Endocrine Abstracts

Introduction: Primary adrenal lymphoma is rare, representing less than 1% of non-Hodgkin lymphomas.¹ The clinical presentation is non-specific, symptoms of adrenal insufficiency may occur with bilateral lesions, and work up can be tricky.²

Background: A 60-year-old man was admitted mid-January 2020 for acute left abdominal pain. He had no medical history and didn’t smoke or take any medication. He had no history of recent trauma. Abdominal CT-scan showed a voluminous (61(5747 mm), heterogeneous, hemorrhagic mass in left adrenal gland with a high spontaneous density (45UH). Pheochromocytoma diagnosis was first suspected although 24-h urine fractionated metanephrines and catecholamines measurements were only slightly increased (NA:1.1(ULN and NMN:1.2(ULN). Hormonal evaluation did not demonstrate excessive cortisol or sexual steroids secretion. 18F-FDG PET-CT imaging revealed intense FDG-uptake in both adrenal glands, heart and liver. With this new information, the differential diagnosis includes carcinoid cardiac tumor, malignant cardiac myxoma, lymphoma, sarcoma and metastases. Tumor markers (5HIAA, NSE, CA 19–9, CA 125, CEA, PSA, β-hCG) were negative. For Valentine’s Day, cardiac MRI confirmed a pathological thickening of right inter-atrioventricular groove with marked late enhancement at this level, corresponding to pathological areas in isotopic imaging. On the other hand, MRI did not show any lesion in the liver. Next day, patient presented acute right flank pain similar to the one he had on left side one month earlier. New abdominal CT-scan showed a large hemorrhage in right adrenal gland that was not present on 18F-FDG PET-CT performed 3 days before. Latest Ga-DOTATATE-PET imaging didn’t show anywhere STT-receptors presence. Morning cortisol level (411 nmol/l) remained non-suggestive of adrenal insufficiency. A research of antiphospholipid syndrome was also negative. The patient never had any sign of infection. This case was discussed by web-conference with the French experts of the national COMETE multidisciplinary committee. It was decided to obtain histology from left adrenal tumor by percutaneous biopsy, which was performed in a specialized center, using a coaxial 16G needle with post-biopsy gelatin injection, and 24H post-biopsy monitoring. This was preferred to adrenalectomy because the radicality of surgery might have changed depending on histological diagnosis. Unfortunately two CT-guided biopsies did not succeed to obtain enough material for required-immunostaining to characterize a suspected T lymphoma. Right adrenalectomy was finally performed and a postoperative hydrocortisone supplementation was prescribed. Histological analysis showed a high grade non-germinal center B-cell like lymphoma with BCL6 rearrangement. The patient started an R-CHOP protocol, reaching a complete metabolic response within three cures.

However the patient presented a primary adrenal insufficiency after the right adrenalectomy, which still requires hydrocortisone supplementation.

Discussion: Adrenal incidentaloma exists in roughly 6% of the population.³ While unilateral incidentaloma are usually benign, bilateral incidentaloma (15% of total incidentaloma³) are more likely to be malignant or functional.³

In a 2002 retrospective review, among 85 patients with an adrenal incidentaloma requiring pathological diagnosis, only one was found with adrenal lymphoma, which presented as a bilateral incidentaloma.⁴

In a recent retrospective study, bilateral adrenal lesions were found in up to 71% of adrenal lymphoma and were more likely to be associated with adrenal insufficiency, supposedly because of lymphomatous infiltration¹. Therefore it is recommended to look for adrenal insufficiency in all patient with bilateral adrenal lesions.¹

Our patient presented adrenal hemorrhage and myocardium involvement, which are rarely present with adrenal lymphoma.¹ FDG-PET is decisive for revealing extra-adrenal locations, even if CT or MRI is negative.¹

Conclusion: This case underlines importance of complementary investigations in all patients with bilateral adrenal lesions, especially to rule out adrenal insufficiency. It also underlines importance of discussing complex cases or atypical presentations with experts as allowed by COMETE or ENDO-ERN networks.

References: 1. Laurent C, Casasnovas O, Martin L, Chauchet A, Ghesquieres H, Aussedat G, et al. Adrenal lymphoma: presentation, management and prognosis. QJM. 18 oct 2016;hcw174.

2. Ellis RD. Bilateral adrenal non-Hodgkin’s lymphoma with adrenal insufficiency. Postgrad Med J. 1 août 2000;76(898):508–9.

3. Carlson AL, Marney AM, Anderson SR, Gilbert MP. Bilateral Adrenal Incidentalomas: A Case Report and Review of Diagnostic Challenges. Case Rep Endocrinol. 2013;2013:1–4.

4. Kasperlik-Zaluska AA, Roslonowska E, Slowinska-Srzednicka J, Migdalska B, Jeske W, Makowska A, et al. Incidentally discovered adrenal mass (incidentaloma): investigation and management of 208 patients. Clin Endocrinol (Oxf). janv 1997;46(1):29–37.

5. Bourdeau I, El Ghorayeb N, Gagnon N, Lacroix A. MANAGEMENT OF ENDOCRINE DISEASE: Differential diagnosis, investigation and therapy of bilateral adrenal incidentalomas. Eur J Endocrinol. août 2018;179(2):R57–67.