Incidental adenocarcinoma after bilateral lung transplant in a patient with scleroderma interstitial lung disease

SESSION TITLE: Diffuse Lung Disease Case Report Posters 3 SESSION TYPE: Case Report Posters PRESENTED ON: 10/09/2023 12:00 pm - 12:45 pm INTRODUCTION: Scleroderma, or systemic sclerosis (SSc), is an autoimmune connective tissue disease characterized by skin fibrosis, vasculopathy, and internal organ involvement including interstitial lung disease (ILD). Significant ILD be present in up to 30-40% of patients and represents the leading cause of death in patients with SSc (1). Lung transplantation can be considered for refractory cases with advanced progression. We describe a patient who underwent bilateral lung transplantation for SSc related ILD and lung adenocarcinoma was incidentally found in the explanted lungs. CASE PRESENTATION: A 62-year-old White woman with an eight-year history of SSc complicated by ILD (combined pattern of fibrotic nonspecific interstitial pneumonia and usual interstitial pneumonia) and chronic hypoxemic respiratory failure presented to the hospital with worsening shortness of breath and hypoxemia. High-resolution chest computed tomography (CT) showed worsening of known ILD and new areas of ground glass opacities involving all lobes. Oxygen requirements increased from baseline to 9-10L at rest and up to 15L with exertion. Bronchoscopy was deferred due to the severe hypoxemia and high risk of mechanical ventilation post-procedure. The patient was placed under venoarterial extracorporeal membrane oxygenation. Three months after her first visit, she underwent double lung transplant without complications. However, histopathologic results from the explanted lung revealed adenocarcinoma (stage III), involving all lobes of both lungs and 7/14 hilar lymph nodes. After discussion with the oncology board, palliative systemic chemotherapy was initiated with carboplatin/pemetrexed. Eighteen months later, the patient presented with dyspnea, hypoxemia, and pancytopenia; a PET scan revealed multifocal osseous metastatic disease, particularly on the spine and pelvis. Nine months later, the patient developed new punctate lesions noted on the brain MRI. The patient expired secondary to metastatic complications. DISCUSSION: This case represents a rare finding of incidentally found lung adenocarcinoma after lung transplantation for SSc ILD. There have been previous cases of incidental adenocarcinoma in the explanted lungs of some transplant recipients (2). However, this is the first case report of a patient with a connective tissue disease-ILD secondary to Scleroderma. This represents a difficult clinical scenario since prior CT-scan did not show pulmonary nodules or other concerns related to malignancy. Due to the poor respiratory status of the patient and high risk of post-procedural complications, further diagnostic evaluation with bronchoscopy had been deferred. CONCLUSIONS: Previous studies have shown that SSc has a higher known incidence of lung cancer. A study by Morrisore et al. found a standardized incidence ratio of 3.07 for lung cancer in patients with SSc, and an association between patients with ILD and lung cancer was also described (3). We suggest that patients who present with a SSc ILD, which has a higher known incidence of lung cancer, should undergo a thorough evaluation to rule out malignancy. A prior PET scan could be considered in patients with structurally abnormal lungs prior to transplantation. REFERENCE #1: Goldin JG, Lynch DA, Strollo DC, et al. High-Resolution CT Scan Findings in Patients With Symptomatic Scleroderma-Related Interstitial Lung Disease. Chest. 2008;134(2):358-367. REFERENCE #2: Ritchie AJ, Mussa S, Sivasothy P, Stewart S. Single-lung transplant complicated by unexpected explant carcinoma: a management dilemma. J Heart Lung Transplant. 2007;26(11):1206-1208. REFERENCE #3: Morrisroe K, Hansen D, Huq M, et al. Incidence, Risk Factors, and Outcomes of Cancer in Systemic Sclerosis. Arthritis Care & Research. 2020;72(11):1625-1635. DISCLOSURES: No relevant relationships by Florentina Berianu No relevant relationships by Maximiliano Diaz-Menindez No relevant relationships by Megan Sullivan