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https://www.readbyqxmd.com/read/28650885/aggressive-imaging-features-in-a-malignant-pheochromocytoma-with-a-novel-mutation-of-the-sdhb-gene
#1
Soon-Ah Park, Dae Ho Lee, Hun Soo Kim
We describe a 15-year-old boy with a huge bilateral adrenal pheochromocytoma that had a de novo germline mutation in the succinate dehydrogenase subunit B (SDHB) gene. F-FDG PET/CT revealed bilateral metabolically active large masses in the adrenal glands and the activated brown adipose tissues. The I-MIBG scintigraphic findings revealed only a mild accumulation of MIBG in the right adrenal mass, but a high uptake in the left adrenal mass. Thus, F-FDG PET/CT imaging may be more effective than I-MIBG scintigraphy for the evaluation of pheochromocytomas that are associated with highly malignant characteristics resulting from mutations of the SDHB gene...
June 24, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28649613/early-onset-parkinsonism-in-a-pedigree-with-phosphoglycerate-kinase-deficiency-and-a-heterozygous-carrier-do-pgk-1-mutations-contribute-to-vulnerability-to-parkinsonism
#2
Satoshi Sakaue, Takashi Kasai, Ikuko Mizuta, Masaya Suematsu, Shinya Osone, Yumiko Azuma, Toshihiko Imamura, Takahiko Tokuda, Hitoshi Kanno, Omar M A El-Agnaf, Masafumi Morimoto, Masanori Nakagawa, Hajime Hosoi, Toshiki Mizuno
Phosphoglycerate kinase 1 (PGK-1) is a glycolytic enzyme encoded by PGK-1, which maps to the X chromosome. PGK-1 deficiency causes X-linked recessive hereditary chronic hemolytic anemia, myopathy, and neurological disorders due to insufficient ATP regeneration. Early-onset parkinsonism has occasionally been reported as a neurological complication of this condition. However, heterozygous carriers of PGK-1 deficiency were thought to be neurologically asymptomatic. Here, we report a boy with PGK-1 deficiency and his mother, a carrier of a heterozygous mutation in PGK-1, both of whom presented with early-onset parkinsonism...
2017: NPJ Parkinson's Disease
https://www.readbyqxmd.com/read/28646015/determination-of-the-heart-to-mediastinum-ratio-of-123-i-mibg-uptake-using-dual-isotope-123-i-mibg-99m-tc-tetrofosmin-multi-pinhole-czt-spect-in-patients-with-heart-failure
#3
Tanguy Blaire, Alban Bailliez, Fayçal Ben Bouallègue, Dimitri Bellevre, Denis Agostini, Alain Manrique
The aim of this retrospective study was to determine the heart-to-mediastinum ratio (HMR) of (123)I-metaiodobenzylguanidine (MIBG) uptake obtained using a multi-pinhole cadmium-zinc-telluride (CZT)-based camera (Discovery NM 530c, DNM-530c, GE Healthcare, Milwaukee, WI, USA) in comparison with that obtained using conventional planar imaging (Infinia, GE Healthcare, Milwaukee, WI, USA). Methods: Forty consecutive heart failure patients underwent planar acquisition 4 h after (123)I-MIBG injection (191±41 MBq)...
June 23, 2017: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
https://www.readbyqxmd.com/read/28632699/inadvertent-arterial-injection-of-123i-mibg-does-not-necessarily-cause-any-symptoms
#4
Wei Zhang, Hongming Zhuang
An I-MIBG scan was obtained to evaluate a right adrenal mass in a 10-year-old boy, which showed no elevated activity in the lesion. However, diffuse increased activity was noted in the right forearm with typical manifestation of an "arterial injection." Different from a prior case report, no symptoms or signs related to arterial injection were observed in our patient.
June 19, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28625940/prodromal-stage-of-disease-dementia-with-lewy-bodies-how-to-diagnose-in-practice
#5
Frédéric Blanc, Marc Verny
Disease with Lewy bodies or dementia with Lewy bodies (DLB), particularly at the prodromal stage, is a complex disease to diagnose because of different clinical beginnings and variable paths in terms of clinical expression. Thus DLB can be entcountered in different input modes: mild cognitive impairment, depression, acute behavioral disorders, confusion and delirium, or sleep disorders. In the aim to better diagnose the disease, should be sought obviously to search for the key symptoms: fluctuations, hallucinations, extra-pyramidal syndrome, and REM sleep behavior disorder...
