gallium-ga-68-dotatate and Paraganglioma

Neuroectodermal tumours arise from chromaffin cells and possess the ability to secrete catecholamines. They are generally rare and may occur in association with a variety of hereditary syndromes such as MEN-2A and 2B, neurofibromatosis type 1 and von Hippel-Lindau disease. The most common types are phaeochromocytoma arising from the adrenal medulla and paraganglioma of extra-adrenal origin. Phaeochromocytomas tend to be benign and are often associated with a gene mutation if the disease is bilateral, while paragangliomas are often malignant, have a more aggressive nature and tend to metastasize. There are no specific histological or immunohistochemical features that indicate the malignant potential and the diagnosis of malignancy can only be established by the presence of distant metastases. Therefore, imaging can play a vital role in the diagnosis, localization, staging and assessment of spread. Traditionally, this is achieved with a combination of cross-sectional (CT and MRI) and functional ((123)I-MIBG or (111)In-octreotide) imaging. However, these modalities are not adequate and achieve moderate sensitivity. The introduction of (68)Ga-DOTA peptide in PET/CT imaging has led to improved receptor targeting and superb PET resolution, as well as accurate localization of lesions. The use of this technique in neuroectodermal tumours has been shown to be superior to all available modalities, but the available data are limited and larger studies are awaited to establish its role in the management of these tumours.

Topics: Female; Gallium Radioisotopes; Humans; Male; Multimodal Imaging; Neuroectodermal Tumors; Organometallic Compounds; Paraganglioma; Peptides; Pheochromocytoma; Positron-Emission Tomography; Radiopharmaceuticals; Tomography, X-Ray Computed

Pheochromocytomas/paragangliomas (PHEOs/PGLs) overexpress somatostatin receptors and recent studies have already shown excellent results in the localization of these tumors using (68)Ga-labeled somatostatin analogs ((68)Ga-DOTA-SSA), especially in patients with germline succinate dehydrogenase subunit B gene (SDHB) mutations and head and neck PGLs (HNPGLs). The value of (68)Ga-DOTA-SSA has to be established in sporadic cases, including PHEOs. Thus, the aim of this study was to compare (68)Ga-DOTATATE PET/CT, (18)F-FDOPA PET/CT, and conventional imaging in patients with various PHEOs/PGLs with a special emphasis on sporadic cases, including those located in the adrenal gland.. (68)Ga-DOTATATE, (18)F-FDOPA PET/CT, and conventional imaging (contrast-enhanced CT and MRI with MR angiography sequences) were prospectively performed in 30 patients (8 with SDHD mutations, 1 with a MAX mutation and 21 sporadic cases) with PHEO/PGL at initial diagnosis or relapse.. The patient-based sensitivities were 93 % (28/30), 97 % (29/30), and 93 % (28/30) for (68)Ga-DOTATATE PET/CT, (18)F-FDOPA PET/CT, and conventional imaging, respectively. The lesion-based sensitivities were 93 % (43/46), 89 % (41/46), and 76 % (35/46) for (68)Ga-DOTATATE PET/CT, (18)F-FDOPA PET/CT, and conventional imaging respectively (p = 0.042). (68)Ga-DOTATATE PET/CT detected a higher number of HNPGLs (30/30) than (18)F-FDOPA PET/CT (26/30; p = 0.112) and conventional imaging (24/30; p = 0.024). (68)Ga-DOTATATE PET/CT missed two PHEOs of a few millimeters in size and a large recurrent PHEO. One lesion was considered false-positive on (68)Ga-DOTATATE PET/CT and corresponded to a typical focal lesion of fibrous dysplasia on MRI. Among the 11 lesions missed by conventional imaging, 7 were detected by conventional imaging with knowledge of the PET results (4 HNPGLs, 2 LNs, and 1 recurrent PHEO).. (68)Ga-DOTATATE PET/CT is the most sensitive tool in the detection of HNPGLs, especially SDHD-related tumors, which may be very small and fail to concentrate sufficient (18)F-FDOPA. The present study further expands the use of (68)Ga-DOTATATE for all patients with HNPGLs, regardless of their genotype. (68)Ga-DOTATATE PET/CT may be inferior to (18)F-FDOPA PET/CT in the detection PHEOs.

