CitationSubset: IM. DescriptorName: AdrenalCortexNeoplasms. DescriptorName: AdrenocorticalCarcinoma... DescriptorName: DNA, Neoplasm... AbstractText: The disease course of adrenocorticalcarcinoma (ACC) patients is heterogeneous. A marker for prognosis and treatment response would facilitate choices for diagnosis and therapy. In other cancer types, circulating cell-free tumor DNA predicted tumor dynamics AbstractText: The present pilot study included six patients.
DescriptorName: AdrenalCortexNeoplasms. DescriptorName: AdrenocorticalCarcinoma... DescriptorName: Neoplasm Recurrence, Local... AbstractText: Adrenocorticalcarcinoma (ACC) is a rare endocrine malignancy with overall poor prognosis... local recurrences, eight metastases) from seven European Network for the Study of AdrenalTumors centers were studied AbstractText: H-scores were generated to quantify VAV2 expression ...
Adrenocorticaladenomas (ACA) may lead to the autonomous secretion of aldosterone responsible for ... ionic homeostasis and membrane potential have been identified in aldosterone producing adenoma... In cortisol producing adenomas, recurrent somatic mutations in PRKACA (coding for the cAMP-dependent protein ... have been identified in both aldosterone as well as cortisol producing adenomas.
... serve as a marker of disease severity in patients with adrenaladenomas and glucocorticoid secretory autonomy AbstractText: Retrospective cohort study AbstractText: Twenty ... cortisol excess (MACE), and 32 patients with a non-functioning adrenaltumor (NFAT) were included AbstractText: Medical records were reviewed and body ... objective assessment of glucocorticoid-related disease severity in patients with adrenaladenomas.
... tracers in other malignant and benign lesions has been reported, including faint accumulation of Ga-PSMA-HBED-CC in adrenaladenoma... Non-contrast-enhanced CT scan indicated left adrenaladenoma. Regarding the high positive predictive value of multiphase contrast-enhanced CT (98%), presence of right adrenaladenoma is also likely.
DescriptorName: Adrenal Gland Neoplasms. DescriptorName: Algorithms. DescriptorName: Disease Management. DescriptorName: Endocrine System Diseases. DescriptorName: Flushing. DescriptorName: Hot Flashes. DescriptorName: Humans. DescriptorName: Malignant Carcinoid Syndrome. DescriptorName: Menopause. DescriptorName: Neuroendocrine Tumors. DescriptorName: Pheochromocytoma. DescriptorName: Sweating. AbstractText: Flushing can be defined as a sensation of warmth accompanied by erythema that most commonly is seen on the face and which occurs in episodic attacks.
Women's Health (2), Endocrine Disorders (1) Pheochromocytoma (2), Mastocytosis (2), Endocrine System Diseases (2), more mentions
DescriptorName: Adrenal Gland Neoplasms... AbstractText: Recently, the European Society of Endocrinology (ESE), in collaboration with the European Network for the Study of AdrenalTumors (ENSAT), asserted that adrenal incidentalomas (AIs) <4 cm and ≤10 Hounsfield units (HU) do not require further follow-up imaging. To validate the clinical application of the follow-up strategies suggested by the 2016 ESE-ENSAT ...
Neoplasms (2), Cushing Syndrome (2), Pheochromocytoma (1), more mentions
The discovery of missense mutations of ALK gene identified this receptor tyrosine kinase as a therapeutic target in neuroblastoma (NB). Moreover, a high level of ALK protein has been associated with metastatic NB cases and with a worse prognosis, suggesting that also ALK overexpression is involved in NB tumorigenesis. Since miRNAs play key roles in the regulation of gene expression we aimed at identifying those miRNAs that can regulate ALK in NB. We therefore analyzed the genome-wide expression profile of miRNAs in two sample sets of 16 NB cell lines and 22 NB samples by using miRNA microarrays. Both sample sets were then divided into two subgroups showing high (ALK+) or low/absent (ALK-) expression of ALK. Results showed a down-regulation of 30 and 23 miRNAs (p-value <0.05) in the ALK+ group in NB cell lines and samples, respectively. Validation analysis indicated that miR-424-5p and miR-503-5p, belonging to the same cluster, were differentially expressed in both NB cell lines and tumor samples. Although only miR-424-5p showed a direct binding to ALK 3'-UTR, both miRNAs led to a remarkable decreasing of ALK protein as well as to the inhibition of cell viability in ALK+ NB cell lines. In conclusion, our data indicate that both miR-424-5p and miR-503-5p are involved in regulating ALK expression in NB, either by directly targeting ALK receptor or indirectly, and may thus serve as potential therapeutic tools in ALK dependent NBs.
