Investig Magn Reson Imaging.  2019 Dec;23(4):361-366. 10.13104/imri.2019.23.4.361.

MRI Finding of Retroperitoneal Desmoplastic Small Round Cell Tumor with Hepatic Metastasis and Portal Vein Thrombosis: a Case Report

• Kim YR
• Lee YH
• Choi KH

• KMID: 2468053
• DOI: http://doi.org/10.13104/imri.2019.23.4.361

Abstract

Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignancy common in young male patient. Typical imaging features of DSRCT include multiple soft tissue masses in the peritoneal cavity, omentum, or mesentery without an organ of origin. This report presents a rare manifestation of DSRCT revealing a solitary large retroperitoneal mass with hepatic metastasis and malignant portal vein thrombosis in 70-year-old women together with the review of literature. The tumor showed a hemorrhagic and necrotic mass with peripheral portion of T2 hypo-intensity and delayed enhancement that indicated desmoplastic stroma with dense cellularity.

Keyword

Desmoplastic small round cell tumor; Retroperitoneum; Metastasis

MeSH Terms

Aged
Desmoplastic Small Round Cell Tumor*
Female
Humans
Magnetic Resonance Imaging*
Male
Mesentery
Neoplasm Metastasis*
Omentum
Peritoneal Cavity
Portal Vein*
Venous Thrombosis*

Figure

• 

  Fig. 1 A 70-year-old woman with retroperitoneal desmoplastic small round cell tumor (DSRCT), liver metastasis, and malignant portal vein thrombosis. (a) Axial CT venous image showing a large solitary retroperitoneal mass demonstrating mild peripheral enhancing portion and central enhancing part. Multiple mild enhancing hepatic masses and malignant portal vein thrombosis at the umbilical section of left portal vein (arrow) were indicated. (b, c) Coronal T2 weighted image and axial T2 fat suppressed image showing mild hyperintense peripheral portion and more hyperintense central part in the retroperitoneal mass. The left adrenal gland was displaced and externally compressed by the mass (arrow). (d) Axial precontrast T1 fat suppressed gradient echo image showing ill-defined hyperintensity in central part suggesting hemorrhagic necrosis. (e-g) Dynamic enhanced T1 fat suppressed images showing arterial nodular enhancement and delayed enhancement of peripheral part. Liver masses and portal vein thrombosis presented mild delayed enhancement. (h, i) Diffusion weighted images (b factor = 800) showing diffusion restriction at peripheral solid part, high and low ADC at central part that resulted from hemorrhagic necrosis. (j, k) Photomicrograph (× 40, Hematoxylin and Eosin) showing desmoplastic stroma and dense speckled cells made up of small round cell and immunohistochemistry (× 400) showing positive results of WT-1, Vimentin, pan-CK, and NSE.

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