EXTRAMEDIASTINAL LESION IN PATIENTS WITH PRIMARY MEDIASTINAL B-CELL LYMPHOMA

Background. Extramediastinal and bone marrow involvement in PMBCL patients in the onset of the disease is an exception to the rules and complete information, except for the word “rare”, in Russian and international literature is not available.

Objective: to characterize PMBCL patients with extramediastinal involvement.

Materials and methods. From 2007 to 2017 diagnosis of PMBCL was established in 157 patients according to WHO criteria with extramediastinal involvement in 16 (10.2 %) patients, 3 of them were at different stages of pregnancy. The median age was 27 years (23–69). Patients received different therapy protocols: m-NHL-BFM-90, R-DA-EPOCH и VACOP-B.

Results. One extramediastinal lesion was verified in 11/16 (68.7 %) patients, multiple – in 5/11 (31.3 %). The most common localizations were: pancreas – 6 (37.5 %), kidneys – 5 (31.2 %), ovaries – 3 (18.7 %), liver – 3 (18.7 %), bone marrow – 3 (18.7 %) and breast – 2 (12 %) cases. Involvement of stomach, bones, soft tissues, spleen, pelvis, adrenal gland was revealed in one case each. In 15/16 cases, extramediastinal lesions were combined with antero-superior mediastinum involvement and only in 1 cases an isolated lesion of the soft thorax tissues without involvement of mediastinal structures was revealed. Five-year overall survival in the group of patients with classical PMBCL who received R-DA-EPOCH, m-NHL-BFM-90 and cohort of patients with extramediastinal lesions was comparable and was 93 %. As a result of the analysis, in 10.2 % (16/157) of cases extramediastinal involvement was revealed. In all cases, there is involvement of organs and tissues, but not the lymph nodes. In 18.7 % (3/16) of cases there was bone marrow involvement, confirmed by molecular and histological studies.

Conclusion. Involvement of the antero-superior mediastinum and the presence of extramediastinal lesion, bone marrow involvement is not excluding criterion for PMBCL, but requires differential diagnosis with DBCL, including standard and molecular methods. Is isolated extramediastinal involvement in PMBCL a poor prognostic factor, is uncertain because of the small number of observations.

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