scholarly journals Paraneoplastic Pemphigus Associated with Follicular Dendritic Cell Tumor in the Mediastinum

2016 ◽  
Vol 2016 ◽  
pp. 1-4
Author(s):  
Aparna Mullangath Prakasan ◽  
Anne Jennifer Prabhu ◽  
Kanmani Velarasan ◽  
Selvamani Backianathan ◽  
Thomas Samuel Ram

Paraneoplastic Pemphigus (PNP) is an autoimmune bullous disease characterized by severe stomatitis, polymorphous skin eruptions, and underlying neoplasms. Diagnosis of cutaneous paraneoplastic disorders requires high index of suspicion. We describe a patient with PNP associated with follicular dendritic cell (FDC) tumor in the mediastinum, a rare neoplasm originating from follicular dendritic cells. Its management requires identification of underlying malignancy and treatment of the same. Our patient showed remission of PNP upon excision of the tumor and remained disease-free for 8 years.

1997 ◽  
Vol 288 (2) ◽  
pp. 381-389 ◽  
Author(s):  
Rikiya Tsunoda ◽  
Alain Bosseloir ◽  
Kikuo Onozaki ◽  
Ernst Heinen ◽  
Katsuya Miyake ◽  
...  

2020 ◽  
Author(s):  
Erlei Zhang ◽  
Wenqiang Wang ◽  
Jian Li ◽  
Dong Chen ◽  
Zhiyong Huang

Abstract BackgroundInflammatory pseudotumor (IPT)-like follicular dendritic cell (FDC) sarcoma of the liver is very rare and mostly occurs in females. Up to now, 19 cases were reported in the English literature. Surgical resection remains the preferred method for the management of this kind of tumor. Case presentationWe reported a case of a 47-year-old male patient who had hepatitis B virus infection history and presented with up-abdominal discomfort. He underwent liver resection of segment VII for hepatic non-hodgkin lymphoma (B-cell lymphoma) 19 years ago. Serum alpha fetoprotein was normal. Imaging studies revealed a well-circumscribed, solid mass in the right hepatic lobe and was diagnosed as HCC or hepatic lymphoma. Right hemihepatectomy was performed and the patient was disease free after a follow-up of 20 months. Histological feature showed a mixture of chronic inflammatory cells and variable amounts of spindle cells. Immunohistochemical studies demonstrated that all the tumor cells showed strong nuclear in situ labeling for EBV-encoded small RNAs (EBER) and strongly positive staining with CD21 and CD35. A diagnosis of hepatic IPT-like FDC sarcoma was rendered. The coexistence of IPT-like sarcoma and non-Hodgkin’s lymphoma in same patient is extremely rare. He remains disease-free more than 40 months after surgical resection.ConclusionTo our knowledge, this is the first report to demonstrate hepatic IPT-like FDC sarcoma in a patient with primary hepatic non-hodgkin lymphoma history. Complete surgical resection should be pursued.


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