Adrenal ganglioneuroma: features and outcomes of 27 cases at a referral cancer centre.

BACKGROUND: Adrenal ganglioneuroma (AGN) is a rare neurogenic tumour that can mimic other adrenal neoplasms. Limited information, mostly derived from small cases series, is available for AGN. METHODS: A retrospective review for AGNs seen at a tertiary referral centre describing important features to distinguish AGN from other adrenal neoplasms. RESULTS: Of 53 ganglioneuromas, 27 were AGNs. Median age was 31 years (range, 1·7-64 years) and median tumour size was 8 cm (range, 1·5-20 cm). Seventeen AGNs (63%) were detected incidentally and nine patients (33%) presented with abdominal/back discomfort. Catecholamine levels, available for 21 patients, were normal. On computed tomography (CT), most AGNs were homogenous and well circumscribed with a median density of 32·5 Hounsfield units (HU) on unenhanced CT; 40 HU on postcontrast venous phase; and 66·5 HU on delayed postcontrast phase. On magnetic resonance imaging (MRI), AGNs had hypo-intense signal on T1-weighted images with heterogeneous hyperintense signal on T2-weighted images. In four patients, there was no tumour growth during median follow-up of 48 months (range, 21-60 months). One patient had malignant peripheral nerve sheath tumour arising from AGN. Thirteen patients with resected AGN had no recurrence during a median follow-up of 50 months (range, 2-135 months). CONCLUSIONS: We herein describe the largest AGN series reported to date. Isolated AGNs do not produce catecholamines and have CT imaging characteristics that can help in distinguishing them from other adrenal and para-adrenal neoplasms. The natural history of AGNs is usually benign, although local extra-adrenal extension or malignant transformation can rarely occur.

Clinical and radiologic features of pheochromocytoma/ganglioneuroma composite tumors: a case series with comparative analysis.

OBJECTIVE: To describe and compare the clinical, biochemical, radiologic, and pathologic features of adrenal pheochromocytoma-ganglioneuroma (PC-GN) composites with the features of isolated pheochromocytomas (PCs) and adrenal ganglioneuromas (AGNs). METHODS: We reviewed data for PC-GN composite cases seen at a single tertiary center between 1993 and 2012 and compared them with cases of isolated AGN and relatively similar median-size PCs. RESULTS: Nine PC-GN composites were included. The median age at diagnosis was 52 years (range, 28 to 83 years) for PC-GN compared with 55 years (range, 24 to 78 years) for PC patients and 40 years (range, 18 to 64 years) for AGN patients. Similar to PCs, all PC-GN composites were associated with catecholamine overproduction, whereas AGNs were nonfunctioning. On pathology, the median tumor sizes were 7 cm (range, 2.5 to 13 cm) for PC-GN tumors, 6.5 cm (range, 3.5 to 7 cm) for PCs, and 8 cm (range, 3.2 to 20 cm) for AGNs. On computed tomography (CT) imaging, PC-GN composites and PCs were heterogeneous, with both having significantly higher postcontrast density values than AGNs, which typically looked homogeneous and had a progressive enhancement pattern without contrast washout in most cases. CONCLUSION: The presence of a PC component significantly increases tumor heterogeneity and postcontrast density values. CT imaging could be very helpful in distinguishing AGNs from both PC-GN and PC tumors, but only pathologic examination can yield the diagnosis. Clinically and radiologically, PC-GN composites are indistinguishable from PCs and need to be managed similarly.

Knowledge of osteoporosis among men in the primary care setting.

OBJECTIVES: Osteoporosis has been often viewed as a disease affecting women; however, men are at risk for osteoporosis, and the mortality after hip fracture in men older than 75 years of age is significantly higher than in women. We aimed to assess knowledge of osteoporosis and its risk factors in men. METHODS: A questionnaire assessing knowledge of osteoporosis was presented to 136 male patients randomly in Internal Medicine and Family Practice clinics at Saint Joseph Hospital. RESULTS: A total of 136 men agreed to fill in the questionnaire. Only 130 patients who completed the entire questionnaire were included in the data analysis. Twenty-three percent of men surveyed had never heard about osteoporosis. Thirty-nine percent were unaware that osteoporosis is directly responsible for disabling hip fractures. Sixty-seven percent did not know that a potential outcome of hip fracture is death. Only 38% recognized that smoking is a risk factor, 20% knew that excessive caffeine intake can put them at risk, and 35% realized that excessive alcohol intake is a risk factor. Only 21% could correctly identify the calcium-rich foods among the choices. There was a positive relationship (P < 0.05) between receiving an official discussion with a physician regarding osteoporosis and the actual score on the "knowledge of osteoporosis" questionnaire. CONCLUSION: A significant percentage of men are unaware of the complications and risk factors for osteoporosis. Receiving educational information about osteoporosis from a physician should be considered at an early stage, especially for those patients with modifiable risk factors.

