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Garg N, Pandey S, Madan S, Yadav S, Majumdar G, Jain N, Jain M. Isolated Cardiac Paraganglioma Encasing Right Coronary Artery With Evidence of Succinyl Dehydrogenase Gene Mutation: Successful Management Using Multimodality Imaging. Tex Heart Inst J 2023; 50:490667. [PMID: 36745744 PMCID: PMC9969784 DOI: 10.14503/thij-21-7639] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
This report involves a young woman with isolated cardiac paraganglioma that was diagnosed using 68Gallium-labeled [1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid]-1-NaI3-octreotide positron emission tomographic scintigraphy. For the preoperative evaluation, multimodality imaging accurately described the anatomic location of the tumor and its relationship with the surrounding tissues. The patient underwent successful surgical resection of the tumor along with right coronary artery bypass grafting. The 2-month follow-up scintigraphy was normal. Next-generation sequencing evaluation revealed a novel germline mutation for the succinate dehydrogenase subunit B gene.
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Affiliation(s)
- Naveen Garg
- Department of Cardiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Shashank Pandey
- Department of Cardiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Shiva Madan
- Department of Endocrinology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Subhash Yadav
- Department of Endocrinology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Gurango Majumdar
- Department of Cardiovascular and Thoracic Surgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Neeraj Jain
- Department of Radiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Manoj Jain
- Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
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Laparoscopic resection of a paraganglioma in the greater omentum mimicking a peripancreatic neoplasm: a case report. Clin J Gastroenterol 2021; 14:1364-1370. [PMID: 34053005 DOI: 10.1007/s12328-021-01452-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/29/2021] [Accepted: 05/25/2021] [Indexed: 10/21/2022]
Abstract
A paraganglioma is a tumor originating in the sympathetic or parasympathetic nervous system. Its diagnosis may sometimes be confusing if it occurs in an atypical site. We described herein a case of a peripancreatic paraganglioma originating in the greater omentum. An asymptomatic, 61-year-old, female patient was referred to our hospital for detailed examination of a peripancreatic mass detected incidentally on computed tomography (CT). The differential diagnosis was a neuroendocrine neoplasm (NEN), and a biopsy using EUS-FNA was performed. Histologically, the tumor cells showed proliferation in solid cell nests and were positive for CD56, chromogranin A, and synaptophysin. These findings and the hypervascularity of the tumor on imaging studies were compatible with NEN. Since the imaging studies did not clearly demonstrate the continuity of the tumor with the pancreas, laparoscopic tumor resection without a pancreatectomy and sampling of the enlarged peripancreatic lymph nodes were planned as treatment. The absence of continuity with the pancreas was later confirmed by intraoperative observation, and the resection was carried out as planned. The resected tumor was pathologically considered as NEN at first in agreement with the preoperative diagnosis. However, several histological findings (such as a zelleballen-like growth pattern, pseudo-inclusion, and strong nuclear atypia compared with the cells' proliferative ability) were atypical for NEN, and paraganglioma was included in the differential diagnosis. Additional immunostainings of S-100 and AE1/AE3 were performed, leading to the final diagnosis of paraganglioma. Paragangliomas should be included in the differential diagnosis of an intraperitoneal mass of uncertain identity with hypervascularity.
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Jandou I, Moataz A, Mouqtassid MH, Dakir M, Debbagh A, Aboutaieb R. The ectopic vesical pheochromocytoma a diagnostic and therapeutic challenge case report and literature review. Int J Surg Case Rep 2020; 77:857-861. [PMID: 33395912 PMCID: PMC7726245 DOI: 10.1016/j.ijscr.2020.11.132] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/21/2020] [Revised: 11/25/2020] [Accepted: 11/25/2020] [Indexed: 11/03/2022] Open
Abstract
INTRODUCTION Extra-adrenal localization of pheochromocytoma is rare. Its clinical revelation is paroxysmal but maybe in the form of permanent hypertension resistant to treatment. The main problem with these tumors is to affirm their benignity or malignancy, ectopic pheochromocytomas have a malignant development once in two. CASE PRESENTATION We report the observation of a 39-year-old woman hypertensive on Amlodipine. She presents for sweating, headache, and palpation. The dosage of urinary metabolites revealed a marked increase in metanephrine and normetanephrine. Imagery had shown, at the expense of the left lateral wall of the bladder, a budding tissue formation with irregular contours which was significantly enhanced after injection of PDC. The diagnosis of an ectopic pheochromocytoma was accepted. The patient underwent a partial cystectomy, the postoperative consequences of which were unremarkable. DISCUSSION Threatening secretory syndromes are rare medical situations that require urgent management. Pheochromocytoma can be seen at any age from childhood to old age. The treatment of pheochromocytoma is surgical, once the stages of positive and topographic diagnosis have been carried out, the patient must be immediately entrusted to a team of trained surgeons and anesthetists. The prognosis remains unpredictable, hence the interest in prolonged monitoring. CONCLUSION Pheochromocytoma is a tumor developed at the expense of chromaffin tissues, extra-adrenal localizations exist, but remain rare. It is a pathology that presents a diagnostic and therapeutic dilemma in which patients require regular and prolonged postoperative monitoring to detect a possible metastasis or recurrence.
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Affiliation(s)
- Issam Jandou
- University Hospital Center Ibn Rochd Casablanca, Morocco; Faculté de médicine et de pharmacie Casablanca, Morocco.
| | - Amine Moataz
- University Hospital Center Ibn Rochd Casablanca, Morocco; Faculté de médicine et de pharmacie Casablanca, Morocco
| | - Mohammed Hicham Mouqtassid
- University Hospital Center Ibn Rochd Casablanca, Morocco; Faculté de médicine et de pharmacie Casablanca, Morocco
| | - Mohammed Dakir
- University Hospital Center Ibn Rochd Casablanca, Morocco; Faculté de médicine et de pharmacie Casablanca, Morocco
| | - Adil Debbagh
- University Hospital Center Ibn Rochd Casablanca, Morocco; Faculté de médicine et de pharmacie Casablanca, Morocco
| | - Rachid Aboutaieb
- University Hospital Center Ibn Rochd Casablanca, Morocco; Faculté de médicine et de pharmacie Casablanca, Morocco
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Abstract
OBJECTIVE. Loss of the neurofibromatosis type 1 (NF1) tumor suppressor protein causes uninhibited activation of the RAS oncogene, which leads to tumorigenesis in patients with NF1. This case-based review discusses imaging manifestations of NF1 in the abdomen and pelvis, highlighting key genetic associations and management to elucidate features different from the general population. CONCLUSION. The spectrum of pathologic findings includes gastrointestinal tumors such as gastrointestinal stromal tumors, genitourinary lesions including urogenital neurofibromas, vascular entities such as renal artery stenosis, and less common associations like lymphoma.
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Chen HT, Cheng YY, Tsao TF, Peng CM, Hsu JD, Tyan YS. Abdominal Ultrasound in the Detection of an Incidental Paraganglioma. J Med Ultrasound 2020; 29:119-122. [PMID: 34377644 PMCID: PMC8330680 DOI: 10.4103/jmu.jmu_25_20] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/14/2020] [Revised: 04/29/2020] [Accepted: 05/15/2020] [Indexed: 11/26/2022] Open
Abstract
Paraganglioma is a tumor that originates from neuroendocrine cells of the sympathetic or parasympathetic systems. Patients may suffer from headaches, palpitations, diaphoresis, and hypertension due to catecholamine excess or symptoms from the mass effect of the tumor. In the absence of typical symptoms of catecholamine excess, the diagnosis of a nonfunctional paraganglioma is often delayed. Herein, we report a case of a 63-year-old female patient with a nonfunctional paraganglioma which is an accidental finding during investigation of a fever. Abdominal ultrasonography incidentally detected this lesion as a complex, solid, cystic mass in the left suprarenal retroperitoneum.
