A CASE OF PULMONARY HYALINIZING GRANULOMA WITH RETROPERITONEAL FIBROSIS

Abstract

SESSION TITLE: Lung Pathology 2 SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Pulmonary hyalinizing granuloma (PHG) is a rare benign lung disease characterized by single or multiple lung nodules mimicking lung neoplasm. It is characterized by fibrosing nodules with central concentric deposits of lamellar collagen, surrounded by collections of plasma cells, histiocytes and lymphocytes. Its etiopathogenesis is unknown, although it could be associated with an autoimmune, fibrotic or previous infectious process. CASE PRESENTATION: A 64 year old male with PMH of anemia, renal insufficiency and retroperitoneal fibrosis (RPF diagnosed by imaging study in 2009) presented to pulmonary clinic in September 2013 with dyspnea on exertion, cough with intermittent hemoptysis and chest discomfort. Chest radiograph and CT chest showed RLL (5cm mass) and LLL lesions. Subsequent bronchoscopy with lung biopsy of RLL (TBBx) showed fungal elements felt by pathologist to be Rhizopus. Patient was treated with amphotericin for two weeks but was stopped due to worsening renal function. Slight improvement in lung mases noted on repeat CT chest in 2 months. Infectious disease team recommended resection of the RLL mass since medical therapy of Rhizopus with amphotericin was not clear. Patient refused surgery and lost in follow up. Patient presented to pulmonary clinic for follow up in 6/2017 and CT chest showed increasing size of RLL and LLL masses and new LUL mass. Patient was asymptomatic. Laboratory work up with CBC, BMP, rheumatology work up (including ANA, ENA, RF, CCP), immunoglobulin and fungal serology were unremarkable. PET scan demonstrated increased FDG uptake in RLL, LUL and LLL masses. Subsequent LUL biopsy was consistent with pulmonary hyalinizing granuloma. Patient is asymptomatic and is being followed with serial CT chest to make sure densities do not increase in size. DISCUSSION: PHG represents a hyperfibrotic response to an infectious agent such as histoplasma or associated with mediastinal or retroperitoneal fibrosis. In our case PHG was diagnosed many years after RPF. The association between two disorders is obscure. Abnormal immune response might form a range of conditions common to the two diseases. The prognosis for patients with PHG is generally excellent with no significant impact on longevity. Successful resolution of the nodules with the administration of glucocorticoids has been reported. CONCLUSIONS: PHG is closely associated with fibrotic disease, autoimmune and infectious process. The clinicians should consider the possibility of PHG when encountering a patient with RPF or vice versa. Currently there is no established treatment. Asymptomatic patient can be managed with serial imaging study. Reference #1: 1. Brandao, Viviane, et al. "Hyalinizing granuloma: an unusual case of a pulmonary mass." Case reports in medicine 2010 (2010). Reference #2: 2. Saint-Pierre, Mathieu, et al. "Pulmonary Hyalinizing Granuloma (PHG): An Uncommon Cause of Pulmonary Nodules." Chest 152.4 (2017): A435. Reference #3: 3. Young, Adam S., et al. "Pulmonary hyalinizing granuloma and retroperitoneal fibrosis in an adolescent." Pediatric radiology 37.1 (2007): 91-95. DISCLOSURES: No relevant relationships by James Finley, source=Web Response No relevant relationships by Mohammed Nagori, source=Web Response