CC BY-NC-ND 4.0 · Experimental and Clinical Endocrinology & Diabetes Reports 2018; 05(01): e1-e5
DOI: 10.1055/s-0043-122492
Case Report
Eigentümer und Copyright ©Georg Thieme Verlag KG 2018

Lymphocytic Adrenal Medullitis: Case Report of A Patient with Pheochromocytoma-Like Hypertensive Paroxysms

Jan Calissendorff
1   Department of Endocrinology, Metabolism and Diabetes, Karolinska University Hospital, Stockholm, Sweden.
2   Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden
,
Carl Christofer Juhlin
3   Department of Clinical Pathology and Cytology, Karolinska University Hospital, Stockholm, Sweden
4   Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden
› Institutsangaben
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Publikationsverlauf

received 08. August 2017
first decision 18. Oktober 2017

accepted 30. Oktober 2017

Publikationsdatum:
22. Januar 2018 (online)

Abstract

Lymphocytic adrenal medullitis (LAM) denote the histological finding of lymphocytic infiltrates in the adrenal medulla, a rare phenomenon that previously has been deemed asymptomatic and linked to autopsy findings in patients with type I diabetes. We describe the finding of LAM in a 66-year old female presenting with hyperaldosteronism and a 17 mm lesion in the right adrenal gland. The patient displayed secondary hypertension as well as intermittently occurring exaggerated hypertensive episodes with systolic peaks above 220 mm Hg. Preoperative catecholamine screening was negative, diminishing the possibility of a pheochromocytoma. Unilateral adrenalectomy was performed, and histology revealed an aldosterone producing adrenocortical adenoma. No signs of adrenomedullary hyperplasia or pheochromocytoma were found; however, prominent B- and T-lymphocytic infiltrates were observed across the medulla. The inflammation was almost absent in the surrounding adrenocortical tissue. Postoperatively, the patient remained hypertensive, but without paroxysmal excessive blood pressure elevations. A review of our pathology records between the years 2000 to present time as well as focused histopathological re-examination of 40 cases revealed no signs of LAM in any adrenal gland investigated, confirming the occurrence in living patients as exceedingly rare. This is the first report of LAM in a living patient, with a possible clinical association to intermittent episodes of markedly increased blood pressure not commonly seen in patients with hyperaldosteronism. A coupling between LAM and exaggerated hypertensive episodes cannot be ruled out.

 
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