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Han, Chae, Lee, Park, Sung, Han, Sim, and Kim: A Rare Case of Small Bowel Undifferentiated Pleomorphic Sarcoma: Imaging Findings on CT and Magnetic Resonance Enterography
Case Report

J Korean Soc Radiol 2019;80(1):122-128.

Published online: 20 January 2019

DOI: https://doi.org/10.3348/jksr.2019.80.1.122

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

Yeo Eun Han, MD1, Soo Young Chae, MD1, Jeong Hyeon Lee, PhD2, Beom Jin Park, PhD1, Deuk Jae Sung, PhD1, Na Yeon Han, PhD1, Ki Choon Sim, MD1, Min Ju Kim, PhD1, ∗

1Department of Radiology, Korea University Anam Hospital, Korea University College of Medicine, Seoul, Korea

2Department of Pathology, Korea University Anam Hospital, Korea University College of Medicine, Seoul, Korea

∗Corresponding author Min Ju Kim, PhD Department of Radiology, Korea University Anam Hospital, KoreaUniversity College of Medicine, 73 Inchon-ro, Seongbuk-gu, Seoul 02841, Korea. Tel 82-2-920-5578 Fax 82-2-929-3796 E-mail dr.minjukim@gmail.com

Received 12 June 2018       Revised 19 July 2018       Accepted 29 July 2018

Copyright © 2019 The Korean Society of Radiology

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.

References

1. Karki B, Xu YK, Wu YK, Zhang WW. Primary malignant fibrous histiocytoma of the abdominal cavity: CT findings and pathological correlation. World J Radiol. 2012; 4:151–158.
crossref
2. Kim YR, Lee YH, Yoon KH, Yun KJ. CT findings of primary undifferentiated pleomorphic sarcoma in the small bowel: a case report. J Korean Soc Radiol. 2015; 73:323–327.
crossref
3. Giuliani J. Primary malignant fibrous histiocytoma of Vater's Papilla: first reported case.J Gastrointest Cancer. 2013; 44:366–367.
4. Kobayashi K, Narita H, Morimoto K, Hato M, Ito A, Sugiyama K. Primary malignant fibrous histiocytoma of the ileum: report of a case. Surg Today. 2001; 31:727–731.
crossref
5. Meyers SP.MRI of bone and soft tissue tumors and tumorlike lesions: differential diagnosis and atlas. Stuttgart and. New York: Thieme;2008.
6. Amzallag-Bellenger E, Oudjit A, Ruiz A, Cadiot G, Soyer PA, Hoeffel CC. Effectiveness of MR enterography for the assessment of small-bowel diseases beyond Crohn disease.Radiograph/iics. 2012; 32:1423–1444.
7. Kroep JR, Bovée JV, van der Molen AJ, Hogendoorn PC, Gelderblom H. Extra-abdominal subcutaneous metastasis of a gastrointestinal stromal tumor: report of a case and a review of the literature.J Cutan Pathol. 2009; 36:565–569.
8. Tran T, Davila JA, El-Serag HB. The epidemiology of malignant gastrointestinal stromal tumors: an analysis of 1458 cases from 1992 to 2000. Am J Gastroenterol. 2005; 100:162–168.
crossref
9. Wang ZS, Xiong CL, Zhan N, Xiong GS, Li H, Hu H. Primary malignant fibrous histiocytoma of the small bowel: a report of an additional case in duodenum. Int J Gastrointest Cancer. 2005; 36:105–112.
crossref
10. Katsourakis A, Noussios G, Hadjis I, Evangelou N, Chatzitheoklitos E. Primary malignant fibrous histiocytoma: a rare case.Case Rep Med. 2011; 2011:134801.

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
jksr-80-122f1.tif
Table 1.
Previously Reported CT Findings of Small Bowel Undifferentiated Pleomorphic Sarcoma in the English Literature
Author Year of Publication Sex Age (Years) Location CT Findings
Fukunaga et al. 1999 F 50 Ileum Distal small intestine in the pelvic cavity, colonic dilatation, and ascites
Farinon et al. 1999 F 61 Duodenum Infiltrating duodenal mass (6 cm)
Umehara et al. 2000 M 16 Jejunum Intussusception with small intestinal tumor
Kobayashi et al. (4) 2001 M 71 Ileum Oval mass (8 cm) with diffuse enhancement in the pelvis
Kotan et al. 2002 M 58 Ileum Intussusceptions of the small intestine with suspected tumor
Jiao et al. 2002 M 76 Jejunum Focal thickening of the wall of the small intestine
Hasegawa et al. 2004 M 52 Ileum Intussusception of the small intestine
Wang et al. (9) 2005 M 61 Duodenum Duodenal tumor
Spanos et al. 2005 M 41 Jejunum Intussusception of the jejunum
Fu et al. 2007 M 43 Small intestine Multiple intussusceptions of the small intestine
Katsourakis et al. (10) 2011 F 67 Jejunum Tumor in the pelvic cavity near the small intestine
Karki et al. (1) 2012 M 79 Ileum Intraluminal and intramural mass without necrosis in the ileum
Giuliani (3) 2013 M 67 Vaters papilla Mass in the pancreatic head and duodenum with enhancement
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