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Affiliation(s)
- Hsiao-Ting Chen
- Department of Medical Imaging, Chung Shan Medical University Hospital, Taichung, Taiwan.,Department of Medical Imaging and Radiological Sciences, Chung Shan Medical University, Taichung, Taiwan
| | - Yung-Yin Cheng
- Department of Medical Imaging, Chung Shan Medical University Hospital, Taichung, Taiwan.,Department of Medical Imaging and Radiological Sciences, Chung Shan Medical University, Taichung, Taiwan
| | - Teng-Fu Tsao
- Department of Medical Imaging, Chung Shan Medical University Hospital, Taichung, Taiwan.,Department of Medical Imaging and Radiological Sciences, Chung Shan Medical University, Taichung, Taiwan.,School of Medicine, Chung Shan Medical University, Taichung, Taiwan
| | - Cheng-Ming Peng
- School of Medicine, Chung Shan Medical University, Taichung, Taiwan.,Division of General Surgery, Department of Surgery, Chung Shan Medical University Hospital, Taichung, Taiwan
| | - Jeng-Dong Hsu
- School of Medicine, Chung Shan Medical University, Taichung, Taiwan.,Department of Pathology, Chung Shan Medical University Hospital, Taichung, Taiwan
| | - Yeu-Sheng Tyan
- Department of Medical Imaging, Chung Shan Medical University Hospital, Taichung, Taiwan.,Department of Medical Imaging and Radiological Sciences, Chung Shan Medical University, Taichung, Taiwan.,School of Medicine, Chung Shan Medical University, Taichung, Taiwan
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Schreiner F, Beuschlein F. Disease monitoring of patients with pheochromocytoma or paraganglioma by biomarkers and imaging studies. Best Pract Res Clin Endocrinol Metab 2020; 34:101347. [PMID: 31662271 DOI: 10.1016/j.beem.2019.101347] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors, a large proportion of which secrete catecholamines. PPGL are associated with a high cardiovascular morbidity and come with a risk of malignancy. The therapy of choice is surgical resection. Nevertheless, PPGL are associated with a lifelong risk of tumor persistence or recurrence. Currently, there are no clinical, biochemical, histopathological or imaging characteristics, which can predict or exclude malignant behavior or tumor recurrence. Therefore, long-term follow-up is recommended even after apparent complete surgical removal. Early detection of recurrence is essential to reduce cardiovascular morbidity and mortality due to catecholamine secretion, to prevent morbidity by mass effects of paraganglioma (PGL) or by metastatic spread of disease. Due to the rarity of these tumors, no prospective data on long-term surveillance exist. In fact, current recommendations are based on retrospective analyses, expert opinions and case studies. The aim of this review is to provide an overview on the current state of knowledge with regard to known factors that increase the risk of recurrence and might impact disease monitoring as well as the available possibilities for biochemical and imaging follow-up. Based on this overview, we aim to propose a practical approach for a patient-oriented follow-up after surgical removal of a PPGL.
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Affiliation(s)
- Florentine Schreiner
- Klinik für Endokrinologie, Diabetologie und Klinische Ernährung, UniversitätsSpital Zürich, Zurich, Switzerland
| | - Felix Beuschlein
- Klinik für Endokrinologie, Diabetologie und Klinische Ernährung, UniversitätsSpital Zürich, Zurich, Switzerland; Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, Munich, Germany.
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Abstract
Pheochromocytomas are rare neuroendocrine chromaffin-derived tumors that arise within the adrenal medulla. They are usually benign, but if not diagnosed or if left untreated, they can have devastating consequences. Clinical consideration of the diagnosis is paramount, as they may have protean manifestations, and a high index of suspicion is essential if serious consequences are to be avoided. An accurate biochemical diagnosis is crucial for the management of these patients: either plasma or urinary metanephrines are both highly sensitive and specific if correctly employed, but knowledge of pre- and post-analytic interference is essential. Diagnostic imaging with cross-sectional CT and/or MRI offers high sensitivity in their detection, but lack specificity. The introduction of PET/CT/MR has led to a dramatic improvement in the localization of both pheochromocytomas and paragangliomas, together with the increasing availability of new functional imaging radionuclides. Optimal investigation and accurate diagnosis is best achieved at 'centers of excellence' with expert multidisciplinary teams.
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Affiliation(s)
- Emilia Sbardella
- Department of Experimental Medicine, Sapienza University of Rome, Viale del Policlinico, 155, 00161, Rome, Italy.
| | - Ashley B Grossman
- Department of Endocrinology, Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, University of Oxford, Oxford, OX3 7LE, UK; Centre for Endocrinology, Barts and the London School of Medicine, London, EC1M 6BQ, UK; ENETS Centre of Excellence, Royal Free Hospital, London, NW3 2QG, UK
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Adrenal Cavernous Hemangioma: A Rarely Perceived Pathology-Case Illustration and Review of Literature. Case Rep Pathol 2019; 2019:8463890. [PMID: 31949968 PMCID: PMC6944974 DOI: 10.1155/2019/8463890] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/12/2019] [Revised: 10/03/2019] [Accepted: 11/29/2019] [Indexed: 12/02/2022] Open
Abstract
Cavernous hemangiomas are endothelial tumors that rarely affect the adrenal glands. Most of these tumors remain silent and are incidentally found on abdominal imaging. Hardly ever, these tumors are endocrinologically functional. They may present as vague abdominal pain. Surgical resection remains the mainstay for large masses. In this paper, we are presenting a case of adrenal cavernous hemangioma in a 83-year-old male patient who initially presented for workup of vague abdominal and bilateral flank pain. A computed tomography scan of the abdomen showed an 8 cm right adrenal adenoma which was metabolically nonfunctional. The mass was completely resected through an open subcostal incision, with no encountered postoperative complications. A highlight of all published cases of adrenal hemangiomas since 1955 is also presented and reviewed.
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Bhargava P, Sangster G, Haque K, Garrett J, Donato M, D'Agostino H. A Multimodality Review of Adrenal Tumors. Curr Probl Diagn Radiol 2019; 48:605-615. [DOI: 10.1067/j.cpradiol.2018.10.002] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/08/2018] [Accepted: 10/16/2018] [Indexed: 12/15/2022]
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Barbosa BC, Parente DB, Macedo FNA. Uncharacteristic diffuse abdominal pain in a 14-year-old girl. Frontline Gastroenterol 2019; 10:442-443. [PMID: 31656574 PMCID: PMC6788122 DOI: 10.1136/flgastro-2018-101117] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/03/2018] [Revised: 12/05/2018] [Accepted: 12/14/2018] [Indexed: 02/04/2023] Open
Affiliation(s)
| | - Daniella Braz Parente
- Radiology, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil,Radiology, D’Or Institute for Research and Education, Rio de Janeiro, Brazil
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Pandit-Taskar N, Modak S. Norepinephrine Transporter as a Target for Imaging and Therapy. J Nucl Med 2017; 58:39S-53S. [PMID: 28864611 DOI: 10.2967/jnumed.116.186833] [Citation(s) in RCA: 58] [Impact Index Per Article: 7.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/11/2017] [Accepted: 07/19/2017] [Indexed: 01/01/2023] Open
Abstract
The norepinephrine transporter (NET) is essential for norepinephrine uptake at the synaptic terminals and adrenal chromaffin cells. In neuroendocrine tumors, NET can be targeted for imaging as well as therapy. One of the most widely used theranostic agents targeting NET is metaiodobenzylguanidine (MIBG), a guanethidine analog of norepinephrine. 123I/131I-MIBG theranostics have been applied in the clinical evaluation and management of neuroendocrine tumors, especially in neuroblastoma, paraganglioma, and pheochromocytoma. 123I-MIBG imaging is a mainstay in the evaluation of neuroblastoma, and 131I-MIBG has been used for the treatment of relapsed high-risk neuroblastoma for several years, however, the outcome remains suboptimal. 131I-MIBG has essentially been only palliative in paraganglioma/pheochromocytoma patients. Various techniques of improving therapeutic outcomes, such as dosimetric estimations, high-dose therapies, multiple fractionated administration and combination therapy with radiation sensitizers, chemotherapy, and other radionuclide therapies, are being evaluated. PET tracers targeting NET appear promising and may be more convenient options for the imaging and assessment after treatment. Here, we present an overview of NET as a target for theranostics; review its current role in some neuroendocrine tumors, such as neuroblastoma, paraganglioma/pheochromocytoma, and carcinoids; and discuss approaches to improving targeting and theranostic outcomes.
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Affiliation(s)
| | - Shakeel Modak
- Memorial Sloan Kettering Cancer Center, New York, New York
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12
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Kligerman SJ, Auerbach A, Franks TJ, Galvin JR. Castleman Disease of the Thorax: Clinical, Radiologic, and Pathologic Correlation: From the Radiologic Pathology Archives. Radiographics 2017; 36:1309-32. [PMID: 27618318 DOI: 10.1148/rg.2016160076] [Citation(s) in RCA: 34] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Castleman disease is a complex lymphoproliferative disease pathologically divided into two subtypes, the hyaline vascular variant (HVV) and the plasma cell variant (PCV). The HVV is the most common, is thought to represent a benign neoplasm of lymph node stromal cells, and is treated with surgical resection. It is most commonly found in the mediastinum, where it classically appears as a unicentric, avidly enhancing mass at computed tomography (CT) and magnetic resonance imaging. This appearance can mimic other avidly enhancing mediastinal masses, and location, clinical history, laboratory data, and nuclear medicine single photon emission CT (SPECT) and positron emission tomography (PET) studies can help narrow the differential diagnosis. Multicentric Castleman disease (MCD), which in the majority of cases is composed of the PCV, is an aggressive lymphoproliferative disorder associated with human herpesvirus infection, interleukin 6 dysregulation, and other systemic disorders. While it can be difficult to differentiate MCD from lymphoma, the presence of avidly enhancing lymph nodes can suggest the diagnosis. The purpose of this article is to review the clinical, immunologic, and pathologic findings associated with both unicentric Castleman disease and MCD and discuss how the imaging findings correlate with the pathophysiology of the disease.