Neuroblastoma (2), Neoplastic Cell Transformation (1), Neoplasms (1), more mentions
Adrenergic myocarditis is an uncommon presentation of pheochromocytoma and extremely rare cause of de novo acute heart failure (AHF). We present a case of a 31-year-old Caucasian woman with a history of hypertension and recurrent occipital headaches who was admitted to the emergency department due to severe de novo AHF presenting as pulmonary edema and cardiogenic shock. During the hospital admission the patient experienced asystolic cardiac arrest and was successfully resuscitated, intubated, and mechanically ventilated. Bedside transthoracic echocardiography revealed severe diffuse left ventricular hypokinesis with ejection fraction (LVEF) of 10%. Coronary angiography disclosed normal epicardial coronary arteries. The diagnosis of fulminant myocarditis was based on clinical, laboratory and imaging findings including cardiac magnetic resonance imaging (cMRI) Lake Louise criteria. STIR-cMRI sequences revealed myocardial edema in the lateral, inferior and posterior walls of the left ventricle, whereas T1-weighted early contrast-enhanced sequences showed myocardial hyperemia and capillary leak. An ultrasound and computed tomographic scan of the abdomen disclosed a solid, heterogeneous mass (3.6×3.2×2.8-cm) in the right suprarenal area. Urinary and plasma catecholamines and metanephrines were markedly elevated. A pheochromocytoma was suspected and laparoscopic resection of the tumor was performed after pharmacological preparation with phenoxybenzamine. The histopathological findings were consistent with pheochromocytoma. Follow-up cMRI showed complete reversal of myocardial edema and hyperemia. At 12-month follow-up, the patient has remained asymptomatic and normotensive with no recurrence of cardiovascular symptoms.
CONTEXT: Pheochromocytoma and paraganglioma are rare neuroendocrine tumors which overproduce catecholamines and arise from the adrenal gland or extra-adrenal chromaffin cells of the sympathetic and parasympathetic ganglia (1). Excessive catecholamine-induced stimulation of cardiac myocytes leads to damage which manifests in several forms ranging from Takotsubo to dilated cardiomyopathy. Diagnosis of pheochromocytoma-related cardiomyopathies is often delayed due to the atypical presentation associated with many cases.
OBJECTIVE: Limited data exists on the presentation and outcomes of the various forms of pheochromocytoma-induced cardiomyopathies. We performed a literature review to assess the association of pheochromocytoma and cardiomyopathy to aide in further understanding this clinical entity.
DESIGN: 163 cases from 150 articles published between 1991 and November 2016 were included from a PubMed search.
RESULTS: There were 163 occurrences of pheochromocytoma and cardiomyopathy (63 dilated cardiomyopathy, 38 Takotsubo cardiomyopathy, 30 inverted Takotsubo cardiomyopathy, 10 HOCM, 8 myocarditis, and 14 unspecified cardiomyopathy). Many patients lacked classic signs or symptoms of pheochromocytoma with hypertension as a presenting symptom in 65% and the triad of headache, palpitations, and diaphoresis only in 4%. Resection of the pheochromocytoma led to improvement of the cardiomyopathy in 96% while lack of resection was associated with death or cardiac transplantation in 44%.
CONCLUSION: Pheochromocytoma should be considered in the evaluation of non-ischemic, non-valvular cardiomyopathy even in the absence of symptoms of catecholamine excess. Our study highlights the importance of early suspicion and diagnosis of pheochromocytoma in cases of idiopathic heart failure as early resection may prevent progression to irreversible myocardial remodeling and death.
Background: This retrospective analysis of patients who underwent adrenalectomy for pheochromocytoma aimed to determine preoperative risk factors for intraoperative massive blood loss. Preoperative identification of patients at high-risk of massive blood loss may be helpful in anesthesia management and preoperative preparation.
Materials and Methods: The study involved data of 268 patients who had undergone pheochromocytoma surgery at the Peking Union Medical College Hospital between January 1, 2013 and October 31, 2016. For analysis, the patients were grouped according to intraoperative blood loss: ≥ 20% of estimated blood volume (group A, n = 38) and < 20% of estimated blood volume (group B, n = 230). Perioperative characteristics were compared between the two groups. Significant variables were selected for a forward stepwise binary logistic regression analysis to determine the independent risk factors for massive blood loss.
Results: The two groups showed significant differences in tumor location, tumor size, operative approach, preoperative 24-hour urine level of total noradrenaline, preoperative hemoglobin concentration, phenoxybenzamine maximum daily dose, preoperative preparation time, intraoperative urine volume, crystalloid and colloidal fluid volumes, allogeneic red blood cell transfusion, plasma and autologous blood transfusion volumes, incidence of prolonged hypotension, postoperative drainage volume, lowest and discharge hemoglobin concentrations, length of stay in intensive care unit and length of postoperative hospitalization. Binary logistic regression analysis indicated increased risk of intraoperative massive blood loss in subjects with tumors proximal to vessels or other organs (odds ratio (OR): 4.227), with tumors ≥ 5 cm (OR: 7.321), or with preoperative preparation time of ≤ 14 days (OR: 17.747).
