Central-type primitive neuroectodermal tumor of the uterus: Case report and literature review
- 1Department of Pathology, Zhanjiang Central Hospital, Guangdong Medical University (Central People’s Hospital of Zhanjiang)
- 2Department of Pathology and Research Center, Affiliated Hospital of Guangdong Medical University
- 3Department of Pathology, Foshan Hospital of Traditional Chinese Medicine
Abstract
Primitive neuroectodermal tumors (PNETs) arising in the uterus, particularly those classified as the central-type (cPNET), are exceedingly rare. To date, approximately 30 cases of uterine cPNET have been reported, and their clinicopathological features remain poorly characterized. These tumors typically present as highly aggressive small round cell neoplasms with variable neuroectodermal differentiation, often posing significant diagnostic challenges owing to their overlap with other uterine malignancies, including neuroendocrine carcinoma, endometrial stromal sarcoma, and undifferentiated carcinoma. Here, we report a rare case of uterine cPNET characterized by a diffuse small round cell morphology and prominent neuroendocrine differentiation. Immunohistochemical analysis demonstrated positivity for synaptophysin, INSM1, and CD56, whereas markers associated with Ewing’s sarcoma (CD99, FLI1 and NKX2.2) were negative. Next-generation sequencing revealed no Ewing sarcoma breakpoint region 1 rearrangements, supporting the exclusion of peripheral PNET. This case highlights the diagnostic complexity of uterine tumors with neuroectodermal differentiation and expands the current morphological and immunophenotypic spectra of this entity. Accurate recognition requires the integration of histopathology, immunoprofile, and molecular findings, with particular attention paid to the differential diagnosis of other high-grade uterine malignancies.
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BibTeX
@article{lin2026centraltype,
title = {{Central-type primitive neuroectodermal tumor of the uterus: Case report and literature review}},
author = {Yi-Ying Lin and Ming-Yu Xie and Rong-Jun Mao and Bo-Tao Luo and Yan Qi},
journal = {Frontiers in Oncology},
year = {2026},
month = oct,
doi = {10.3389/fonc.2026.1926715},
url = {https://www.frontiersin.org/articles/10.3389/fonc.2026.1926715/full},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Central-type primitive neuroectodermal tumor of the uterus: Case report and literature review
AU - Yi-Ying Lin
AU - Ming-Yu Xie
AU - Rong-Jun Mao
AU - Bo-Tao Luo
AU - Yan Qi
PY - 2026
DA - 2026/10/23
JO - Frontiers in Oncology
DO - 10.3389/fonc.2026.1926715
UR - https://www.frontiersin.org/articles/10.3389/fonc.2026.1926715/full
AB - Primitive neuroectodermal tumors (PNETs) arising in the uterus, particularly those classified as the central-type (cPNET), are exceedingly rare. To date, approximately 30 cases of uterine cPNET have been reported, and their clinicopathological features remain poorly characterized. These tumors typically present as highly aggressive small round cell neoplasms with variable neuroectodermal differentiation, often posing significant diagnostic challenges owing to their overlap with other uterine malignancies, including neuroendocrine carcinoma, endometrial stromal sarcoma, and undifferentiated carcinoma. Here, we report a rare case of uterine cPNET characterized by a diffuse small round cell morphology and prominent neuroendocrine differentiation. Immunohistochemical analysis demonstrated positivity for synaptophysin, INSM1, and CD56, whereas markers associated with Ewing’s sarcoma (CD99, FLI1 and NKX2.2) were negative. Next-generation sequencing revealed no Ewing sarcoma breakpoint region 1 rearrangements, supporting the exclusion of peripheral PNET. This case highlights the diagnostic complexity of uterine tumors with neuroectodermal differentiation and expands the current morphological and immunophenotypic spectra of this entity. Accurate recognition requires the integration of histopathology, immunoprofile, and molecular findings, with particular attention paid to the differential diagnosis of other high-grade uterine malignancies.
SN - 2234-943X
ER -