68ga-dotanoc and Paraganglioma

Gallium-68 DOTANOC is a high affinity somatostatin receptor ligand, first introduced in 2005 for imaging neuroendocrine tumors. Due to its technically simple production, broad availability, favourable biodistribution and advantageous dosimetry, although not approved yet in all European countries, gallium-68 DOTANOC has rapidly gained acceptance in the diagnostic and therapeutic work-flow of different types of neuroendocrine tumors. Principal indications in clinical practice in countries where it is officially approved include diagnosis and staging, restaging after treatment, identification of sites of unknown primary and selection of patients with neuroendocrine tumors eligible for therapy with somatostatin analogues.

Topics: Adrenal Gland Neoplasms; Humans; Image Enhancement; Organometallic Compounds; Paraganglioma; Radionuclide Imaging; Radiopharmaceuticals

The primary aim of study was to compare role of iodine-131 (I-131)-labeled metaiodobenzylguanidine ([I]MIBG) and gallium-68 (Ga-68)-labeled DOTA-l-Nal3-octreotide ([Ga]DOTANOC) PET/computed tomography (CT) in patients with pheochromocytoma (PCC) and paraganglioma (PGL), subsequent follow-up to see management. The secondary aim was to see association of germline mutation in histopathologically proven patients.. We performed [Ga]DOTANOC PET/CT and [I]MIBG in 106 patients (61 men; age: 38.5 ± 16.2 years) of known or suspected PCC/PGL. Following scans, 16 histopathologically proven patients were screened for germline mutations.. [I]MIBG detected 41 lesions in 34 patients and [Ga]DOTANOC PET/CT detected more than 79 lesions in 55 patients. The mean duration of follow-up was 20.6 ± 16.5 months. Management following scans: surgery in 35 patients (positive histopathology in 34 patients, negative in 1 patient); lutecium-177 (Lu-177)-labeled DOTA-0-Tyr-3 octreotate ([Lu]DOTATATE) therapy in 2 patients; chemotherapy in 1 patient; conservative therapy in 34 patients; no therapy in 17 patients; 2 patients have died and 3 were lost to follow-up. Among 12 previously operated, 2 patients showed metastatic disease and 1 showed residual disease. Out of 16 patients who underwent genotypic analysis (15 operated), 8 were positive for germline mutations. Mutations were seen in SDHB, RET, VHL, MDH2 and SDHA genes, including two germline mutations in two patients. Deletion was observed in one patient in SDHB gene and substitution in all other mutations. Four novel mutations in MDH2 (c.1005G>C, c.916G>A, c.580G>A) and SDHB (c.378_380delAAT) were observed (SRA accession: PRJNA551457).. [Ga]DOTANOC PET/CT should be considered as a first-line investigation in PCC/PGL especially at high risk of metastasis and screening of persons with familial syndrome.

Topics: 3-Iodobenzylguanidine; Adrenal Gland Neoplasms; Adult; Female; Humans; Male; Middle Aged; Mutation; Organometallic Compounds; Paraganglioma; Pheochromocytoma; Positron Emission Tomography Computed Tomography; Prospective Studies; Young Adult

We present the case of a 33-year-old woman with complaints of headache and palpitations with raised urinary catecholamines. Ultrasound of the abdomen was noncontributory, and the patient was referred for 68Ga-DOTANOC PET/CT, which revealed tracer accumulation in the thecal sac/spinal canal at D5-D7 level, suggestive of a thoracic paraganglioma. MRI of the spine subsequently confirmed the presence of an extradural mass in the spinal canal extending from D4 to D8.

