A Rare Cause of a Case of a Rapidly Growing Adrenal Incidentaloma

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: MON 1-36-Adrenal Incidentaloma & Carcinoma
Clinical
Monday, June 17, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board MON-15
Veronica Crawford*1, Jade AU Tamatea1, Marianne Susan Elston1, Michael J Swarbrick2, Louise Wolmarans1, John V Conaglen3 and Goswin Y Meyer-Rochow1
1Waikato Hospital, Hamilton, New Zealand, 2Waikato Hospital, New Zealand, 3Waikato Clinical School, University of Auckland, Hamilton, New Zealand
Background: Incidental adrenal lesions (adrenal incidentalomas [AI]) are identified on approximately 5% of abdominal computed tomography (CT) and magnetic resonance imaging (MRI) scans. While most are benign and nonfunctioning, some AIs may be malignant and/or hormonally active. As such, current guidelines recommend further investigation of AIs.

Clinical Case: A 53 year old woman presented with 24 hours of right sided loin pain. Renal calculi were excluded by a non-contrast CT urogram however a 20 mm right adrenal nodule of 40 Hounsfield units was identified.

Her past medical history included longstanding mild hirsutism, acne, asthma, type 2 diabetes, with a three year history of hypertension and dyslipidaemia. There were no symptoms suggestive of pheochromocytoma and no prior malignancy. On examination BMI was 45kg/m2, with acanthosis nigricans but no features of Cushing’s syndrome, or stigmata of familial endocrine syndromes. Biochemical testing confirmed normal renin, aldosterone,  ACTH, cortisol, androgens and 24 hour urinary free cortisol levels. Initial plasma free normetadrenaline was marginally elevated (914pmol/L, RR 0-900) with a normal metadrenaline level but normal on subsequent testing.

As the lesion was small and appeared non-functioning an interval scan was arranged which revealed an increase in maximum diameter from 20mm to 64 mm over a four month period. She was referred for a right adrenalectomy. A staging CT chest, abdomen and pelvis performed 14 days after the second scan showed a further increase in size to 74 mm with enlarged para-aortic lymph nodes but no evidence of distant metastatic disease.

Given the high suspicion of malignancy an open right adrenalectomy with retroperitoneal lymph node excision was performed. Histology demonstrated an adrenal plasmacytoma with lymph node metastasis. In situhybridisation for mRNA of kappa and lambda light chains was strongly positive for lambda light chains. Bone marrow aspirate showed 5% plasma cells of a clonal population. Elevated lambda serum free light chains and beta2 microglobulin were identified. She underwent chemotherapy(melphalan, prednisone, bortezomib) and radiotherapy which was ineffective and she subsequently developed a plasma cell leukaemia for which she is currently receiving cyclophosphamide and dexamethasone.   Adrenal plasmacytomas are rare with only six previous case reports of this condition in the literature.

Conclusion: This case highlights the value of early interval imaging in patients with an atypical adrenal incidentaloma if a conservative approach is being taken.

Nothing to Disclose: VC, JAT, MSE, MJS, LW, JVC, GYM

*Please take note of The Endocrine Society's News Embargo Policy at http://www.endo-society.org/endo2013/media.cfm