ECE2016 Eposter Presentations Adrenal cortex (to include Cushing's) (85 abstracts)
Primary bilateral diffuse large B-cell lymphoma of adrenals presenting as incidental adrenal masses
Cristina Alina Silaghi 1, , Delia Dima 3 , Carmen Georgiu 4 , Delia Lupu 5 , Ionela Lungu 1 , Anca Elena Craciun 6 , Daniel Tudor Cosma 6 , Cristina Sorina Catana 7 , Horatiu Silaghi 8 & Romeo Chira 9
1County Emergency Hospital, Cluj Napoca, Romania; 2Department of Endocrinology, ‘Iuliu Hatieganu’ University of Medicine and Pharmacy, Cluj Napoca, Romania; 3Departament of Hematology, Institute of Oncology ‘Prof. Dr Ion Chiricuta’, Cluj Napoca, Romania; 4Discipline of Pathology, Department of Morphological Sciences, ‘Iuliu Hatieganu’ University of Medicine and Pharmacy, Cluj Napoca, Romania; 5IInd Internal Medecine Departament ‘Iuliu Hatieganu’ University of Medicine and Pharmacy, Cluj Napoca, Romania; 6Diabet, Nutrition and Metabolic Diseases Clinical Center, Cluj Napoca, Romania; 7Department of Medical Biochemistry ‘Iuliu Hatieganu’ University of Medicine and Pharmacy, Cluj Napoca, Romania; 85th Departament of Surgery ‘Iuliu Hatieganu’ University of Medicine and Pharmacy, Cluj Napoca, Romania; 9Ist Internal Medecine Departament, ‘Iuliu Hatieganu’ University of Medicine and Pharmacy, Cluj Napoca, Romania.
Primary adrenal non-Hodgkin lymphoma (PANHL) is extremely rare and constitutes <1% of all NHLs and 3% of extranodal lymphoma. PANHL affects predominantly elderly and males and could arises in the presence of a preexisting autoimmune adrenalitis. Diffuse large lymphomas of B-cell origin (DLBCL) histology is reported in 70% of PANHL cases.
The present case is a primary, bilateral DLBCL of adrenal in 75-year-old male patient admitted for abdominal pain, weight loss and fatigue. He had no known past history of lymphoma or other carcinoma. Abdominal CT scan revealed an incidental, bilateral, marked, homogenous adrenal gland enlargement, measuring 15×8 cm on the right and 9×4.8 cm on the left (both masses >10 HU), with no lymphadenopathy or visceral involvement.
Serum metanephrines, renine, aldosterone and 24-h urine cortisol levels were normal. Depot-ACTH stimulation test demonstrated an insufficient response with an increase from baseline plasma cortisol of 15.2 μg/dl (normal range: 7–22 μg/dl) to 17.8 μg/dl after 4 h and an elevated plasma ACTH of 112.7 pg/ml (normal range 7.2–63.3 pg/ml), indicating a primary adrenal insufficiency (AI). Testosteron and 17OH progesterone were normal. Serum lactate dehydrogenase (LDH) was increased. A biopsy of the adrenals confirmed DLBCL (intense positive CD 20 and negative CD10, CD 99, synaptophisin, Hep-Par expression). Ki 67 was 90%. His bone marrow was not involved at diagnosis. Following hydrocortisone substitution therapy, the patient completed six R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone) courses of chemotherapy. FDG PET-CT revealed no tracer uptake in both adrenal masses or in lymph nodes and the patient is still in remision after 8 months. The prognosis is poor and is worsened by increased LDH, increased age, AI, and large tumor size. This case should remind clinicians that PANHL may be a cause of bilateral adrenal incidentaloma with or without AI.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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