pain, cramps of the lower abdomen and vaginal bleeding.

A 31-year-old primigravida (8 week gestation) arrived to our attenction with lower abdomen cramps and vaginal bleeding. Transvaginal ultrasound (TVS) scan showed uterine cavity enlargement and hydropic changes suggestive of hydatidiform mole, without intrauterine gestational sac. Surgical uterine evacuation was performed, followed by weekly human chorionic gonadotropin (hCG) follow-up. Light microscopic examination revealed normal villi coexisting with villi showing hydropic changes, cistern formations and diffuse circumferential trophoblastic hyperplasia consistent in partial molar change (Fig. 1). Because of increasing hCG level (21135 mUI/ml) during follow-up, transabdominal US was performed showing uterine enlargement and echogenic masses inside (Fig. 2). The patient underwent thorax and abdomen CT staging examination (Fig. 3). Basal scans demonstrated an enlarged inhomogeneous uterus with a peripherical area of low attenuation, well marked after contrast medium administration, without extrauterine metastases. After six cycles of methotrexate and folinic acid therapy, serum hCG levels remained high. Another CT examination was performed (Fig. 4). It revealed a greater involvement of the uterus because of the lesions and a marked pelvic venous congestion; it also showed enlarged ovaries with multiple cysts and inhomogeneity of the right lobe of thyroid because of hCG stimulation.
The patient started multiagent chemotherapy.

Hydatidiform mole (HM) is a rare disorder of trophoblast proliferation occurring in 1:2,500 pregnancies. It is a gestational trophoblastic tumour (GTT), also considered as a pre-neoplastic disorder, because it can develop into malignant tumour (choriocarcinoma). It is important to distinguish between a complete hydatidiform mole (CHM), which is usually androgenetic and displays vestigial embryonic development, and a partial hydatidiform mole (PHM), which is typically triploid and shows coexistence of an embryo and trophoblast hyperplasia. In fact, a complete hydatidiform mole has a greater malignant potential (15-20%). In all cases, the presence and course of the disease can be monitored with quantitative levels of hCG, the first hormone secreted by the trophoblast during pregnancy. Serial determinations of HCG levels will indicate a rapidly mounting level that climbs even faster than during either a normal single or normal multiple pregnancy.
Clinical signs and symptoms like abdominal pain, cramps of the lower abdomen and vaginal bleeding during pregnancy, are usually insufficient for predicting the extent of disease. The uterus is large for gestational age, and foetal heart tones are usually absent. US is the examination of choice for initial diagnosis. In partial moles, the uterus is enlarged and contains areas of multiple, diffuse anechoic lesions. Although the diagnosis is made with a high degree of certainty at sonography, it should be confirmed pathologically. Suction curettage is regarded as the preferred treatment method. At histologic examination, hydatidiform moles are characterised by abnormalities in the chorionic villi consisting of varying degrees of trophoblastic proliferation and oedema of villous stroma. Complete and partial moles remit spontaneously in most cases, following evacuation of the uterine cavity. However, either persistent trophoblastic disease or a frank trophoblastic tumour can follow a complete hydatidiform mole. Computed Tomography (CT) and Magnetic Resonance (MR) imaging are not usually performed initially but may be used to determine if there is extension of molar tissue outside the uterus. CT examination usually demonstrates an enlarged uterus with areas of low attenuation, or hypo-attenuating foci surrounded by highly enhanced areas in the myometrium and also extrauterine metastases. On T2-weighted MR images, hydatidiform mole appears as a heterogeneous mass of high signal intensity that distends the endometrial cavity. Numerous cystic spaces may be present in the uterine mass and in the ovaries because of hCG stimulation. Management of both partial mole and complete mole is similar: surgical evacuation followed by weekly gonadotropin follow-up until hCG levels become undetectable. Patients are then followed for 6 months: an increase or a plateau in the hCG level during this period defines persistent gestational trophoblastic neoplasia (GTN). Choice of a chemotherapeutic regimen is based upon the characteristics of the presenting GTN. Patients are stratified into low and high risk GTN by combining the International Federation of Obstetrics and Gynecology (FIGO) stage with the World Health Organization (WHO) prognostic scoring system. Single-agent therapy is indicated for low risk GTN, while multi-agent chemotherapies are utilized for high risk GTN successfully.