SESSION TITLE: Fellows Disorders of the Mediastinum Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Sarcoidosis is a multi-organ system granulomatous disease of unknown etiology and, although it commonly affects the lungs, may be highly variable in its presentation. We will describe here a case of retroperitoneal fibrosis and ureteral obstruction secondary to sarcoidosis. CASE PRESENTATION: A 69 year old male with a history of prior episodes of nephrolithiasis presented with recurrent flank pain and nausea. Computed tomography (CT) of the abdomen revealed a new 4 mm stone at the left ureterovesicular junction with periureteral stranding. Cystoscopy was significant for distal left ureteral extramural compression. Subsequent magnetic resonance imaging (MRI) of the abdomen showed infiltrative soft tissue encasing the distal left ureter and iliac vessels. Patient underwent CT guided biopsy with pathology showing inflammatory myofibroblastic tumor. Initially, only observation was advised. The patient later developed recurrent urethral obstruction requiring new cystoscopy and stent placement. Subsequent MRI showed progressive infiltrative soft tissue throughout the retroperitoneum encasing the infrarenal aorta, IVC, and bilateral ureters. Multiple enlarged lymph nodes were also partially visualized in the chest, then dedicated CT chest showed diffuse nonspecific mediastinal and bilateral hilar lymphadenopathy. Endobronchial ultrasound guided fine needle aspiration of the mediastinal lymph nodes, stations 7, 11R, 4R and 4L revealed non-necrotizing granulomas consistent with sarcoidosis, without evidence of infection, malignancy or any additional inflammatory process as an explanation for the pathologic findings. DISCUSSION: Retroperitoneal fibrosis is a rare fibro-inflammatory disorder comprising the periabdominal aorta which expands to involve the retroperitoneum and frequently causes ureteral obstruction. It is an extremely rear manifestation of sarcoidosis, and always other more common etiologies such as immunoglobulin G4-related disease (IgG4-RD), malignancy, connective tissue disease, abdominal aortic aneurysm, and medications such as ergots, beta blockers, and non-steroidal anti-inflammatory drugs need to be ruled out. Symptoms are non-specific and include a dull low back, flank, or abdominal pain as well as constipation, fatigue, and weight loss. The diagnosis can be made with CT or MRI and biopsy is used to exclude malignancy and diagnose IgG4-RD. First line therapy is corticosteroids. CONCLUSIONS: Although experience with retroperitoneal fibrosis as a manifestation of sarcoidosis is lacking, treatment may help to reduce inflammation especially in the case of recurrent ureteral obstruction. Reference #1: Runowska M, Majewski D, Puszczewicz M. Retroperitoneal fibrosis – the state-of-the-art. Reumatologia 2016; 54, 5: 256–263. Reference #2: Spagnolo P, Rossi G, Trisolini R, et al. Pulmonary sarcoidosis. Lancet Respir Med 2018; 6: 389–402. DISCLOSURES: no disclosure on file for David Abia Trujillo; no disclosure on file for Isabel Mira-Avendano; No relevant relationships by Murat Tatari, source=Web Response