Hypothyroidism to hyperthyroidism: an immunological pendulum swing from two extreme poles - a case series.

We report two women who were diagnosed with hypothyroidism due to what was thought to be Hashimoto's thyroiditis 18 and 16 years ago, respectively. They had been euthyroid on stable doses of levothyroxine for many years, and they presented to our clinic with clinically and biochemically overt hyperthyroidism that persisted even after stopping levothyroxine. Immunological and imaging workups were consistent with Graves' disease. Both patients were treated medically and then received definitive treatment. To our knowledge, the intervals for these two conversions are among the longest conversion intervals reported in the medical literature.

A Unique Case of Metastatic, Functional, Hereditary Paraganglioma Associated With an SDHC Germline Mutation.

Context: Mutations in genes encoding for the succinate dehydrogenase (SDH) complex are linked to hereditary paraganglioma syndromes. Paraganglioma syndrome 3 is associated with mutations in SDHC and typically manifests as benign, nonfunctional head and neck paragangliomas. Design: We describe a case of a 51-year-old woman who initially presented with diarrhea and hypertension and was found to have a retroperitoneal mass, which was resected with a pathology consistent with paraganglioma. Five years later, her symptoms recurred, and she was found to have new retroperitoneal lymphadenopathy and lytic lesions in the first lumbar vertebral body and the right iliac crest, which were visualized on CT scan and octreoscan but not on iodine-123-meta-iodobenzylguanidine (123I-MIBG) and bone scans. She had significantly elevated 24-hour urine norepinephrine and dopamine. The patient received external beam radiation and a series of different antineoplastic agents. Her disease progressed, and she eventually expired within 2 years. Genetic testing revealed a heterozygous SDHC c.43C>T, p.Arg15X mutation, which was also detected in her daughter and her grandson, both of whom have no biochemical or imaging evidence of paraganglioma syndrome yet. Conclusion: We report a unique case of functional, metastatic abdominal paraganglioma associated with SDHC germline mutation. Our case exemplifies that SDHC germline mutation has variable penetrance, which may manifest with an aggressive biology that could be missed by a 123I-MIBG scan.

Drug-induced hypersensitivity reaction: A case of simultaneous thyroiditis and fulminant type 1 diabetes.

A 43-year-old incarcerated man with AIDS was hospitalized for 30 pounds weight loss and diffuse pruritic rash. Three months prior, he was started on dapsone for Pneumocystis jiroveci pneumonia prevention. Biochemical evaluation was remarkable for eosinophilia, thrombocytopenia, acute renal insufficiency, transaminitis, thyrotoxicosis, and significant hyperglycemia (450 mg/dl; nl, 65-99). His hemoglobin A1c level was 5.9% (nl, 4.1-5.6). Thyroid-stimulating immunoglobulin, glutamic acid decarboxylase, and islet cell autoantibodies were within the normal range. He was found to have acute interstitial nephritis based on renal biopsy. He was diagnosed with hypersensitivity reaction due to dapsone. The patient was managed with a tapering dose of corticosteroid, beta-blocker, and multiple daily injections of insulin. The symptoms and biochemical disturbances including thyrotoxicosis resolved within a few weeks. Insulin requirements decreased but diabetes did not resolve with hemoglobin A1c of 6.1% a year after hospitalization. To our knowledge, this is the first case of hypersensitivity reaction due to dapsone causing simultaneous fulminant type 1 diabetes and thyroiditis.

Pheochromocytoma-induced atrial tachycardia leading to cardiogenic shock and cardiac arrest: resolution with atrioventricular node ablation and pacemaker placement.