June 1, 2017: Gériatrie et Psychologie Neuropsychiatrie du Vieillissement
https://www.readbyqxmd.com/read/28605448/efficacy-of-peptide-receptor-radionuclide-therapy-prrt-for-functional-metastatic-paraganglioma-and-phaeochromocytoma
#6
Grace Kong, Simona Grozinsky-Glasberg, Michael S Hofman, Jason Callahan, Amichay Meirovitz, Ofra Maimon, David A Pattison, David J Gross, Rodney J Hicks
Purpose: Treatment options for unresectable Paraganglioma (PGL)/Phaeochromocytoma (PCC), especially with uncontrolled secondary hypertension (HTN) are limited. Preliminary studies with PRRT suggest efficacy but data on HTN control and survival are lacking. We assessed PRRT outcomes in such patients from 2 referral centres. Methods: 20 consecutive patients (M:F=13:7; 21-77y.o.) with high somatostatin receptor (SSTR) expression treated with 177Lu-DOTA-octreotate, 9 with radiosensitising chemotherapy, were retrospectively reviewed...
June 9, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28603516/iodine-123-meta-iodobenzylguanidine-myocardial-scintigraphy-in-isolated-autonomic-failure-potential-red-flag-for-future-multiple-system-atrophy
#7
Francesca Baschieri, Giovanna Calandra-Buonaura, Annagrazia Cecere, Giorgio Barletta, Manuela Contin, Piero Parchi, Pietro Cortelli
Pure autonomic failure is challenging as it can be the presenting feature of a central nervous system syncleinopathy such as Parkinson's disease (PD) or multiple system atrophy (MSA). Because the prognosis of MSA and PD is so different, predictive features for a possible conversion can be extremely valuable. In this paper, we report three cases (two with autopsy-proven diagnosis) that had isolated AF for many years before converting to MSA or PD. Of all the tests that were performed during the premotor stage, Iodine-123-meta-iodobenzylguanidine (MIBG) myocardial scintigraphy was predictive of the conversion to MSA...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28596948/standardization-of-123-i-meta-iodobenzylguanidine-myocardial-sympathetic-activity-imaging-phantom-calibration-and-clinical-applications
#8
REVIEW
Kenichi Nakajima, Derk O Verschure, Koichi Okuda, Hein J Verberne
PURPOSE: Myocardial sympathetic imaging with (123)I-meta-iodobenzylguanidine ((123)I-mIBG) has gained clinical momentum. Although the need for standardization of (123)I-mIBG myocardial uptake has been recognized, the availability of practical clinical standardization approaches is limited. The need for standardization includes the heart-to-mediastinum ratio (HMR) and washout rate with planar imaging, and myocardial defect scoring with single-photon emission computed tomography (SPECT)...
2017: Clinical and Translational Imaging: Reviews in Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28595752/improved-outcome-of-131-i-mibg-treatment-through-combination-with-external-beam-radiotherapy-in-the-sk-n-sh-mouse-model-of-neuroblastoma
#9
Aurélien Corroyer-Dulmont, Nadia Falzone, Veerle Kersemans, James Thompson, Danny P Allen, Sarah Able, Christiana Kartsonaki, Javian Malcolm, Paul Kinchesh, Mark A Hill, Boris Vojnovic, Sean C Smart, Mark N Gaze, Katherine A Vallis
PURPOSE: To assess the efficacy of different schedules for combining external beam radiotherapy (EBRT) with molecular radiotherapy (MRT) using (131)I-mIBG in the management of neuroblastoma. MATERIALS AND METHODS: BALB/c nu/nu mice bearing SK-N-SH neuroblastoma xenografts were assigned to five treatment groups: (131)I-mIBG 24h after EBRT, EBRT 6days after (131)I-mIBG, EBRT alone, (131)I-mIBG alone and control (untreated). A total of 56 mice were assigned to 3 studies...