Conclusions: Tumors proximal to vessels and other organs or with maximum diameter of ≥ 5 cm (as shown by preoperative radiographic evidence), and preoperative preparation time of ≤ 14 days were independent risk factors of intraoperative massive blood loss in patients treated with adrenalectomy for pheochromocytoma.
Pheochromocytoma (5), Neoplasms (5), Hypotension (1), more mentions
... histopathological features of olfactory neuroblastoma (ONB) and other sinonasal neuroendocrine neoplasms (NENs), in order to refine diagnostic criteria, analyze treatment outcomes ... The neoplasms were ultimately classified into four groups with different immunohistochemical profiles ... cases (5-year-OS, 42.6%); MiNEN (mixed neuroendocrine/non-neuroendocrine neoplasm) in five cases (5-year-OS, 0%,0/5 cases ...
PURPOSE OF REVIEW: The purpose of this manuscript is to review the progress in the field of therapeutics for malignant pheochromocytomas and sympathetic paraganglioma (MPPG) over the past 5 years.
RECENT FINDINGS: The manuscript will describe the clinical predictors of survivorship and their influence on the first TNM staging classification for pheochromocytomas and sympathetic paragangliomas, the treatment of hormonal complications, and the rationale that supports the resection of the primary tumor and metastases in patients with otherwise incurable disease. Therapeutic options for patients with bone metastasis to the spine will be presented. The manuscript will also review chemotherapy and propose a maintenance regimen with dacarbazine for patients initially treated with cyclophosphamide, vincristine, and dacarbazine. Finally, the manuscript will review preliminary results of several phase 2 clinical trials of novel radiopharmaceutical agents and tyrosine kinase inhibitors. MPPGs are very rare neuroendocrine tumors. MPPGs are usually characterized by a large tumor burden, excessive secretion of catecholamines, and decreased overall survival. Recent discoveries have enhanced our knowledge of the pathogenesis and phenotypes of MPPG. This knowledge is leading to a better understanding of the indications and limitations of the currently available localized and systemic therapies as well as the development of phase 2 clinical trials for novel medications.
Oncology (1) Pheochromocytoma (4), Paraganglioma (4), Neoplasms (2), more mentions
... after adrenalectomy in patients with benign versus malignant nonfunctional primary adrenaltumors AbstractText: A retrospective cross-sectional analysis was performed using the ... On risk-adjusted multivariate analysis, malignant nonfunctional primary adrenaltumors were independently associated with increased risk of complications following adrenalectomy AbstractText: Patients with malignant nonfunctional primary adrenaltumors have higher perioperative morbidity and mortality compared to patients with benign nonfunctional adrenaltumors ...
Cardiovascular Diseases (1), Immune System Diseases (1) Neoplasms (11), Hematoma (1), Acute Kidney Injury (1), more mentions
PURPOSE: Neuroblastoma may be treated with molecular radiotherapy, (131)I meta-Iodobenzylguanidine and (177)Lu Lutetium DOTATATE, directed at distinct molecular targets: Noradrenaline Transporter Molecule (NAT) and Somatostatin Receptor (SSTR2), respectively. This study used immunohistochemistry to evaluate target expression in archival neuroblastoma tissue, to determine whether it might facilitate clinical use of molecular radiotherapy.
METHODS: Tissue bank samples of formalin fixed paraffin embedded neuroblastoma tissue from patients for whom clinical outcome data were available were sectioned and stained with haematoxylin and eosin, and monoclonal antibodies directed against NAT and SSTR2. Sections were examined blinded to clinical information and scored for the percentage and intensity of tumour cells stained. These data were analysed in conjunction with clinical data.
RESULTS: Tissue from 75 patients was examined. Target expression scores varied widely between patients: NAT median 45%, inter-quartile range 25% - 65%; and SSTR2 median 55%, interquartile range 30% - 80%; and in some cases heterogeneity of expression between different parts of a tumour was observed. A weak positive correlation was observed between the expression scores of the different targets: correlation coefficient = 0.23, p = 0.05. MYCN amplified tumours had lower SSTR2 scores: mean difference 23% confidence interval 8% - 39%, p < 0.01. Survival did not differ by scores.
CONCLUSIONS: As expression of both targets is variable and heterogeneous, imaging assessment of both may yield more clinical information than either alone. The clinical value of immunohistochemical assessment of target expression requires prospective evaluation. Variable target expression within a patient may contribute to treatment failure.
