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Langenbeck's Archives of Surgery Mar 2022Composite phaeochromocytoma is a tumour containing a separate tumour of neuronal origin in addition to a chromaffin cell tumour. This study reports on two cases from a... (Review)
Review
INTRODUCTION
Composite phaeochromocytoma is a tumour containing a separate tumour of neuronal origin in addition to a chromaffin cell tumour. This study reports on two cases from a single centre's records and presents a systematic literature review of composite phaeochromocytomas.
METHODS
In addition to describing 2 case reports, a systematic search of the Medline database from inception up to April 2020 was done for human case reports on composite phaeochromocytomas. Relevant titles and/or abstracts were screened, and full texts were reviewed to identify appropriate studies. Data was extracted and a descriptive analysis of presentation, clinical features, management strategies and outcomes was performed. The quality of included studies was assessed using a critical appraisal checklist.
RESULTS
There were 62 studies included, with a total of 94 patients. Of 91 patients where data was available, the median (range) age of patients was 48 (4-86) years. Of 90 patients where information was provided, 57% were female. In at least 28% of patients, a genetic cause was identified. Common presenting features include abdominal pain, palpable mass, cardiovascular and gastrointestinal symptoms. The most common tumour component with phaeochromocytoma is ganglioneuroma; other components include ganglioneuroblastoma, neuroblastoma and malignant peripheral nerve sheath tumours. In patients with follow-up data (n=48), 85% of patients were alive and well at a median (range) follow-up time of 18 (0.5-168) months.
CONCLUSION
Composite phaeochromocytoma is a rare tumour, with a significant genetic predisposition. This review summarises available epidemiological data, which will be useful for clinicians managing this rare condition.
Topics: Adrenal Gland Neoplasms; Aged; Aged, 80 and over; Brain Neoplasms; Female; Humans; Middle Aged; Pheochromocytoma
PubMed: 33651160
DOI: 10.1007/s00423-021-02129-5 -
BMJ Open Dec 2020Accurate subtype classification in primary aldosteronism (PA) is critical in assessing the optimal treatment options. This study aimed to evaluate the diagnostic... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
Accurate subtype classification in primary aldosteronism (PA) is critical in assessing the optimal treatment options. This study aimed to evaluate the diagnostic accuracy of adrenal imaging for unilateral PA classification.
METHODS
Systematic searches of PubMed, EMBASE and the Cochrane databases were performed from 1 January 2000 to 1 February 2020, for all studies that used CT or MRI in determining unilateral PA and validated the results against invasive adrenal vein sampling (AVS). Summary diagnostic accuracies were assessed using a bivariate random-effects model. Subgroup analyses, meta-regression and sensitivity analysis were performed to explore the possible sources of heterogeneity.
RESULT
A total of 25 studies, involving a total of 4669 subjects, were identified. The overall analysis revealed a pooled sensitivity of 68% (95% CI: 61% to 74%) and specificity of 57% (95% CI 50% to 65%) for CT/MRI in identifying unilateral PA. Sensitivity was higher in the contrast-enhanced (CT) group versus the traditional CT group (77% (95% CI 66% to 85%) vs 58% (95% CI 50% to 66%). Subgroup analysis stratified by screening test for PA showed that the sensitivity of the aldosterone-to-renin ratio (ARR) group was higher than that of the non-ARR group (78% (95% CI 69% to 84%) vs 66% (95% CI 58% to 72%)). The diagnostic accuracy of PA patients aged ≤40 years was reported in four studies, and the overall sensitivity was 71%, with 79% specificity. Meta-regression revealed a significant impact of sample size on sensitivity and of age and study quality on specificity.
CONCLUSION
CT/MRI is not a reliable alternative to invasive AVS without excellent sensitivity or specificity for correctly identifying unilateral PA. Even in young patients (≤40 years), 21% of patients would have undergone unnecessary adrenalectomy based on imaging results alone.
Topics: Adrenal Glands; Adrenalectomy; Aged; Humans; Hyperaldosteronism; Magnetic Resonance Imaging; Tomography, X-Ray Computed
PubMed: 33384386
DOI: 10.1136/bmjopen-2020-038489 -
The Lancet. Diabetes & Endocrinology Jan 2021Phaeochromocytoma or paraganglioma (collectively known as PPGL) in pregnant women can lead to severe complications and death due to associated catecholamine excess. We...