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Affiliation(s)
- Seth J Kligerman
- From the Department of Diagnostic Radiology and Nuclear Medicine, Division of Chest Imaging (S.J.K., J.R.G.), and Department of Internal Medicine, Division of Pulmonary/Critical Care (J.R.G.), University of Maryland School of Medicine, 22 S Greene St, Baltimore, MD 21201; Department of Hematopathology (A.A.) and Department of Pulmonary and Mediastinal Pathology (T.J.F.), the Joint Pathology Center, Department of Defense, Defense Health Agency, Silver Spring, Md; and Department of Chest Imaging, American Institute for Radiologic Pathology, Silver Spring, Md (J.R.G.)
| | - Aaron Auerbach
- From the Department of Diagnostic Radiology and Nuclear Medicine, Division of Chest Imaging (S.J.K., J.R.G.), and Department of Internal Medicine, Division of Pulmonary/Critical Care (J.R.G.), University of Maryland School of Medicine, 22 S Greene St, Baltimore, MD 21201; Department of Hematopathology (A.A.) and Department of Pulmonary and Mediastinal Pathology (T.J.F.), the Joint Pathology Center, Department of Defense, Defense Health Agency, Silver Spring, Md; and Department of Chest Imaging, American Institute for Radiologic Pathology, Silver Spring, Md (J.R.G.)
| | - Teri J Franks
- From the Department of Diagnostic Radiology and Nuclear Medicine, Division of Chest Imaging (S.J.K., J.R.G.), and Department of Internal Medicine, Division of Pulmonary/Critical Care (J.R.G.), University of Maryland School of Medicine, 22 S Greene St, Baltimore, MD 21201; Department of Hematopathology (A.A.) and Department of Pulmonary and Mediastinal Pathology (T.J.F.), the Joint Pathology Center, Department of Defense, Defense Health Agency, Silver Spring, Md; and Department of Chest Imaging, American Institute for Radiologic Pathology, Silver Spring, Md (J.R.G.)
| | - Jeffrey R Galvin
- From the Department of Diagnostic Radiology and Nuclear Medicine, Division of Chest Imaging (S.J.K., J.R.G.), and Department of Internal Medicine, Division of Pulmonary/Critical Care (J.R.G.), University of Maryland School of Medicine, 22 S Greene St, Baltimore, MD 21201; Department of Hematopathology (A.A.) and Department of Pulmonary and Mediastinal Pathology (T.J.F.), the Joint Pathology Center, Department of Defense, Defense Health Agency, Silver Spring, Md; and Department of Chest Imaging, American Institute for Radiologic Pathology, Silver Spring, Md (J.R.G.)
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Yuan Y, Shi H, Tao X. Head and neck paragangliomas: diffusion weighted and dynamic contrast enhanced magnetic resonance imaging characteristics. BMC Med Imaging 2016; 16:12. [PMID: 26833065 PMCID: PMC4736670 DOI: 10.1186/s12880-016-0114-3] [Citation(s) in RCA: 27] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/01/2015] [Accepted: 01/22/2016] [Indexed: 12/20/2022] Open
Abstract
Background To determine the feature values of head and neck paragangliomas on diffusion-weighted imaging (DWI) and dynamic contrast enhanced (DCE) magnetic resonance imaging (MRI). Methods Patients with primary head and neck paraganglioma who underwent both DWI and DCE-MRI before treatment between January 2010 and June 2013 were identified. Two radiologists assessed apparent diffusion coefficient (ADC) values on DWI and kinetic characteristics on DCE-MRI. The time intensity curves (TICs) and dynamic parameters, including peak height (PH), maximum enhancement ratio (ERmax), time to peak enhancement (Tpeak) and maximum rise slope (Slopemax), were generated and evaluated. Results Ten patients with head and neck paraganglioma were retrospectively analyzed. On conventional MRI, the tumors demonstrated as well-circumscribed, strongly enhanced lesions. Mean ADC value of the lesions was 1.12 ± 0.15 × 10−3 mm2/s. The TICs demonstrated washout pattern (type-III) in all lesions. The mean PH, ERmax, Tpeak and Slopemax value was 121.24 ± 63.99, 193.79 ± 67.18, 8.16 ± 3.29 and 25.42 ± 7.91, respectively. Conclusions Head and neck paragangliomas demonstrate distinctive DWI and DCE-MRI results than for other benign tumors which should be taken into account in further evaluation of MRI on head and neck lesions.
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Affiliation(s)
- Ying Yuan
- Department of Radiology, Shanghai Ninth People's Hospital, Affiliated to JiaoTong University School of Medicine, 639 Zhizaoju Road, Shanghai, 200011, China.
| | - Huimin Shi
- Department of Radiology, Shanghai Ninth People's Hospital, Affiliated to JiaoTong University School of Medicine, 639 Zhizaoju Road, Shanghai, 200011, China.
| | - Xiaofeng Tao
- Department of Radiology, Shanghai Ninth People's Hospital, Affiliated to JiaoTong University School of Medicine, 639 Zhizaoju Road, Shanghai, 200011, China.
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Knie B, Plotkin M, Zschieschang P, Prasad V, Moskopp D. A family with pheochromocytoma-paraganglioma inherited tumour syndrome. Serial 18F-DOPA PET/CT investigations. Nuklearmedizin 2016; 55:34-40. [PMID: 26740102 DOI: 10.3413/nukmed-0755-15-07] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/03/2015] [Accepted: 12/04/2015] [Indexed: 01/26/2023]
Abstract
AIM Hereditary pheochromocytoma-paraganglioma syndromes are characterized by multiple pheochromocytomas (PCC) and paragangliomas (PGLs), inherited in an autosomal dominant manner. Early detection and removal of tumours may prevent or minimize complications related to mass effects and malignant transformation. Having confirmed the diagnosis, it is important to localize the tumours and reveal their extent preoperatively. This study aimed to introduce 18F-DOPA PET/CT as a highly sensitive non-invasive diagnostic tool for early detection of mass lesions in patients with pheochromocytoma-paraganglioma inherited tumour syndrome and to report about its impact on patient management. PATIENTS, METHODS We are currently supervising one of the largest documented families in Germany with genetically determined SDHD gene mutation. We performed 18F-DOPA PET/CT in order to detect tumours in asymptomatic gene carriers and enable subsequent surgical therapy. RESULTS In seven patients undergoing 12 18F-DOPA PET/CT scans 17 lesions have been detected. Three of these lesions, located in the head and neck region, have had no morphologic correlate in CT and one had also no morphologic correlate in MRI. Of the six histologically analyzed lesions five have been tumors (PGL or PCC) and one has been a nodular hyperplasia. This means the 18F-DOPA PET/CT scan in our study group had a sensitivity of 83%. 18F-DOPA PET/CT investigations lead to change in the management in 5/7 studied patients (70%). CONCLUSION The benefits of PET/CT in detection of pheochromocytoma and paraganglioma are well documented, but we are the first to use this technique for screening of a rare hereditary disease (estimated prevalence 0.3/100 000).
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Affiliation(s)
- B Knie
- Bettina Knie, Klinik für Neurochirurgie, Vivantes Klinikum im Friedrichshain, Landsberger Allee 49, 10249 Berlin, Germany, Tel. +49/(0)30/130 23-15 26, Fax -20 57,
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Maurea S, Mainenti PP, Romeo V, Mollica C, Salvatore M. Nuclear imaging to characterize adrenal tumors: Comparison with MRI. World J Radiol 2014; 6:493-501. [PMID: 25071890 PMCID: PMC4109101 DOI: 10.4329/wjr.v6.i7.493] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/21/2014] [Revised: 05/30/2014] [Accepted: 06/16/2014] [Indexed: 02/06/2023] Open
Abstract
AIM: To describe the role of nuclear imaging modalities using nor-cholesterol, metaiodobenzylguanidine (MIBG) and fluorine-deoxy-glucose (FDG) in adrenal tumors for lesion characterization in comparison with magnetic resonance (MR).
METHODS: Population was classified in group 1 consisting of 30 patients with non-hypersecreting unilateral adrenal masses, in group 2 consisting of 34 patients with hypersecreting (n = 19) or non-hypersecreting (n = 15) adrenal adenomas and in group 3 consisting of 18 patients with chromaffin-tissue tumors (CTT), of which 14 were pheochromocytomas while 4 were paragangliomas (n = 4). All patients underwent MR and nuclear studies (nor-cholesterol, MIBG and FDG). Pathology samples (n = 63) or follow-up data in adenomas (n = 19) were used as standard of reference for imaging studies interpretation.