Topics: Adrenal Gland Neoplasms; Adult; Aged; Aged, 80 and over; Dihydroxyphenylalanine; False Positive Reactions; Female; Humans; Image Processing, Computer-Assisted; Male; Middle Aged; Organometallic Compounds; Paraganglioma; Pheochromocytoma; Positron Emission Tomography Computed Tomography; Prospective Studies; Young Adult

Somatostatin receptor (SSTR) imaging with 68Ga-labeled somatostatin analogs has been used for many tumors with high SSTR expression. Increased uptake mostly depends on the SSTR status of tumors; however, false-positive uptake can be demonstrated in benign diseases or other malignancies because of the increased SSTR expression. We present a case of a 50-year-old man with increased SSTR activity on cervical lesion located in internal jugular chain in 68Ga-DOTATATE PET/CT, which was performed for suspicion of paraganglioma. Biopsy revealed lymph node metastasis of squamous cell carcinoma other than paraganglioma. 18F-FDG PET/CT showed primary malignancy on larynx and lymph node metastasis, which were correlated surgically.

Topics: Biopsy; Carcinoma, Squamous Cell; Diagnosis, Differential; Head and Neck Neoplasms; Humans; Lymphatic Metastasis; Male; Middle Aged; Organometallic Compounds; Paraganglioma; Positron Emission Tomography Computed Tomography

We report a case of an adult male patient with multifocal urinary bladder paragangliomas, which were negative on Ga-DOTATATE PET/CT scan, but positive on I-MIBG SPECT/CT scan. While the Ga-DOTA analog PET/CT exhibits superior performance in diagnosis and staging of pheochromocytoma and paraganglioma, our case demonstrates negative somatostatin receptor expression in this rare entity and indicates that I-MIBG SPECT/CT still plays a vital role in characterization of bladder paraganglioma.

Topics: 3-Iodobenzylguanidine; Biological Transport; Humans; Male; Middle Aged; Organometallic Compounds; Paraganglioma; Positron Emission Tomography Computed Tomography; Receptors, Somatostatin; Urinary Bladder Neoplasms

Paragangliomas are rare tumours of neural crest origin that express high levels of somatostatin receptor. Ga-68 DOTATATE PET/CT is a widely accepted method for imaging of neuroendocrine tumours. This study was performed to review a Ga-68 DOTATATE PET/CT patient database and to establish the impact of the modality on patient treatment.. Demographic data, imaging data and change in management after Ga-68 DOTATATE PET/CT were evaluated.. Ga-68 DOTATATE PET/CT scans were performed in 21 patients in whom paragangliomas had been confirmed after biopsy or surgery and in one patient with suspected paraganglioma. In most patients, the primary site was the organ of Zuckerkandl (12/22). Of the 22 Ga-68 DOTATATE PET/CT scans completed, 19 (86.4%) were positive and three (13.6%) negative. In 12 of 14 recurrent cases (90.9%), the treatment plan was changed after the Ga-68 DOTATATE PET/CT scan owing to new, unexpected findings, while it remained unchanged in two (9.1%). Regarding the change in treatment plan, in most instances the new treatment comprised peptide receptor radionuclide therapy (PRRT).. Ga-68 DOTATATE PET/CT findings led to a change in the scheduled treatment plan in 90.9% of patients with suspected recurrence. The most frequent change consisted in initiation of PRRT due to disease recurrence or progression or detection of multiple metastases.

Topics: Adolescent; Adult; Aged; Biopsy; Female; Humans; Male; Middle Aged; Organometallic Compounds; Paraganglioma; Positron Emission Tomography Computed Tomography; Retrospective Studies; Young Adult

After dedicated CT and MRI, Ga-DOTATATE PET/CT was performed in a patient with a temporal bone mass with primary diagnostic considerations of an endolymphatic sac tumor versus a glomus jugulotympanicum paraganglioma. The Ga-DOTATATE PET showed mild radiotracer uptake in the mass (SUVmax, 10.9). After surgical resection, pathology revealed an endolymphatic sac tumor. Immunohistochemical staining demonstrated somatostatin receptor type 2A expression in the vasculature of the mass, but not in the tumor cells.

