Splenic Inflammatory Myofibroblastic Tumor (Inflammatory Pseudotumor)

2001 ◽  
Vol 125 (3) ◽  
pp. 379-385 ◽  
Author(s):  
Thomas S. Neuhauser ◽  
Gregory A. Derringer ◽  
Lester D. R. Thompson ◽  
Julie C. Fanburg-Smith ◽  
Nadine S. I. Aguilera ◽  
...  

Abstract Context.—Inflammatory pseudotumor is an uncommon and enigmatic lesion. The spindle cells found in this tumor have features of myofibroblasts. Because of the indefinite relationship of these lesions with inflammatory fibrosarcoma and their indefinite biologic behavior, inflammatory pseudotumor is currently classified as inflammatory myofibroblastic tumor (IMT). To date, only case reports or small series have been published on these tumors, which are primary in the spleen. Design.—In this study, we describe the clinical, morphologic, and immunophenotypic findings of 12 cases of splenic IMT and examine their relationship to Epstein-Barr virus (EBV). Results.—The patients included 8 women and 3 men, ranging from 19 to 77 years of age (mean, 53 years; median, 60 years). Demographic data were unavailable for 1 patient. Patients generally presented with abdominal pain (n = 5) and fever (n = 4). Associated lesions included renal cell carcinoma (n = 2), colonic adenocarcinoma (n = 1), and cholecystitis (n = 1). All tumors were composed of a bland spindle cell proliferation in association with a variable mixed inflammatory component. There were 2 growth patterns, namely, a cellular spindle cell pattern and a hypocellular fibrous pattern. An immunohistochemical panel confirmed the myofibroblastic nature of the spindle cells. The spindle cells of 2 cases were immunoreactive for EBV latent membrane protein 1, whereas 6 of 10 cases were positive for EBV-encoded RNA using in situ hybridization. Follow-up was available for 8 patients; 6 were alive with no evidence of recurrence and 2 were dead of other causes. Conclusion.—Splenic IMTs are uncommon lesions that can be distinguished from other conditions using a combination of clinical, histologic, and immunophenotypic findings. Epstein-Barr virus may play a role in the pathogenesis of splenic IMT, and there may be an association of splenic IMT with concomitant disease or malignancy. Most splenic IMTs have an excellent long-term prognosis.

2015 ◽  
Vol 93 (6) ◽  
pp. e41-e43 ◽  
Author(s):  
Antonio Palomeque Jiménez ◽  
Montserrat Reyes Moreno ◽  
Salvador Calzado Baeza ◽  
Sergio Antonio Ranea Jimena ◽  
Paul Sebastian Robayo Soto

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S77-S78
Author(s):  
H Bharadwaj ◽  
J Freeman

Abstract Casestudy: Inflammatory pseudotumor-like follicular/fibroblastic dendritic cell sarcoma (IP-L FDC/FRC) is a rare dendritic cell neoplasm recognized as a specific entity in the WHO classification of tumors of the hematopoietic and lymphoid tissues. The tumor has specific clinical features, including a predominance in young to middle-aged women typically involving the liver or spleen, with neoplastic cells typically positive for Epstein-Barr virus. Morphologically, the tumor poses a diagnostic challenge to distinguish it from inflammatory pseudotumor and inflammatory myofibroblastic tumor, with which it shares considerable morphologic and immunohistochemical overlap. We present a case report of a 35-year-old female who presented with weight loss, malaise, and intraabdominal mass. MRI revealed a 14 cm large ovoid solid mass with central hemorrhage and necrosis. Biopsy and subsequent resection of the tumor confirmed IP-L FDC/FRC sarcoma. Histologically, the tumor was composed of spindle cells admixed with many lymphocytes and prominent plasma cells. Immunohistochemical stains showed neoplastic spindle cells focally positive for dendritic cell markers CD21, CD23, and CD35, diffuse positivity for SMA, and striking in situ hybridization for Epstein-Barr virus (EBER). Due to its rarity, biologic behavior and outcomes are not well established. The tumors are generally regarded as indolent with a risk of repeated intraabdominal recurrence, although recurrences may be related to the ease and completeness of surgical resection. It is essential to distinguish this tumor from other high- grade sarcomas with greater metastatic potential and overall worse prognosis.


2003 ◽  
Vol 120 (1) ◽  
pp. 56-61 ◽  
Author(s):  
Jason T. Lewis ◽  
Robyn L. Gaffney ◽  
Mary B. Casey ◽  
Michael A. Farrell ◽  
William G. Morice ◽  
...  

2014 ◽  
Vol 03 (08) ◽  
pp. 443-449
Author(s):  
Slim Jarboui ◽  
Abelwaheb Hlel ◽  
Med Ali Bekkay ◽  
Habib BelHaadj Khalifa ◽  
Moncef Mokni

2006 ◽  
Vol 63 (8) ◽  
pp. 757-760 ◽  
Author(s):  
Natasa Colovic ◽  
Radoje Colovic ◽  
Nikica Grubor ◽  
Marijan Micev ◽  
Vladimir Radak ◽  
...  

Background. Inflammatory pseudotumors are benign tumor lesions of an unknown etiology that can appear almost at any organ, sometimes along with infectious agens, like Epstein-Barr virus. They are extremely rare in the spleen, with less than 80 cases described in the world literature. It is a single, very rarely multinodal lesion. Middle aged patients are mostly affected. Symptoms are either absent or uncharacteristic, so that the tumors are discovered by chance or during investigations of other diseases. Case report. We presented a 56-year-old woman in whom a nodal lesion of 2 cm in diameter had been discovered in the spleen during a routine check-up after a mild traffic accident. The lesion had grown up to 6 cm in diameter during a 5-year follow-up causing a mild pain over the upper left abdomen and left shoulder. Fine needle biopsy failed to show the exact nature of the lesion. Splenectomy was performed due to a suspected lymphoproliferative disorder. Histology and immunohistochemistry of the lesion of the upper pole of the spleen showed inflammatory pseudotumor. Epstein-Barr virus was not present. The patient developed postsplenectomy thrombocytosis (up to 850 ? 109/l) which settled down within 10 weeks. Conclusion. Inflammatory pseudotumors of the spleen are extremely rare. The exact diagnosis can be established only by histology and/or immunohistochemistry of the spleen removed for a suspected lymphoproliferative disorder, most frequently lymphoma. The patient was cured with splenectomy.


2005 ◽  
Vol 129 (11) ◽  
pp. 1480-1483 ◽  
Author(s):  
Ulises Torres ◽  
William G. Hawkins ◽  
Cristina R. Antonescu ◽  
Ronald P. DeMatteo

Abstract Follicular dendritic cell sarcoma of the liver is an uncommon pathologic entity, and only 5 cases have been reported previously. Herein, we report the first case, to our knowledge, of hepatic follicular dendritic cell sarcoma without evidence of Epstein-Barr virus infection. The patient is an elderly man who was found to have an incidental liver mass and then developed weight loss and fever. The diagnosis was based on the typical morphologic appearance of spindle cell proliferation associated with a brisk lymphoplasmacytic infiltrate and strong immunoreactivity to CD21 and CD35. Based on our experience and a review of the published reports, we summarize the clinical and pathologic features of hepatic follicular dendritic cell sarcoma and its surgical management.


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