June 5, 2017: Radiotherapy and Oncology: Journal of the European Society for Therapeutic Radiology and Oncology
https://www.readbyqxmd.com/read/28592453/diagnosis-and-management-of-dementia-with-lewy-bodies-fourth-consensus-report-of-the-dlb-consortium
#10
REVIEW
Ian G McKeith, Bradley F Boeve, Dennis W Dickson, Glenda Halliday, John-Paul Taylor, Daniel Weintraub, Dag Aarsland, James Galvin, Johannes Attems, Clive G Ballard, Ashley Bayston, Thomas G Beach, Frédéric Blanc, Nicolaas Bohnen, Laura Bonanni, Jose Bras, Patrick Brundin, David Burn, Alice Chen-Plotkin, John E Duda, Omar El-Agnaf, Howard Feldman, Tanis J Ferman, Dominic Ffytche, Hiroshige Fujishiro, Douglas Galasko, Jennifer G Goldman, Stephen N Gomperts, Neill R Graff-Radford, Lawrence S Honig, Alex Iranzo, Kejal Kantarci, Daniel Kaufer, Walter Kukull, Virginia M Y Lee, James B Leverenz, Simon Lewis, Carol Lippa, Angela Lunde, Mario Masellis, Eliezer Masliah, Pamela McLean, Brit Mollenhauer, Thomas J Montine, Emilio Moreno, Etsuro Mori, Melissa Murray, John T O'Brien, Sotoshi Orimo, Ronald B Postuma, Shankar Ramaswamy, Owen A Ross, David P Salmon, Andrew Singleton, Angela Taylor, Alan Thomas, Pietro Tiraboschi, Jon B Toledo, John Q Trojanowski, Debby Tsuang, Zuzana Walker, Masahito Yamada, Kenji Kosaka
The Dementia with Lewy Bodies (DLB) Consortium has refined its recommendations about the clinical and pathologic diagnosis of DLB, updating the previous report, which has been in widespread use for the last decade. The revised DLB consensus criteria now distinguish clearly between clinical features and diagnostic biomarkers, and give guidance about optimal methods to establish and interpret these. Substantial new information has been incorporated about previously reported aspects of DLB, with increased diagnostic weighting given to REM sleep behavior disorder and (123)iodine-metaiodobenzylguanidine (MIBG) myocardial scintigraphy...
June 7, 2017: Neurology
https://www.readbyqxmd.com/read/28585398/genetic-status-determines-18-f-fdg-uptake-in-pheochromocytoma-paraganglioma
#11
Ankita Tiwari, Nalini Shah, Vijaya Sarathi, Gaurav Malhotra, Ganesh Bakshi, Gagan Prakash, Kranti Khadilkar, Reshma Pandit, Anurag Lila, Tushar Bandgar
INTRODUCTION: Although few studies have demonstrated utility of (18) F- fluoro-2-deoxy-d-glucose positron emission tomography/computerised tomography ((18) F-FDG PET/CT) in benign pheochromocytoma/paragangliomas (PCC/PGLs), there limited data on factors predicting the FDG uptake in PCC/PGL. METHODS: The study was conducted at a tertiary health care centre. In addition to the routine investigations, all patients (n = 96) with PCC/PGL were evaluated with (18) F-FDGPET/CT and majority (n = 78) underwent (131) I-metaiodobenzyl guanidine ((131) I-MIBG) scintigraphy...