Topics: Adult; Aged; Diagnosis, Differential; Ear Neoplasms; Endolymphatic Sac; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Organometallic Compounds; Paraganglioma; Positron Emission Tomography Computed Tomography; Receptors, Somatostatin

Neuroendocrine tumors (NETs) express somatostatin receptors, which can be targeted with radiolabeled peptides. In a variety of solid tumors, radioguided surgery (RGS) has been used to guide surgical resection. Gallium 68 (68Ga) dota peptides have been shown to be more accurate than other radioisotopes for detecting NETs. A pilot study previously demonstrated the feasibility and safety of 68Ga-dotatate RGS for patients with NETs.. To evaluate what intraoperative techniques and thresholds define positive lesions that warrant resection during 68Ga-dotatate RGS.. This prospective cohort study, conducted between October 23, 2013, and February 14, 2018, included 44 patients with NETs who underwent 68Ga-dotatate RGS.. Gallium 68-dotatate RGS.. The in vivo and ex vivo tumor to background ratio (TBR) was assessed for resected lesions and correlated with the histopathologic findings.. Forty-four patients (22 women and 22 men; mean [SD] age, 51.0 [13.7] years) had 133 lesions detected on preoperative imaging scans, with a diagnosis of a pancreatic NET (19 of 44 [43%]), gastrointestinal NET (22 of 44 [50%]), and pheochromocytoma or paraganglioma (3 of 44 [7%]). The TBR was obtained by normalizing to the omentum (106 of 133 [79.7%]) or other solid organs (27 of 133 [20.3%]). The omentum had a significantly lower mean (SD) count than other solid organs for background count activity 3 hours after injection (22.1 [17.0] vs 34.5 [39.0]; P < .001). The lesions containing NETs had a higher TBR than those that did not contain NETs (18.9 vs 4.4; P < .001). On a receiver operating characteristic curve analysis, a TBR of 2.5 had a sensitivity of 90% and a specificity of 25%, and a TBR of 16 had a sensitivity of 54% and a specificity of 81%.. A TBR of 2.5 or greater is a highly sensitive threshold for indicating a lesion to be consistent with a NET on histologic findings and thus warranting surgical resection. The omentum should be used as the background count activity for 68Ga-dotatate RGS for patients with abdominal NETs.

Topics: Female; Gastrointestinal Neoplasms; Humans; Male; Middle Aged; Neuroendocrine Tumors; Organometallic Compounds; Pancreatic Neoplasms; Paraganglioma; Pheochromocytoma; Positron Emission Tomography Computed Tomography; Positron-Emission Tomography; Prospective Studies; Radiopharmaceuticals; Surgery, Computer-Assisted; Treatment Outcome

Germline succinate dehydrogenase (SDHx) mutation carriers, especially SDHB, are at increased risk for malignancy and require life-long surveillance. Current guidelines recommend periodic whole-body MRI imaging. We assessed the incremental value of 68Ga-DOTA-octreotate (GaTate) positron emission tomography (PET)/CT compared with conventional imaging in such patients.. SDHx mutation carriers who had GaTate PET/CT were retrospectively reviewed. Detection of lesions were compared with MRI or CT on a per-patient and per-lesion basis. Proof of lesions were based on histopathology or clinical/imaging follow-up.. Twenty consecutive patients (median age, 46 years; 10 males) were reviewed. Fourteen patients had SDHB, four, SDHD, one SDHC, and one SDHA mutation. Fifteen had prior surgery and/or radiotherapy. Indications for PET/CT were as follows: 7 patients for surveillance for previously treated disease, 9 residual disease, 2 asymptomatic mutation carriers, and 2 for elevated catecholamines. Median time between modalities was 1.5 months.GaTate PET/CT had higher sensitivity and specificity than conventional imaging. On a per-patient basis: PET/CT sensitivity 100%, specificity 100%; MRI/CT 85% and 50%. Per-lesion basis: PET/CT sensitivity 100%, specificity 75%; MRI/CT 80% and 25%. PET/CT correctly identified additional small nodal and osseous lesions. MRI/CT had more false-positive findings. Change of management resulted in 40% (8/20 patients): 3 received localized treatment instead of observation, 1 changed to observation given extra disease detected, 4 with metastases had radionuclide therapy.. GaTate PET/CT provided incremental diagnostic information with consequent management impact in SDHx-pheochromocytoma and paraganglioma. Incorporating this modality as part of a surveillance program seems prudent. Further research is needed to define the optimal surveillance strategy including use of MRI.

