J Korean Soc Radiol.  2019 Jan;80(1):122-128. 10.3348/jksr.2019.80.1.122.

A Rare Case of Small Bowel Undifferentiated Pleomorphic Sarcoma: Imaging Findings on CT and Magnetic Resonance Enterography

• Han YE
• Chae SY
• Lee JH
• Park BJ
• Sung DJ
• Han NY
• Sim KC
• Kim MJ

• KMID: 2442471
• DOI: http://doi.org/10.3348/jksr.2019.80.1.122

Abstract

Gastrointestinal tract involvement in undifferentiated pleomorphic sarcoma (UPS) is extremely rare. To the authors' knowledge, only 21 cases of primary small bowel UPS have been reported in the literature available in English. Reported CT findings in primary small bowel UPS have been nonspecific, and MRI findings have been reported in only one case. The present article describes a case involving a 72-year-old male with histologically confirmed primary UPS arising from the ileum, focusing on both CT and magnetic resonance enterography findings. On CT, primary small bowel UPS was noted as a heterogeneously enhanced small bowel mass without obstruction. Magnetic resonance enterography revealed heterogeneous intermediate T1 and T2 signal intensity, with hemorrhagic or necrotic foci within the mass and heterogeneous enhancement. The differential diagnosis included malignant gastrointestinal tumor; however, the prognosis of UPS is worse, with higher incidences of extra-abdominal metastasis.

MeSH Terms

Aged
Diagnosis, Differential
Gastrointestinal Tract
Histiocytoma
Histiocytoma, Malignant Fibrous
Humans
Ileum
Incidence
Intestine, Small
Magnetic Resonance Imaging
Male
Neoplasm Metastasis
Prognosis
Sarcoma*
Tomography, X-Ray Computed

Figure

• 

  Fig. 1. Primary undifferentiated pleomorphic sarcoma of the small bowel in a 72-year-old male patient. A. CT enterography revealing a mass 6.8 cm in size at the ileum, exhibiting endoluminal and exoluminal growth. On the portal phase, the mass exhibits heterogeneous enhancement (arrow). B. On T1WI in MR enterography, the mass exhibited heterogeneous intermediate signal intensity (arrow). C. On T2WI, the mass exhibited heterogeneous intermediate signal intensity with T2 hyperintense foci (arrow). D. On T1WI (fat suppressed), 70 s after intravenous contrast administration, the mass exhibited heterogeneous enhancement with non-enhancing foci (arrow). E. On the ADC map, the mass exhibited diffuse restriction with a low ADC value (arrow). F. On gross examination, a 12.4 × 7.9 × 3.8 cm exophytic soft mass (arrow) was identified in the small intestinal wall. Microscopic examination (hematoxylin and eosin, × 100) revealed solid sheets of malignant tumor cells, which are mixture of spindle cells with vague fascicular pattern, pleomorphic cells, and a considerable number of bizarre multinucleated tumor giant cells. ADC = apparent diffusion coefficient, WI = weighted imaging

Reference

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