RESULTS: In group 1, MR findings were not highly accurate for lesion characterization, while the results of nuclear scans showed abnormal nor-cholesterol, MIBG and FDG concentration in all cases of adenomas, pheos and malignant tumors, respectively. In group 2, no differences in MR parameters were found between hyperfunctioning and non-hyperfunctioning adenomas, while nor-cholesterol uptake was significantly higher in hyperfunctioning compared to non-hyperfunctioning lesions. In group 3, no differences in MR parameters were found between benign and malignant CCT, while MIBG uptake was significantly higher in malignant compared to benign tumors.
CONCLUSION: On the basis of our findings, nuclear imaging modalities using specific target agents are able to better characterize adrenal tumors, compared with MR. In particular, radionuclide techniques are able to identify the nature of adrenal incidentalomas and to differentiate between hypersecreting and non-hypersecreting adenomas as well as between benign and malignant CTT.
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Lenders JWM, Duh QY, Eisenhofer G, Gimenez-Roqueplo AP, Grebe SKG, Murad MH, Naruse M, Pacak K, Young WF. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab 2014; 99:1915-42. [PMID: 24893135 DOI: 10.1210/jc.2014-1498] [Citation(s) in RCA: 1715] [Impact Index Per Article: 155.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
OBJECTIVE The aim was to formulate clinical practice guidelines for pheochromocytoma and paraganglioma (PPGL). PARTICIPANTS The Task Force included a chair selected by the Endocrine Society Clinical Guidelines Subcommittee (CGS), seven experts in the field, and a methodologist. The authors received no corporate funding or remuneration. EVIDENCE This evidence-based guideline was developed using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system to describe both the strength of recommendations and the quality of evidence. The Task Force reviewed primary evidence and commissioned two additional systematic reviews. CONSENSUS PROCESS One group meeting, several conference calls, and e-mail communications enabled consensus. Committees and members of the Endocrine Society, European Society of Endocrinology, and Americal Association for Clinical Chemistry reviewed drafts of the guidelines. CONCLUSIONS The Task Force recommends that initial biochemical testing for PPGLs should include measurements of plasma free or urinary fractionated metanephrines. Consideration should be given to preanalytical factors leading to false-positive or false-negative results. All positive results require follow-up. Computed tomography is suggested for initial imaging, but magnetic resonance is a better option in patients with metastatic disease or when radiation exposure must be limited. (123)I-metaiodobenzylguanidine scintigraphy is a useful imaging modality for metastatic PPGLs. We recommend consideration of genetic testing in all patients, with testing by accredited laboratories. Patients with paraganglioma should be tested for SDHx mutations, and those with metastatic disease for SDHB mutations. All patients with functional PPGLs should undergo preoperative blockade to prevent perioperative complications. Preparation should include a high-sodium diet and fluid intake to prevent postoperative hypotension. We recommend minimally invasive adrenalectomy for most pheochromocytomas with open resection for most paragangliomas. Partial adrenalectomy is an option for selected patients. Lifelong follow-up is suggested to detect recurrent or metastatic disease. We suggest personalized management with evaluation and treatment by multidisciplinary teams with appropriate expertise to ensure favorable outcomes.
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Affiliation(s)
- Jacques W M Lenders
- Radboud University Medical Center (J.W.M.L.), 6500 HB Nijmegen, The Netherlands; VA Medical Center and University of California, San Francisco (Q.-Y.D.), San Francisco, California 94121; University Hospital Dresden (G.E.), 01307 Dresden, Germany; Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service de Génétique, (A.-P.G.-R.), F-75015 Paris, France; Université Paris Descartes (A.-P.G.-R.), F-75006 Paris, France; Mayo Clinic (S.K.G.G., M.H.M.), Rochester, Minnesota 55905; National Hospital Organisation Kyoto Medical Center (M.N.), Kyoto 612-8555; Japan; Eunice Kennedy Shriver National Institute of Child Health & Human Development (K.P.), Bethesda, Maryland 20892; and Mayo Clinic (W.F.Y.), Rochester, Minnesota 55905
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Sharma P, Singh H, Dhull VS, Suman KC S, Kumar A, Bal C, Kumar R. Adrenal masses of varied etiology: anatomical and molecular imaging features on PET-CT. Clin Nucl Med 2014; 39:251-260. [PMID: 24217551 DOI: 10.1097/rlu.0b013e3182a77c72] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
A wide spectrum of benign and malignant diseases can present as an adrenal mass. Combined PET-CT is useful for evaluation of adrenocortical and adrenomedullary masses. F-FDG has been extensively used as PET radiotracer for this purpose. F-FDOPA PET, Ga-DOTA peptide (Ga-DOTANOC/TATE) PET, and C-HED PET have also been used for imaging of adrenal medullary lesions, whereas C-MTO PET has been used for adrenocortical imaging. We provide a review of imaging characteristics of adrenal gland pathologies on PET-CT using different tracers.
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Affiliation(s)
- Punit Sharma
- From the Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India
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Sharma P, Dhull VS, Arora S, Gupta P, Kumar R, Durgapal P, Malhotra A, Chumber S, Ammini AC, Kumar R, Bal C. Diagnostic accuracy of (68)Ga-DOTANOC PET/CT imaging in pheochromocytoma. Eur J Nucl Med Mol Imaging 2014; 41:494-504. [PMID: 24158184 DOI: 10.1007/s00259-013-2598-1] [Citation(s) in RCA: 39] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/29/2013] [Accepted: 10/01/2013] [Indexed: 01/01/2023]
Abstract
PURPOSE 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. METHODS 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. RESULTS 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). CONCLUSION (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.
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Affiliation(s)
- Punit Sharma
- Department of Nuclear Medicine, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India
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Naswa N, Bal CS. Divergent role of (68)Ga-labeled somatostatin analogs in the workup of patients with NETs: AIIMS experience. Recent Results Cancer Res 2013; 194:321-51. [PMID: 22918767 DOI: 10.1007/978-3-642-27994-2_17] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/27/2022]
Abstract
Neuroendocrine tumors (NETs) encompass a wide range of rare and heterogeneous neoplasms arising from the neural crest. Diagnosis of NETs is conventionally done by a combination of common clinical symptoms and biochemical evidence of hormonal excess, which these tumors are known to secrete. After a diagnosis of NET is established, a search for its localization is carried out using common morphologic imaging methods such as ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI). The main problem with structural imaging is, however, its inability to distinguish between endocrine and exocrine lesions. Functional imaging of NETs started with use of iodine-131-meta-iodobenzylguanidine ((131)I-MIBG) and has come a long way since. From accurate demonstration of functioning tumors to detection of small and occult lesions, functional imaging has penetrated almost every aspect of NET management. Procedures such as (131/123)I-MIBG, (111)In-Octreoscan and others are rapidly giving way to use of PET/CT based on the superior resolution of the system and the availability of target-specific positron-emitting radiotracers. The availability of (68)Ga from generator-based radionuclide systems, namely (68)Ge/(68)Ga generators, opened up a new era of molecular imaging for NETs. A multitude of somatostatin analogs can be easily radioliganded with (68)Ga using heterocyclic macromolecular bifunctional chelating systems for targeted diagnosis of somatostatin receptor-expressing tumors, used most effectively to date for detection of NETs. This chapter focuses on our experience at the All India Institute of Medical Sciences, New Delhi regarding the divergent roles of (68)Ga-labeled somatostatin analogs in the workup of patients with NETs.
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Affiliation(s)
- Niraj Naswa
- Department of Nuclear Medicine, AIIMS, Ansari Nagar, New Delhi, India
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Imaging characterization of benign and malignant pheochromocytoma or paraganglioma: comparison between MIBG uptake and MR signal intensity ratio. Ann Nucl Med 2012; 26:670-5. [PMID: 22752959 DOI: 10.1007/s12149-012-0624-1] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/26/2012] [Accepted: 06/18/2012] [Indexed: 10/28/2022]
Abstract
OBJECTIVE We compared metaiodobenzylguanidine (MIBG) uptake and magnetic resonance (MR) signal intensity ratio in differentiating benign and malignant disease in patients with pheochromocytoma or paraganglioma. METHODS Eighteen patients (9 men, mean age 37 ± 8 years) with pheochromocytoma or paraganglioma underwent MR imaging and iodine-131 MIBG scintigraphy. MR signal intensity ratio was measured on T1 and T2-weighted images using region of interest analysis and intensity ratio of MIBG uptake was calculated for each tumor lesion on 48 h images. RESULTS A total of 28 tumor lesions was analyzed of which 12 were benign and 16 malignant. MIBG uptake intensity ratio was significantly higher in malignant lesions compared to benign (5.2 ± 2.4 and 2.9 ± 1.4, respectively, p < 0.01). On the contrary, no significant difference in tumor size and MR signal intensity ratio between malignant and benign tumor lesions was observed. CONCLUSIONS In patients with pheochromocytoma or paraganglioma, iodine-131 MIBG uptake is able to differentiate between benign and malignant disease, while MR imaging is not useful for this purpose. The higher MIBG uptake observed in malignant lesions could reflect major tumor storage of catecholamines compared to benign lesions.