BACKGROUND
Phaeochromocytoma or paraganglioma (collectively known as PPGL) in pregnant women can lead to severe complications and death due to associated catecholamine excess. We aimed to identify factors associated with maternal and fetal outcomes in women with PPGL during pregnancy.
METHODS
We did a multicentre, retrospective study of patients with PPGL and pregnancy between Jan 1, 1980, and Dec 31, 2019, in the International Pheochromocytoma and Pregnancy Registry and a systematic review of studies published between Jan 1, 2005, and Dec 27, 2019 reporting on at least five cases. The inclusion criteria were pregnancy after 1980 and PPGL before or during pregnancy or within 12 months post partum. Eligible patients from the retrospective study and systematic review were included in the analysis. Outcomes of interest were maternal or fetal death and maternal severe cardiovascular complications of catecholamine excess. Potential variables associated with these outcomes were evaluated by logistic regression.
FINDINGS
The systematic review identified seven studies (reporting on 63 pregnancies in 55 patients) that met the eligibility criteria and were of adequate quality. A further 197 pregnancies in 186 patients were identified in the International Pheochromocytoma and Pregnancy Registry. After excluding 11 pregnancies due to potential overlap, the final cohort included 249 pregnancies in 232 patients with PPGL. The diagnosis of PPGL was made before pregnancy in 37 (15%) pregnancies, during pregnancy in 134 (54%), and after delivery in 78 (31%). Of 144 patients evaluated for genetic predisposition for phaeochromocytoma, 95 (66%) were positive. Unrecognised PPGL during pregnancy (odds ratio 27·0; 95% CI 3·5-3473·1), abdominal or pelvic tumour location (11·3; 1·5-1440·5), and catecholamine excess at least ten-times the upper limit of the normal range (4·7; 1·8-13·8) were associated with adverse outcomes. For patients diagnosed during pregnancy, α-adrenergic blockade therapy was associated with fewer adverse outcomes (3·6; 1·1-13·2 for no α-adrenergic blockade vs α-adrenergic blockade), whereas surgery during pregnancy was not associated with better outcomes (0·9; 0·3-3·9 for no surgery vs surgery).
INTERPRETATION
Unrecognised and untreated PPGL was associated with a substantially higher risk of either maternal or fetal complications. Appropriate case detection and counselling for premenopausal women at risk for PPGL could prevent adverse pregnancy-related outcomes.
FUNDING
US National Institutes of Health.
Topics: Adolescent; Adrenal Gland Neoplasms; Adult; Cohort Studies; Female; Fetal Diseases; Humans; Incidence; Infant, Newborn; Infant, Newborn, Diseases; Male; Middle Aged; Pheochromocytoma; Pregnancy; Pregnancy Complications, Neoplastic; Pregnancy Outcome; Prenatal Exposure Delayed Effects; Retrospective Studies; Young Adult
PubMed: 33248478
DOI: 10.1016/S2213-8587(20)30363-6 -
Advances in Nutrition (Bethesda, Md.) Jun 2021A systematic review was conducted to summarize the absorption, transport, storage, and metabolism of oral neonatal vitamin A supplementation (NVAS). This review focused...
A systematic review was conducted to summarize the absorption, transport, storage, and metabolism of oral neonatal vitamin A supplementation (NVAS). This review focused specifically on the neonatal period (first 28 d of life for humans) to inform guidance by WHO on recommendations related to NVAS. A systematic search of international and regional databases was conducted. Inclusion criteria were human or animal studies that gave oral vitamin A as a single or limited number of doses to apparently healthy neonates. Studies evaluating fortification or food-based approaches, dosing with retinoic acid, or studies of neonatal models of disease were excluded. The search retrieved 8847 unique records. After screening by title and abstract, 88 were screened using the full text, and 35 records met inclusion criteria: 13 human and 22 animal studies. Studies indicate that high-dose NVAS is absorbed well by neonates, typically mirroring fat absorption. Doses were primarily stored in the liver and transiently increased in the lung, kidney, spleen, adrenal glands, brain, skin, and adipose tissue, generally with a dose-response. Serum retinol and retinyl esters also transiently increased following NVAS. Although minimal acute adverse effects are noted, there is a lack of data supporting NVAS for improving organ maturation or sustained delivery to target organs. Research gaps include the physiological effects of the short-term increase of vitamin A concentrations in extrahepatic tissues, or whether there are unknown adverse effects over time.