Topics: Adult; Female; Humans; Multimodal Imaging; Organometallic Compounds; Paraganglioma; Positron Emission Tomography Computed Tomography; Positron-Emission Tomography; Tomography, X-Ray Computed

To evaluate Ga-DOTANOC positron emission tomography/computed tomography (PET/CT) and I-meta-iodobenzylguanidine single-photon emission computed tomography/computed tomography (131I-MIBG SPECT/CT) in patients with paragangliomas, including uncommon primaries.. Ninety patients were prospectively enrolled, and both scans were done within 2 weeks of each other. Lesions were grouped as Head/neck, abdominal, uncommon primary paraganglioma, and metastatic lesions. In most histopathology was used as reference standard.. PET/CT had sensitivity, specificity, positive predictive value, negative predictive value and accuracy of 97%, 94%, 99%,88%,97% respectively on patient wise analysis (90) and 98%, 94%, 99%, 85% and 97% respectively on lesion wise analysis (149). Comparison with MIBG SPECT/CT: Significant difference in sensitivities noted (PET/CT-98%, I-131 MIBG -39%) (P < 0.001), however, no significant difference in specificities (94% and 100%, respectively). Group-wise analysis: Head/Neck: Significant difference noted between PET/CT (sensitivity 100%) and I-131 MIBG SPECT/CT (sensitivity 22%) (P = 0.001). Abdominal: No significant difference noted in sensitivities and specificities of PET/CT and I-131 MIBG SPECT/CT. Uncommon paraganglioma: PET/CT detected 10 of 11, while I-131 MIBG detected only 2 of 11 uncommon paraganglioma. Metastatic sites: Significant difference noted between PET/CT (sensitivity 97%) and I-131 MIBG SPECT/CT (sensitivity 33%) (P < 0.0001).. The study demonstrates high diagnostic accuracy of Ga-DOTANOC PET/CT and superiority over I MIBG SPECT/CT for evaluation of extra-adrenal paraganglioma. The current diagnostic role of I-131 MIBG seems limited to abdominal paragangliomas and for theranostic purpose.

Topics: 3-Iodobenzylguanidine; Abdominal Neoplasms; Adolescent; Adult; Aged; Child; Female; Head and Neck Neoplasms; Humans; Male; Middle Aged; Organometallic Compounds; Paraganglioma; Positron Emission Tomography Computed Tomography; Prospective Studies; Sensitivity and Specificity; Single Photon Emission Computed Tomography Computed Tomography; Young Adult

Topics: Abdominal Neoplasms; Dihydroxyphenylalanine; Head and Neck Neoplasms; Humans; Hypertension; Image Processing, Computer-Assisted; Mutation; Organometallic Compounds; Paraganglioma; Peptides; Positron Emission Tomography Computed Tomography; Succinate Dehydrogenase

Carotid body paragangliomas are mostly benign tumors with very rare propensity to metastasize to lymph nodes, bones, liver, and lungs. Leptomeningeal metastasis from a carotid body paraganglioma is a very rare phenomenon. We describe Ga-DOTANOC PET-CT findings of a 33-year-old man, a known case of metastatic paraganglioma who underwent scan for response assessment to everolimus therapy.

Topics: Adult; Carotid Body Tumor; Humans; Male; Meningeal Carcinomatosis; Organometallic Compounds; Paraganglioma; Positron Emission Tomography Computed Tomography

Sellar and suprasellar paragangliomas are rare. We report the case of a 19-year-old man with sellar paraganglioma with parasellar and posterior pontine extension, requiring multiple transcranial surgeries and radiotherapy. The lesion was avid on Ga DOTANOC PET-CT scan substantiating its neuroendocrine nature.

Topics: Brain Neoplasms; Humans; Male; Organometallic Compounds; Paraganglioma; Pons; Positron Emission Tomography Computed Tomography; Radiopharmaceuticals; Young Adult

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

Topics: Acetabulum; Bone Neoplasms; Female; Humans; Mutation; Neck; Neoplasm Staging; Organometallic Compounds; Paraganglioma; Positron-Emission Tomography; Radiopharmaceuticals; Succinate Dehydrogenase; Tomography, X-Ray Computed

Paragangliomas (PGLs) due to succinate dehydrogenase subunit B (SDH-B) mutation can be aggressive. A 40-year-old woman with normetanephrine-secreting, extra-adrenal, abdominal PGL due to SDH-B genetic mutation underwent surgical excision of primary, followed by whole-body I-MIBG scan after 6 months, which showed no abnormality. However, Ga-DOTANOC and F-FDG PET/CT scans showed primary site recurrence, hepatic and skeletal metastasis, with latter scan revealing more lesions. We suggest that F-FDG PET should be done in all patients with PGL due to SDH-B mutations, as it may show additional unsuspected lesions that may be missed by other tracers.

