DescriptorName: AdrenalCortexNeoplasms. DescriptorName: AdrenocorticalCarcinoma... immunohistochemical expression of several proteins and their relationship with adrenal cortical carcinoma (ACC) diagnosis and progression AbstractText: A total of 83 patients with benign and malignant adrenalcortextumors operated on in a single center were included in the study... Keyword: adrenaltumors.
... 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: Kidney Neoplasms... DescriptorName: Neoplasms... DescriptorName: Thyroid Neoplasms. Abstract: The second article in this 2-part continuing medical education series reviews the following malignant causes of flushing: mastocytosis, medullary thyroid carcinoma, pheochromocytoma, carcinoid tumors, gastroenteropancreatic neuroendocrine tumors, bronchogenic carcinoma, vasointestinal polypeptide secreting tumors, and renal cell carcinoma.
Carcinoma (4), Pheochromocytoma (3), Mastocytosis (3), more mentions
The norepinephrine transporter (NET) is essential for norepinephrine uptake at the synaptic terminals and adrenal chromaffin cells. In neuroendocrine tumors, NET can be targeted for imaging as well as therapy. One of the most widely used theranostic agents targeting NET is metaiodobenzylguanidine (MIBG), a guanethidine analog of norepinephrine. (123)I/(131)I-MIBG theranostics have been applied in the clinical evaluation and management of neuroendocrine tumors, especially in neuroblastoma, paraganglioma, and pheochromocytoma. (123)I-MIBG imaging is a mainstay in the evaluation of neuroblastoma, and (131)I-MIBG has been used for the treatment of relapsed high-risk neuroblastoma for several years, however, the outcome remains suboptimal. (131)I-MIBG has essentially been only palliative in paraganglioma/pheochromocytoma patients. Various techniques of improving therapeutic outcomes, such as dosimetric estimations, high-dose therapies, multiple fractionated administration and combination therapy with radiation sensitizers, chemotherapy, and other radionuclide therapies, are being evaluated. PET tracers targeting NET appear promising and may be more convenient options for the imaging and assessment after treatment. Here, we present an overview of NET as a target for theranostics; review its current role in some neuroendocrine tumors, such as neuroblastoma, paraganglioma/pheochromocytoma, and carcinoids; and discuss approaches to improving targeting and theranostic outcomes.
Neuroblastoma (5), Paraganglioma (3), Neuroendocrine Tumors (3), more mentions
AbstractText: Current adrenalectomy outcomes for functional adrenocorticalcarcinoma (ACC) remain unclear... require appropriate preoperative medical optimization prior to adrenalectomy AbstractText: Patients with functional adrenocorticalcarcinoma (ACC) have significant preoperative comorbidities and experience higher rates of certain postoperative ... These patients should undergo appropriate preoperative counseling in preparation for adrenalectomy Keyword: Adrenocorticalcarcinoma.
Abstract: Compared with left suprarenal splenosis, a right suprarenal accessory spleen is more likely to be misdiagnosed as an adrenaltumor due to its extreme rarity. Especially when splenosis mimics an atypical pheochromocytoma, preoperative diagnosis may become more difficult and elusive. Herein we report the case of a female patient with a right suprarenal mass, which was suspected to be ...
Pheochromocytoma (4), Splenosis (3), Neoplasms (1), more mentions
OBJECTIVE: To determine if the laterality of primary tumors in patients with olfactory neuroblastoma (ONB) influenced the pattern and development of neck disease.
METHODS: Using a retrospective cohort study design from 1994 to 2015, the primary tumors of patients who either presented with or developed neck disease were volumetrically analyzed using iPlan software (version 3.0.0, BrainLAB, Feldkirchen, Germany) by two independent observers. Agreement of volume-derived sidedness was assessed with a kappa statistic, whereas agreement in volume-derived degree of tumor laterality was evaluated with an intraclass correlation coefficient. A one-sample t test was used to assess the difference in dominant percentage between the two observers.
RESULTS: Sixty-one patients with histological diagnosis and treatment of ONB at our institution were identified. Twenty-four patients exhibited neck involvement, 13 of whom could be volumetrically analyzed. Tumors that were greater than 75% eccentric to one side all exhibited contralateral disease, whereas the majority of unilateral neck disease was associated with relatively midline masses. Within the entire cohort, ipsilateral level 2 lymph nodes displayed the highest involvement (83%, 20 of 24), followed by ipsilateral level 1 (54%, 13 of 24), contralateral level 2 (46%, 11 of 24), contralateral level 1 (21%, 5 of 24), and ipsilateral level 3 (21%, 5 of 24).
CONCLUSION: Ipsilateral neck involvement frequently was observed; however, the degree of ONB primary site laterality did not appear to have implications on the development of contralateral neck disease. Therefore, when considering elective therapy to the neck, ONB laterality should not be used to justify unilateral neck treatment.
