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Updated: May 10, 2026

Tumorsphere Derivation and Treatment from Primary Tumor Cells Isolated from Mouse Rhabdomyosarcomas

Tumorsphere Derivation and Treatment from Primary Tumor Cells Isolated from Mouse Rhabdomyosarcomas

Published on: September 13, 2019

[Clinicopathologic features of pleomorphic rhabdomyosarcoma].

Lin Yu¹, Jian Wang

¹Department of Pathology, Fudan University, Shanghai, China.

Zhonghua Bing Li Xue Za Zhi = Chinese Journal of Pathology

|June 18, 2013

Summary

Pleomorphic rhabdomyosarcoma (PRMS) is a high-grade sarcoma often resembling undifferentiated pleomorphic sarcoma. Diagnosis relies on identifying pleomorphic rhabdomyoblasts and specific immunophenotypes, particularly desmin positivity.

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Induction of Mesenchymal-Epithelial Transitions in Sarcoma Cells

Induction of Mesenchymal-Epithelial Transitions in Sarcoma Cells

Published on: April 7, 2017

Area of Science:

Oncology
Pathology
Sarcoma Research

Context:

Pleomorphic rhabdomyosarcoma (PRMS) is a rare and aggressive soft tissue sarcoma.
Distinguishing PRMS from other pleomorphic sarcomas, such as undifferentiated pleomorphic sarcoma (UPS) and pleomorphic leiomyosarcoma, is diagnostically challenging.

Purpose:

To elucidate the clinicopathologic features, diagnostic criteria, and biological behavior of PRMS.
To aid in the differential diagnosis of PRMS by analyzing its histological and immunophenotypic characteristics.

Summary:

A review of 44 PRMS cases revealed a median age of 55 years, with tumors frequently occurring in the extremities and trunk.
Histologically, PRMS often mimics UPS/MFH but contains pleomorphic rhabdomyoblasts. Immunohistochemistry showed diffuse desmin positivity (100%) and variable expression of myogenin, MyoD1, and MSA, with negative α-SMA in most cases.
Follow-up data indicated a poor prognosis, with a median disease-free survival of 6.0 months and median overall survival of 8.0 months, and a high rate of disease progression.

Impact:

This study provides critical insights into the diagnosis and behavior of PRMS, emphasizing the importance of desmin staining for differentiation.
Findings underscore PRMS as a high-grade sarcoma with a poor prognosis, necessitating careful consideration in differential diagnoses, especially in adults.
The research contributes to a better understanding of PRMS, potentially guiding future therapeutic strategies and improving patient outcomes.