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Ingrid Mak, Aimee R Hayes, Bernard Khoo, Ashley Grossman
<br>At present, there is no clinical guideline or standardised protocol for the treatment of metastatic or invasive phaeochromocytoma and paraganglioma (collectively known as PPGL) due to the rarity of the disease and the lack of prospective studies or extended national databases. Prognosis is mainly determined by genetic predisposition, tumour burden, rate of disease progression and location of metastases. For patients with progressive or symptomatic disease that is not amenable to surgery, there are various palliative treatment options available...
March 12, 2019: Neuroendocrinology
Abhishek Jha, Kristine de Luna, Charlene Ann Balili, Corina Millo, Cecilia Angela Paraiso, Alexander Ling, Melissa K Gonzales, Bruna Viana, Rami Alrezk, Karen T Adams, Isabel Tena, Alice Chen, Jiri Neuzil, Margarita Raygada, Electron Kebebew, David Taieb, M Sue O'Dorisio, Thomas O'Dorisio, Ali Cahid Civelek, Constantine A Stratakis, Leilani Mercado-Asis, Karel Pacak
Background: Pheochromocytoma and paraganglioma (PHEO/PGL) are rare neuroendocrine tumors which may cause potentially life-threatening complications, with about a third of cases found to harbor specific gene mutations. Thus, early diagnosis, treatment, and meticulous monitoring are of utmost importance. Because of low incidence of succinate dehydrogenase complex subunit A ( SDHA )-related metastatic PHEO/PGL, currently there exists insufficient clinical information, especially with regards to its diagnostic and treatment characteristics...
2019: Frontiers in Oncology
Thomas A Hope, Jérémie Calais, Li Zhang, William Dieckmann, Corina Millo
Purpose: Eligibility for somatostatin receptor (SSTR) radionuclide therapy uses the qualitative Krenning Score based on 111 In-pentetreotide planar scintigraphy as was performed in the NETTER-1 trial. The purpose of this study was to determine the effect of using SSTR-PET-based Krenning Score in comparison to 111 In-pentetreotide. Methods: This was a post-hoc head-to-head comparison of 68 Ga-DOTATATE-based and 111 In-pentetreotide-based Krenning scores in 150 patients included in a prospective phase 2 study (NCT01967537)...
March 8, 2019: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
Naj L Waseem, Carina Mari Aparici, Pamela L Kunz
The diagnosis and subsequent therapy of neuroendocrine neoplasms (NENs) have long-relied on somatostatin receptor (SSTR) expression. The field of theranostics now uses newer SSTR based Positron Emission Tomography (SSTR-PET) imaging with 68 Ga-DOTA0-Tyr3-Octreotate (68 Ga-DOTATATE) or 68 Ga-DOTA0-Tyr3-Octreotide (68 Ga-DOTATOC), as a prerequisite for the administration of peptide receptor radionuclide therapy (PRRT). In the US, 177 Lu-Dotatate, a form of PRRT, gained FDA approval in 2018 for use in gastroenteropancreatic NENs and was based on prolonged progression-free survival (PFS) vs...
March 8, 2019: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
Sadiye Altun Tuzcu, Zafer Pekkolay
AIM: The aim of this study was to evaluate a new imaging method 68Ga-DOTATATE PET/CT as an alternative method to diagnose evidence of neuroendocrine tumors or their metastasis (if any) in patient with MEN 2A. METHODS: Three patients( 2F, 1M; age 28,46 and 50 years) with MEN 2A syndrome who underwent 68Ga-DOTATATE PET/CT scan were prospectively evaluated. PET/CT images were analyzed with measurement of maximum standardized uptake value (SUVmax). RESULTS: All patients had adrenal masses with increased uptake of 68Ga-DOTATATE (first case adrenal mass SUVmax: 9,1, second case adrenal mass SUVmax: 32,4(right), 30,3(left) and third case SUVmax:12,4)...
March 4, 2019: Annali Italiani di Chirurgia
Stephanie Kim, William P Dillon, Thomas A Hope, Ivan H El-Sayed, Annemieke van Zante, Karin Wu, Umesh Masharani
Ectopic thyroid-secreting hormone (TSH)-secreting pituitary adenomas are rare, with only nine cases reported in the literature. We describe a case of an ectopic TSH-secreting pituitary adenoma located in the nasopharynx in a patient initially presenting with signs and symptoms of hyperthyroidism that persisted despite treatment with anti-thyroid medications. Magnetic resonance imaging (MRI) of the pituitary gland was normal, though subsequent review by neuroradiology revealed a nodule attached to the posterior septum in the nasopharynx that was, in retrospect, seen on several other MR scans...
