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Retroperitoneal Fibrosis Masquerading as Malignancy: A Clinical Dilemma-Case Report

Premanand Ponoth Chief-Cardiothoracic & Vascular Surgery, Hon. Prof. the Tamil Nadu MGR Medical University, Chennai, Dr. Kamakshi Memorial Hospital & Prasanth Super Speciality Hospital Chennai, Tamil Nadu 600100, India. and Consultant at The Karen Hospita , P.M. Ponoth , A.J. Ruturi , S.N.K. Waweru , S.K. Panchal , A.K. Gikonyo , A.W. Kanyua , O.A Morara ,

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Journal of Cardiovascular Medicine and Surgery 4(1):p 50-56, January - March 2018. | DOI: http://dx.doi.org/10.21088/jcms.2454.7123.4118.8

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Abstract

Retroperitoneal fibrosis is a rare vascular connective tissue disease of unknown aetiology, typically presenting as inflammation within the retroperitoneum with the resultant mass effect of the pathological processes involved compress the organs in the retroperitoneum: Kidneys, ureter, aorta and pancreas. The disease was first described by John Kelso Ormond in 1948 on a patient who presented with bilateral ureteral obstruction secondary to an inflammatory retroperitoneal process [2,3]. Its incidence is at 1.38 cases per 100,000 [1]. It has a male predominance of between 1.6 to 3 fold [1,7]. There have been associations of retroperitoneal fibrosis with various autoimmune conditions.Immunosuppressive treatment modalities have been effective on its control and management thus indirectly inferring to an autoimmune process as a key factor behind its pathophysiology [4, 5]. Radiological diagnosis through CT remains a gold standard with biopsy being a controversial option of obtaining diagnosis [5, 17-21]. Main stay treatment has been use of glucocorticoids as 1st line, with the disease modifying anti-rheumatic drugs (DMARDS) as a second option where the disease has been found to be steroid resistant [6]. For the scope of this paper, we will describe a case managed at the Karen hospital in Nairobi, Kenya in September 2017.

 

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DOI: http://dx.doi.org/10.21088/jcms.2454.7123.4118.8

Keywords

Retroperitoneal Fibrosis; Lymphoma; Retroperitoneal Mass. 

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