LEVEL OF EVIDENCE: 4. Laryngoscope, 2017.
Neoplasms (4), Olfactory Esthesioneuroblastoma (4), more mentions
INTRODUCTION: To describe outcomes of patients with metyrosine (MET) pretreatment for abdominal surgical resection of pheochromocytoma or paraganglioma (PCC/PGL) compared with patients who had phenoxybenzamine (PBZ) pretreatment.
METHODS: Retrospective review of perioperative outcomes for PCC/PGL patients treated with MET and propensity-matched comparison of MET and PBZ (MET + PBZ) with PBZ alone.
RESULTS: MET preparation was given in 63 cases (26 laparoscopic and 37 open, of which 55 also received PBZ). All patients had wide perioperative hemodynamic oscillations. Patients with open procedures required more intravenous fluids and blood transfusions; 35% required postoperative vasopressor infusions for hypotension and 38% developed acute kidney injury. One laparoscopic procedure required postoperative vasopressor infusion, and 12% of patients developed acute kidney injury. Forty-five MET + PBZ patients were propensity-matched with PBZ-only patients. Intraoperatively, MET + PBZ patients had lower minimum systolic and diastolic blood pressures than PBZ-only patients (median systolic, 74 vs 80 mm Hg, P = 0.01; median diastolic, 42 vs 46 mm Hg, P = 0.005) and larger intraoperative blood pressure oscillations (median systolic range, 112 vs 93 mm Hg, P = 0.06; median diastolic range, 58 vs 51 mm Hg, P = 0.02). Postoperative vasopressor infusion use was similar between MET + PBZ and PBZ only (16% vs 11%, P = 0.76). Major outcomes were not different between regimens.
CONCLUSION: Large hemodynamic oscillations were present in our PCC/PGL patients treated with MET + PBZ. These patients had a wider range of intraoperative blood pressure variations than PBZ-only patients. No differences in postoperative comorbid outcomes were found between MET + PBZ and PBZ-only groups.
Immune System Diseases (1) Paraganglioma (3), Pheochromocytoma (3), Acute Kidney Injury (2), more mentions
BACKGROUND: Although several studies have been conducted on the role of surgery in localized neuroblastoma, the impact of surgical timing and extent of primary tumor resection on outcome in high-risk patients remains controversial.
METHODS: Patients from the German neuroblastoma trial NB97 with localized neuroblastoma INSS stage 1-3 age > 18 months were included for retrospective analysis. Imaging reports were reviewed by two independent physicians for Image Defined Risk Factors (IDRF). Operation notes and corresponding imaging reports were analyzed for surgical radicality. The extent of tumor resection was classified as complete resection (95-100%), gross total resection (90-95%), incomplete resection (50-90%), and biopsy (<50%) and correlated with local control rate and outcome. Patients were stratified according to the International Neuroblastoma Risk Group (INRG) staging system. Survival curves were estimated according to the method of Kaplan and Meier and compared by the log-rank test.
RESULTS: A total of 179 patients were included in this study. 77 patients underwent more than one primary tumor operation. After best surgery, 68.7% of patients achieved complete resection of the primary tumor, 16.8% gross total resection, 14.0% incomplete surgery, and 0.5% biopsy only. The cumulative complication rate was 20.3% and the surgery associated mortality rate was 1.1%. Image defined risk factors (IDRF) predicted the extent of resection. Patients with complete resection had a better local-progression-free survival (LPFS), event-free survival (EFS) and OS (overall survival) than the other groups. Subgroup analyses showed better EFS, LPFS and OS for patients with complete resection in INRG high-risk patients. Multivariable analyses revealed resection (complete vs. other), and MYCN (non-amplified vs. amplified) as independent prognostic factors for EFS, LPFS and OS.
CONCLUSIONS: In patients with localized neuroblastoma age 18 months or older, especially in INRG high-risk patients harboring MYCN amplification, extended surgery of the primary tumor site improved local control rate and survival with an acceptable risk of complications.
AbstractText: Metastatic AdrenocorticalCarcinoma (ACC) is a rare malignancy with a poor 5-year-survival rate ( <15 ... resection of hepatic metastases of ACC AbstractText: A retrospective analysis of the German AdrenocorticalCarcinoma Registry was conducted... recurrence and single versus multiple metastases are predictive factors for the outcome Keyword: Adrenocorticalcarcinoma.
We describe a family with multiple endocrine neoplasia type 2A (MEN2A) caused by the D631Y RET mutation resulting in an atypical phenotype. The index case was a 24-year-old man with history of recurrent anaplastic ependymoma incidentally found to have the D631Y RET mutation. At first assessment, four family members had evidence of large pheochromocytomas. One patient was found to have micromedullary thyroid cancer at 79 years of age. None of the patients had primary hyperparathyroidism. Patients with MEN2A caused by a D631Y RET mutation most commonly present with pheochromocytomas. Medullary thyroid cancer is a less common part of the syndrome when compared with other RET mutations.
