Hypercalcaemia associated with disseminated cryptococcosis

We report an elderly man who presented with giddiness and right-sided weakness, constipation and constitutional symptoms for 6 months duration. Blood investigations indicated hypercalcaemia with normal serum phosphate and acute kidney injury. Serum intact parathyroid hormone was suppressed. CT revealed bilateral tiny lung nodules with right upper lobe tree in bud appearance and incidental findings of bilateral adrenal lesion. Tuberculosis was ruled out. CT adrenal showed multiseptated hypodense rim enhancement adrenal lesion bilaterally. Adrenal function tests were normal except for low dehydroepiandrosterone (DHEA). Right-sided cervical lymph node biopsy confirmed fungal infection with the presence of intracellular and extracellular fungal yeast. Serum cryptococcus antigen titre was positive. Our final diagnosis was disseminated cryptococcosis with lungs, bilateral adrenal gland and lymph nodes involvement. The patient was then treated with antifungal treatment. Serum calcium was normalised after 1 month with marked clinical improvement.

Biochemically silent phaeochromocytoma presenting with non-specific loin pain

A 55-year-old woman presented with a 4-month history of right-sided non-specific loin pain and 6 kg weight loss. A CT scan of the abdomen and pelvis showed an incidental 4.5 cm right-sided adrenal lesion which was not typical of an adrenal adenoma. This was further confirmed on MRI of the adrenals. Biochemical investigations to investigate for a functional adrenal lesion included serum catecholamines and metanephrines, an aldosterone to renin ratio and an overnight dexamethasone suppression test. These were all negative. A laparoscopic adrenalectomy was performed in view of the large size of the lesion. Histology was consistent with a phaeochromocytoma, which confirmed the diagnosis of a non-secreting phaeochromocytoma. Non-secreting phaeochromocytomas are rare and usually found in patients with known genetic mutations. Adrenal lesions not related to any mutations similar to our case are even rarer and reported even less in the literature.

Nephrotic Syndrome Following Resection of an Adrenal Incidentaloma:A Case Report

A 69 year old man had a 5 cm right adrenal lesion discovered incidentally while being investigated for a deterioration in previously well-controlled hypertension. Routine investigations including serum albumin were normal. Further investigation confirmed a non-functioning adrenal lesion. MRI revealed a ‘non-fat-containing T1 hyperintense indeterminate adrenal lesion with speckling of T2 hyperintensity, not typical for adenoma, hyperplasia, myelolipoma, haemangioma or pheochromocytoma’. An uncomplicated laparoscopic adrenalectomy was performed. Histology revealed a 118 g adrenal neoplasm, modified Weiss score 0, with abundant hyaline deposits.3 months later the patient complained of peripheral oedema. Investigations revealed a serum albumin of 24 g/L and 14 g of proteinuria in 24 hours. Serum protein electrophoresis revealed a monoclonal IgA type lambda band. Renal biopsy revealed amorphous material displaying apple green birefringence on staining with Congo Red, which stained with antibodies to lambda light chains, confirming AL amyloid. Therefore the patient’s resected adrenal specimen was retrieved and stained with Congo Red, revealing apple green birefringence in the walls of the blood vessels, confirming the presence of amyloidosis. Although adrenal gland involvement in secondary amyloidosis is common, adrenal involvement in primary amyloidosis is less well described. This case illustrates the indolent nature of primary amyloidosis, prior to the development of often catastrophic symptoms. Consideration should be given to Congo Red staining of resected pathologic specimens containing hyaline deposition, to potentially allow for earlier recognition of this devastating disease. A pathophysiologic link between the patient’s incidentaloma, adrenalectomy, and onset of nephrotic syndrome remains a matter for conjecture.

Cancer risk in adrenalectomy: are adrenal lesions equal or more than 4 cm a contraindication for laparoscopy?