June 5, 2017: Journal of Medical Imaging and Radiation Oncology
https://www.readbyqxmd.com/read/28567294/pheochromocytomatosis-associated-with-a-novel-tmem127-mutation
#12
Run Yu, Danielle Sharaga, Christopher Donner, M Fernando Palma Diaz, Masha J Livhits, Michael W Yeh
Pheochromocytomatosis, a very rare form of pheochromocytoma recurrence, refers to new, multiple, and often small pheochromocytomas growing in and around the surgical resection bed of a previous adrenalectomy for a solitary pheochromocytoma. We here report a case of pheochromocytomatosis in a 70-year-old female. At age 64 years, she was diagnosed with a 6-cm right pheochromocytoma. She underwent laparoscopic right adrenalectomy, during which the tumor capsule was ruptured. At age 67 years, CT of abdomen did not detect recurrence...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28566531/sdhb-mutation-carriers-with-malignant-pheochromocytoma-respond-better-to-cvd
#13
Lauren Fishbein, Sivan Ben-Maimon, Stephen Keefe, Keith Cengel, Daniel A Pryma, Arturo Loaiza-Bonilla, Douglas L Fraker, Katherine L Nathanson, Debbie L Cohen
Pheochromocytomas and paragangliomas (PCC/PGL) are tumors in the adrenal medulla and extra adrenal sites, respectively. About a quarter are metastatic, often with a long latency period. Some PCC/PGL are defined as clinically aggressive given extensive local invasion into adjacent normal tissue. These tumors, like metastatic PCC/PGL, cannot be surgically cured and patients can have continued secretion of excessive catecholamines with all related sequelae. For patients with metastatic or aggressive tumors, treatment options are limited...
May 31, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28566259/multimodal-approach-in-radioguided-surgery-in-a-case-of-multiple-paraganglioma
#14
N Sánchez, A Tapias, H Bowles, E Delgado, R Almenara, D Fuster, S Vidal-Sicart
The case involves a 34-year-old who underwent surgical removal of a retroperitoneal paraganglioma adjacent to the left kidney lower pole, and visualized by CT and MRI. The (123)I-MIBG scan was positive for this lesion and, in addition, another more caudal uptake, of smaller size and intensity, was observed at L5 level. It was not considered for removal, due to its lack of morphological definition. One week after the surgery, the patient presented with difficult-to-control high blood pressure. A second (123)I-MIBG scan was performed...
May 26, 2017: Revista Española de Medicina Nuclear e Imagen Molecular
https://www.readbyqxmd.com/read/28559940/clinical-significance-of-pretreatment-fdg-pet-ct-in-mibg-avid-pediatric-neuroblastoma
#15
Seo Young Kang, Muhammad Kashif Rahim, Yong-Il Kim, Gi Jeong Cheon, Hyoung Jin Kang, Hee Young Shin, Keon Wook Kang, June-Key Chung, E Edmund Kim, Dong Soo Lee
PURPOSE: (18)F-fluorodeoxyglucose-positron emission tomography (FDG-PET) imaging is well known to have clinical significance in the initial staging and response evaluation of the many kinds of neoplasms. However, its role in the pediatric neuroblastoma is not clearly defined. In the present study, the clinical significance of FDG-PET/computed tomography (CT) in (123)I- or (131)I-metaiodobenzylguanidine (MIBG)-avid pediatric neuroblastoma was investigated. METHODS: Twenty patients with neuroblastoma who undertook pretreatment FDG PET/CT at our institute between 2008 and 2015 and showed MIBG avidity were retrospectively enrolled in the present study...
June 2017: Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28559462/expression-of-five-neuroblastoma-genes-in-bone-marrow-or-blood-of-patients-with-relapsed-refractory-neuroblastoma-provides-a-new-biomarker-for-disease-and-prognosis
#16
Araz Marachelian, Judith G Villablanca, Cathy W Liu, Betty Liu, Fariba Goodarzian, Hollie A Lai, Hiroyuki Shimada, Hung C Tran, Jaime A Parra, Richard Gallego, Nora Bedrossian, Sabrina Young, Scarlett Czarnecki, Rebekah Kennedy, Brian Weiss, Kelly C Goldsmith, Meaghan Granger, Katherine K Matthay, Susan Groshen, Shahab Asgharzadeh, Richard Sposto, Robert C Seeger
We determined if quantifying neuroblastoma (NB)-associated mRNAs (NB-mRNA) in bone marrow (BM) and blood improves assessment of disease and prediction of disease progression in patients with relapsed/refractory NB. <br /><br />Patients and Methods: mRNA for CHGA, DCX, DDC, PHOX2B, and TH was quantified in BM and blood from 101 patients concurrently with clinical disease evaluations. Correlation between NB-mRNA (delta cycle threshold [ΔCt] for the geometric mean of genes from the TaqMan® Low Density Array NB5 assay) and morphologically-defined tumor cell percentage in BM, (123)I-meta-iodobenzylguanidine (MIBG) Curie score, and CT/MRI-defined tumor longest diameter was determined...
