Objective: To present a rare case of placental site trophoblastic tumor and the multidisciplinary care required for successful treatment

Summary: Gestational trophoblastic disease (GTD) is a group of tumors that can occur with any gestational event and is categorized by hydatidiform moles and invasive gestational trophoblastic diseases or neoplasias which include invasive moles, choriocarcinoma, placental site trophoblastic tumor and epithelioid trophoblastic tumor. Placental site trophoblastic tumor (PSTT) is an extremely rare form of GTD, characterized by abnormal proliferation of the intermediate trophoblast at the placental implantation site. Here we present a 34-year-old patient who presented with advanced PSTT that had metastasized to the adrenals and lungs. We discuss clinical presentation, diagnosis including relevant pathology and treatment course which required extensive interdisciplinary care given multiple adverse events the patient experienced while undergoing treatment. We also review related literature on current and evolving treatments for PSTT, including emerging evidence for use of immunotherapy, as well as the treatment of pulmonary arteriovenous malformations that formed after regression of metastatic disease.

Conclusion: PSTT is a very rare form of GTD, which in this case presented seven months after antecedent pregnancy with significant local disease and metastatic disease burden. Effective treatment of this patient required coordination of care between a large multidisciplinary team. Additionally, there is an emerging role for treatment of GTD with immunotherapy.