... To evaluate the proportion of pheochromocytomas meeting the criteria for adenoma on adrenal washout CT and the diagnostic performance of adrenal washout CT for differentiating adenoma from pheochromocytoma AbstractText: MEDLINE and EMBASE were searched to 28 ... The pooled proportion of pheochromocytomas meeting the criteria for adenomas was 35 % (95 % CI 20-51... than washout percentage should be used when diagnosing pheochromocytomas Keyword: Adrenaladenoma.
BACKGROUND: The presence of single nucleotide polymorphisms (SNPs) in the REarranged during Transfection (RET) gene has been investigated with regard to their potential role in the development or progression of medullary thyroid cancer or pheochromocytomas (PHEO) in patients with the multiple endocrine neoplasia type 2 (MEN2) syndrome. The aim of this study was to evaluate the spectrum of RET variants in France between 2003 and 2013, and to evaluate the impact of SNPs on the MEN2 A phenotype.
METHODS: In this retrospective cohort study, RET variants were screened in 5109 index cases, and RET pathogenic variants were screened in 2214 relatives. Exons 5, 8, 10, 11, 13, 14, 15, and 16 were characterized by Sanger sequencing. RET pathogenic variants, RET variants with unknown functional significance (VUS), and four RET SNP variants-G691S (rs1799939), L769L (rs1800861), S836S (rs1800862), and S904S (rs1800863)-were characterized and are reported in index cases. In silico analysis and classification following the recommendation of the American College of Medical Genetics and Genomics was performed for RET VUS. Each patient's age at the time of diagnosis, sex, and the endocrine neoplasias present at molecular diagnosis were recorded.
RESULTS: Twenty-six single VUS in RET without any well-defined risk profiles were found in 33 patients. Nine of these were considered probably pathogenic, 11 of uncertain significance, and six as probably benign. Three double pathogenic variants found in three patients were classified as pathogenic. A study of the entire cohort showed that patients carrying pathogenic variants or VUS in RET together with PHEO were diagnosed earlier than the others. The presence of the G691S SNP, or a combination of SNPs, increased the risk of developing PHEO but did not modify the date of the diagnosis. No association was found between SNPs and medullary thyroid cancer or hyperparathyroidism.
CONCLUSIONS: The findings propose a classification of 15 of the 26 VUS in RET without any well-defined risk profiles and suggest that the G691S SNP, or a combination of SNPs, may be associated with the development of PHEO.
Oncology (3) Thyroid Carcinoma (3), Multiple Endocrine Neoplasia Type 2a (2), Pheochromocytoma (2), more mentions
BACKGROUND: Validation of the prognostic value of the SIOPEN mIBG skeletal scoring system in two independent stage 4, mIBG avid, high-risk neuroblastoma populations.
RESULTS: The semi-quantitative SIOPEN score evaluates skeletal meta-iodobenzylguanidine (mIBG) uptake on a 0-6 scale in 12 anatomical regions. Evaluable mIBG scans from 216 COG-A3973 and 341 SIOPEN/HR-NBL1 trial patients were reviewed pre- and post-induction chemotherapy. The prognostic value of skeletal scores for 5-year event free survival (5 yr.-EFS) was tested in the source and validation cohorts. At diagnosis, both cohorts showed a gradual non-linear increase in risk with cumulative scores. Several approaches were explored to test the relationship between score and EFS. Ultimately, a cutoff score of ≤3 was the most useful predictor across trials. A SIOPEN score ≤ 3 pre-induction was found in 15% SIOPEN patients and in 22% of COG patients and increased post-induction to 60% in SIOPEN patients and to 73% in COG patients. Baseline 5 yr.-EFS rates in the SIOPEN/HR-NBL1 cohort for scores ≤3 were 47% ± 7% versus 26% ± 3% for higher scores at diagnosis (p < 0.007) and 36% ± 4% versus 14% ± 4% (p < 0.001) for scores obtained post-induction. The COG-A3973 showed 5 yr.-EFS rates for scores ≤3 of 51% ± 7% versus 34% ± 4% for higher scores (p < 0.001) at diagnosis and 43% ± 5% versus 16% ± 6% (p = 0.004) for post-induction scores. Hazard ratios (HR) significantly favoured patients with scores ≤3 after adjustment for age and MYCN-amplification. Optimal outcomes were recorded in patients who achieved complete skeletal response.
CONCLUSIONS: Validation in two independent cohorts confirms the prognostic value of the SIOPEN skeletal score. In particular, patients with an absolute SIOPEN score > 3 after induction have very poor outcomes and should be considered for alternative therapeutic strategies.
... preclinical and clinical studies of the immune checkpoint inhibitors as a treatment option for thyroid cancer, ovarian carcinoma, pancreatic adenocarcinoma, adrenocorticalcarcinoma and neuroendocrine tumors... opportunities of using immune checkpoint inhibitors against these endocrine malignancies, highlighting the breakthroughs and pitfalls that have recently emerged Keyword: adrenocorticalcarcinoma. Keyword: endocrine neoplasms.