Oncology (3) Multiple Endocrine Neoplasia Type 2a (5), Pheochromocytoma (3), Thyroid Carcinoma (3), more mentions
Pheochromocytoma (PHEO) and paraganglioma (PGL) (PPGL) may cause acute Takotsubo-like catecholamine cardiomyopathy (TLC).
OBJECTIVE: To determine the prevalence and clinical presentation of TLC in a large cohort of patients with PPGL.
METHODS: We reviewed retrospectively the records of consecutive patients with PPGL investigated in our center from 1995 to 2016. We collected clinical and paraclinical data of patients that had TLC in this cohort. We performed a literature review of cases of TTC-related to PPGL described previously from 1990-2015.
RESULTS: Our cohort included 275 patients with PPGL (151 PHEO and 121 PGL). Acute TLC was found in 4/152 (2.6%) patients with secreting-PPGL. There was no event recorded in 123 patients with unknown presurgical secretion (n=51) or non-secreting PPGL (n=72). Four patients (44 to 79 years old) filled the criteria for TLC, including 2 PHEO and 2 PGL. A precipitating stressor event was identified in 3 cases including surgery (n=2) and upper respiratory tract infection. In all cases the diagnosis of PPGL came after the cardiac event and following the investigation of a lesion incidentally found at imaging. Moreover, we identified 59 cases described previously in the last 25 years and analyzed this cohort together with our 4 new cases.
CONCLUSION: Acute TLC may be found in up to 3% of patients with secreting-PPGL. Considering that the diagnosis of PPGL was performed following incidental finding of radiological mass, the real prevalence of PPGL in TTC remains to be determined.
Paraganglioma (3), Pheochromocytoma (3), Cardiomyopathies (2), more mentions
OBJECTIVE: Various studies have validated plasma free metanephrines as biomarkers for pheochromocytoma and paraganglioma (PPGL). This meta-analysis aimed to estimate the overall diagnostic accuracy of this biochemical test for PPGL.
METHODS: We searched the PubMed, the Cochrane Library, Web of Science, Embase, Scopus, OvidSP, and ProQuest Dissertations & Theses databases from January 1, 1995 to December 2, 2016 and selected studies written in English that assessed plasma free metanephrines in the diagnosis of PPGL. Quality Assessment of Diagnostic Accuracy Studies 2 (QUADAS-2) was used to evaluate the quality of the included studies. We calculated pooled sensitivities, specificities, positive and negative likelihood ratios, diagnostic odds ratios (DORs) and areas under curve (AUCs) with their 95% confidence intervals (95% CIs). Heterogeneity was assessed by I(2). To identify the source of heterogeneity, we evaluated the threshold effect and performed a meta-regression. Deeks' funnel plot was selected for investigating any potential publication bias.
RESULTS: Although the combination of metanephrine and normetanephrine carried lower specificity (0.94, 95% CI 0.90-0.97) than normetanephrine (0.97, 95% CI 0.92-0.99), normetanephrine was generally more accurate than individual tests, with the highest AUC (0.99, 95% CI 0.97-0.99), DOR (443.35, 95% CI 216.9-906.23), and pooled sensitivity (0.97, 95% CI 0.94-0.98) values. Threshold effect and meta-regression analyses showed that different cut-offs, blood sampling positions, study types and test methods contributed to heterogeneity.
CONCLUSIONS: This meta-analysis suggested an effective value for combined plasma free metanephrines for the diagnosis of PPGL, but testing for metanephrines requires more standardization using tightly regulated studies.
All neoplasms were completely resected via transurethral resection of bladder tumor (TURBT), en bloc transurethral resection with thulium-yag laser, or partial cystectomy. Most PUB samples were positive for immunohistochemical (IHC) staining markers such as chromogranin A (CgA), Synaptophysin (Syn), CD56, and S-100 protein, while negative for IHC markers such as cytokeratins (CKs) and Melan A. According to pathological ...
AbstractText: Adrenaladenomas are benigntumors often discovered incidentally, and >70% are hormonally inactive... Malignant adrenaltumors were excluded... 78% (n = 21,279) had hormonally inactive and 22% (n = 6033) had hormonally active adrenaltumors... high-risk patients should undergo appropriate preoperative medical optimization in preparation for adrenalectomy Keyword: Adrenaladenoma.
Deletion of the long arm of chromosome 11 (11q deletion) is one of the most frequent events that occur during the development of aggressive neuroblastoma. Clinically, 11q deletion is associated with higher disease stage and decreased survival probability. During the last 25 years, extensive efforts have been invested to identify the precise frequency of 11q aberrations in neuroblastoma, the recurrently involved genes, and to understand the molecular mechanisms of 11q deletion, but definitive answers are still unclear. In this review, it is our intent to compile and review the evidence acquired to date on 11q deletion in neuroblastoma.
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