Related Experiment Video
Updated: Aug 7, 2026
07:55
Establishment of a Primary Culture of Patient-derived Soft Tissue Sarcoma
Published on: April 11, 2018
Dedifferentiated Liposarcoma Presenting as Primary Diffuse Peritoneal Sarcomatosis: A Case Report and Literature
Jiongyuan Wang1, Quan Jiang1, Weiqi Lu2
1Department of Retroperitoneal Soft Tissue Surgical Oncology, Zhongshan Hospital, Fudan University, Shanghai, China.
Journal of Gastrointestinal Cancer
|August 6, 2026
Summary
Dedifferentiated liposarcoma (DDLPS) can rarely present as diffuse peritoneal sarcomatosis. Prompt diagnosis is crucial to differentiate it from peritoneal carcinomatosis, guiding appropriate treatment.
Area of Science:
- Oncology
- Surgical Pathology
- Genetics
Background:
- Dedifferentiated liposarcoma (DDLPS) typically presents as a solitary retroperitoneal mass.
- Diffuse granular and nodular peritoneal sarcomatosis as the initial presentation of DDLPS is exceptionally rare.
Purpose of the Study:
- To report a rare case of DDLPS presenting as diffuse peritoneal sarcomatosis.
- To emphasize the importance of distinguishing DDLPS from epithelial peritoneal carcinomatosis.
Main Methods:
- A 72-year-old man presented with multifocal intra-abdominal masses.
- CT-guided biopsy confirmed DDLPS with MDM2 and CDK4 amplification via FISH.
- Surgical exploration revealed diffuse peritoneal deposits, followed by cytoreductive surgery.
Main Results:
- Histopathology confirmed FNCLCC Grade III DDLPS.
- Next-generation sequencing showed high-level co-amplification of CDK4, MDM2, TSPAN31, CCND2, MDM4, and RAC1.
- The patient had microsatellite stability and a low tumor mutational burden.
Conclusions:
- DDLPS can manifest as primary diffuse peritoneal sarcomatosis, even with a retroperitoneal component.
- When biopsy shows an epithelial-marker-negative spindle cell neoplasm, sarcoma should be considered.
- FISH for MDM2/CDK4 is essential to avoid misdiagnosis as peritoneal carcinomatosis.
