Primary uterine non-gestational placental site trophoblastic tumor as a distinct entity: a report of five cases
File(s)
Published version
Author(s)
Type
Journal Article
Abstract
Uterine placental site trophoblastic tumors (PSTTs) are rare trophoblastic neoplasms, presumed to be of gestational origin. Herein, using a comprehensive morphological, immunohistochemical, and molecular approach, we describe five cases of primary uterine non-gestational PSTTs. The median age at presentation was 32 years old (range: 25-45). All tumors were initially expected to be of gestational origin as all were located in the uterus and all patients had a history of pregnancy (5/5, 100%). The median size of the primary uterine tumors was 6.3 cm (range: 4.8-7.5). Three patients (3/5, 60%) had metastatic disease at presentation or revealed
during initial workup (1/5 [20%] patients with lymph node metastasis only and 2/5 [40%] with distant metastases). All tumors showed similar histopathological and immunohistochemical features to those of gestational PSTTs. The tumor cells expressed hPL in 5/5 (100%) tumors, hCG in 5/5 (100%; focal in all tumors), and GATA3 in 5/5 (100%). However, short tandem repeat (STR) genotyping did not identify any non-patient alleles in the tumors, indicating a non-gestational origin. The median progression-free survival was 18 months (range: 0 to 85) and 2/5 (40%) patients died from disease, highlighting the potential poor prognosis of this non-gestational tumor. Thus, in the same way as gestational and non-gestational choriocarcinomas are recognized as different entities, non-gestational PSTTs could be viewed as a distinct entity from their gestational counterparts, although further investigation and more cases are needed. Furthermore, we propose recommendations for diagnosing and staging of non-gestational PSTTs to improve patient stratification and management.
during initial workup (1/5 [20%] patients with lymph node metastasis only and 2/5 [40%] with distant metastases). All tumors showed similar histopathological and immunohistochemical features to those of gestational PSTTs. The tumor cells expressed hPL in 5/5 (100%) tumors, hCG in 5/5 (100%; focal in all tumors), and GATA3 in 5/5 (100%). However, short tandem repeat (STR) genotyping did not identify any non-patient alleles in the tumors, indicating a non-gestational origin. The median progression-free survival was 18 months (range: 0 to 85) and 2/5 (40%) patients died from disease, highlighting the potential poor prognosis of this non-gestational tumor. Thus, in the same way as gestational and non-gestational choriocarcinomas are recognized as different entities, non-gestational PSTTs could be viewed as a distinct entity from their gestational counterparts, although further investigation and more cases are needed. Furthermore, we propose recommendations for diagnosing and staging of non-gestational PSTTs to improve patient stratification and management.
Date Issued
2026-04-01
Date Acceptance
2025-11-26
Citation
American Journal of Surgical Pathology, 2026, 50 (4), pp.435-447
ISSN
0147-5185
Publisher
Lippincott, Williams & Wilkins
Start Page
435
End Page
447
Journal / Book Title
American Journal of Surgical Pathology
Volume
50
Issue
4
Copyright Statement
© 2026 The Author(s). Published by Wolters Kluwer Health, Inc. This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unre stricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
License URL
Identifier
10.1097/PAS.0000000000002502
Subjects
PSTT
nongestational trophoblastic tumor
uterine neoplasm
trophoblastic differentiation
STR genotyping
Publication Status
Published