Mediastinal gray zone lymphoma in a pregnant woman presenting with cardiac tamponade

Abstract

Background: Mediastinal gray zone lymphoma is a newly recognized rare B cell neoplasm, which is challenging in diagnosis and treatment.

Case presentation: In the current study, we aimed to report a 25-year-old pregnant woman at 25 weeks of gestation who presented with chronic cough and progressive shortness of breath, hypotension, tachycardia, and tachypnea. A large circumferential pericardial effusion with compressive effect on the right atrium and right ventricle and a large extracardiac mass with external pressure to mediastinal structures were seen on trans thoracic echocardiography. The emergency pericardiocentesis was performed with the diagnosis of cardiac tamponade. Also, CMR revealed a huge heterogeneous anterior mediastinal mass, and the pathology and the immunohistochemistry of the mass biopsy revealed gray zone lymphoma with positive CD3, CD20, CD30, CD45, PAX5, and negative CD15 expression. Three courses of chemotherapy with the CHOP regimen were performed with an acceptable response every three weeks before delivery. A caesarian section was performed at 37 weeks without any problem for the patient and fetus, and chemotherapy will be started three weeks after delivery.

Conclusion: Cardiac tamponade as an emergency condition occurred in this pregnant patient by malignant pericardial effusion and mediastinal mass pressure. Accurate diagnosis and on time interventions caused a significant improvement and a successful delivery.

Keywords: Cardiac tamponade; Chemotherapy; Gray zone lymphoma; Pericardial effusion; Pregnancy.

Fig. 1

Cardiac magnetic resonance A Short axis T1-W sequence view shows iso-signal tumor. B, C Short axis and axial SSFP sequence views reveal heterogeneous high signal tumor. D Short axis STIR sequence view demonstrates high signal tumor. E Coronal Late Gadolinium Enhancement (LGE) shows heterogeneous enhancement and necrosis (arrow). SSFP = steady-state free precession, STIR = Short tau inversion recovery)

Fig. 2

Mediastinal mass pathology A, B Microscopic examination (H&E staining) shows diffuse and nodular infiltration of lymphocytes, neutrophils, eosinophils and some large atypical cells with enlarged hyperchromatic nuclei with irregular contour and prominent nucleoli in fibrotic stroma. C All lymphoid cells including some large atypical cells are positive for CD45. D Many large atypical cells are positive for CD20. E Many small lymphocytes are positive for CD3. F Large atypical cells are negative for CD15. G Large atypical cells are positive for CD30. H Large atypical cells are strongly positive for PAX5)