Topics: Animals; Dietary Supplements; Humans; Infant, Newborn; Liver; Retinyl Esters; Vitamin A; Vitamin A Deficiency
PubMed: 33216111
DOI: 10.1093/advances/nmaa137 -
Cancer Immunology, Immunotherapy : CII Jun 2021To summarize the clinical characteristics and immunological and genetic features of patients who developed autoimmune polyendocrine syndrome type II (APS-2) after...
OBJECTIVE
To summarize the clinical characteristics and immunological and genetic features of patients who developed autoimmune polyendocrine syndrome type II (APS-2) after treatment with immune checkpoint inhibitors (ICIs).
DESIGN AND METHODS
Several databases (MEDLINE/EMBASE/Cochrane) were searched for studies published between January 2000 and February 2020 involving patients with two or more endocrine disorders after ICI therapy.
RESULTS
Our final review included 22 articles comprising 23 patients (median age 56 years; 65.2% male patients). Of these patients, 60.9% received anti-programmed cell death 1 (PD-1) therapy, 17.4% received anti-programmed cell death ligand 1 (PD-L1) therapy, and 4.3% received anti-cytotoxic T-lymphocyte antigen 4 (CTLA-4) monotherapy. Patients underwent a median of four treatment cycles before the onset of the primary adverse event; the median time of onset was 8.5 weeks. Endocrine organs affected by ICI administration included the thyroid gland (18/23, 78.3%), pancreatic islets (17/23, 73.9%), pituitary gland (11/23, 47.8%), and adrenal gland (2/23, 8.7%). Related autoantibodies were detected in 65.2% of patients. In patients with diabetes, glutamic acid decarboxylase antibody was closely related to the development of diabetes ketoacidosis. The human leukocyte antigen genotype was reported in 34.8% (8/23) of patients, 5 (62.5%) of which had risk genotypes.
CONCLUSIONS
As a serious adverse event of ICI treatment, APS-2 is presented with abrupt initiation time and rapid development. Physicians should be aware of potential endocrine disorders and continue monitoring hormone status when treating cancer patients with ICIs.
Topics: Humans; Immune Checkpoint Inhibitors; Neoplasms; Polyendocrinopathies, Autoimmune; Prognosis
PubMed: 33200250
DOI: 10.1007/s00262-020-02699-1 -
Human Vaccines & Immunotherapeutics May 2021Programmed cell death protein 1 (PD-1) inhibitors are the first-line treatment for advanced non-small-cell lung cancer (NSCLC) patients. However, their efficacy in... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Programmed cell death protein 1 (PD-1) inhibitors are the first-line treatment for advanced non-small-cell lung cancer (NSCLC) patients. However, their efficacy in metastatic NSCLC patients remains controversial.
AIM OF THE STUDY
The aim of our study was to evaluate the prognosis of advanced metastatic NSCLC patients treated with PD-1 inhibitors, and discuss the predictive effect of metastatic site on the long-term outcome.
METHODS
The Embase, Ovid Medline, Cochrane Central Register of Controlled Trials, and PubMed databases were systematically screened up to February 10, 2020. Twenty-five eligible studies, involving 8,067 patients that assessed the impact of metastatic sites on survival outcome were incorporated in our study. Overall survival (OS) and progression-free survival (PFS) were described as hazard ratio (HR) with 95% confidence interval (CI).
RESULTS
Among the advanced NSCLC patients, the median proportion of brain, liver, bone, and adrenal gland metastases were 21%, 17%, 35%, and 21%, respectively. Patients with metastases to the brain, liver, and bone had worse OS compared to patients without these metastases when treated with PD-1 inhibitors. Similarly, patients with metastasis to the brain and liver were more likely to progress when treated with PD-1 inhibitors. Besides, patients with multiple metastatic sites had worse PFS compared to patients with one metastatic site, while no significant difference was found in terms of OS.
CONCLUSIONS
Based on the findings of our systematic review and meta-analysis, metastatic sites were independent predictors of the survival outcome for advanced NSCLC patients treated with PD-1 inhibitors.
