Case Report
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Apr 26, 2020; 8(8): 1495-1501
Published online Apr 26, 2020. doi: 10.12998/wjcc.v8.i8.1495
Rare anaplastic sarcoma of the kidney: A case report
Jsun-Liang Kao, Swei-Hsiung Tsung, Chih-Chung Shiao
Jsun-Liang Kao, Chih-Chung Shiao, Division of Nephrology, Department of Internal Medicine, Camillian Saint Mary’s Hospital Luodong, Yilan 26546, Taiwan
Swei-Hsiung Tsung, Department of Pathology, Camillian Saint Mary’s Hospital Luodong, Yilan 26546, Taiwan
Chih-Chung Shiao, Saint Mary’s Junior College of Medicine, Nursing and Management, Yilan 26546, Taiwan
Author contributions: Kao JL and Shiao CC gathered the information of the patients, wrote the manuscript and approved the manuscript; Tsung SH performed the pathological interpretation, wrote the manuscript, revised the manuscript and approved the manuscript.
Informed consent statement: Informed written consent was obtained from the patient’s family member for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Chih-Chung Shiao, MD, Associate Professor, Deputy superintendent, Camillian Saint Mary’s Hospital Luodong, No. 160 Chong-Cheng South Road, Loudong, Yilan 26546, Taiwan. chungyy2001@smc.edu.tw
Received: January 23, 2020
Peer-review started: January 23, 2020
First decision: February 26, 2020
Revised: March 29, 2020
Accepted: April 17, 2020
Article in press: April 17, 2020
Published online: April 26, 2020
Processing time: 78 Days and 20.8 Hours
Abstract
BACKGROUND

Anaplastic sarcoma of the kidney (ASK) is a rare and newly recognized renal neoplasm. The tumor usually is extensive and cystic, characterized by pleomorphic spindle cells with marked atypia and associated with multinucleated cells. To date, only 27 cases have been reported in the literature. The authors present an additional case and summarize the relevant knowledge in the literature.

CASE SUMMARY

A 27-year-old previously healthy woman presented with a palpable mass over the abdomen and right flank soreness for one year. After the computed tomography study, the patient underwent right radical nephrectomy obtaining a 1680-g tumor with a size of 18.4 cm × 14.5 cm × 11 cm. The tumor is chiefly composed of anaplastic spindle cells with marked nuclear atypia admixed with multinucleated cells. Immunohistochemical evaluation of tumor cells exhibited diffuse positivity for CD56, p53, and vimentin, and focally positive for desmin. The diagnosis of ASK was established. Unfortunately, a local tumor recurrence followed by a distant metastasis developed within months. The patient died 26 months after the initial surgery. Comparing to the previously 27 cases of ASK, the current case had a relatively worse prognosis, which might be potentially associated with older patient age, larger tumor size, and the lack of en-bloc resection of adjacent organs during the initial radical nephrectomy.

CONCLUSION

This case points out the featured pathological findings for diagnosing ASK and suggests more aggressive management for patients with ASK.

Keywords: Anaplastic sarcoma of the kidney; En bloc resection; Local recurrence; Metastasis; Mortality; Radical nephrectomy; Case report

Core tip: The current case report points out the crucial features to differentiate the rare tumor, anaplastic sarcoma of the kidney (ASK), from other renal malignancies. Besides, the authors summarized the demographic, clinical, and prognostic information of the 28 ASK cases reported in the literature. Based on the lethal outcome in a 5-year old patient and two patients with stage 1 of the disease at presentation, we suggest that all patients with ASK should receive radical nephrectomy, en bloc resection of adjacent organs, chemotherapy, and long-term close follow-up. Further experience is essential to modify this approach in the future.