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Kim, Lee, Yoon, and Yun: CT Findings of Primary Undifferentiated Pleomorphic Sarcoma in the Small Bowel: A Case Report
Abdominal Radiology

Journal of the Korean Society of Radiology 2015; 73(5): 323-327.

Published online: 28 October 2015

DOI: https://doi.org/10.3348/jksr.2015.73.5.323

CT Findings of Primary Undifferentiated Pleomorphic Sarcoma in the Small Bowel: A Case Report

Youe Ree Kim, MD1, Young Hwan Lee, MD1, Kwon-Ha Yoon, MD1, Ki Jung Yun, MD2

1Department of Radiology, Wonkwang University School of Medicine & Hospital, Institute of Wonkwang Medical Science, Iksan, Korea.

2Department of Pathology, Wonkwang University School of Medicine & Hospital, Institute of Wonkwang Medical Science, Iksan, Korea.

Corresponding author: Young Hwan Lee, MD. Department of Radiology, Wonkwang University Hospital, 895 Muwang-ro, Iksan 54538, Korea. Tel. 82-63-859-1920, Fax. 82-63-851-4749, yjyh@wonkwang.ac.kr

Received 4 May 2015       Revised 11 June 2015       Accepted 14 July 2015

Copyright © 2015 The Korean Society of Radiology

(open-access):

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma, is a soft tissue sarcoma arising from mesenchymal tissue of the body. UPS of the gastrointestinal tract is known to be rare and only a few cases have been reported in the literature. Based on our case and review of the other relevant literature, the CT findings of primary UPS of the small bowel included nodular bowel wall thickening with homogeneous enhancement. It presents as a rapidly growing tumor without bowel obstruction, and it may be accompanied by distant metastasis.

Keywords: Primary Undifferentiated Pleomorphic Sarcoma, Primary Malignant Fibrous Histiocytoma, Small Intestine, Ileum, Multidetector Computed Tomography

Figures and Tables

Fig. 1

Primary undifferentiated pleomorphic sarcoma (UPS) of the small bowel with adrenal metastases in a 51-year-old man.

A. The initial computed tomography (CT) image reveals segmental nodular wall thickening of the ileum with homogeneous enhancement (arrows). The degree of tumor enhancement is lower than that of normal mural enhancement. The size of the tumor is 3.6 × 2.7 cm.
B. The initial CT image presents two adrenal masses of medial (arrow) and lateral (not shown) limbs with heterogeneous enhancement. The size of upper limb tumor is 3.1 cm.
C. On a follow-up CT image 80 days later, the ileal tumor grew rapidly to 6.5 × 4.2 cm on the axial image. The tumor showing enhancing nodular wall thickening became more heterogeneous (arrows). Though the thickness of the wall is 2.0 cm or more, the patent lumen is seen as an aneurysmal dilatation.
D. On a follow-up CT image 80 days later, the right adrenal gland mass is 5.2 cm in size. It presents with central low-attenuation with a mild enhancing inner solid portion (arrow).
E. The gross pathological findings of small bowel UPS. A 6 × 5 cm tumor with diffuse necrotic changes and ulceration is noted.
F. A photomicrograph reveals the hypercellularity and storiform architecture of the pleomorphic spindle cells (hematoxylin and eosin, × 200).
G. These tumor cells are immunoreactive for alpha-1-antichymotrysin (× 200).
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