May 30, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28553188/incidental-detection-of-synchronous-medullary-thyroid-carcinoma-with-bilateral-adrenal-pheochromocytoma-on-iodine-123-metaiodobenzylguanidine-scintigraphy-leading-to-diagnosis-of-multiple-endocrine-neoplasia-2a
#17
Asif Ali Fakhri, Paul David Rodrigue, Mustafa Aladin, Aun Hussain
We report a case of a 29-year-old female with the family history of medullary thyroid carcinoma (MTC) presenting with hematuria and tachycardia, who was found to have bilateral adrenal masses on abdominal computed tomography and biochemical testing compatible with pheochromocytoma. Iodine-123 (I-123) metaiodobenzylguanidine (MIBG) scintigraphy for preoperative planning prior to planned adrenalectomy revealed incidental synchronous unifocal MTC, along with expected bilateral adrenal pheochromocytomas. Pathology confirmed these findings, and subsequent genetic testing confirmed a rearranged during transfection proto-oncogene mutation on exon 11, confirming the clinical diagnosis of multiple endocrine neoplasia 2A (MEN 2A)...
April 2017: World Journal of Nuclear Medicine
https://www.readbyqxmd.com/read/28552865/new-development-of-diagnosis-and-treatment-for-parkinson-s-disease
#18
Satoshi Orimo
New methods for the diagnosis and new treatments for Parkinson's disease (PD) were explained. As imaging tools, neuromelanin imaging using brain MRI, meta-iodobenzylguanidine (MIBG) cardiac scintigraphy, dopamine transporter scintigraphy, and transcranial sonography were introduced. Olfactory dysfunction and REM sleep behavior disorders (RBD), which are important non-motor symptoms, and the new Clinical Criteria for PD launched by Movement Disorder Society (MDS) were also described. Investigative new medications and new anti-PD medications, which recently became available in Japan, were introduced...
May 26, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28538394/123i-ioflupane-brain-spect-and-123i-mibg-cardiac-planar-scintigraphy-combined-use-in-uncertain-parkinsonian-disorders
#19
Susanna Nuvoli, Angela Spanu, Maria Rita Piras, Antonio Nieddu, Aldo Mulas, Gaia Rocchitta, Grazia Galleri, Pier Andrea Serra, Giuseppe Madeddu
We evaluated the clinical usefulness of the combined use of I-ioflupane brain single photon emission computed tomography (SPECT) and I-metaiodobenzylguanidine (MIBG) cardiac scintigraphy in discriminating uncertain parkinsonism with vascular lesions in striatal nuclei at magnetic resonance imaging (MRI). Forty-three consecutive patients with uncertain parkinsonism and vascular lesions at MRI in striatal nuclei were retrospectively evaluated; the uncertain differential diagnosis was between Parkinson's disease and vascular parkinsonism (PD/VP) in 22 patients, between PD and other neurodegenerative parkinsonism (PD/PS) in 11 patients and between Lewy body dementia and Alzheimer disease (LBD/AD) in the remaining 10 cases...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28525458/unusual-presentation-of-relapse-in-neuroblastoma-pancreatic-metastases-detected-by-123i-mibg-scintigraphy
#20
Maria Felicia Villani, Maria Luisa D'Andrea, Aurora Castellano, Milena Pizzoferro, Maria Carmen Garganese
Neuroblastoma is the most common extracranial solid tumor in children and accounts for 8% to 10% of all childhood cancers. The spread of metastases is both lymphatic and hematogenous. Hematogenous route occurs most commonly in bone marrow and cortical bone; pancreatic metastases are very rare, and there are few reports about this topic in medical literature. We report the case of a 14-year-old boy who suffers a third relapse of neuroblastoma: in this patient I-MIBG confirms that lesions detected by CT were due to a relapse in pancreatic tissue and influenced subsequent therapeutic management...
May 19, 2017: Clinical Nuclear Medicine
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