Background Some authors consider adrenal lesions size of less than 4 cm as a positive cut-off limit to set the indications for minimally invasive surgery due to a lower risk of malignancy. Aim of this study is to report the risk of cancer for adrenal lesions measuring 4 cm or more in diameter, assessed as benign at preoperative workup (primary outcome), and to evaluate the feasibility and safety of laparoscopic adrenalectomy (LA) in these cases (secondary outcome). Methods From January 1994 to February 2019, 579 patients underwent adrenalectomy. Fifty patients with a preoperative diagnosis of primary adrenal cancer or metastases were excluded. The remaining 529 patients were included and divided in five subgroups based on adrenal lesion size at definitive histology: group A, 4–5.9 cm (137 patients); group B, 6–7.9 cm (64 patients); group C, 8–9.9 cm (13 patients); group D, ≥ 10 cm (11 patients); group E, < 4 cm (304 patients). Each group was further divided based on diagnosis of benign or malignant lesions at definitive histology. Results Four (2.9%) malignant lesions were observed in group A, 5 (7.8%) in group B, 2 (15.4%) in Groups C and D (18.2%) and 13 (4.3%) in Group E. Comparing the cancer risk among the groups, no statistically significant differences were observed. Operative time increased with increasing lesion size. However, no statistically significant differences were observed between benign and malignant lesions in each group comparing operative time, conversion and complication rates, postoperative hospital stay and mortality rate. Conclusions Adrenal lesions measuring 4 cm or more in diameter are not a contraindication for LA neither in terms of cancer risk nor of conversion and morbidity rates, even if the operative time increases with increasing adrenal lesion diameter. Further prospective studies with a larger number of patients are required to draw definitive conclusions.

Adrenal medullary hyperplasia mimicking pheochromocytoma

A 59-year-old woman, a known case of hypertension, was incidentally diagnosed with a large right-sided adrenal mass. Investigations for a functional adrenal lesion resulted in very high preoperative norepinephrine levels. A right adrenalectomy was performed. Histology showed adrenal medullary hyperplasia (AMH). AMH is a rare diagnosis and its incidence is poorly documented in the literature. This is a benign entity which resembles pheochromocytoma (PCC) in both clinical and biochemical manner. AMH is usually bilateral and may occur in isolation or in association with PCC. In fact, some authors consider it to be a precursor to PCC. Thus, these patients need long-term follow-up in view of the risk of development of PCC later.

SAT-LB307 Case of the Broken Hearted

Phaeochromocytomas are rare neuroendocrine tumours which, usually present with headache, diaphoresis, episodic tachycardia and hypertension. Takotsubo-pattern cardiomyopathy is an unusual initial presentation of phaeochromocytomas, however has been reported1. Approximately 10% of phaeochromocytomas present with cardiomyopathy. It is associated with a high mortality risk and early intervention with surgery is the key to recovery. We report a case of a previously healthy, 68 year old man who presented with symptoms of left ventricular failure, and feeling anxious. Physical examination revealed signs of left ventricular failure, with a normal blood pressure and heart rate. A transthoracic echocardiogram (TTE) revealed dilated left ventricle with severe global hypokinesis and a left ventricular ejection fraction (LVEF) of 15%. A CTPA performed to exclude pulmonary embolism revealed an incidental right sided adrenal lesion measuring 3.9 X 3.4 X 3.7 cm. Subsequently a dedicated abdominal CT confirmed the adrenal lesion. Biochemistry revealed elevated 24 hour urine catecholamines and metanephrines and an elevated plasma metanephrines. Subsequently he proceeded to have adrenalectomy. Pre-operatively the patient was managed with alpha and beta blocking agents for 3 weeks prior to surgery. Post-operative course was uneventful and histopathology confirmed right sided phaeochromocytoma. TTE performed 12 months post-operatively showed a significant improvement in his LVEF to 40 % with subsequent TTE showing maintenance of LVEF. This case demonstrates highlights the unusual presentations of phaeochromocytomas and that early recognition and early intervention with surgery is the key to recovery to avoid catastrophic cardiac events.1. Chiang YL, Chen PC, Lee CC, et al. Adrenal pheochromocytoma presenting with Takotsubo-pattern cardiomyopathy and acute heart failure: A case report and literature review. Medicine (Baltimore)2016;95(36):e4846. doi: 10.1097/MD.0000000000004846 [published Online First: 2016/09/08]