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A Mouse Model of Incompletely Resected Soft Tissue Sarcoma for Testing Neoadjuvant Therapies

A Mouse Model of Incompletely Resected Soft Tissue Sarcoma for Testing Neoadjuvant Therapies

Published on: July 28, 2020

CIC-Rearranged Sarcoma.

Naohiro Makise¹, Akihiko Yoshida²

¹Division of Surgical Pathology, Chiba Cancer Center, 666-2 Nitona-cho, Chuo-ku, Chiba-shi, Chiba, 260-8717, Japan.

Surgical Pathology Clinics

|January 26, 2024

Summary

CIC-rearranged sarcoma is an aggressive small round cell cancer affecting deep soft tissues. Diagnosis relies on characteristic histology and immunohistochemistry, though genetic testing for CIC fusion can be challenging.

Keywords:

CIC CIC-rearranged sarcoma DUX4 ETV4 Ewing-like sarcoma Fusion gene

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Area of Science:

Oncology
Pathology
Genetics

Background:

CIC-rearranged sarcoma is a rare, aggressive small round cell sarcoma.
It affects deep soft tissues across a wide age range.
This sarcoma has a poorer prognosis than Ewing sarcoma and chemoresistance.

Purpose of the Study:

To summarize the key features of CIC-rearranged sarcoma.
To highlight diagnostic challenges and methods.

Main Methods:

Review of histological and immunohistochemical findings.
Discussion of genetic detection methods for CIC fusion.

Main Results:

Characteristic histology includes lobulated growth, myxoid changes, and specific cell morphology.
ETV4 and WT1 nuclear expression are valuable immunohistochemical markers.
Genetic testing, including next-generation sequencing, can detect CIC fusion but has limitations, especially for CIC::DUX4.

Conclusions:

CIC-rearranged sarcoma presents distinct histological and immunohistochemical features.
Accurate diagnosis requires integrating clinical, histological, and molecular data.
Challenges remain in sensitive molecular detection of all CIC fusion types.