ECE2021 Audio Eposter Presentations Adrenal and Cardiovascular Endocrinology (80 abstracts)
A very rare case of extranodal lymphoma with adrenal and heart involvement
Labrini Papanastasiou 1 , Spyridon Nikitas Skarakis 1 , Eurydiki Michalis 2 , Maria Vardaka 2 , Pinelopi Lampropoulou 3 , Konstantinos Lymperopoylos 3 , Theodosia Choreftaki 4 , Anastasia Dimitriadi 4 , Anastasia Perpinia 5 , Eirini Papadimitriou 6 , Athina Markou 6 , Theodoros Marinakis 2 , Ioannis-Anastasios Vatalas 7 & Theodora Kounadi 1
1Unit of Endocrinology and Diabetes Center, ’G. Gennimatas’ General Hospital, Athens, Greece; 2Department of Hematology, General Hospital of Athens ’G Gennimatas’, Athens, Greece; 3Department of Radiology, General Hospital of Athens ’G Gennimatas’, Athens, Greece; 4Department of Pathology, ’G. Gennimatas’ General Hospital, Athens, Greece; 5Department of Cardiology, ’G. Gennimatas’ General Hospital, Athens, Greece; 6Unit of Endocrinology and Diabetes Center, ’G. Gennimatas’ General Hospital, Athens, Greece; 7TYPET-Mutual Health Fund of National Bank of Greece Personnel, Athens, Greece
Background
Extranodal non-Hodgkin lymphoma (NHL) is a rare condition that accounts for less than one-third of patients with NHL at diagnosis. The heart or the endocrine organs (adrenals) involvement is extremely rare.
Objective
We report an unusual case of extranodal B-cell NHL: DLBCL (diffuse large B-cell non-Hodgkin lymphoma) presenting with heart and bilateral adrenal involvement.
Design-results
A 72-year-old male patient presented to our Endocrinology Unit for investigation of 3.8 cm right adrenal mass, diagnosed by abdominal ultrasound and thereafter by computed tomography (CT) because of abdominal pain. On admission, a repeated adrenal CT scan identified bilateral adrenal masses of 6.7 cm on right adrenal gland compressing the inferior vena cava and 3.7 and 3.6cm on left adrenal gland. Thorax CT scan revealed a 4.6cm mass in the right atrium wall extending from the drainage of the superior vena cava to the drainage of the inferior vena cava. Heart MRI confirmed the previous finding. Left ventricular ejection fraction was 55%. Adrenal hormonal baseline and dynamic investigation [cortisol post-overnight dexamethasone suppression test and aldosterone post-saline infusion test] excluded autonomous cortisol or aldosterone secretion. Urinary metanephrine and normetanephrine excretion were normal. Cortisol response after a short synacthen test excluded adrenal insufficiency. A CT-guided adrenal biopsy revealed the presence of a DLBCL, with triple expression of bcl2, bcl6, C-MYC(+70%). Cell proliferation index Ki-67 was 99%. Positron emission tomography scan (PET-CT) showed hyper-metabolic infiltrative masses involving the right atrium (SUVmax 21.2) and both adrenal glands (SUVmax 20.5). Virology tests for Epstein Barr virus and HIV were negative. Bone marrow aspiration, trephine biopsy and cerebrospinal fluid analysis were negative for lymphoma infiltration. Karyotype was normal and MRI brain spectroscopy was negative for CNS lymphoma detection. The patient was asymptomatic. Under the care of the haematology team systemic immunochemotherapy with R-DA-EPOCH (rituximab, dose adjustment etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin) and high dose methotrexate for CNS prophylaxis were administrated. After the completion of six cycles of immunochemotherapy, a marked decrease of lymphoma infiltration in the post-chemotherapy imaging CT and MRI scans was found. Left ventricular ejection fraction was 66%. The treatment was complicated with reversible paralytic ileus, due to vincristine neurotoxicity, which was omitted from the next immunochemotherapy cycles.
Conclusions
An extremely rare case of an extranodal DLBCL stage IVE with both adrenal and heart involvement is described. The selection of the appropriate treatment modality can lead to profound response and improve patient’s outcome.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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