February 21, 2019: World Neurosurgery
Jiangyuan Yu, Nan Li, Jie Li, Ming Lu, Jeffrey P Leal, Huangying Tan, Hua Su, Yang Fan, Yan Zhang, Wei Zhao, Hua Zhu, Martin G Pomper, Yun Zhou, Zhi Yang
PURPOSE: Objectives of the study are to analyze the correlation between [68 Ga]DOTATATE positron emission tomography (PET)/X-ray computed tomography (CT) measurements and various biological characteristics of gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs), and to determine optimal cutoff value of SUVmax (standard uptake value) to differentiate neuroendocrine tumors (NETs) and neuroendocrine cancers (NECs). PROCEDURES: Of the GEP-NEN cases (73 males, 53 females; age 18-77 years) with pathologically proven primary and/or metastatic lesions, 126 were studied...
February 22, 2019: Molecular Imaging and Biology: MIB: the Official Publication of the Academy of Molecular Imaging
Fuad Novruzov, Leyla Mehmetbeyli, Jamil A Aliyev, Bahadur Abbasov, Elnur Mehdi
A 54-year-old man with multiple endocrine neoplasia type 1 had previous history of parathyroid surgery and left thyroid lobectomy 5 years earlier, and was referred for recurrent hypoglycemic episodes. Ga-DOTATATE PET/CT had showed multiple lesions in the right lung, liver, and pancreas. Biopsy from pancreas revealed low-grade neuroendocrine neoplasia. After 2 fractions of Lu-DOTATATE therapy, the size of lesions and its activity reduced on the Ga-DOTATATE scan and the hypoglycemic episodes manifested every day have scaled down to 1 time over 1-year follow-up...
February 15, 2019: Clinical Nuclear Medicine
Jasmine Shell, Amit Tirosh, Corina Millo, Samira M Sadowski, Yasmine Assadipour, Patience Green, Dhaval Patel, Naris Nilubol, Electron Kebebew
PURPOSE: Patients with von Hippel-Lindau (VHL) disease may develop various tumors, including neuroendocrine tumors of the pancreas (PNETs) and adrenal, central nervous system and retinal hemangioblastomas, kidney tumors and more. 68 Ga-DOTATATE positron emission tomography (PET)/computerized tomography (CT) has been shown to be highly accurate for tumors with cells expressing somatostatin receptors. We aimed to assess the performance of 68 Ga-DOTATATE PET/CT in patients with VHL disease...
March 2019: European Journal of Radiology
Frederik A Verburg, Martin Wiessmann, Georg Neuloh, Felix M Mottaghy, Marc-Alexander Brockmann
AIM: Here we describe the results of superselective intraarterial application of diagnostic Ga-68-DOTA0,Tyr3-octreotate (Ga-68-DOTATATE) in a series of patients with inoperable WHO grade II meningiomas which were no longer amenable to radiotherapy. METHODS: Four patients with inoperable WHO grade II meningioma underwent systemic venous infusion of Ga-68-DOTATATE followed by PET/CT. Ga-68-DOTATATE application was repeated intraarterially via transfemoral catheterization of vessels supplying the meningioma and another PET/CT was performed...
February 2019: Nuklearmedizin. Nuclear Medicine
Reto M Kaderli, Marko Spanjol, Attila Kollár, Lukas Bütikofer, Viktoria Gloy, Rebecca A Dumont, Christian A Seiler, Emanuel R Christ, Piotr Radojewski, Matthias Briel, Martin A Walter
Importance: Multiple therapies are currently available for patients with neuroendocrine tumors (NETs), yet many therapies have not been compared head-to-head within randomized clinical trials (RCTs). Objective: To assess the relative safety and efficacy of therapies for NETs. Data Sources: PubMed, Embase, the Cochrane Central Register of Controlled Trials, trial registries, meeting abstracts, and reference lists from January 1, 1947, to March 2, 2018, were searched...
February 14, 2019: JAMA Oncology
Michael Clark, Mark Nathan, Stephen Broski
Carcinoid is a subtype of neuroendocrine tumor, a rare group of tumors that are known to express somatostatin receptor 2. Ga-DOTATATE is a somatostatin analog that is specific for somatostatin receptor 2 and therefore allows visualization of neuroendocrine tumors. We present 2 cases of primary multifocal small bowel carcinoid evaluated using Ga-DOTATATE PET/CT, along with contrast-enhanced CT corollary findings. Given the increased sensitivity of Ga-DOTATATE PET/CT compared with CT and In-octreotide scintigraphy, we expect multifocal carcinoid to become increasingly recognized...
February 8, 2019: Clinical Nuclear Medicine
Amr Mohamed, Jonathan R Strosberg
Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are relatively rare neoplasms, characterized by a propensity to secrete hormones which cause distinct clinical syndromes. During the past decade, the systemic treatment landscape has improved significantly: new options include everolimus, an inhibitor of the mammalian target of rapamycin (mTOR), sunitinib, an angiogenesis inhibitor, and cytotoxic regimens such as capecitabine and temozolomide. Moreover, the recent approval of the radiolabeled somatostatin analog 177 Lutetium(Lu)-dotatate has had a significant impact on management of neuroendocrine malignancies...