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Amin MF, El Ameen NF. Diagnostic efficiency of multidetector computed tomography versus magnetic resonance imaging in differentiation of head and neck paragangliomas from other mimicking vascular lesions: comparison with histopathologic examination. Eur Arch Otorhinolaryngol 2012; 270:1045-53. [PMID: 22736054 DOI: 10.1007/s00405-012-2084-6] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/19/2012] [Accepted: 06/06/2012] [Indexed: 11/28/2022]
Abstract
The purpose of this study was to determine the efficiency of Multidetector Computed Tomography (MDCT) versus MRI in detection and characterization of paragangliomas and differentiating them from other vascular mimicking conditions in the neck and skull base in comparison with histo-pathological results as a gold standard. A prospective study included 30 patients with vascular neck lesions. They were susceptible for MDCT and MRI for characterization of the nature of the lesions. Histo-pathological evaluation was performed in all lesions for confirmation. As a result of this study included 30 patients: 22 males and 8 females. Paragangliomas were the commonest detectable lesions; 12/30 patient had glomus tumor (1 glomus tympanicum, 2 glomus vegale, 4 glomus jugulo-tempanicum, and 5 glomus jugular), 6 carotid body tumor, 2 hemangiopericytoma, 3 vegal Schwanoma, 4 lymphadenopathy, 2 juvenile angiofibroma, and one neurofibroma. The sensitivity of MDCT was higher than MRI in differentiation of paragangliomas from other mimicking lesions, where MDCT sensitivity was 83.33 % and the NPV was 80 % while that of MRI was 77.7 % and the NPV 75 %, but both techniques have moderate agreement between them in differentiating paragangliomas from other mimicking vascular lesion. MDCT with its new utilities has near degree of accuracy in detection and localization of paragangliomas as the same that of MRI. Both techniques have moderate agreement between them in differentiating paragangliomas from other mimicking vascular lesions. So, it is better to use both of them as complementary techniques for accurate diagnosis and grading of paraganglioma.
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Affiliation(s)
- Mohammed Farghally Amin
- Department of Radiodiagnosis, ElMinia University Hospital, ElMinya High Road, ElMinya, Egypt.
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Taffel M, Haji-Momenian S, Nikolaidis P, Miller FH. Adrenal imaging: a comprehensive review. Radiol Clin North Am 2012; 50:219-43, v. [PMID: 22498440 DOI: 10.1016/j.rcl.2012.02.009] [Citation(s) in RCA: 62] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
The discovery of an incidental adrenal mass (adrenal incidentaloma) continues to rise with the increasing use of cross-sectional imaging. Although most adrenal lesions are benign and asymptomatic, radiologists should guide evaluation of these lesions, whether benign or malignant. This article reviews the various imaging techniques used to evaluate adrenal masses and their relative strengths and weaknesses. It focuses on the most prevalent adrenal pathologies and their typical imaging characteristics, and concludes with a brief discussion of developing techniques, including diffusion-weighted imaging and dual-energy CT.
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Affiliation(s)
- Myles Taffel
- Department of Radiology, George Washington University Medical Center, Washington, DC, USA.
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Naswa N, Sharma P, Nazar AH, Agarwal KK, Kumar R, Ammini AC, Malhotra A, Bal C. Prospective evaluation of ⁶⁸Ga-DOTA-NOC PET-CT in phaeochromocytoma and paraganglioma: preliminary results from a single centre study. Eur Radiol 2012; 22:710-719. [PMID: 21971823 DOI: 10.1007/s00330-011-2289-x] [Citation(s) in RCA: 52] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/05/2011] [Revised: 08/21/2011] [Accepted: 08/23/2011] [Indexed: 01/08/2023]
Abstract
OBJECTIVE 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. METHODS 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. RESULTS 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. CONCLUSION (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. KEY POINTS • ( 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.
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Affiliation(s)
- Niraj Naswa
- Department of Nuclear Medicine, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India
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A paraganglioma of the organ of Zuckerkandl. UROLOGICAL SCIENCE 2012. [DOI: 10.1016/j.urols.2011.12.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022] Open
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Kastelan D. Management of adrenal incidentaloma. Expert Rev Endocrinol Metab 2011; 6:811-817. [PMID: 30780866 DOI: 10.1586/eem.11.75] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/08/2022]
Abstract
The term adrenal incidentaloma covers a wide spectrum of adrenal pathologies sharing the method of discovery. The management of adrenal incidentaloma is controversial, and clinicians should determine whether the patient's health is affected by the presence of a tumor. Critical points in management include differentiating benign from malignant and hormonally active from nonfunctional adrenal masses. The probability of malignancy is related to tumor size and imaging phenotype. Masses smaller than 4 cm in diameter that exhibit low attenuation by unenhanced CT scan and rapid contrast washout by delayed contrast-enhanced CT scan are probably benign. All patients with adrenal incidentaloma should undergo biochemical evaluations for pheochromocytoma and subclinical or overt Cushing's syndrome, and hypertensive patients should undergo biochemical testing for hyperaldosteronism. Laparoscopic adrenalectomy is the treatment of choice in all hypersecretory tumors. If adrenocortical carcinoma is suspected, an open adrenalectomy is recommended. Patients who are not candidates for surgical resection of the tumor should be monitored radiographically for 2 years, and hormonal follow-up should be performed annually for 4 years.
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Affiliation(s)
- Darko Kastelan
- a School of Medicine, University of Zagreb, Zagreb, Croatia and Department of Endocrinology, University Hospital Zagreb, Zagreb, Croatia.
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Use of Fluorine-18-Labelled Deoxyglucose Positron Emission Tomography with Computed Tomography to Localize a Paraganglioma in Pregnancy. South Med J 2010; 103:1238-42. [DOI: 10.1097/smj.0b013e3181eda0de] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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Benhammou JN, Boris RS, Pacak K, Pinto PA, Linehan WM, Bratslavsky G. Functional and oncologic outcomes of partial adrenalectomy for pheochromocytoma in patients with von Hippel-Lindau syndrome after at least 5 years of followup. J Urol 2010; 184:1855-9. [PMID: 20846682 PMCID: PMC3164541 DOI: 10.1016/j.juro.2010.06.102] [Citation(s) in RCA: 46] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/25/2010] [Indexed: 01/20/2023]
Abstract
PURPOSE Although the safety and feasibility of partial adrenalectomy in patients with von Hippel-Lindau syndrome have been established, long-term outcomes have not been examined. In this study we evaluate the recurrence and functional outcomes in a von Hippel-Lindau syndrome cohort treated for pheochromocytoma with partial adrenalectomy with a followup of at least 5 years. MATERIALS AND METHODS We reviewed the records of patients with von Hippel-Lindau syndrome treated with partial adrenalectomy for pheochromocytoma at the National Cancer Institute. Demographic, germline mutation status, surgical indication, oncologic and functional outcome data were collected. Local recurrence was defined as radiographic evidence of recurrent tumor on the ipsilateral side of partial adrenalectomy. Patients were considered steroid dependent if they required steroids at most recent followup. RESULTS A total of 36 partial adrenalectomies for pheochromocytoma were performed in 26 patients with von Hippel-Lindau syndrome between September 1995 and December 2003. Of these cases 23 were performed open and 13 were performed laparoscopically. Prior surgical history was obtained for all patients. At a median followup of 9.25 years (range 5 to 46) metastatic pheochromocytoma had not developed in any patients. In 3 patients (11%) there were 5 local recurrences treated with surgical extirpation or active surveillance. All recurrences were asymptomatic and detected by radiographic imaging on followup. In addition, 3 of 26 patients (11%) subsequently required partial adrenalectomy for pheochromocytoma on the contralateral adrenal gland. In the entire cohort only 3 patients became steroid dependent (11%). CONCLUSIONS Outcomes of partial adrenalectomy in patients with von Hippel-Lindau syndrome with pheochromocytoma are encouraging at long-term followup and should be recommended as a primary surgical approach whenever possible. Adrenal sparing surgery can obviate the need for steroid replacement in the majority of patients. Local recurrence rates appear to be infrequent and can be managed successfully with subsequent observation or intervention.
