Pancreatic follicular dendritic cell sarcoma: a rare case report and systematic literature review of 7 cases

World Journal of Surgical Oncology

Case Report

Huichao Wu¹  Shuwen Han¹  Qingyun He¹  Xu Li¹  Jin Gu² 

[1]Department of Gastroenterology, Digestive Disease Hospital, The Affiliated Hospital of Zunyi Medical University, Zunyi City, Guizhou Province, People’s Republic of China

[2]Department of General Surgery, The Affiliated Hospital of Zunyi Medical University, Zunyi City, Guizhou Province, People’s Republic of China

关键词: Follicular dendritic cell sarcoma;    Pancreas;    CT;    MRI;    Diagnosis;    Treatment;   

DOI  :  10.1186/s12957-023-03115-5

 received in 2023-04-12, accepted in 2023-07-13,  发布年份 2023

来源: Springer

【 摘 要 】

IntroductionPancreatic follicular dendritic cell sarcoma (FDCS) is an exceptionally rare and low-to-moderate malignancy, with only seven reported cases to date. Clinical diagnosis of FDCS is challenging due to the lack of distinct biological and radiographic features.Case presentationA 67-year-old woman presented to the hospital with a 4-day history of severe abdominal pain. Imaging studies (CT and MRI) revealed a large cystic mass located at the tail of the pancreas, which was suspected to be myeloid sarcoma (MS) based on EUS and CT-guided pancreatic puncture. Postoperative pathology and immunohistochemistry confirmed the diagnosis of pancreatic FDCS. After the diagnosis was confirmed, the patient received postoperative chemotherapy with the CHOP regimen. At 11 months of follow-up, there was no evidence of recurrence. Seven published cases have been reviewed to comprehensively summarize the clinical characteristics, diagnosis, and treatment options of FDCS.ConclusionWhile imaging can be useful in detecting pancreatic FDCS, it should be interpreted with caution as it can be challenging to differentiate from other pancreatic tumors. Pathology and immunohistochemistry are considered the gold standard for diagnosis, with CD21, CD23, and CD35 being specific tumor cell markers. However, preoperative diagnosis of pancreatic FDCS remains difficult, and the pancreatic puncture may further increase the risk of misdiagnosis. The disease is highly prone to recurrence and metastasis, and surgery is the preferred method for both diagnosis and treatment of localized disease.

【 授权许可】

CC BY   
© The Author(s) 2023

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