Topics: Adolescent; Adrenal Gland Neoplasms; Adult; Aged; Catecholamines; False Positive Reactions; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Multimodal Imaging; Mutation; Neoplasm Metastasis; Organometallic Compounds; Paraganglioma; Pheochromocytoma; Positron Emission Tomography Computed Tomography; Radiopharmaceuticals; Retrospective Studies; Sensitivity and Specificity; Treatment Outcome; Young Adult

The aim of this study was to present our experience in the baseline evaluation of carotid body paragangliomas (CBP) with. Five patients (4F, 1M; age 24-73 years) with CBPs who underwent. All patients had unilateral CBP lesion, showed intense

Topics: Adult; Aged; Carotid Body Tumor; Female; Humans; Male; Middle Aged; Organometallic Compounds; Paraganglioma; Positron Emission Tomography Computed Tomography; Retrospective Studies

Anatomical imaging modalities including CT and MRI are the mainstay of evaluation of primary pheochromocytoma or paraganglioma. However, nuclear medicine imaging is frequently necessary to determine the nature of the lesions. The purpose of this investigation is to assess which commonly used nuclear medicine modality might have a better diagnostic value in this clinical setting.. Eight patients who had been suspected of having either primary pheochromocytoma or primary paraganglioma and 1 patient with known pheochromocytoma were included in the analysis. Among the 8 patients without known diagnosis, 7 had been suggested by anatomical imaging modalities, whereas one of them presented with initial negative anatomical imaging interpretation. All of 9 patients underwent Ga-DOTATATA PET/CT, FDG PET/CT, and MIBG SPECT/CT for further evaluation. The imaging findings were compared with postsurgical pathology and follow-up.. Both Ga-DOTATATA PET/CT and MIBG SPECT/CT accurately identified 9 primary tumors, whereas FDG PET/CT showed increased activity in 8 of 9 primary tumors. Both Ga-DOTATATA and FDG PET/CT are able to detect associated extra-adrenal lesions not shown on MIBG study in patients with multiple endocrine neoplasia syndrome.. Ga-DOTATATA PET/CT could be the nuclear medicine imaging choice to evaluate suspected primary pheochromocytoma or paraganglioma, especially in the situation of multiple endocrine neoplasia syndrome.

Topics: 3-Iodobenzylguanidine; Adult; Aged; Aged, 80 and over; Female; Fluorodeoxyglucose F18; Humans; Male; Middle Aged; Organometallic Compounds; Paraganglioma; Pheochromocytoma; Positron Emission Tomography Computed Tomography; Retrospective Studies; Single Photon Emission Computed Tomography Computed Tomography

Topics: Humans; Magnetic Resonance Imaging; Organometallic Compounds; Paraganglioma; Positron Emission Tomography Computed Tomography

A 60-year-old woman was referred to contrast-enhanced CT for evaluation of jugular vein thrombosis incidentally detected by ultrasound. Contrast-enhanced CT showed an enhanced tumor of the right skull base highly suspicious of jugulotympanic paraganglioma. However, the jugular veins showed a nearly symmetric contrast enhancement without clear evidence of thrombosis. Consecutive Ga-DOTATATE PET/CT depicted high tumor uptake, which comprised the entire internal jugular vein. Endovascular growth of paraganglioma might be missed on contrast-enhanced CT because of high vascularization of the lesion. Ga-DOTATATE PET is suited for accurate determination of tumor extent.

Topics: Female; Glomus Jugulare Tumor; Humans; Middle Aged; Organometallic Compounds; Paraganglioma; Positron Emission Tomography Computed Tomography; Radiopharmaceuticals

Pheochromocytomas/paragangliomas are somatostatin receptor 2-overexpressing tumors. Ga-DOTA-peptide imaging has recently shown excellent results in the detection of metastatic lesions in these tumors. However, currently used Ga-DOTA peptides show different somatostatin receptor affinities. Here, we report the remarkable differences in a patient who was imaged with Ga-DOTANOC and Ga-DOTATATE PET/CT within a 7-month period. The patient presented with a nearly negative Ga-DOTANOC PET/CT scan, whereas on Ga-DOTATATE PET/CT, multiple highly positive lesions were identified.

