Dunya Yang , Shouyue Chen , Xiaoyu Zhang , Xiangwu Lin , Shaoqin Lin , Qunxiang Chen , Zongjing Zhang , Jie Li , Xi Chen
{"title":"Pancreatic panniculitis associated with presumed advanced pancreatic tumor: A case report and literature review","authors":"Dunya Yang , Shouyue Chen , Xiaoyu Zhang , Xiangwu Lin , Shaoqin Lin , Qunxiang Chen , Zongjing Zhang , Jie Li , Xi Chen","doi":"10.1016/j.prp.2025.156214","DOIUrl":null,"url":null,"abstract":"<div><div>Pancreatic tumor-associated panniculitis is a rare paraneoplastic syndrome, primarily caused by systemic release of pancreatic enzymes (e.g., lipase) from malignancies like acinar cell carcinoma (ACC), leading to necrotizing inflammation in subcutaneous and other adipose tissues. This report details an 87-year-old female presenting with progressive skin lesions (erythematous rash → indurated swelling → ulceration with purulent drainage), lower limb pain, fever, and markedly elevated lipase (3686.2 U/L). Skin biopsy confirmed lobular panniculitis with characteristic \"ghost cells\". Imaging revealed a pancreatic tail mass and liver lesions. Despite sequential anti-infectives, glucocorticoids, Octreotide, Somatostatin, Ulinastatin, and Cimetidine, her condition deteriorated, culminating in death. Literature review confirms that ACC is the predominant pancreatic tumor type causing panniculitis (accounting for 85 %). Diagnosis hinges on recognizing \"ghost cells\" pathologically and significant hyperlipasemia (often >4000 U/L). Crucially, skin manifestations frequently precede abdominal symptoms, leading to misdiagnosis and delayed cancer management. Controlling panniculitis fundamentally requires treating the primary tumor: surgical resection (including metastases) significantly improves survival; platinum-based chemotherapy (e.g., FOLFOX/FOLFIRINOX) is preferred for ACC; and molecular profiling (BRCA/RAF/NTRK) guided targeted therapy is promising. Prognosis is poor; early recognition via clinician vigilance for unexplained panniculitis and pathologist identification of \"ghost cells\", coupled with multidisciplinary collaboration and aggressive tumor-directed therapy, is essential for improving outcomes.</div></div>","PeriodicalId":19916,"journal":{"name":"Pathology, research and practice","volume":"275 ","pages":"Article 156214"},"PeriodicalIF":3.2000,"publicationDate":"2025-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Pathology, research and practice","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0344033825004078","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"PATHOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Pancreatic tumor-associated panniculitis is a rare paraneoplastic syndrome, primarily caused by systemic release of pancreatic enzymes (e.g., lipase) from malignancies like acinar cell carcinoma (ACC), leading to necrotizing inflammation in subcutaneous and other adipose tissues. This report details an 87-year-old female presenting with progressive skin lesions (erythematous rash → indurated swelling → ulceration with purulent drainage), lower limb pain, fever, and markedly elevated lipase (3686.2 U/L). Skin biopsy confirmed lobular panniculitis with characteristic "ghost cells". Imaging revealed a pancreatic tail mass and liver lesions. Despite sequential anti-infectives, glucocorticoids, Octreotide, Somatostatin, Ulinastatin, and Cimetidine, her condition deteriorated, culminating in death. Literature review confirms that ACC is the predominant pancreatic tumor type causing panniculitis (accounting for 85 %). Diagnosis hinges on recognizing "ghost cells" pathologically and significant hyperlipasemia (often >4000 U/L). Crucially, skin manifestations frequently precede abdominal symptoms, leading to misdiagnosis and delayed cancer management. Controlling panniculitis fundamentally requires treating the primary tumor: surgical resection (including metastases) significantly improves survival; platinum-based chemotherapy (e.g., FOLFOX/FOLFIRINOX) is preferred for ACC; and molecular profiling (BRCA/RAF/NTRK) guided targeted therapy is promising. Prognosis is poor; early recognition via clinician vigilance for unexplained panniculitis and pathologist identification of "ghost cells", coupled with multidisciplinary collaboration and aggressive tumor-directed therapy, is essential for improving outcomes.
期刊介绍:
Pathology, Research and Practice provides accessible coverage of the most recent developments across the entire field of pathology: Reviews focus on recent progress in pathology, while Comments look at interesting current problems and at hypotheses for future developments in pathology. Original Papers present novel findings on all aspects of general, anatomic and molecular pathology. Rapid Communications inform readers on preliminary findings that may be relevant for further studies and need to be communicated quickly. Teaching Cases look at new aspects or special diagnostic problems of diseases and at case reports relevant for the pathologist''s practice.