A sarcoidosis–lymphoma syndrome revealed by hypopituitarism

Abstract

Summary A 26-year-old woman presented with persistent headache and tiredness. Biological investigations disclosed a moderate inflammatory syndrome, low PTH-hypercalcemia and complete anterior hypopituitarism. A magnetic resonance imaging (MRI) of the pituitary gland was performed and revealed a symmetric enlargement with a heterogeneous signal. Ophthalmological examination showed an asymptomatic bilateral anterior and posterior uveitis, and a diagnosis of pituitary sarcoidosis was suspected. As the localization of lymphadenopathies on the fused whole-body FDG-PET/computerized tomography (CT) was not evoking a sarcoidosis in first instance, an excisional biopsy of a left supraclavicular adenopathy was performed showing classic nodular sclerosis Hodgkin’s lymphoma (HL). A diagnostic transsphenoidal biopsy of the pituitary gland was proposed for accurate staging of the HL and surprisingly revealed typical granulomatous inflammation secondary to sarcoidosis, leading to the diagnosis of a sarcoidosis–lymphoma syndrome. The co-existence of these diseases constitutes a diagnostic challenge and we emphasize the necessity of exact staging of disease in order to prescribe adequate treatment. Learning points: The possibility of a sarcoidosis–lymphoma syndrome, although rare, should be kept in mind during evaluation for lymphadenopathies. In the case of such association, lymphoma usually occurs after sarcoidosis. However, sarcoidosis and lymphoma can be detected simultaneously and development of sarcoidosis in a patient with previous lymphoma has also been reported. An accurate diagnosis of the disease and the respective organ involvements, including biopsy, is necessary in order to prescribe adequate treatment.

Diffuse large B-cell lymphoma in the course of systemic sarcoidosis: A case report and review of 30 Japanese patients with sarcoidosis-lymphoma syndrome

“…1,2 Sarcoidosis has been known to be complicated rarely with lymphoma, and the condition is designated as sarcoidosis-lymphoma syndrome. [3][4][5][6][7][8][9][10][11] In this report, we describe a patient with pathologically-proven sarcoidosis in the lung who later developed diffuse large B-cell lymphoma and showed a good response to chemotherapy. We also reviewed 30 Japanese patients, including this case, with sarcoidosis who developed lymphoma in the literature.…”