Topics: Abdominal Neoplasms; Child; Female; Humans; Lung Neoplasms; Neoplasm Metastasis; Organometallic Compounds; Paraganglioma; Positron Emission Tomography Computed Tomography; Protein Binding; Radiopharmaceuticals; Receptors, Somatostatin; Succinate Dehydrogenase

Pheochromocytomas/paragangliomas overexpress somatostatin receptors, and recent studies have already shown excellent results in the localization of sympathetic succinate dehydrogenase complex, subunit B, mutation-related metastatic pheochromocytomas/paragangliomas using (68)Ga-DOTATATE PET/CT. Therefore, the goal of our study was to assess the clinical utility of this functional imaging modality in parasympathetic head and neck paragangliomas (HNPGLs) compared with anatomic imaging with CT/MRI and other functional imaging modalities, including (18)F-fluorohydroyphenylalanine ((18)F-FDOPA) PET/CT, currently the gold standard in the functional imaging of HNPGLs.. (68)Ga-DOTATATE PET/CT was prospectively performed in 20 patients with HNPGLs. All patients also underwent (18)F-FDOPA PET/CT, (18)F-FDG PET/CT, and CT/MRI, with 18 patients also undergoing (18)F-fluorodopamine ((18)F-FDA) PET/CT. (18)F-FDOPA PET/CT and CT/MRI served as the imaging comparators.. Thirty-eight lesions in 20 patients were detected, with (18)F-FDOPA PET/CT identifying 37 of 38 and CT/MRI identifying 23 of 38 lesions (P < 0.01). All 38 and an additional 7 lesions (P = 0.016) were detected on (68)Ga-DOTATATE PET/CT. Significantly fewer lesions were identified by (18)F-FDG PET/CT (24/38, P < 0.01) and (18)F-FDA PET/CT (10/34, P < 0.01).. (68)Ga-DOTATATE PET/CT identified more lesions than other imaging modalities. With the results of the present study, and the increasing availability and use of DOTA analogs in the therapy of neuroendocrine tumors, we expect that (68)Ga-DOTATATE PET/CT will become the preferred functional imaging modality for HNPGLs in the near future.

Topics: Adult; Aged; Dihydroxyphenylalanine; Female; Fluorodeoxyglucose F18; Head and Neck Neoplasms; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Multimodal Imaging; Organometallic Compounds; Paraganglioma; Positron-Emission Tomography; Prospective Studies; Radiopharmaceuticals; Succinate Dehydrogenase; Tomography, X-Ray Computed

Pheochromocytomas/paragangliomas (PPGLs) and their metastases are tumors that predominantly express somatostatin receptor 2 (SSR2). (68)Ga-DOTA(0)-Tyr(3)-octreotate ((68)Ga-DOTATATE) is a PET radiopharmaceutical with both high and selective affinity for SSRs. The purpose of this study was to evaluate the utility of (68)Ga-DOTATATE in comparison with other specific and nonspecific radiopharmaceuticals recommended in the current guidelines for the localization of metastatic sporadic PPGL by PET/CT.. This prospective study included 22 patients (15 men, 7 women; aged 50.0 ± 13.9 years) with confirmed metastatic PPGL, a negative family history for PPGL, and negative genetic testing, who underwent (68)Ga-DOTATATE, (18)F-fluoro-2-deoxy-D-glucose ((18)F-FDG) PET/CT, and CT/MRI. Only 12 patients underwent an additional (18)F-fluorodihydroxyphenylalanine ((18)F-FDOPA) PET/CT scan and only 11 patients underwent an additional (18)F-fluorodopamine ((18)F-FDA) PET/CT scan. The rates of detection of metastatic lesions were compared among all the imaging studies. A composite of all functional and anatomical imaging studies served as the imaging comparator.. (68)Ga-DOTATATE PET/CT showed a lesion-based detection rate of 97.6 % (95 % confidence interval, CI, 95.8 - 98.7 %). (18)F-FDG PET/CT, (18)F-FDOPA PET/CT, (18)F-FDA PET/CT, and CT/MRI showed detection rates of 49.2 % (CI 44.5 - 53.6 %; p < 0.01), 74.8 % (CI 69.0 - 79.9 %); p < 0.01), 77.7 % (CI 71.5 - 82.8 %; p < 0.01), and 81.6 % (CI 77.8 - 84.8 %; p < 0.01), respectively.. The results of this study demonstrate the superiority of (68)Ga-DOTATATE PET/CT in the localization of sporadic metastatic PPGLs compared to all other functional and anatomical imaging modalities, and suggest modification of future guidelines towards this new imaging modality.

