AB0926 JUVENILE SYSTEMIC SCLEROSIS AND MUCINOUS ADENOCARCINOMA OF THE LUNG IN PATIENT WITH CYSTIC ADENOID MALFORMATION-PARANEOPLASTIC SYNDROME OR JUST A COINCIDENCE?
Authors: Amra Adrovic, Kenan Barut, Mehmet Yildiz, Oya Koker, Sezgin Sahin, Ozgur Kasapcopur
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Juvenile systemic sclerosis (JSS) is a rare auto-immune condition characterized by the skin stiffness and internal organ fibrosis. In adult patients, systemic sclerosis has been related to an increased risk of malignancy with lung cancer being most prevalent. Up-to-date, we haven't found any report on lung cancer in patient with JSS. <h3>Objectives</h3> Herein, we aimed to present a case with juvenile systemic sclerosis and coincidentally detected cystic adenoid malformation complicated by the mucinous adenocarcinoma of the lung. <h3>Methods</h3> Data on disease history are taken from patient's chart. Clinical and laboratory findings are recorded during the last visit at our outpatient department. <h3>Results</h3> A 14 years old previously healthy female patient, admitted to our outpatient department due to puffy fingers, Raynaud's phenomenon and stiffness of the skin proximally to metacarpophalangeal joints. Her anti-nuclear antibody was positive (1/80) but the other auto-antibodies (including Anti Scl 70, anti-centromere antibody) were negative. Patient was diagnosed as progressive systemic sclerosis and the prednisolone, methotrexate and nifedipine were started. Routinely performed echocardiography was normal. Respiratory function tests were appropriate for the age and hemoglobin level: FVC 80%, DLCO 92%. Surprisingly, thorax HRCT revealed cystic adenoid malformation on the left lower lobe of the lung so pediatric surgery department has been consulted. The decision for surgical intervention has been made and the lesion has been removed. Tissue sample has been sent to the pathological investigation and the result was consistent with mucinous adenocarcinoma of the lung. There were 2 focus (0.4 cm in diameter) of the adenocarcinoma in the cystic adenoid malformation without invasion to the local perineural, lymphatic or vascular tissues, with positive KRAS (KRAS12-612C, 612S) mutation. Cranial MRI and PET-CT show no residual, relapse nor the metastatic lesions. Clinical follow-up has been suggested, without need for further oncological treatment. <h3>Conclusion</h3> Juvenile systemic sclerosis is a rare disease characterized with multiorgan involvement. The paraneoplastic syndrome with clinical presentation of systemic sclerosis should be kept on mind among adolescents, especially those with negative autoantibodies. <h3>References</h3> [1] Adrovic A, şahin S, Barut K, Kasapçopur ö. Juvenile Scleroderma: A Referral Center Experience. Arch Rheumatol. 2018;33:344-351. [2] Bento J, Fernandes G, Barbosa MA, Magalhães A, Santos AR, Hespanhol V. Lung adenocarcinoma associated with systemic sclerosis: a case report. Rev Port Pneumol. 2009;15:93-9. [3] Yoshimi R, Takeno M, Yamanaka S, et al. Systemic sclerosis and pseudomesotheliomatous adenocarcinoma of the lung. Mod Rheumatol. 2006;16:165-8. <h3>Disclosure of Interests</h3> None declared