A 52- year old perimenopausal caucasian woman, gravida 3 para 3, with a 10-week long vaginal bleeding, bloating, fatigue, weight gain (>7 kg), and hypogastric mass was admitted to the local emergency room for an epigastric pain and a mild dyspnea. She also observed breast tenderness for the last 3 months. Her personal history included: appendectomy, amiodarone-induced hypothyroidism, chronic atrial fibrillation and breast abscess but not hypertension. Her last delivery was 23 years ago and she discontinued oral contraceptive pill at least 18 months back. She then observed hot flashes and menstrual irregularity with longer menstrual cycles and her last menses occurred 4 months ago.
A computed tomography (CT) scan of the chest, abdomen and pelvis showed a uterine enlargement (15 × 12 cm axial) with a heterogeneous hypodense endometrium punctuated with nodular areas enhanced by iodinated contrast (Fig. 1a & b). Neither fetus nor adnexal mass was detected. Diffuse bilateral pulmonary nodules were observed (Fig. 1c). While her serum hCG level was 0.96 × 106 IU/L, an endometrial trans-cervical biopsy showed two non-malignant chorionic villi.
When she was referred to the regional cancer center ten days later, she was further diagnosed with the following signs of early onset preeclampsia (EOP): new onset of severe hypertension (170/100 mmHg), proteinuria, oliguria, headache, hyper reflexivity in lower limbs and growing epigastric pain radiating to both hypochondria. While her fundal height was measured to be 18 cm, her laboratory tests including TBC, kidney and liver function were initially normal except a serum hCG test rising to 1.266 × 106 IU/L. Blood pressure and diuresis were stabilized after a parenteral treatment of severe hypertension and pain. Her biological results, however, rapidly deteriorated with hemolytic anemia (hemoglobin 97 g/L, haptoglobin <0.06 g/L), thrombocytopenia (75 G/L), and elevated liver enzymes (ALT 120 IU/L, AST 252 IU/L). Additionally, she developed severe proteinuria (1.85 g/24 h). Together, she presented a complete form of HELLP syndrome. The patient was transferred to the intensive care unit and a salvage total abdominal hysterectomy with bilateral salpingo-oophorectomy was immediately decided in accordance with the French Trophoblastic Disease Reference Centre advice. Gross examination of the specimen showed an enlarged and tensed uterine body (22 × 20 × 10 cm, Fig. 2a) whose longitudinal incision released macroscopic vesicles without any identifiable fetus (Fig. 2b). Histological examination further revealed a complete and invasive hydatidiform mole (Fig. 2 c & d). Histology was reviewed within the French network of trophoblastic disease referent pathologists.
One week after hysterectomy, her biological results markedly improved (hemoglobin 105 g/L, haptoglobin 2.7 g/L, platelets 311 G/L, ALT 24 IU/L, AST 23 IU/L and hCG 31.240 × 103 IU/L.
Thoraco-abdomino-pelvic CT scan, liver MRI, brain CT and 18 F-FDG PET/CT detected diffuse metastases limited to the lungs (visible on chest CT scan but not by chest-X-ray). She was thus considered to develop a post-molar high-risk GTN with a FIGO stage/score of III:7 [8]. Therefore, an EMA-CO multi-agent chemotherapy was initiated at day 20 post-operative, as the patient left the intensive care unit [9]. She was subsequently registered in the national database with her informed signed consent [10]. Complete remission of GTN was ascertained by the rapid hCG regression within 10 weeks (Fig. 3). She was administered five EMA/CO cycles with two more consolidation courses after normalization of serum hCG levels that were periodically followed up to 24 months [11]. A complete response was observed on the thoracic CT four months after diagnosis. The patient is disease-free for ten years.
