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Updated: Jul 9, 2026

Establishment of Cancer Stem Cell Cultures from Human Conventional Osteosarcoma

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Histiocytic Sarcoma: A Review and Update.

Yuki Shinohara¹, Shizuhide Nakayama², Mikiko Aoki³

¹Section of Orthopaedic Surgery, Department of Medicine, Fukuoka Dental College, 2-15-1 Tamura, Sawara-ku, Fukuoka 814-0193, Japan.

International Journal of Molecular Sciences

|September 13, 2025

Summary

Histiocytic sarcoma (HS) is a rare, aggressive cancer. This review covers its features, diagnosis using immunohistochemistry, genetic mutations, and limited treatment options, highlighting PD-L1 as a potential target.

Keywords:

diagnosis hematopoietic neoplasm histiocytic sarcoma malignant histiocytosis pathogenesis treatment

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Published on: January 13, 2023

Area of Science:

Hematology
Oncology
Pathology

Background:

Histiocytic sarcoma (HS) is an ultra-rare hematopoietic neoplasm.
It commonly presents in extranodal sites in adults and exhibits aggressive clinical behavior.
HS has a poor prognosis, with a median overall survival of approximately six months.

Purpose of the Study:

To provide a comprehensive overview of histiocytic sarcoma.
To discuss current knowledge on clinicoradiological features, histopathology, pathogenesis, and management.
To highlight recent findings and potential therapeutic targets.

Main Methods:

Review of current literature on histiocytic sarcoma.
Analysis of diagnostic criteria, including histopathology and immunohistochemistry.
Summary of identified genomic alterations and signaling pathway involvement.

Main Results:

HS is characterized by large cells, often misdiagnosed.
Immunohistochemistry is crucial, showing markers like CD163 and CD68.
High PD-L1 expression suggests a therapeutic target; common mutations include RAS/MAPK, PI3K/AKT/mTOR, CDKN2A, and TP53.

Conclusions:

Management of HS lacks a standard protocol, with surgery and radiotherapy for localized disease.
Systemic treatment options for disseminated HS are limited.
Further research into targeted therapies based on genetic alterations and PD-L1 expression is warranted.