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Affiliation(s)
| | | | | | | | | | - Gennady Bratslavsky
- Correspondence: Gennady Bratslavsky, M.D., Urologic Oncology Branch, National Cancer Institute, Building 10 Room 1-5940, Bethesda, Maryland 20892-1107, Tel: 301 496-6353, Fax: 301 402-0922,
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Magnetic resonance characterization of pheochromocytomas in the abdomen and pelvis: imaging findings in 18 surgically proven cases. J Comput Assist Tomogr 2010; 34:548-53. [PMID: 20657223 DOI: 10.1097/rct.0b013e3181d529f2] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
PURPOSE To study the magnetic resonance imaging characteristics of adrenal and extra-adrenal pheochromocytomas in the abdomen and pelvis. METHODS We retrospectively reviewed 18 cases of pathologically proven cases of pheochromocytomas in the abdomen and pelvis. These patients have undergone magnetic resonance imaging evaluation before surgery. The study population included 10 men and 7 women (age range, 19-68 years; mean, 38 years). A consensus review of the magnetic resonance images was performed by 2 blinded expert observers. A qualitative evaluation was completed, and the tumors were classified by anatomical location, shape, T2 signal, contrast enhancement, and signal dropout on chemical shift pulse sequences. RESULTS On T2-weighted images, most lesions demonstrated mild to moderate increased signal intensity (SI) (n = 12), 5 lesions demonstrated a markedly increased SI, and only 1 lesion demonstrated an isointense SI on T2-weighted images.Five lesions demonstrated marked postcontrast enhancement. Three lesions demonstrated moderate enhancement, and 5 lesions demonstrated mild postcontrast enhancement.The pattern of enhancement was variable: 4 salt and pepper, 4 homogeneous, 3 heterogeneous, and 2 target with central necrosis and hemorrhage. None of the lesions contained significant amount of intracellular lipid, as no lesions demonstrated greater than 16.5% signal dropout on out-of-phase compared with in-phase pulse sequences.
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Surgical treatment of cardiac pheochromocytoma: a case report. Ann Thorac Surg 2009; 88:278-81. [PMID: 19559246 DOI: 10.1016/j.athoracsur.2008.11.029] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/06/2008] [Revised: 11/01/2008] [Accepted: 11/13/2008] [Indexed: 11/27/2022]
Abstract
Primary cardiac pheochromocytoma is an extremely rare neoplasm. We report a 15-year-old girl who was presented with paroxysmal hypertension. An iodine-131 metaiodobenzylguanidine scintigraphy scanning showed a pheochromocytoma in her right atrial and ventricular wall. The tumor was subsequently confirmed by magnetic resonance imaging and coronary angiogram. This patient underwent a successful surgical resection of the tumor, a reconstruction of the atrial ventricular wall and right coronary artery bypass grafting. Her blood pressure remained normal thereafter. A follow-up coronary angiogram revealed a patent saphenous vein graft 4 months after the operation.
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Re: Adrenal pheochromocytoma: correlation of MRI appearances with histology and function. Eur Radiol 2009; 19:2310-1; author reply 2312-3. [PMID: 19367407 DOI: 10.1007/s00330-009-1387-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/16/2009] [Accepted: 03/12/2009] [Indexed: 10/20/2022]
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Jacques AET, Sahdev A, Sandrasagara M, Goldstein R, Berney D, Rockall AG, Chew S, Reznek RH. Adrenal phaeochromocytoma: correlation of MRI appearances with histology and function. Eur Radiol 2008; 18:2885-92. [PMID: 18641999 DOI: 10.1007/s00330-008-1073-z] [Citation(s) in RCA: 93] [Impact Index Per Article: 5.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/05/2008] [Revised: 05/01/2008] [Accepted: 05/14/2008] [Indexed: 12/14/2022]
Abstract
The purpose of this study was to describe the range of appearances of adrenal phaeochromocytomas on T2-weighted MRI, correlate appearances with histopathology, and quantify the incidence of the previously described hyperintense appearance. The appearance and MR characteristics of 44 phaeochromocytomas were reviewed retrospectively. T2-weighted appearances were grouped: (1) 'classical', homogeneous, high signal intensity, isointense to CSF; (2) homogeneous, isointense or minimally hyperintense to spleen, hypointense to CSF; (3) heterogeneous, marbled appearance; (4) heterogeneous, multiple high signal intensity pockets. All 44 adrenal phaeochromocytomas were well circumscribed, 1.2-15 cm in maximum diameter, with no visual or quantitative signal loss on chemical shift imaging. On T2-weighted MRI 5/44 (11%) had group 1 appearance; 15/44 (34%) group 2, 7/44 (16%) group 3; and 17/44 (39%) group 4. Homogeneous group 1 and 2 lesions were smaller (mean 4.5 cm) than heterogeneous group 3 and 4 lesions (mean 6.3 cm). Increasing MRI heterogeneity correlated pathologically with increasing amounts of haemorrhage, necrosis and fibrosis. No MRI features were predictive of malignancy. Non-functioning phaeochromocytomas were larger than functioning lesions. No size difference was seen between syndrome and sporadic lesions. In this large series we report a wide range of appearances of adrenal phaeochromocytomas on T2-weighted MRI. The previously described classical hyperintense phaeochromocytoma is relatively uncommon.
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Affiliation(s)
- Audrey E T Jacques
- Cancer Imaging, St Bartholomew's Hospital, West Smithfield, London, EC1A 7BE, UK.
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Lekanidi K, Koumellis P, Bungay P, Birchall JD. An organ of Zuckerkandl tumour shown by MRA (2007: 12b). Eur Radiol 2008; 18:639-42. [PMID: 18264736 DOI: 10.1007/s00330-007-0656-4] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/30/2007] [Accepted: 04/02/2007] [Indexed: 11/29/2022]
Abstract
Pheochromocytomas are catecholamine-releasing tumors that can be found in an extraadrenal location in 10% of the cases. Almost half of all pheochromocytomas are now discovered incidentally during cross-sectional imaging for unrelated causes. We present a case of a paragaglioma of the organ of Zuckerkandl that was discovered incidentally during a magnetic resonance angiogram performed for intermittent claudication. Subsequent investigation with computed tompgraphy and I-123 metaiodobenzylguanine scintigraphy as well as an overview of the literature are also presented.
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Affiliation(s)
- Katerina Lekanidi
- Department of General Medicine, University Hospitals of Leicester, Leicester General Hospital, Gwendolen Road, Leicester, LE5 4PW, UK.
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Abstract
Paragangliomas are neoplasms that arise from neural crest cells and histologically resemble their adrenal counterpart, the phaeochromocytoma. The majority of extra-adrenal tumours develop within the abdomen and are associated with the coeliac, superior and inferior mesenteric ganglia, which run parallel to the aorta. The organ of Zuckerkandl origin is most common. Pararectal paragangliomas are extremely rare. This case report presents ultrasound, magnetic resonance and histological features of such a case.
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Affiliation(s)
- S Bhatt
- Department of Imaging Sciences, University of Rochester School of Medicine, Rochester, NY 14642, USA
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Mittendorf EA, Evans DB, Lee JE, Perrier ND. Pheochromocytoma: advances in genetics, diagnosis, localization, and treatment. Hematol Oncol Clin North Am 2007; 21:509-25; ix. [PMID: 17548037 DOI: 10.1016/j.hoc.2007.04.012] [Citation(s) in RCA: 47] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
Pheochromocytomas are rare, catecholamine-secreting tumors arising most frequently in the chromaffin cells of the adrenal glands. Recent studies have suggested that genetic mutations are more frequent than previously appreciated in patients with these lesions. Advances continue to be made not only in the genetic evaluation of these patients but also in the biochemical confirmation and tumor localization. Surgery remains the definitive treatment, and advances in laparoscopic techniques as well as cortical-sparing procedures have reduced the morbidity associated with tumor resection.
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Affiliation(s)
- Elizabeth A Mittendorf
- Department of Surgical Oncology, The University of Texas M. D. Anderson Cancer Center, Unit 444, Houston, TX 77030, USA
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Mizuta E, Hamada T, Taniguchi SI, Shimoyama M, Nawada T, Miake J, Kaetsu Y, Peili L, Ishiguro K, Ishiguro S, Igawa O, Shigemasa C, Hisatome I. Small extra-adrenal pheochromocytoma causing severe hypertension in an elderly patient. Hypertens Res 2006; 29:635-8. [PMID: 17139794 DOI: 10.1291/hypres.29.635] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
We report the case of a 67-year-old woman with severe hypertension caused by an extra-adrenal pheochromocytoma. The tumor was detected by 131I metaiodobenzylguanidine scintigraphy and it was found to be small (2 cm ø) by enhanced CT. After the extirpation of the tumor, the blood pressure of the patient immediately normalized. It should be taken into account that a small extra-adrenal pheochromocytoma can be one of the causes of secondary hypertension in elderly patients. Since small extra-adrenal pheochromocytomas are difficult to detect, it is also important to perform suitable examinations to establish the diagnosis. Furthermore, we emphasize the importance of an accurate diagnosis in elderly patients with pheochromocytoma, for they often have less symptomatology and more severe cardiovascular complications due to refractory hypertension than younger patients.
