Diagnosis and management of pheochromocytoma: a practical guide to clinicians.

Pheochromocytomas (PCCs) are rare catecholamine producing neuroendocrine tumors. The majority of these tumors (85 %) arise from the adrenal medulla. Those arising from the extra-adrenal neural ganglia are called paragangliomas (PGLs). Paroxysmal hypertension with sweating, headaches and palpitation are the usual presenting features of PCCs/ PGLs. Gene mutations are reported in 32-79 % of cases, making genetic screening mandatory in all the cases. The malignancy rates are 10-15 % for PCCs and 20-50 % for PGLs. Measurement of plasma or 24-hour urinary fractionated metanephrines is the best biochemical diagnostic test. Computed tomography or magnetic resonance imaging has high sensitivity (90-100 %) and reasonable specificity (70-90 %) for the anatomical localization. The functionality is assessed by different radionuclide imaging modalities such as metaiodobenzylguanidine (MIBG) scintigraphy, positron emission tomography or single photon emission computed tomography. The only modality of curative treatment is tumor excision. Proper peri-operative management improves the surgical outcomes. Annual follow up with clinical and biochemical assessment is recommended in all the cases after treatment. Children, pregnant women and older people have higher morbidity and mortality risk. De-bulking surgery, chemotherapy, radiotherapy, molecular agents like sunitinib and everolimus, radionuclide agents and different ablation procedures may be useful in the palliation of inoperable/metastatic disease. An update on the diagnostic evaluation and management of PCCs and PGLs is presented here.

Metastatic involvement of the maxillary antrum from an uncommon source.

UNLABELLED: Metastatic involvement of the jawbones is uncommon, particularly in the maxilla. Case reports of such metastases from renal cell primaries are few, making a consensus on treatment difficult to establish. We present a case of metastatic involvement of the maxilla two years following a nephrectomy for renal cell carcinoma. The case exemplifies the broad range of symptoms attributable to metastases in the maxilla and the management dilemmas. CLINICAL RELEVANCE: The case highlights the role of practitioners in primary dental care in identifying the potential for such pathology based on clinical and radiographic features.

Excess mortality associated with hypopituitarism in adults: a meta-analysis of observational studies.

CONTEXT: Several previous observational studies showed an association between hypopituitarism and excess mortality. Reports on reduction of standard mortality ratio (SMR) with GH replacement have been published recently. OBJECTIVE: This meta-analysis assessed studies reporting SMR to clarify mortality risk in hypopituitary adults and also the potential benefit conferred by GH replacement. DATA SOURCES: A literature search was performed in Medline, Embase, and Cochrane library up to March 31, 2014. ELIGIBILITY CRITERIA: Studies with or without GH replacement reporting SMR with 95% confidence intervals (95% CI) were included. DATA EXTRACTION AND ANALYSIS: Patient characteristics, SMR data, and treatment outcomes were independently assessed by two authors, and with consensus from third author, studies were selected for analysis. Meta-analysis was performed in all studies together, and those without and with GH replacement separately, using the statistical package metafor in R. RESULTS: Six studies reporting a total of 19 153 hypopituiatary adults with a follow-up duration of more than 99,000 person years were analyzed. Hypopituitarism was associated with an overall excess mortality (weighted SMR, 1.99; 95% CI, 1.21-2.76) in adults. Female hypopituitary adults showed higher SMR compared with males (2.53 vs 1.71). Onset of hypopituitarism at a younger age was associated with higher SMR. GH replacement improved the mortality risk in hypopituitary adults that is comparable to the background population (SMR with GH replacement, 1.15; 95% CI, 1.05-1.24 vs SMR without GH, 2.40; 95% CI, 1.46-3.34). GH replacement conferred lower mortality benefit in hypopituitary women compared with men (SMR, 1.57; 95% CI, 1.38-1.77 vs 0.95; 95% CI, 0.85-1.06). LIMITATIONS: There was a potential selection bias of benefit of GH replacement from a post-marketing data necessitating further evidence from long-term randomized controlled trials. CONCLUSIONS: Hypopituitarism may increase premature mortality in adults. Mortality benefit from GH replacement in hypopituitarism is less pronounced in women than men.

Amiodarone-induced thyrotoxicosis: type 1 or type 2?

A 69-year-old man presented with a 6-week history of shortness of breath on exertion, unintentional weight loss, altered bowel habits and lethargy. He was diagnosed with atrial fibrillation 2 years previously and started on amiodarone and warfarin. He subsequently reverted to sinus rhythm and his medication was stopped 1 year prior to presentation. He denied history of thyroid dysfunction. On examination, he had a small firm goitre of grade I. His skin was warm and sweaty. Thyroid function tests showed him to be thyrotoxic. He was started on carbimazole 20 mg; however, he further became significantly thyrotoxic. Given the severity of his presentation and lack of any clinical or biochemical improvement, oral prednisolone 30 mg once daily was coadministered. His symptoms subsequently resolved and he became euthyroid. The prednisolone was tapered down and stopped while the carbimazole was incrementally reduced to 10 mg daily.