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Imagens em Pneumologia

Sarcoidosis-lymphoma syndrome: a diagnostic dilemma

Sarcoidosis-lymphoma syndrome: a diagnostic dilemma

Clara Perini Fiorot1, Rosana Souza Rodrigues1,2, Edson Marchiori1

A 57-year-old patient who had been diagnosed with pulmonary sarcoidosis 10 years prior and had received irregular corticosteroid treatment was admitted for investigation due to dyspnea on minimal effort, dry cough, dysphagia, and a 12-kg weight loss over 8 months. Physical examination revealed collateral circulation in the anterior thoracic region. Laboratory tests performed at admission showed only thrombocytopenia. Chest CT revealed a bulky lobulated mass in the anterior mediastinum (Figure 1A), calcified “eggshell” lymph nodes in various mediastinal chains, and fibrotic interstitial changes consistent with chronic sarcoidosis (Figures 1B and C). PET/CT demonstrated glycolytic hypermetabolism suggesting the presence of neoplastic tissue within the mass (Figure 1D). Biopsy of the mediastinal mass with immunohistochemical analysis led to the diagnosis of B-cell lymphoma. The patient underwent a 1-year regimen of chemotherapy combined with corticosteroid treatment, which resulted in marked reduction in mediastinal mass volume and glycolytic metabolism (Figure 1E).


 
The diagnosis of sarcoidosis is based on clinical presentation, complementary examination findings, and biopsy. Sarcoidosis increases the risk of lymphoma development, possibly via lymphocytic proliferation associated with high mitotic activity in the active chronic form, ultimately leading to the rare sarcoidosis-lymphoma syndrome.(1-4) The understanding of this relationship remains limited, and the condition is documented sparsely in the medical literature.
 
REFERENCES
 
1.            Alzghoul BN, Zayed Y, Obeidat A, Alzghoul B, Naser A, Shilbayeh AR, et al. Clinical Characteristics of Sarcoidosis Patients with Self-Reported Lymphoma: A US Nationwide Registry Study. Lung. 2021;199(6):611-618. https://doi.org/10.1007/s00408-021-00485-z
2.            Faurschou M, Omland LH, Obel N, Lindhardsen J, Baslund B. Risk of Cancer Among Sarcoidosis Patients With Biopsy-verified Nonnecrotizing Granulomatous Inflammation: Population-based Cohort Study. J Rheumatol. 2022;49(2):186-191. https://doi.org/10.3899/jrheum.210588
3.            Brincker H. The sarcoidosis-lymphoma syndrome. Br J Cancer. 1986;54(3):467-473. https://doi.org/10.1038/bjc.1986.199
4.            Papanikolaou IC, Sharma OP. The relationship between sarcoidosis and lymphoma. Eur Respir J. 2010;36(5):1207-1209. https://doi.org/10.1183/09031936.00043010

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