Objectives The aim of this study was to evaluate the temporal association between the diagnosis of neoplasia and sarcoidosis in a large cohort of Spanish sarcoidosis patients.
Methods We included 170 patients consecutively diagnosed with sarcoidosis. The diagnosis of sarcoidosis was based on clinical criteria and imaging studies, together with the histopathological demonstration of non-necrotizing granulomas, and excluding other granulomatous diseases. The diagnosis of neoplasia was recorded before and after the diagnosis of sarcoidosis. Hematological neoplasias were classified according to the 2008 WHO Classification.
Results There were 111 (65%) women and 59 (35%) men (mean age at diagnosis, 47 years); 23 (13.5%) patients developed 25 neoplasias (two patients developed 2 neoplasias, before and after the diagnosis of sarcoidosis). There were 13 (52%) solid neoplasias, including pulmonary (n=3), thyroid (n=2), breast (n=2), melanoma (n=2) and cervix, testicular, renal and gastric cancer (1 case each). The remaining 12 (48%) were hematological neoplasias, mainly mature B-cell neoplasms (3 chronic lymphocytic leukemia, 3 extranodal MALT lymphomas and 2 diffuse large B-cell lymphoma), but also 2 acute leukemias (1 acute promyelocytic PML-PARA, 1 acute myeloid leukemia), 1 mature T-cell neoplasia (mycosis fungoide) and 1 myeloid neoplasia (essential thrombocytopenia). No significant differences were found in the main epidemiological, clinical or radiological features of sarcoidosis in patients with or without associated neoplasia, except for a statistical trend to a higher frequency of extrathoracic sarcoidosis in patients with associated cancer (70% vs 50%, p=0.11). Of the 23 patients with cancer, 5 were diagnosed before sarcoidosis and 16 after the diagnosis of sarcoidosis (10 solid and 6 hematological neoplasia); the 2 remaining patients developed a second neoplasia after the diagnosis of sarcoidosis.
Conclusions We identified four states of association between sarcoidosis and malignancy: i) patients with sarcoidosis who develop solid tumors (43%); ii) development of hematologic malignancies, mainly sarcoidosis-lymphoma syndrome (26%); iii) development of sarcoidosis in patients with neoplasia, including paraneoplastic sarcoidosis (cancer discovered concurrently ±1 year of the diagnosis of sarcoidosis) in 3 patients (22%); iv) patients with sarcoidosis and previous cancer who developed a second and different neoplasia (9%).
Disclosure of Interest None declared