Undifferentiated pleomorphic sarcoma of the retroperitoneum

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retroperitoneal malignant fibrous histiocytoma in childhood. At arterial phase, the tumour showed a non -omogeneous hypodense mass.
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retroperitoneal malignant fibrous histiocytoma in childhood. The tumohr showed an abundance of tortuous blood vessels. The right lobe of the liver was compressed by the mass and visibly deformed.
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retroperitoneal malignant fibrous histiocytoma in childhood. The tumour had variously enhanced patchy scattered areas. Similarly, the right kidney was compressed and deformed.
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retroperitoneal malignant fibrous histiocytoma in childhood. At delayed phases, the tumour was intensified more lastingly and broader in range than on arterial period.
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retroperitoneal malignant fibrous histiocytoma in childhood. The mass had an intact capsule and was located on the entire right side of the abdomen without infiltration of surrounding visceral organs.
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retroperitoneal malignant fibrous histiocytoma in childhood. The mass had an intact capsule and was located on the entire right side of the abdomen without infiltration of surrounding visceral organs.
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retroperitoneal malignant fibrous histiocytoma in childhood. The delayed enhancement of solid components, fibrous septa and tumour capsule was seen inside the tumour.
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Primary
retroperitoneal neoplasms • Undifferentiated pleomorphic sarcoma of the retroperitoneum - Ganzer Fall bei Radiopaedia
Glick Y, Undifferentiated pleomorphic sarcoma of the retroperitoneum. Case study, Radiopaedia.org (Accessed on 06 Oct 2023) https://doi.org/10.53347/rID-172448 • CC by-nc-sa 3.0 (modified)
Radiopaedia • CC-by-nc-sa 3.0 • de

Undifferentiated pleomorphic sarcomas (UPS), previously known as malignant fibrous histiocytoma (MFH), are an uncommon tumor overall but are considered as one of the most common types of primary retroperitoneal neoplasms.

For a broad discussion of this type of a tumor, which commonly involves the extremities, please refer to the main article on undifferentiated pleomorphic sarcoma.

Epidemiology

Retroperitoneal malignant fibrous histiocytomas correspond to approximately 15% of all UPS . They occur with predominance in men.

Pathology

They are categorized as sarcomas and are often high-grade tumors.

There are six histological subtypes: storiform, pleomorphic, myxoid, giant cell, inflammatory and angiomatoid. Most metastasize and common sites include lung and lymph nodes.

Radiographic features

Imaging is not characteristic and clarification pathologically is required. Reassessment of a UPS diagnosis in some series result in re-classification as dedifferentiated liposarcomas.

25% of cases include dystrophic calcification which is uncommon amongst the remaining primary retroperitoneal malignancies.

Siehe auch:
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Assoziationen und Differentialdiagnosen zu pleomorphes undifferenziertes Sarkom des Retroperitoneums:
retroperitoneale
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