Jiang H, Ta N, Huang XY, Zhang MH, Xu JJ, Zheng KL, Jin G, Zheng JM. Pancreatic perivascular epithelioid cell tumor: A case report with clinicopathological features and a literature review. World J Gastroenterol 2016; 22(13): 3693-3700 [PMID: 27053862 DOI: 10.3748/wjg.v22.i13.3693]
Corresponding Author of This Article
Jian-Ming Zheng, MD, Department of Pathology, Changhai Hospital, Second Military Medical University, Shanghai 200433, China. jmzheng1962@163.com
Research Domain of This Article
Pathology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
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/
World J Gastroenterol. Apr 7, 2016; 22(13): 3693-3700 Published online Apr 7, 2016. doi: 10.3748/wjg.v22.i13.3693
Pancreatic perivascular epithelioid cell tumor: A case report with clinicopathological features and a literature review
Hui Jiang, Na Ta, Xiao-Yi Huang, Ming-Hua Zhang, Jing-Jing Xu, Kai-Lian Zheng, Gang Jin, Jian-Ming Zheng
Hui Jiang, Na Ta, Xiao-Yi Huang, Ming-Hua Zhang, Jing-Jing Xu, Jian-Ming Zheng, Department of Pathology, Changhai Hospital, Second Military Medical University, Shanghai 200433, China
Kai-Lian Zheng, Gang Jin, Department of General Surgery, Changhai Hospital, Second Military Medical University, Shanghai 200433, China
Author contributions: Jiang H and Ta N contributed equally to this paper and wrote the paper; Jiang H diagnosed the case and interpreted the data; Ta N assisted with the literature retrieval and clinical data collection; Zheng KL and Jin G carried out the operation and patient follow-up; Huang XY, Zhang MH and Xu JJ helped perform the cytological and immunohistochemical diagnosis; all authors approved the final manuscript for publication.
Institutional review board statement: The study was reviewed and approved by the Shanghai Changhai Hospital Ethnic Committee.
Informed consent statement: The study was performed after obtaining the patient’s informed consent. The patient was treated according to the provisions of the Helsinki criteria.
Conflict-of-interest statement: We declare that we have no commercial or associative interest that represents a conflict of interest in connection with the work submitted.
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: Jian-Ming Zheng, MD, Department of Pathology, Changhai Hospital, Second Military Medical University, Shanghai 200433, China. jmzheng1962@163.com
Telephone: +86-21-81873689 Fax: +86-21-81873689
Received: November 10, 2015 Peer-review started: November 12, 2015 First decision: December 11, 2015 Revised: December 27, 2015 Accepted: January 11, 2016 Article in press: January 11, 2016 Published online: April 7, 2016
Abstract
Perivascular epithelioid cell tumor (PEComa) of the pancreas is an unusual tumor deriving from mesenchyma. This paper described a case of pancreatic PEComa, which was initially suspected as neuroendocrine carcinoma by biopsy, and therefore surgical treatment was recommended due to undetermined diagnosis. Examination of the surgical specimen under a microscope showed that the tumor cell’s morphology was epithelioid or spindle-shaped, and ranged in a nested pattern. Additionally, these cells had a large extent of acidophilic cytoplasm, no mitotic figures, and expressed HMB-45, melan-p, and smooth muscle actin immunohistochemically. Pathological examination indicated that PEComa originated from the pancreas, but symptoms related to tuberous sclerosis were absent. Since PEComa is extremely rare in the pancreas, it is likely to be ignored in differential diagnosis. In conclusion, our article highlighted the clinicopathological features of PEComa, and we conducted a literature review focusing on PEComa so as to deepen the understanding of this tumor type.
Core tip: Perivascular epithelioid cell tumor (PEComa) of the pancreas is an unusual tumor deriving from mesenchyma. Via describing a rare case of pancreatic PEComa, we highlight the clinicopathological features of PEComa and conduct a literature review focusing on this tumor type so as to deepen the understanding of the subject. We also reviewed the biological behavior, prognosis, and therapeutic strategy of PEComa.
