Case Report: A case of early rapid recurrence of a primary intracranial alveolar soft part sarcoma with high proliferative activity and complex molecular variations
- 1Department of Radiation Oncology, General Hospital of Northern Theater Command
- 2Oncology Department, No. 968 Hospital of the Joint Logistics Support Force of the People’s Liberation Army
Abstract
Alveolar soft part sarcoma (ASPS) is a rare type of soft tissue sarcoma; ASPS originating in the central nervous system is even rarer. Previous literature has generally described it as having an indolent course with a favorable prognosis following surgical resection. This report presents a case of a 31-year-old woman with primary intracranial alveolar soft part sarcoma (PIASPS) that exhibited the opposite characteristics, demonstrating high invasiveness. The patient underwent gross total resection during the initial surgery, and postoperative histopathological examination and immunohistochemistry confirmed the diagnosis of PIASPS. Rapid local recurrence occurred just over two months after surgery; the tumor exhibited abnormally high proliferative activity, with an initial Ki-67 index of approximately 65%, which rose to 75% following recurrence. Molecular testing also revealed a complex mutation profile, including a BRAF p.V600E mutation, a TP53 p.D281E missense mutation, a TERT promoter mutation, an NBN truncating mutation, an HGF nonsense mutation, and an RPSAP52-HMGA2 fusion; these molecular events may have contributed to the rapid tumor recurrence, although their individual and combined effects require further functional validation. Following recurrence, the patient underwent a second surgery, followed by adjuvant radiotherapy and concurrent oral anlotinib; however, the patient died two months following the completion of radiotherapy, with an overall survival of eight months. Characterized by rapid early postoperative recurrence, sustained high proliferative activity, and complex molecular features, this case challenges the traditional perception of PIASPS as an indolent tumor. This case suggests that a high Ki-67 index combined with molecular subtyping may help identify potentially aggressive subtypes; its value in guiding decisions regarding adjuvant therapy may be further explored in future studies.
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BibTeX
@article{ma2026case,
title = {{Case Report: A case of early rapid recurrence of a primary intracranial alveolar soft part sarcoma with high proliferative activity and complex molecular variations}},
author = {Xinying Ma and Jing Liu and Defu Yang and WanLiang Sun and Ying Xu},
journal = {Frontiers in Oncology},
year = {2026},
month = oct,
doi = {10.3389/fonc.2026.1943604},
url = {https://www.frontiersin.org/articles/10.3389/fonc.2026.1943604/full},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Case Report: A case of early rapid recurrence of a primary intracranial alveolar soft part sarcoma with high proliferative activity and complex molecular variations
AU - Xinying Ma
AU - Jing Liu
AU - Defu Yang
AU - WanLiang Sun
AU - Ying Xu
PY - 2026
DA - 2026/10/15
JO - Frontiers in Oncology
DO - 10.3389/fonc.2026.1943604
UR - https://www.frontiersin.org/articles/10.3389/fonc.2026.1943604/full
AB - Alveolar soft part sarcoma (ASPS) is a rare type of soft tissue sarcoma; ASPS originating in the central nervous system is even rarer. Previous literature has generally described it as having an indolent course with a favorable prognosis following surgical resection. This report presents a case of a 31-year-old woman with primary intracranial alveolar soft part sarcoma (PIASPS) that exhibited the opposite characteristics, demonstrating high invasiveness. The patient underwent gross total resection during the initial surgery, and postoperative histopathological examination and immunohistochemistry confirmed the diagnosis of PIASPS. Rapid local recurrence occurred just over two months after surgery; the tumor exhibited abnormally high proliferative activity, with an initial Ki-67 index of approximately 65%, which rose to 75% following recurrence. Molecular testing also revealed a complex mutation profile, including a BRAF p.V600E mutation, a TP53 p.D281E missense mutation, a TERT promoter mutation, an NBN truncating mutation, an HGF nonsense mutation, and an RPSAP52-HMGA2 fusion; these molecular events may have contributed to the rapid tumor recurrence, although their individual and combined effects require further functional validation. Following recurrence, the patient underwent a second surgery, followed by adjuvant radiotherapy and concurrent oral anlotinib; however, the patient died two months following the completion of radiotherapy, with an overall survival of eight months. Characterized by rapid early postoperative recurrence, sustained high proliferative activity, and complex molecular features, this case challenges the traditional perception of PIASPS as an indolent tumor. This case suggests that a high Ki-67 index combined with molecular subtyping may help identify potentially aggressive subtypes; its value in guiding decisions regarding adjuvant therapy may be further explored in future studies.
SN - 2234-943X
ER -