Topics: 3-Iodobenzylguanidine; Adrenal Gland Neoplasms; Adult; Female; Fluorodeoxyglucose F18; Humans; Multimodal Imaging; Mutation; Organometallic Compounds; Paraganglioma; Radionuclide Imaging; Radiopharmaceuticals; Succinate Dehydrogenase; Tomography, X-Ray Computed

Urinary bladder paraganglioma constitute only a small fraction of urinary bladder tumors. These rare tumors are well known for recurrences even after many years of surgery. Functional imaging with different radiotracers has been found to be useful to evaluate the extent, metastasis, and recurrence of paragangliomas. We report a case where Ga DOTANOC PET/CT successfully detected recurrence of a paraganglioma in the urinary bladder along with pelvic lymph nodal metastasis.

Topics: Adult; Humans; Male; Multimodal Imaging; Neoplasm Recurrence, Local; Organometallic Compounds; Paraganglioma; Positron-Emission Tomography; Radiopharmaceuticals; Tomography, X-Ray Computed; Urinary Bladder Neoplasms

Glomus laryngicum (laryngeal paraganglioma) are extremely rare neoplasm of the larynx. We here present the 68Ga-DOTANOC PET/CT images of a 40-year-old male patient with glomus laryngicum. In this report, we have highlighted the potential utility and limitations of 68Ga-DOTANOC PET/CT imaging in patients with glomus laryngicum.

Topics: Adult; Humans; Laryngeal Neoplasms; Male; Multimodal Imaging; Organometallic Compounds; Paraganglioma; Positron-Emission Tomography; Tomography, X-Ray Computed

Urinary bladder paragangliomas are exceedingly rare tumors. A 22-year-old woman with bladder paraganglioma underwent Ga DOTANOC PET/CT for proper localization of the primary tumor and to rule out locoregional and distant metastases. Ga DOTANOC-avid bladder mass was detected with no other abnormal site of radiotracer uptake elsewhere. Although radioactive urine can mask urinary paragangliomas, diuretic method can aid tumor detection by Ga DOTANOC PET/CT.

Topics: Adrenal Gland Neoplasms; Adult; Diagnosis, Differential; Diuretics; Female; Humans; Multimodal Imaging; Organometallic Compounds; Paraganglioma; Pheochromocytoma; Positron-Emission Tomography; Radiopharmaceuticals; Tomography, X-Ray Computed; Urinary Bladder Neoplasms