Pheochromocytoma should be considered in young patients who have acute cardiac decompensation, even if they have no history of hypertension. Atrioventricular node ablation and pacemaker placement should be considered for stabilizing pheochromocytoma patients with cardiogenic shock due to atrial tachyarrhythmias. A 38-year-old black woman presented with cardiogenic shock (left ventricular ejection fraction, <0.15) that did not respond to the placement of an intra-aortic balloon pump. A TandemHeart(®) Percutaneous Ventricular Assist Device was inserted emergently. After atrioventricular node ablation and placement of a temporary pacemaker, the TandemHeart was removed. Computed tomography of the abdomen revealed a pheochromocytoma. After placement of a permanent pacemaker, the patient underwent a right adrenalectomy. This is, to our knowledge, the first reported case of pheochromocytoma-induced atrial tachyarrhythmia that led to cardiogenic shock and cardiac arrest unresolved by the placement of 2 different ventricular assist devices, but that was completely reversed by radiofrequency ablation of the atrioventricular node and the placement of a temporary pacemaker. We present the patient's clinical, laboratory, and imaging findings, and we review the relevant literature.

Melanoma of unknown primary origin presenting as a rapidly enlarging adrenal mass.

Metastasis to the adrenal can be seen in the context of metastatic melanoma, but primary adrenal melanoma is very uncommon. We present a case of a rapidly enlarging adrenal mass that mimicked non-functioning primary adrenal malignancies but later proved to be part of a widely metastatic melanoma of unknown primary origin. Careful physical examination of the patient led to the discovery of a subcutaneous metastatic focus that was not seen on [(18)F]-fluorodeoxyglucose positron emission tomography/CT imaging. The presence of subcutaneous metastases raised the suspicion for metastatic melanoma; however, pathological confirmation remained the ultimate tool to reach the final diagnosis.

Unilateral renal artery stenosis with renal atrophy in a patient with metastatic papillary thyroid carcinoma treated with sorafenib.

Tyrosine kinase inhibitors (TKIs) have been recently introduced for treatment of different malignancies. Various cardiovascular toxicities have been reported with TKIs with hypertension being the most common adverse cardiovascular event. We report a case of a 60-year-old woman who developed left renal artery stenosis associated with renal atrophy in the context of metastatic papillary thyroid carcinoma treated with sorafenib. Renal atrophy was noticed during serial imaging studies to monitor cancer therapy. Clinically, she was asymptomatic without significant change in blood pressure. The glomerular filtration rate dropped from 88 ml/min/1.73 m(2) at baseline to 56 ml/min/1.73 ml/min and partially recovered to 71 ml/min/1.73 m(2) after renal artery stenting. To our knowledge, this will be the first known case of renal artery stenosis associated with TKI use. Physicians may need to investigate the possibility of developing renal artery stenosis in patients with unexplained worsening in kidney functions while on TKIs.

Mitotane-induced hyperlipidemia: a retrospective cohort study.

Limited data are available about mitotane-nduced hyperlipidemia. We retrospectively analyzed lipid data in 38 patients with adrenocortical carcinoma (ACC) who received mitotane therapy with emphasis on HDL cholesterol (HDL-c) and clinical predictors of lipid changes. At baseline, the mean levels of HDL-c, LDL-c, and triglycerides were 53.3 mg/dL, 114.4 mg/dL, and 149 mg/dL, respectively. HDL-c, LDL-c, and triglyceride concentrations significantly increased with mitotane therapy to a mean HDL peak (HDL-P) of 86.3 mg/dL (P < 0.001), a mean LDL peak of 160.1 mg/dL (P < 0.001), and a mean triglyceride peak (Tg-P) of 216.7 mg/dL (P = 0.042). HDL-P positively correlated with mitotane concentration (r = 0.52, P < 0.001), while LDL-P levels and Tg-P did not. Gender, body mass index, cortisol overproduction, baseline levels of HDL-c, and triglyceride did not predict change in HDL-c. Similar changes were noticed in subgroup analysis after excluding patients who were using lipid-lowering agents. In conclusion, in ACC patients, mitotane caused significant increases in HDL-c that may counteract the deleterious atherosclerotic effects of LDL-c and Tg rise. Understanding the mechanism of HDL change may lead to the discovery of novel HDL-c-elevating drugs.