Topics: B7-H1 Antigen; Carcinoma, Non-Small-Cell Lung; Humans; Immune Checkpoint Inhibitors; Lung Neoplasms; Progression-Free Survival
PubMed: 33079622
DOI: 10.1080/21645515.2020.1823779 -
BMC Surgery Jul 2020Surgical resection is the main treatment for pheochromocytoma (PHEO). Although open surgery (OS) has been shown to be safe and feasible, the safety and efficacy of... (Comparative Study)
Comparative Study Meta-Analysis
BACKGROUND
Surgical resection is the main treatment for pheochromocytoma (PHEO). Although open surgery (OS) has been shown to be safe and feasible, the safety and efficacy of laparoscopic surgery (LS) for PHEO remain controversial due to the uncertain effects of pneumoperitoneum on haemodynamics and the complexity of the tumour itself. This study was performed to compare the treatment outcomes of OS with those of LS for patients with PHEO.
METHODS
A systematic search through November 11, 2019, was conducted. All studies comparing outcomes of LS and OS for PHEO were included according to eligibility criteria. This meta-analysis was conducted using Review Manager Software, version 5.3, and STATA software, version 12.0. The quality of the included studies was assessed using the Newcastle-Ottawa scale.
RESULTS
Fourteen studies involving 626 patients were included in this meta-analysis. LS was associated with lower rates of intraoperative haemodynamic instability (IHD) [odds ratio (OR) = 0.61, 95% CI: 0.37 to 1.00, P = 0.05], less intraoperative blood loss [weighted mean difference (WMD) = - 115.27 ml, 95% confidence interval (CI): - 128.54 to - 101.99, P < 0.00001], lower blood transfusion rates [OR = 0.33, 95% CI: 0.21 to 0.52, P < 0.00001], earlier ambulation (WMD = - 1.57 d, 95% CI: - 1.97 to - 1.16, P < 0.00001) and food intake (WMD = - 0.98 d, 95% CI: - 1.36 to - 0.59, P < 0.00001), shorter drainage tube indwelling time (WMD = - 0.51 d, 95% CI: - 0.96 to - 0.07, P = 0.02) and postoperative stay (WMD = - 3.17 d, 95% CI: - 4.76 to - 1.58, P < 0.0001), and lower overall complication rates (OR = 0.56, 95% CI: 0.35 to 0.88, P = 0.01). However, no significant differences in operative time, postoperative blood pressure control, rates of severe complications, postoperative hypotension or cardiovascular disease (CVD) were found between the two groups.
CONCLUSIONS
LS is safe and effective for PHEO resection. Compared with OS, LS caused less IHD, providing an equal chance to cure hypertension while also yielding a faster and better postoperative recovery.
Topics: Adrenal Gland Neoplasms; Humans; Laparoscopy; Laparotomy; Length of Stay; Operative Time; Pheochromocytoma; Retrospective Studies; Treatment Outcome
PubMed: 32711496
DOI: 10.1186/s12893-020-00824-6 -
The Journal of Clinical Endocrinology... Jul 2020Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, autonomic dysregulation and neural crest tumor (ROHHHAD[NET]) is a rare and potentially fatal...
CONTEXT
Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, autonomic dysregulation and neural crest tumor (ROHHHAD[NET]) is a rare and potentially fatal disease. No specific diagnostic biomarker is currently available, making prompt diagnosis challenging. Since its first definition in 2007, a complete clinical analysis leading to specific diagnosis and follow-up recommendations is still missing.
OBJECTIVE
The purpose of this work is to describe the clinical timeline of symptoms of ROHHAD(NET) and propose recommendations for diagnosis and follow-up.
DESIGN
We conducted a systematic review of all ROHHAD(NET) case studies and report a new ROHHAD patient with early diagnosis and multidisciplinary care.
METHODS
All the articles that meet the definition of ROHHAD(NET) and provide chronological clinical data were reviewed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis individual patient data guidelines. The data were grouped into 7 categories: hypothalamic dysfunction, autonomic dysregulation, hypoventilation, NET, psychiatric symptoms, other clinical manifestations, and outcome.
RESULTS
Forty-three individual patient data descriptions were analyzed. The timeline of the disease shows rapid-onset obesity followed shortly by hypothalamic dysfunction. Dysautonomia was reported at a median age of 4.95 years and hypoventilation at 5.33 years, or 2.2 years after the initial obesity. A NET was reported in 56% of the patients, and 70% of these tumors were diagnosed within 2 years after initial weight gain.