The purpose of this study was to evaluate the role of (68)Ga-labeled DOTANOC PET/CT for baseline evaluation of patients with head and neck paragangliomas (HNPs).. The data for 26 patients (mean age ± SD, 34.3 ± 10.4 y; 50% men) with known or suspected HNPs who underwent (68)Ga-DOTANOC PET/CT for staging were retrospectively analyzed. PET/CT was performed after intravenous injection of 132-222 MBq of (68)Ga-DOTANOC. The images were evaluated by 2 experienced nuclear medicine physicians in consensus, both qualitatively and quantitatively. The PET/CT findings were grouped as HNPs, paraganglioma at other sites (non-HNPs), and metastatic disease. The size and maximum standardized uptake values (SUVmax) were measured for all lesions. All of the patients also underwent whole-body (131)I-metaiodobenzylgunanidine ((131)I-MIBG) scintigraphy and conventional imaging (CT/MR imaging) of the head and neck region. Their results were compared with those of (68)Ga-DOTANOC PET/CT.. (68)Ga-DOTANOC PET/CT findings were positive in all 26 patients, and 78 lesions were detected. PET/CT imaging demonstrated 45 HNPS, 10 non-HNPs, and 23 metastatic sites. Fifteen patients (57.6%) had more than one site of disease on PET/CT. Among 45 HNPs, 26 were carotid body tumors (CBTs), 15 glomus jugulare, 3 glomus tympanicum, and 1 laryngeal paraganglioma. A positive correlation was seen between size and SUVmax of HNPs (ρ = 0.323; P = 0.030). The SUVmax of the CBTs was higher than that of jugulotympanic paragangliomas (P = 0.026). No correlation was seen between size and SUVmax (ρ = 0.069; P = 0.854) of non-HNPs. The size and SUVmax of non-HNPs were significantly less than those of HNPs (P = 0.029 and 0.047, respectively). (131)I-MIBG scintigraphy showed only 30 of the 78 lesions and was inferior to PET/CT (P < 0.0001). Conventional imaging (CT/MR imaging) was positive for 42 of 49 head and neck lesions and was inferior to PET/CT on direct comparison (P = 0.015). A combination of CT/MR imaging and (131)I-MIBG scintigraphy detected only 53 of 78 (67.9%) lesions and was also inferior to PET/CT (P < 0.0001).. (68)Ga-DOTANOC PET/CT is useful for the baseline evaluation of patients with HNPs and can demonstrate synchronous paragangliomas at other sites and distant metastases. It is superior to (131)I-MIBG scintigraphy and conventional imaging (CT/MR imaging) for this purpose.

Topics: 3-Iodobenzylguanidine; Adolescent; Adult; Female; Head and Neck Neoplasms; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Multimodal Imaging; Organometallic Compounds; Paraganglioma; Positron-Emission Tomography; Retrospective Studies; Tomography, X-Ray Computed; Young Adult

To evaluate the role of (68)Ga-labelled [1, 4, 7, 10-tetraazacyclododecane-1, 4, 7, 10-tetraacetic acid]-1-NaI(3)-Octreotide ((68)Ga-DOTA-NOC) whole body positron emission tomography-computed tomography (PET-CT) as a functional imaging approach for phaeochromocytoma and paraganglioma.. Thirty-five unrelated patients (Median age-34.4 years; range: 15-71) were evaluated in this prospective study. PET-CT was performed after injection of 132-222 MBq of (68)Ga-DOTA-NOC. Images were evaluated by two experienced nuclear medicine physicians both qualitatively as well as quantitatively (standardised uptake value-SUVmax). In addition we compared the findings with (131)I Metaiodobenzylguanidine (MIBG) scintigraphy, which was available for 25 patients. Histopathology and/or conventional imaging with biochemical markers were taken as the reference standard.. 44 lesions were detected on (68)Ga-DOTA-NOC PET-CT imaging with an additional detection of 12 lesions not previously known, leading to a change in management of 6 patients. Sensitivity, specificity and accuracy were 100%, 85.7%, and 97.1% on a per patient basis and 100%, 85.7% and 98% on per lesion basis, respectively.(131)I MIBG scintigraphy was concordant with (68)Ga-DOTA-NOC PET-CT in 16 patients and false negative in 9 patients.. (68)Ga-DOTA-NOC PET-CT is highly sensitive and specific for the detection of phaeochromoctyomas and paragangliomas. It seems better than (131)I MIBG scintigraphy for this purpose.. • ( 68 ) Ga-DOTA-NOC PET-CT seems useful in patients with phaeochromocytoma and paraganglioma. • This prospective single centre study showed that it has high diagnostic accuracy. • (68) Ga-DOTA-NOC PET-CT seems superior to (131) I-MIBG in these patients.

Topics: Adolescent; Adrenal Gland Neoplasms; Adult; Aged; Female; Humans; Male; Middle Aged; Multimodal Imaging; Organometallic Compounds; Paraganglioma; Pheochromocytoma; Positron-Emission Tomography; Prospective Studies; Radiopharmaceuticals; Sensitivity and Specificity; Statistics, Nonparametric; Tomography, X-Ray Computed