Intraplacental choriocarcinoma: Systematic review and management guidance

• Intraplacental choriocarcinoma is easily missed as most patients are asymptomatic.
• We present a systematic review of 62 cases including four previously unreported.
• In cases of maternal metastatic disease, prognosis is excellent with modern therapy.
• 60% of affected pregnancies result in live birth with low neonatal mortality.
• A clinical algorithm has been developed to aid diagnosis and management decisions.

BackgroundIntraplacental choriocarcinoma (IC) is a rare form of malignant gestational trophoblastic disease (GTD). We present a review of 62 cases, including four previously unreported, and a suggested management algorithm.Patients and methodsIC cases and clinical data were identified within the Charing Cross Hospital (CXH) national GTD database (1986–2014) and by systematic literature search (1949–2014).Results62 cases were identified including eight from CXH representing 0.03% of all GTD (n = 27,101) diagnosed between 1986 and 2014. Most cases were identified in the third trimester (n = 52; 84%) among asymptomatic women (n = 31; 50%) and with macroscopically normal placenta in 29% (18/62). In 29 non-metastatic cases with available data, 4 (14%) underwent adjuvant chemotherapy and 25 (86%) surveillance only, one of whom relapsed with metastatic disease cured with multi-agent chemotherapy. In 32 patients with metastatic disease (31 at presentation and one with relapse during surveillance), all 18 treated since 1990 achieved complete remission with multi-agent chemotherapy. Among 58 cases with available data, there were 20 fetal deaths and 38 live births with 2 neonatal deaths. Of the two (5%) cases of infantile choriocarcinoma, one was cured with intensive therapy and the other died shortly after commencing single agent treatment. A further neonatal death was due to fetomaternal haemorrhage.ConclusionsIC usually occurs in the third trimester and is often asymptomatic with no macroscopic placental abnormalities. Prognosis with current therapy is generally excellent, even for patients presenting with metastatic disease. Around 60% of pregnancies affected by IC result in a live birth with a low neonatal mortality.