February 8, 2019: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
Vijay Alexander, Maria Koshy, Riddhi Dasgupta, Ronald Albert Carey
Cushing's syndrome is known to present with a characteristic set of clinical manifestations and complications, well described in literature. However, hypercoagulability remains an under recognised entity in Cushing's syndrome. A 31-year-old woman from Southern India presented with history of fever, left upper quadrant pain and progressive breathing difficulty for 3 weeks. Clinical examination revealed discriminatory features of Cushing's syndrome. Laboratory investigations showed biochemical features of endogenous ACTH-dependent Cushing's syndrome...
February 6, 2019: BMJ Case Reports
Madhav Prasad Yadav, Sanjana Ballal, Chandrasekhar Bal
BACKGROUND: The role of concomitant peptide receptor radionuclide therapy (PRRT) and capecitabine therapy has shown benefit in gastroenteropancreatic neuroendocrine tumors. However, data reporting its role in paraganglioma (PGL) patients is lacking. The aim of this study was to evaluate the role of combined capecitabine and 177 Lu-DOTATATE in malignant PGL patients. METHODS: In this retrospective, single-institutional, single-arm, observational study, data of consecutive advanced stage PGL patients treated with concomitant 177 Lu-DOTATATE-capecitabine therapy, between July 2009 and March 2017, were collected and analyzed...
February 6, 2019: EJNMMI Research
Halil Komek, Canan Can, Zuhat Urakçi, Ferat Kepenek
OBJECTIVE: This study was designed to compare the 18-Fluorodeoxyglucose positron emission tomography/computed tomography [(F)FDG PET/CT] and 1,4,7,10-tetraazacyclododecane-NI,NII,NIII,NIIII-tetraacetic acid (D)-Phe1-thy3-octreotate [(Ga)DOTATATE PET/CT) findings in patients with pulmonary carcinoid (PC) tumors. MATERIALS AND METHODS: A total of 20 patients [mean (SD) age: 52.2 (12.9) years, 60% women] with PC tumors and available records on digital images of (F)FDG PET/CT and (Ga)DOTATATE PET/CT and histopathological analysis were included in this retrospective single-center study...
January 28, 2019: Nuclear Medicine Communications
Gary A Ulaner, Lisa Bodei
Ga-DOTATATE is a radiolabeled somatostatin analog used for the detection and characterization of somatostatin receptor (SSR)-overexpressing tumors, particularly well-differentiated neuroendocrine tumors. We present a case of a 65-year-old man with well-differentiated pancreatic neuroendocrine tumor post-Whipple surgery and a new liver lesion on CT. Ga-DOTATATE PET/CT was performed for SSR characterization and restaging, which demonstrated the lesion to be intensely SSR positive and interpreted as a neuroendocrine metastasis...
April 2019: Clinical Nuclear Medicine
Taymeyah Al-Toubah, Stefano Partelli, Mauro Cives, Valentina Andreasi, Franco Silvestris, Massimo Falconi, Daniel Anaya Saenz, Jonathan Strosberg
New systemic treatments have improved the therapeutic landscape for patients with metastatic gastroenteropancreatic neuroendocrine tumors (GEP-NETs). While drugs such as everolimus, sunitinib, temozolomide, and 177Lutetium-dotatate are appropriate for patients with widespread disease progression, local treatment approaches may be more appropriate for patients with unifocal progression. Surgical resection, radiofrequency ablation (RFA), hepatic arterial embolization (HAE), or radiation, can control discrete sites of progression, allowing patients to continue their existing therapy, and sparing them toxicities of a new systemic treatment...
January 1, 2019: Endocrine-related Cancer
Jiaqiong Wang
A 79-year-old man had carcinoid syndrome and bilateral pulmonary nodules, which were biopsy confirmed as well-differentiated neuroendocrine tumor. PET/CT with Ga-[DOTA-Tyr]-octreotate (Ga-DOTATATE) showed multiple Ga-DOTATATE-avid bilateral pulmonary nodules, left hilar lymph node, as well as diffuse increased Ga-DOTATATE uptake in the prostate. A review of the history revealed that this patient had elevated prostate-specific antigen level. The prostate biopsy did not reveal evidence of prostate cancer but showed benign prostate hyperplasia...
March 2019: Clinical Nuclear Medicine
Andreas Rinscheid, Jeesoo Lee, Peter Kletting, Ambros J Beer, Gerhard Glatting
AIM: For dosimetry in radioligand therapy, the time-integrated activity coefficients (TIACs) for organs at risk and for tumour lesions have to be determined. The used sampling scheme affects the TIACs and therefore the calculated absorbed doses. The aim of this work was to develop a general and flexible method, which analyses numerous clinically applicable sampling schedules using true time-activity curves (TACs) of virtual patients. METHOD: Nine virtual patients with true TACs of the tumours were created using a physiologically-based pharmacokinetic (PBPK) model and individual biokinetic data of five patients with neuroendocrine tumours and four with meningioma...
January 2, 2019: Zeitschrift Für Medizinische Physik
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