CONCLUSION
Because early diagnosis improves the clinical management and the prognosis in ROHHAD(NET), this diagnosis should be considered for any child with rapid and early obesity. We propose guidance for systematic follow-up and advise multidisciplinary management with the aim of improving prognosis and life expectancy.
Topics: Adrenal Gland Neoplasms; Autonomic Nervous System Diseases; Ganglioneuroblastoma; Ganglioneuroma; Humans; Hypothalamic Diseases; Hypoventilation; Obesity; Prognosis; Syndrome
PubMed: 32407531
DOI: 10.1210/clinem/dgaa247 -
La Clinica Terapeutica 2020Laparoscopic adrenalectomy (LA) has become the treatment of choice for benign adrenal lesions. Lateral Transperitoneal Laparoscopic Adrenalectomy (LTLA) is considered...
BACKGROUND
Laparoscopic adrenalectomy (LA) has become the treatment of choice for benign adrenal lesions. Lateral Transperitoneal Laparoscopic Adrenalectomy (LTLA) is considered the gold standard. The number of LTLAs a surgeon must perform, in order to complete his learning curve, is not well defined in Literature. Moreover, the few papers dealing with the learning curve for LTLAs show controversial results and consider different evaluation parameters.
METHODS
The systematic review has been carried out according to PRISMA statement. The literature search included PubMed and Scopus database. Hand searching of reference lists of previous review articles and relevant studies was also performed. The search string was "learning curve AND laparoscopic adrenalectomy".
RESULTS
A total of 9 papers met the inclusion criteria out of 94 non duplicate citations. The aim of this systematic review is to provide a multidimensional evaluation by bringing into focus evaluation parameters of surgical performance, (operative time, intraoperative complications, conversion rate and blood loss), factors related to patient's pathology (side, size, adrenal pathology) and surgeon-specific properties.
CONCLUSIONS
Operative time, intraoperative bleeding, intraoperative complications and conversion rate are the main parameters that have been considered for the achievement of learning curve, and for each there are discrepancies, mainly due to the relative rarity of adrenal tumors, and so for difficulties in obtaining approper analysis that could establish an effective learning curve. So, further evaluations in larger experience are needed.
Topics: Adrenal Gland Neoplasms; Adrenalectomy; Humans; Intraoperative Complications; Laparoscopy; Learning Curve; Length of Stay; Operative Time
PubMed: 32141491
DOI: 10.7417/CT.2020.2209 -
Oman Medical Journal Jan 2020Histoplasmosis is uncommon in many parts of the world, including Bangladesh, where, in recent years, cases are increasingly reported. We sought to describe the... (Review)
Review
Histoplasmosis is uncommon in many parts of the world, including Bangladesh, where, in recent years, cases are increasingly reported. We sought to describe the sociodemographic characteristics, clinical presentation, investigations, treatment, and outcome of histoplasmosis in Bangladesh. We conducted a retrospective data review of published literature from 1962 to 2017, containing information on histoplasmosis in and/or from Bangladesh. Unpublished, well-documented histoplasmosis cases were also included. A total of 26 male patients aged 8-75 years, with a diagnosis of histoplasmosis were included; nine were farmers, seven had diabetes, one was a renal transplant recipient, and four had HIV/AIDS. Fever (n = 20), weight loss (n = 17), anemia (n = 15), lymphadenopathy (n = 9), and hepatosplenomegaly (n = 7) were common. Eleven patients had bilateral adrenal enlargement. Diagnosis was confirmed by histo/cytopathology from skin (n = 1), oropharyngeal ulcers (n = 8), lymph nodes (n = 3), adrenal glands (n = 11), paravertebral soft tissue (n = 2), and bone marrow (n = 4). Cultures of representative samples and antibodies were detected in three and two cases, respectively. Twenty-two patients had disseminated histoplasmosis and four patients had localized oropharyngeal disease. Nine patients were prescribed anti-tuberculosis drugs empirically before establishing the diagnosis of histoplasmosis. Treatment consisted of amphotericin B and itraconazole. Six patients died in hospital, 14 patients recovered with relapse in two cases, and the outcome of the other patients could not be ascertained. Histoplasmosis is thought to be endemic in Bangladesh, but few cases are reported to date, which may be due to many asymptomatic, undiagnosed, misdiagnosed, or under-reported cases. Histoplasmosis should be considered as a differential in appropriate clinical scenarios.
PubMed: 32095275
DOI: 10.5001/omj.2020.09