Case Report
Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Sep 14, 2018; 24(34): 3958-3964
Published online Sep 14, 2018. doi: 10.3748/wjg.v24.i34.3958
Unicentric Castleman disease presenting as a retroperitoneal peripancreatic mass: A report of two cases and review of literature
Jia-Lin Cheng, Jing Cui, Yi Wang, Zong-Zhen Xu, Feng Liu, Shu-Bin Liang, Hu Tian
Jia-Lin Cheng, Shu-Bin Liang, Taishan Medical University, Tai’an 271016, Shandong Province, China
Jia-Lin Cheng, Zong-Zhen Xu, Feng Liu, Shu-Bin Liang, Hu Tian, Department of General Surgery, Shandong Provincial Qianfoshan Hospital, Shandong University, Jinan 250014, Shandong Province, China
Jing Cui, Department of Pathology, Shandong Provincial Qianfoshan Hospital, Shandong University, Jinan 250014, Shandong Province, China
Yi Wang, Department of Medical Imaging, Shandong Provincial Qianfoshan Hospital, Shandong University, Jinan 250014, Shandong Province, China
Author contributions: Cheng JL designed the report and wrote the paper; Cheng JL, Xu ZZ, Liu F and Tian H performed the surgery; Cheng JL, Cui J, Wang Y and Liang SB collected the patient’s clinical data; Cheng JL and Tian H analyzed the data and revised the paper; all authors have read and approved the final version of this manuscript.
Supported by the Key Research and Development Plan of Shandong Province, No. 2016GSF201108.
Informed consent statement: The patients included in this study gave written informed consent for the use and disclosure of their protected health information.
Conflict-of-interest statement: The authors of this manuscript have no conflicts of interest to disclose.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (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/
Correspondence to: Hu Tian, MD, PhD, Chief Doctor, Professor, Surgeon, Department of General Surgery, Shandong Provincial Qianfoshan Hospital, Shandong University, No. 16766, Jingshi Road, Jinan 250014, Shandong Province, China. tianhu6585@163.com
Telephone: +86-531-89269817 Fax: +86-531-82963647
Received: April 23, 2018
Peer-review started: April 27, 2018
First decision: July 4, 2018
Revised: July 10, 2018
Accepted: July 22, 2018
Article in press: July 22, 2018
Published online: September 14, 2018
Processing time: 145 Days and 13 Hours
ARTICLE HIGHLIGHTS
Case characteristics

Case 1 is a 48 year old female patient who presented with lymph node enlargement; and Case 2 is a 57 year old female patient who presented with tiredness and fever.

Clinical diagnosis

In Case 1, the patient initially had no apparent discomfort or noteworthy positive physical signs. In Case 2, the patient felt tired and had a fever with no other discomfort.

Differential diagnosis

Neuroendocrine tumor, paraganglioma and lymphoma.

Laboratory diagnosis

In Case 1, laboratory examinations revealed that the HIV antibody was negative. Carbohydrate antigen 19-9, carcinoembryonic antigen and other laboratory test results were also within the normal range. In Case 2, laboratory examinations showed that the HIV antibody was negative and the white blood cell count was 14.6 × 109/L. Carbohydrate antigen 19-9, carcinoembryonic antigen and other laboratory test results were also within the normal range.

Imaging diagnosis

In Case 1, MRI of the epigastrium showed that there was an elliptical hyperintense signal below the pancreas on the T1WI, and slight hyperintensity on the T2WI. In addition, marked enhancement on the arterial phase and reduced enhancement on the delayed phase were observed. In Case 2, MRI of the epigastrium showed that there was an elliptical hyperintense signal below the pancreas on the T1WI and slight hyperintensity on the T2WI. Marked enhancement on the arterial phase was inhomogeneous. In addition, the venous phase and delayed phase continued to intensify.

Pathological diagnosis

In Case 1, the retroperitoneal single lymph node was compatible with Castleman disease. In addition, immunohistochemical staining revealed that cells were slightly positive for CD20, CD3, CD21, bcl-6, and bcl-2, a small number of cells were positive for CD138, and 20% of cells were positive for Ki-67. In Case 2, the diagnosis was retroperitoneal Castleman disease together with a follicular dendritic cell tumor. Immunohistochemical staining showed that a portion of cells were positive for CD21 and cells were negative for CK, a small number of cells were positive for S-100 and slightly positive for CD 1a and CD23, and about five percent of cells were positive for Ki-67.

Treatment

In Case 1, laparotomy was performed to remove the retroperitoneal peripancreatic tumor without subsequent radiochemotherapy. In Case 2, laparoscopic surgery was performed to remove the retroperitoneal peripancreatic tumor without subsequent radiochemotherapy.

Related reports

Unicentric Castleman disease (UCD) is a rare disease of lymph nodes and related tissues, and occurrence in the retroperitoneal peripancreatic region is quite rare. Only a few cases of UCD together with follicular dendritic cell tumor have been identified. At present, complete resection of the tumor is still the most effective treatment for retroperitoneal peripancreatic UCD with low recurrence and high cure rates.

Term explanation

Follicular dendritic cell tumor is an uncommon neoplasm that has features of follicular dendritic cell differentiation, and typically presents as a slow-growing, painless mass without systemic symptoms. The tumor can occur on various parts of the body such as lymph nodes, tonsils, armpits and mediastinum, but is most common in the neck lymph nodes.

Experiences and lessons

At present, complete resection of the tumor is still the most effective treatment for retroperitoneal peripancreatic UCD with low recurrence and high cure rates. The diagnosis of retroperitoneal peripancreatic UCD is still dependent on histopathology. The two rare cases of retroperitoneal peripancreatic UCD included in this report will improve our understanding of this disease and provide a significant reference for future cases.