Topics: Adult; Aged; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Neoplasm Staging; Organometallic Compounds; Paraganglioma; Positron Emission Tomography Computed Tomography; Radiopharmaceuticals; Reproducibility of Results; Sensitivity and Specificity; Tomography, X-Ray Computed; Young Adult

Topics: Abdominal Neoplasms; Carcinoma, Squamous Cell; Diagnosis, Differential; Fluorodeoxyglucose F18; Head and Neck Neoplasms; Humans; Male; Middle Aged; Nasopharyngeal Neoplasms; Neoplasms, Multiple Primary; Organometallic Compounds; Paraganglioma; Positron Emission Tomography Computed Tomography; Radiopharmaceuticals; Squamous Cell Carcinoma of Head and Neck; Tissue Distribution

Pheochromocytomas (PCC) and paragangliomas (PGL) are neuroendocrine tumours arising from pluripotent neural crest stem cells and are associated with neurons of the autonomic nervous system. PCCs/PGLs are often hereditary and multifocal, and their biologic behaviour and metabolic activity vary making imaging of these tumours challenging. The imaging gold standard has been I-123 MIBG complemented by CT or MRI. PGLs being neuroendocrine tumours express somatostatin receptors enabling imaging with Ga-68 DOTA-coupled peptides such as DOTATATE. Imaging with F-18 FDG also provides additional information regarding metabolic activity and biologic aggressiveness of these tumours, or, in some situations, reflecting metabolic reprogramming of these tumours. We report our experience using both Ga-68 DOTATATE and F-18 FDG PET/CT imaging in patients with PGLs and PCCs.. This was a retrospective review of 23 patients with proven PGL/PCC who underwent both DOTATATE and FDG PET/CT. Seven patients also had I-123 MIBG SPECT/CT and 1 patient had I-124 MIBG PET/CT. Lesional intensity and patterns of uptake were analysed.. DOTATATE and FDG were positive at most sites of disease (96.2 % vs 91.4 %), although uptake intensity was significantly higher on DOTATATE with a median SUV of 21 compared to 12.5 for FDG (p < 0.001). SUVmax on F-18 FDG was significantly higher (p < 0.001) in clinically aggressive cases. I-123/I-124 MIBG detected fewer lesions (30.4 %).. Overall, Ga-68 DOTATATE PET/CT detected similar number but has significantly greater lesion-to-background contrast compared to F-18 FDG PET/CT. Combined with high specificity, patient convenience and relatively low cost, DOTATATE PET/CT should be considered the ideal first line investigation for imaging PGL/PCC. Depending on DOTATATE findings and the clinical question, FDG and MIBG remain useful and, in selected cases, may provide more accurate staging, disease characterisation and guide treatment choices.

Topics: Adrenal Gland Neoplasms; Adult; Aged; Female; Fluorodeoxyglucose F18; Humans; Male; Middle Aged; Organometallic Compounds; Paraganglioma; Pheochromocytoma; Positron Emission Tomography Computed Tomography; Radiopharmaceuticals; Sensitivity and Specificity

Pancreatic neuroendocrine tumors (PNETs) represent a small percentage of all pancreatic malignancies, and most of these present as metastatic disease. Somatostatin receptor scintigraphy had been used successfully for the assessment of patients with NET. Somatostatin receptor scintigraphy is indispensable for localization of ectopic NET and the distribution of NET throughout the body.

Topics: Female; Humans; Male; Middle Aged; Multimodal Imaging; Neoplasm Metastasis; Organometallic Compounds; Pancreatic Neoplasms; Paraganglioma; Positron-Emission Tomography; Radiopharmaceuticals; Tomography, X-Ray Computed