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Affiliation(s)
- Einosuke Mizuta
- Department of Genetic Medicine and Regenerative Medicine and Therapeutics, Institute of Regenerative Medicine and Biofunction, Tottori University Graduate School of Medical Science, Yonago, Japan.
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Lee KY, Oh YW, Noh HJ, Lee YJ, Yong HS, Kang EY, Kim KA, Lee NJ. Extraadrenal paragangliomas of the body: imaging features. AJR Am J Roentgenol 2006; 187:492-504. [PMID: 16861555 DOI: 10.2214/ajr.05.0370] [Citation(s) in RCA: 152] [Impact Index Per Article: 8.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
Abstract
OBJECTIVE This article illustrates the anatomic distribution of the healthy paraganglion system and the imaging and pathologic features of various extraadrenal paragangliomas occurring in the head and neck, chest, and abdomen. CONCLUSION Although paragangliomas can occur in a variety of anatomic locations, the majority are seen in relatively predictable regions of the body. Extraadrenal paragangliomas have nearly identical imaging features, including a homogeneous or heterogeneous hyperenhancing soft-tissue mass at CT, multiple areas of signal void interspersed with hyperintense foci (salt-and-pepper appearance) within tumor mass at MRI, and an intense tumor blush with enlarged feeding arteries at angiography.
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Affiliation(s)
- Ki Yeol Lee
- Department of Radiology, Korea University Anam Hospital, College of Medicine Korea University; #126-1, 5-Ka, Anam-Dong, Sungbuk-Ku, Seoul 136-705, Korea
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Grossman A, Pacak K, Sawka A, Lenders JWM, Harlander D, Peaston RT, Reznek R, Sisson J, Eisenhofer G. Biochemical Diagnosis and Localization of Pheochromocytoma: Can We Reach a Consensus? Ann N Y Acad Sci 2006; 1073:332-47. [PMID: 17102103 DOI: 10.1196/annals.1353.038] [Citation(s) in RCA: 67] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022]
Abstract
Pheochromocytomas can have a highly variable presentation, making diagnosis challenging. To think of the tumor represents the crucial initial step, but establishing the diagnosis requires biochemical evidence of excessive catecholamine production and imaging studies to localize the source. Currently, however, there exist no generally agreed upon guidelines based on which tests and testing algorithms should be used to confirm and locate or exclude a suspected pheochromocytoma. Choice of biochemical tests and imaging studies instead usually depends on institutional experience. At the First International Symposium on Pheochromocytoma (ISP2005), held in Bethesda in October 2005, a panel of experts and patient representatives discussed current problems and available options for tumor diagnosis and localization and formulated recommendations, which were subsequently agreed upon by those in attendance at the meeting. This article summarizes the discussion and recommendations derived from that session.
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Affiliation(s)
- Ashley Grossman
- Department of Endocrinology, St. Bartholomew's Hospital, West Smithfield, London, EC1A 7BE, UK, and Department of Medicine, University of Toronto, Ontario, Canada.
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Bhansali A, Rajput R, Behra A, Rao KLN, Khandelwal N, Radotra BD. Childhood sporadic pheochromocytoma: clinical profile and outcome in 19 patients. J Pediatr Endocrinol Metab 2006; 19:749-56. [PMID: 16789642 DOI: 10.1515/jpem.2006.19.5.749] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
Sporadic pheochromocytoma is a rare tumor of childhood and accounts for less than 1% of cases of hypertension. We describe the presentation and outcome of 19 adolescents with sporadic pheochromocytoma seen over past 10 years at a tertiary care center in north India. The mean age (+/- SD) at presentation was 15.1 +/- 2.4 years with range from 9-18 years. The male to female ratio was 12:7. The lag time between onset of symptoms to diagnosis ranged from 1 month to 5 years with mean (+/- SD) of 1.09 +/- 1.02 years. The majority of children presented with hypertension and paroxysms. Paroxysms, characterized by the triad of headache, palpitations and sweating, was present in 13 (68%) of these patients. Twelve (63%) patients had postural fall in blood pressure, ten (53%) had abdominal pain, four (21%) had visual blurring, and three (16%) each had palpable abdominal mass and significant weight loss at presentation. Nausea and vomiting are common symptoms in children with pheochromocytoma and were present in six (32%) and three (16%) patients, respectively. Café-au-lait macule was present in only two (11%) patients. Urinary vanilyl mandelic acid (VMA) was found to be significantly high in ten (53%) patients, and urinary epinephrine and norepinephrine in eight (42%). Six (32%) patients had both VMA and urinary epinephrine and norepinephrine within normal limits and five (26%) had significant elevation of both. The tumor was localized by ultrasonography in 17 (89%) patients and by computed tomography in 18 (95%), and in one patient it was localized by 131I-MIBG scan. Sixteen (84%) patients had adrenal pheochromocytoma (including four with bilateral masses), while the remaining three (16%) had abdominal extra-adrenal pheochromocytoma originating from sympathetic ganglions. The mean (+/- SD) diameter of the tumor was 4.4 +/- 1.7 cm, ranging from 2.2-7.5 cm. Pre-operatively, hypertension was managed by phenoxybenzamine in six (32%), sustained release prazosin in 12 (63%), beta-blockers in 14 (74%), calcium channel blockers in 12 (63%), and angiotensin converting enzyme inhibitors and diuretics in only two (11%) patients. Eighteen (85%) patients underwent exploratory laparotomy for removal of the tumor. On follow-up, 13 (72%) patients became normotensive, while six (32%) patients continued to have hypertension. In conclusion, childhood pheochromocytoma is characterized by atypical symptomatology; ultrasonography is a useful modality in localizing the lesions in the majority of patients; surgery is rewarding in most patients.
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Affiliation(s)
- A Bhansali
- Department of Endocrinology, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.
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Guller U, Turek J, Eubanks S, Delong ER, Oertli D, Feldman JM. Detecting pheochromocytoma: defining the most sensitive test. Ann Surg 2006; 243:102-7. [PMID: 16371743 PMCID: PMC1449983 DOI: 10.1097/01.sla.0000193833.51108.24] [Citation(s) in RCA: 74] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
OBJECTIVE To define the most sensitive biochemical test to establish the diagnosis of pheochromocytoma and also to assess the potential role of iodine 131-labeled metaiodobenzylguanidine scintigraphy (I-MIBG) in the diagnosis of this tumor. SUMMARY BACKGROUND DATA Pheochromocytoma is a rare, catecholamine-producing tumor with preferential localization in the adrenal gland. Despite its importance, the most sensitive test to establish the diagnosis remains to be defined. METHODS Prospective data collection was done on patients with pheochromocytoma treated at the Duke University Medical Center and the Durham Veterans Affairs Medical Center, Durham, NC. All urinary, plasma, and platelet analyses were highly standardized and supervised by one investigator (J.M.F.). I-MIBG scans were independently reviewed by 2 nuclear medicine physicians. RESULTS A total of 152 patients (55.3% female) were enrolled in the present analysis. Patients were predominantly white (73.7%). Spells (defined as profuse sweating, tachycardia, and headache) and hypertension at diagnosis were present in 51.4% and 66.6%, respectively. Bilateral disease was found in 12.5%, malignant pheochromocytoma in 29.6%, and hereditary forms in 23.0%. The most sensitive tests were total urinary normetanephrine (96.9%), platelet norepinephrine (93.8%), and I-MIBG scintigraphy (83.7%). In combination with I-MIBG scintigraphy, platelet norepinephrine had a sensitivity of 100%, plasma norepinephrine/MIBG of 97.1%, total urine normetanephrine/MIBG of 96.6%, and urine norepinephrine/MIBG of 95.3%. CONCLUSIONS The tests of choice to establish the diagnosis of pheochromocytoma are urinary normetanephrine and platelet norepinephrine. A combination of I-MIBG scintigraphy and diagnostic tests in urine, blood, or platelets does further improve the sensitivity. We thus advocate performing an MIBG scan if the diagnosis of pheochromocytoma is clinically suspected and catecholamine measurements are within the normal range.
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Affiliation(s)
- Ulrich Guller
- Department of Surgery, Divisions of General Surgery and Surgical Research, University Hospital Basel, Basel, Switzerland.
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Sabharwal GK, Strouse PJ. Posterior mediastinal hemangioma. Pediatr Radiol 2005; 35:1263-6. [PMID: 16163502 DOI: 10.1007/s00247-005-1571-y] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/30/2005] [Revised: 07/16/2005] [Accepted: 07/21/2005] [Indexed: 10/25/2022]
Abstract
We report posterior mediastinal hemangiomas in a 4-month-old and a 6-month-old girl. The masses were identified on radiographs of the chest followed by contrast-enhanced CT. Histological evaluation of the surgical specimens established the final diagnosis. Although mediastinal hemangiomas have been described, they remain a rare entity. A diagnosis can be suggested by relatively high attenuating masses on contrast-enhanced CT. Posterior mediastinal hemangiomas sometimes mimic neuroblastomas, which is the most common posterior mediastinal in this age group.
