The Rare of the Rarest: Placental Site Trophoblastic Tumor, Epithelioid Trophoblastic Tumor, Atypical Placental Site Nodule

Abstract

Background: Epithelioid Trophoblastic Tumor (ETT) and Placental Site Trophoblastic Tumor (PSTT) are two of the rarest GTNs that share certain features at diagnosis and management. Atypical Placental Site Nodule (APSN) is a relatively new entity considered as a premalignant lesion.

Objectives and methods: The aim of this review was to summarize the main characteristics of each of these entities, their diagnostic features, and their treatment's standard of care including fertility-sparing treatments.

Outcome: This study provides a thorough review of ETT, PSTT, and APSN.

Conclusions: The reader will gain an insight view of these rare tumors arising from the intermediate trophoblast.

Keywords: Atypical placental site nodule; Epithelioid trophoblastic tumor; Gestational trophoblastic disease; Gestational trophoblastic neoplasia; Placental site trophoblastic tumor; Rare diseases.