Patients with succinate dehydrogenase subunit B(SDHB) mutation-related pheochromocytoma/paraganglioma (PHEO/PGL) are at a higher risk for metastatic disease than other hereditary PHEOs/PGLs. Current therapeutic approaches are limited, but the best outcomes are based on the early and proper detection of as many lesions as possible. Because PHEOs/PGLs overexpress somatostatin receptor 2 (SSTR2), the goal of our study was to assess the clinical utility of [(68)Ga]-DOTA(0)-Tyr(3)-octreotate ([(68)Ga]-DOTATATE) positron emission tomography/computed tomography (PET/CT) and to evaluate its diagnostic utility in comparison with the currently recommended functional imaging modalities [(18)F]-fluorodopamine ([(18)F]-FDA), [(18)F]-fluorodihydroxyphenylalanine ([(18)F]-FDOPA), [(18)F]-fluoro-2-deoxy-d-glucose ([(18)F]- FDG) PET/CT as well as CT/MRI.. [(68)Ga]-DOTATATE PET/CT was prospectively performed in 17 patients with SDHB-related metastatic PHEOs/PGLs. All patients also underwent [(18)F]-FDG PET/CT and CT/MRI, with 16 of the 17 patients also receiving [(18)F]-FDOPA and [(18)F]-FDA PET/CT scans. Detection rates of metastatic lesions were compared between all these functional imaging studies. A composite synthesis of all used functional and anatomical imaging studies served as the imaging comparator.. [(68)Ga]-DOTATATE PET/CT demonstrated a lesion-based detection rate of 98.6% [95% confidence interval (CI), 96.5%-99.5%], [(18)F]-FDG, [(18)F]-FDOPA, [(18)F]-FDA PET/CT, and CT/MRI showed detection rates of 85.8% (CI, 81.3%-89.4%; P < 0.01), 61.4% (CI, 55.6%-66.9%; P < 0.01), 51.9% (CI, 46.1%-57.7%; P < 0.01), and 84.8% (CI, 80.0%-88.5%; P < 0.01), respectively.. [(68)Ga]-DOTATATE PET/CT showed a significantly superior detection rate to all other functional and anatomical imaging modalities and may represent the preferred future imaging modality in the evaluation of SDHB-related metastatic PHEO/PGL.

Topics: Adult; Female; Humans; Male; Middle Aged; Mutation; Neoplasm Metastasis; Organometallic Compounds; Paraganglioma; Pheochromocytoma; Positron-Emission Tomography; Radiopharmaceuticals; Sensitivity and Specificity; Succinate Dehydrogenase; Tomography, X-Ray Computed; Young Adult

Paragangliomas of the urinary bladder are very rare. They are extra-adrenal autonomic nervous system tumors. This report presents a case of a 13-year-old boy with urinary bladder paraganglioma and the role of somatostatin receptor imaging with simultaneous positron emission tomography magnetic resonance (PET/MR). We have also described appearances of iodine-123 metaiodobenzylguanidine ((123)I-MIBG) single photon emission computed tomography (SPECT/CT) in comparison to somatostatin receptor imaging.

Topics: 3-Iodobenzylguanidine; Adolescent; Humans; Magnetic Resonance Imaging; Male; Multimodal Imaging; Organometallic Compounds; Paraganglioma; Radiopharmaceuticals; Tomography, Emission-Computed, Single-Photon; Tomography, X-Ray Computed; Urinary Bladder Neoplasms

The coexistence of a pituitary neoplasm and pheochromocytoma is a rare condition, which may be another undefined variant of Multiple endocrine neoplasia (MEN) syndrome. Moreover, the coexistence of pituitary macroadenoma and multiple paragangliomas is more uncommon and only few authors have reported these findings. We are reporting the use of Ga DOTATATE PET/CT in a rare case of coexisting pituitary macroadenoma and multiple paragangliomas.

Topics: Adenoma; Female; Humans; Middle Aged; Multimodal Imaging; Organometallic Compounds; Paraganglioma; Pituitary Neoplasms; Positron-Emission Tomography; Tomography, X-Ray Computed

Topics: Carotid Body; Child; Female; Humans; Magnetic Resonance Imaging; Organometallic Compounds; Paraganglioma; Peripheral Nervous System Neoplasms; Positron-Emission Tomography