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Affiliation(s)
- Gauravi K Sabharwal
- Section of Pediatric Radiology, C.S. Mott Children's Hospital, University of Michigan Health System, 1500 East Medical Center Drive, Ann Arbor, MI 48109-0252, USA.
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Lin DD, Loughlin KR. Diagnosis and management of surgical adrenal diseases. Urology 2005; 66:476-83. [PMID: 16140061 DOI: 10.1016/j.urology.2005.03.010] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/17/2004] [Revised: 02/03/2005] [Accepted: 03/02/2005] [Indexed: 11/22/2022]
Affiliation(s)
- Darlene D Lin
- Department of Urology, Brigham and Women's Hospital, Boston, Massachusetts 02115, USA.
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Brink I, Hoegerle S, Klisch J, Bley TA. Imaging of pheochromocytoma and paraganglioma. Fam Cancer 2005; 4:61-8. [PMID: 15883712 DOI: 10.1007/s10689-004-2155-y] [Citation(s) in RCA: 101] [Impact Index Per Article: 5.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/13/2004] [Accepted: 03/04/2004] [Indexed: 11/26/2022]
Abstract
Paragangliomas are tumours that arise within the sympathetic nervous system originating from the neural crest. These tumours can be found anywhere from the neck to the pelvis in locations of sympathetic ganglions. Although in the majority of paragangliomas the diagnosis is based on measuring catecholamines and metabolites in plasma or urine, imaging plays an important preoperative role. Today, there are several morphological and radionuclide imaging methods available that predict tumour localisation and tumour extent and give anatomic information to the surgeon. MRI is the morphological imaging modality of choice in localising pheochromocytomas and extra-adrenal paragangliomas. It provides excellent anatomic detail and has the advantage of lacking ionising radiation. The overall accuracy of computed tomography (CT) in detecting primary adrenal pheochromocytomas is very high, but CT lacks in specificity as difficulties may occur in distinguishing between paragangliomas and other tumour entities. The major advantages of radionuclide imaging are very high specificity and routinely performed whole-body scanning. Furthermore, metabolic imaging is not influenced by artifacts like scar tissue or metallic clips in post-surgical follow-up. Currently, a reported specificity of 99% and a cumulative sensitivity of about 90% in paragangliomas make (123)I-MIBG the most important nuclear imaging method. However, (18)F-DOPA-PET seems to be a very promising procedure which offers higher accuracy. The higher spatial resolution of PET-scanners enables the detection of small lesions not visualised with (123)I-MIBG. Both use of radiolabelled somatostatin analogue like (111)In-pentetreotide and (18)F-FDG is limited due to low specificity of the tracers and should be restricted to MIBG- and F-DOPA-negative cases.
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Affiliation(s)
- I Brink
- Division of Nuclear Medicine, University Hospital Freiburg, Hugstetterstr. 55, 79106 Freiburg, Germany.
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Daneman D, Daneman A. Diagnostic imaging of the thyroid and adrenal glands in childhood. Endocrinol Metab Clin North Am 2005; 34:745-68, xi. [PMID: 16085169 DOI: 10.1016/j.ecl.2005.04.006] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/25/2022]
Abstract
This article reviews the clinical utility of modern imaging techniques, particularly ultrasound (US), CT, and MRI in children with disorders of the thyroid and adrenal glands. Radionuclide scanning is the modality of choice in making the anatomic diagnosis in neonates with congenital hypothyroidism, while US is most useful in defining nodular thyroid disease. CT and MRI of the thyroid tend to be limited to defining the extent of thyroid carcinoma. Adrenal US is an essential step in the differential diagnosis of ambiguous genitalia or salt-losing crises in the newborn, while CT and MRI are more useful in defining the anatomy of the adrenals in older children with tumors or diffuse hyperplasia of the glands.
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Affiliation(s)
- Denis Daneman
- Department of Pediatrics, Hospital for Sick Children, Toronto, ON, Canada.
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Abstract
Technological developments in cross-sectional imaging have revolutionized the localization and characterization of functioning adrenal pathology. With effective use of modern imaging, the diagnosis of the cause and nature of functioning adrenal pathology can be reached speedily, accurately and efficiently in the majority of patients. We review the appearance of primary and secondary adrenal pathology, evaluate the diagnostic performance of imaging modalities, highlight newer technical developments, and propose a rational use of these tests in identifying functioning adrenal disease.
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Affiliation(s)
- S A Sohaib
- Department of Diagnostic Radiology, Royal Marsden Hospital, Fulham Road, London SW3 6JJ, UK.
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Affiliation(s)
- L Fernández-Cruz
- Department of Surgery, Institute of Digestive and Metabolic Diseases, Hospital Clinic, Barcelona, Spain.
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47
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Abstract
Evaluation of a functional adrenal mass may be initiated based on symptomatic presentation or the detection of an incidental adrenal mass. Recent literature suggests that 10% to 20% of adrenal incidentalomas demonstrate subclinical hormonal dysfunction, which may place patients at a higher risk for metabolic or cardiovascular disorders. Many diagnostic algorithms have been proposed for the evaluation of pheochromocytoma, Cushing's adenoma, aldosteronoma, and hormonally active adrenal cortical carcinoma. In this article, the available literature on functional adrenal masses is reviewed and up-to-date methods of efficient diagnosis are proposed.
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Affiliation(s)
- Michael D Stifelman
- New York University School of Medicine, 150 East 32nd Street, New York, NY 10016, USA.
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Abstract
Phaeochromocytomas of the heart are very rare. This report describes the case of a 69 year old woman presenting with persistent hypertension and a left atrial phaeochromocytoma diagnosed by 131I-metaiodobenzylguanidine scintigraphy scanning. She was successfully treated by surgical excision with the aid of cardiopulmonary bypass and perioperative alpha and beta adrenergic blockade.
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Affiliation(s)
- N Moorjani
- Department of Cardiothoracic Surgery, Derriford Hospital, Plymouth PL6 8DH, UK.
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Abstract
MR imaging is used commonly for imaging the adrenal glands. Its high-contrast resolution and multiplanar imaging capability enables the detection and characterization of many adrenal masses. The advent of chemical-shift imaging revolutionized the role of MR imaging in characterizing adrenal masses. In this article, the authors discuss the range of MR appearances of common and uncommon adrenal masses, focusing on the nonfunctioning incidentally discovered mass and its characterization methods. MR imaging is continuously improving. The increasing use of higher strength magnets and the introduction of newer coils, sequences, and techniques will help detect and characterize very small adrenal masses, quantify their fat content, and provide exquisite morphologic images of the gland and its vascular supply.
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Affiliation(s)
- Hero K Hussain
- Department of Radiology/MRI B2B311, University of Michigan Hospitals, 1500 East Medical Center Drive, Ann Arbor, MI 48109, USA.
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Sahdev A, Sohaib A, Monson JP, Grossman AB, Chew SL, Reznek RH. CT and MR imaging of unusual locations of extra-adrenal paragangliomas (pheochromocytomas). Eur Radiol 2004; 15:85-92. [PMID: 15290072 DOI: 10.1007/s00330-004-2412-3] [Citation(s) in RCA: 84] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/13/2004] [Accepted: 06/07/2004] [Indexed: 11/25/2022]
Abstract
Our review was undertaken to describe CT and MRI features of unusual extra-adrenal paragangliomas (pheochromocytomas). We retrospectively reviewed CT and MRI findings in 29 patients with 39 extra-adrenal paragangliomas. For each tumour, site, size, MRI characteristics, CT appearances and enhancement after gadolinium and iohexol were recorded. There were 17 carotid body tumours, 1 mediastinal, 1 intra-cardiac, 15 retroperitoneal extra-adrenal paragangliomas, 2 bladder, 1 pelvic sidewall and 2 intra-spinal paragangliomas within the lumbo-sacral spine. All 39 paragangliomas were shown on MRI. Of the 32 lesions studied by MRI and CT, CT detected 30. Of the two lesions missed on CT, one was an intra-cardiac paraganglioma and the second a bladder wall paraganglioma. At detection, 25 tumours were larger than 4 cm, of which 20 were heterogeneous lesions on CT and MRI with variable contrast enhancement. The 14 smaller paragangliomas were smooth in contour and demonstrated avid, homogeneous contrast enhancement. Our review of extra-adrenal paragangliomas highlights their unusual sites and appearances. MRI demonstrated the greatest variability in the appearances of larger tumours, provided additional information compared to CT for surgical planning and is a useful screening tool for patients at high risk of extra-adrenal paragangliomas.
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Affiliation(s)
- Anju Sahdev
- Department of Radiology, St Bartholomew's Hospital, West Smithfield, London, EC1A 7BE, UK.
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