68ga-dotanoc and Pheochromocytoma

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

Pheochromocytomas are rare tumors which can be malignant in 10% of cases. We present the case of a 75-year-old woman who presented with headache and palpitation for 1 year. She had a past history of right adrenalectomy for pheochromocytoma 20 years back. In between, the patient was asymptomatic. Twenty-four-hour urinary vanillylmandelic acid was raised. Noncontrast CT and ultrasound of abdomen were unremarkable. The patient underwent 68Ga-DOTANOC PET/CT that showed metastasis to left ilium, which was confirmed on biopsy.

Topics: Adrenal Gland Neoplasms; Aged; Bone Neoplasms; Female; Humans; Multimodal Imaging; Organometallic Compounds; Pheochromocytoma; Positron-Emission Tomography; Recurrence; Tomography, X-Ray Computed

The purpose of the present study was to evaluate the diagnostic accuracy of (68)Ga-DOTANOC positron emission tomography (PET)/CT in patients with suspicion of pheochromocytoma.. Data of 62 patients [age 34.3 ± 16.1 years, 14 with multiple endocrine neoplasia type 2 (MEN2)] with clinical/biochemical suspicion of pheochromocytoma and suspicious adrenal lesion on contrast CT (n = 70), who had undergone (68)Ga-DOTANOC PET/CT, were retrospectively analyzed. PET/CT images were analyzed visually as well as semiquantitatively, with measurement of maximum standardized uptake value (SUVmax), SUVmean, SUVmax/SUVliver, and SUVmean/SUVliver. Results of PET/CT were compared with (131)I-metaiodobenzylguanidine (MIBG) imaging, which was available in 40 patients (45 lesions). Histopathology and/or imaging/clinical/biochemical follow-up (minimum 6 months) was used as reference standard.. The sensitivity, specificity, and accuracy of (68)Ga-DOTANOC PET/CT was 90.4, 85, and 88.7%, respectively, on patient-based analysis and 92, 85, and 90%, respectively, on lesion-based analysis. (68)Ga-DOTANOC PET/CT showed 100% accuracy in patients with MEN2 syndrome and malignant pheochromocytoma. On direct comparison, lesion-based accuracy of (68)Ga-DOTANOC PET/CT for pheochromocytoma was significantly higher than (131)I-MIBG imaging (91.1 vs 66.6%, p = 0.035). SUVmax was higher for pheochromocytomas than other adrenal lesions (p = 0.005), MEN2-associated vs sporadic pheochromocytoma (p = 0.012), but no difference was seen between benign vs malignant pheochromocytoma (p = 0.269).. (68)Ga-DOTANOC PET/CT shows high diagnostic accuracy in patients with suspicion of pheochromocytoma and is superior to (131)I-MIBG imaging for this purpose. Best results of (68)Ga-DOTANOC PET/CT are seen in patients with MEN2-associated and malignant pheochromocytoma.

Topics: 3-Iodobenzylguanidine; Adrenal Gland Neoplasms; Adult; Female; Humans; Male; Middle Aged; Multimodal Imaging; Organometallic Compounds; Pheochromocytoma; Positron-Emission Tomography; Radiopharmaceuticals; Reference Standards; Sensitivity and Specificity; 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

A 35-year-old man with clinical suspicion of adrenal pheochromocytoma was evaluated using Ga DOTANOC PET/CT. PET/CT demonstrated Ga DOTANOC-avid right adrenal mass and cerebellar lesion, raising the suspicion of adrenal pheochromocytoma with cerebellar hemangioblastoma suggesting von Hippel-Lindau (VHL) syndrome. Cerebellar lesion on further evaluation with MRI was suggestive of cerebellar hemangioblastoma. Surgical resection of the adrenal mass revealed pheochromocytoma, and genetic analysis revealed mutation involving the chromosome 3p, confirming the diagnosis of VHL syndrome. Ga DOTANOC PET/CT in our patient helped in the diagnosis of VHL syndrome and changed the disease management.

Topics: Adrenal Gland Neoplasms; Adult; Cerebellar Neoplasms; Chromosomes, Human, Pair 3; Hemangioblastoma; Humans; Male; Multimodal Imaging; Mutation; Organometallic Compounds; Pheochromocytoma; Positron-Emission Tomography; Radiopharmaceuticals; Tomography, X-Ray Computed; von Hippel-Lindau Disease

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

We observed a 34-year-old man who was incidentally found to have an adrenal mass during surgical follow-up for perforated ulcer. The patient was subjected to I-123 MIBG scintigraphy, 68Ga-DOTANOC PET/CT, and F-18 DOPA PET/CT. Only F-18 DOPA PET/CT showed evidence of an avid adrenal mass. A CT-guided biopsy was performed and it was suggestive for pheochromocytoma. He underwent surgery and a pheochromocytoma, about 40 mm in diameter, was detected. Traditionally, I-123 MIBG scintigraphy has been used in detecting chromaffin cell tumors, but more recently it had been demonstrated that a certain part of pheochromocytoma could be false-negative on scintigraphy.

Topics: 3-Iodobenzylguanidine; Adrenal Gland Neoplasms; Adult; Dihydroxyphenylalanine; False Negative Reactions; Humans; Male; Organometallic Compounds; Pheochromocytoma; Positron-Emission Tomography; Tomography, X-Ray Computed