To compare the sensitivity of (123)I-metaiodobenzylguanidine (MIBG) SPECT and (68)Ga-DOTATATE PET/CT in detecting phaeochromocytomas (PCC) and paragangliomas (PGL) in the initial diagnosis and follow-up of patients with PCC and PGL disease.. Retrospective analysis of 15 patients with PCC/PGL who had contemporaneous (123)I-MIBG and (68)Ga-DOTATATE imaging.. Of the 15 patients in the series, 8 were concordant with both modalities picking up clinically significant lesions. There were no patients in whom both modalities failed to pick up clinically significant lesions. There was discordance in seven patients: 5 had positive (68)Ga-DOTATATE and negative (123)I-MIBG, and 2 (12 and 14) had negative (68)Ga-DOTATATE and positive (123)I-MIBG. Utilizing (123)I-MIBG as the gold standard, (68)Ga-DOTATATE had a sensitivity of 80 % and a positive predictive value of 62 %. The greatest discordance was in head and neck lesions, with the lesions in 4 patients being picked up by (68)Ga-DOTATATE and missed by (123)I-MIBG. On a per-lesion analysis, cross-sectional (CT and MRI) and (68)Ga-DOTATATE was superior to (123)I-MIBG in detecting lesions in all anatomical locations, and particularly bony lesions.. First, (68)Ga-DOTATATE should be considered as a first-line investigation in patients at high risk of PGL and metastatic disease, such as in the screening of carriers for mutations associated with familial PGL syndromes. Second, if (123)I-MIBG does not detect lesions in patients with a high pretest probability of PCC or PGL, (68)Ga-DOTATATE should be considered as the next investigation. Third, (68)Ga-DOTATATE hould be considered in preference to (123)I-MIBG in patients in whom metastatic spread, particularly to the bone, is suspected.

Topics: 3-Iodobenzylguanidine; Adolescent; Adrenal Gland Neoplasms; Adult; Follow-Up Studies; Humans; Middle Aged; Multimodal Imaging; Organometallic Compounds; Paraganglioma; Pheochromocytoma; Positron-Emission Tomography; Retrospective Studies; Sensitivity and Specificity; Tomography, Emission-Computed, Single-Photon; Tomography, X-Ray Computed; Young Adult

Topics: 3-Iodobenzylguanidine; Adrenal Gland Neoplasms; Humans; Multimodal Imaging; Organometallic Compounds; Paraganglioma; Pheochromocytoma; Positron-Emission Tomography; Tomography, Emission-Computed, Single-Photon; Tomography, X-Ray Computed

We aimed to compare imaging with (123)I-MIBG and (68)Ga-DOTA-TATE in neural crest tumours (NCT) to see if the latter could offer more advantage in detecting extra lesions and have higher sensitivity for malignant lesions.. We retrospectively reviewed 12 patients (M = 10, F = 2; age range 20-71 years) with NCT (phaeochromocytomas = 7, paragangliomas = 4, medullary thyroid cancer = 1) who underwent both (68)Ga-DOTA-TATE positron emission tomography (PET) or PET/computed tomography (CT) and (123)I-MIBG single-photon emission computed tomography within 6 months. Visual assessment of all lesions and measurement of target/non-target (T/N) ratio in selected lesions were performed. Five patients (aged 50 or less) had SDHB screening results correlated with imaging results of both radiopharmaceuticals. All patients had contrast-enhanced CT and/or other cross-sectional imaging.. (68)Ga-DOTA-TATE PET showed tumour lesions in ten out of 12 patients with confirmed disease, while (123)I-MIBG showed lesions in five out of 12 patients. In one patient, both (68)Ga-DOTA-TATE PET and (123)I-MIBG were negative, but CT, magnetic resonance imaging, and 2-deoxy-2-[(18)F]fluoro-D-glucose PET scans identified a lesion in the thorax. (68)Ga-DOTA-TATE and (123)I-MIBG detected a total of 30 lesions, of which 29/30 were positive with (68)Ga-DOTA-TATE and 7/30 with (123)I-MIBG. We also found higher incidence of SDHB positive results in patients with positive (68)Ga-DOTA-TATE.. Our limited data suggest that (68)Ga-DOTA-TATE is a better imaging agent for NCT and detects significantly more lesions with higher T/N ratio compared to (123)I-MIBG. (68)Ga-DOTA-TATE was more likely to detect malignant lesions as indicated by correlating imaging results with SDHB screening.

Topics: 3-Iodobenzylguanidine; Adrenal Gland Neoplasms; Adult; Aged; Female; Humans; Iodine Radioisotopes; Lymphatic Metastasis; Male; Middle Aged; Mutation; Organometallic Compounds; Paraganglioma; Positron-Emission Tomography; Retrospective Studies; Succinate Dehydrogenase; Young Adult

Topics: Adult; Female; Head and Neck Neoplasms; Humans; Organometallic Compounds; Paraganglioma; Positron-Emission Tomography; Tomography, X-Ray Computed

Topics: Abdominal Neoplasms; Feasibility Studies; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Organometallic Compounds; Paraganglioma; Positron-Emission Tomography; Radiopharmaceuticals; Soft Tissue Neoplasms