Case Report
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jul 26, 2020; 8(14): 3097-3107
Published online Jul 26, 2020. doi: 10.12998/wjcc.v8.i14.3097
Intra-abdominal inflammatory pseudotumor-like follicular dendritic cell sarcoma associated with paraneoplastic pemphigus: A case report and review of the literature
Jia-Yi Zhuang, Fang-Fei Zhang, Qing-Wen Li, Yong-Feng Chen
Jia-Yi Zhuang, Fang-Fei Zhang, Qing-Wen Li, Yong-Feng Chen, Department of Dermatology, Dermatology Hospital, Southern Medical University/Guangdong Provincial Dermatology Hospital, Guangzhou 510091, Guangdong Province, China
Author contributions: All authors contributed to the study; Zhuang JY wrote the manuscript and analysed the data; Chen YF was the patient’s treating doctor and was responsible for the revision of the manuscript for important intellectual content; Zhang FF and Li QW collected the data; all authors read and approved the final manuscript.
Conflict-of-interest statement: The authors declare that they have no conflict of interest related to this manuscript.
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: Yong-Feng Chen, MD, Professor, Department of Dermatology, Dermatology Hospital, Southern Medical University/Guangdong Provincial Dermatology Hospital, No. 2, Lujing Road, Guangzhou 510091, Guangdong Province, China. gdcyf@163.com
Received: May 18, 2020
Peer-review started: May 18, 2020
First decision: June 4, 2020
Revised: June 9, 2020
Accepted: June 29, 2020
Article in press: June 29, 2020
Published online: July 26, 2020
Processing time: 67 Days and 8.4 Hours
Abstract
BACKGROUD

Follicular dendritic cell (FDC) sarcomas are rare neoplasms that occur predominantly in the lymph nodes. They can also occur extranodally. Extranodal FDC sarcomas most commonly present as solitary masses. Inflammatory pseudotumor (IPT)-like FDC sarcomas, a subcategory of FDC sarcomas, are rarer than other sarcoma subtypes. They are composed of spindle or ovoid neoplastic cells and exhibit an admixture of plasma cells and prominent lymphoplasmacytic infiltration. Paraneoplastic pemphigus (PNP), also known as paraneoplastic autoimmune multiorgan syndrome, is a rare autoimmune bullous disease that is associated with underlying neoplasms. PNP has a high mortality, and its early diagnosis is usually difficult.

CASE SUMMARY

We describe a 27-year-old woman who presented with stomatitis, conjunctivitis, and skin blisters and erosions as her first symptoms of PNP with an intra-abdominal IPT-like FDC sarcoma. The patient underwent surgical tumor resection and received tapering oral corticosteroid treatment. She showed no recurrence at the 1-year follow-up.

CONCLUSION

IPT-like FDC sarcomas are rare underlying neoplasms that have an uncommon association with PNP. PNP-associated FDC sarcomas predominantly occur in intra-abdominal sites and suggest a poor prognosis. Surgical resection is an essential and effective treatment for PNP and primary and recurrent FDC sarcomas.

Keywords: Inflammatory pseudotumor-like follicular dendritic cell sarcoma; Paraneoplastic pemphigus; Follicular dendritic cell sarcoma; Resection; Case report

Core tip: To date, 32 cases of paraneoplastic pemphigus (PNP)-associated follicular dendritic cell (FDC) sarcomas have been reported in the English literature. Inflammatory pseudotumor-like FDC sarcoma was described as an underlying neoplasm of PNP in only two cases. Here, we report a case that PNP was the patient’s first symptom of an intra-abdominal inflammatory pseudotumor-like FDC sarcoma, and review the related literature.