Sympathetic Paraganglioma: A Single-Center Experience from Western India |
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| Institution: | 1. Department of Endocrinology, Seth G.S. Medical College & KEM Hospital, Mumbai, India;2. Department of Endocrinology, Narayana Medical College, Nellore, Andhra Pradesh, India;3. Department of Uro-oncology, Tata Memorial Hospital, Mumbai, India;4. Department of General Surgery, Seth G.S. Medical College & KEM Hospital, Mumbai India.;1. Centre for Inherited Cardiovascular Diseases, Transplant Research Area;2. Cardiac Surgery, University Hospital Policlinico San Matteo, Pavia, Italy;3. Cardiovascular Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, Tokyo, Japan;4. Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA;5. Surgery, Fondazione IRCCS, University Hospital Policlinico San Matteo, Pavia, Italy;1. Endocrinology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.;2. Radiodiagnosis, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.;3. Dermatology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.;1. Department of Neurosurgery, Huashan Hospital, Shanghai, China; Harvard Medical School, 25 Shattuck Street, Boston, MA 02115 |
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| Abstract: | Objective: Most of the Indian studies on pheochromocytoma/paraganglioma (PCC/PGL) have focused on PCC, and there is a paucity of information regarding sympathetic paraganglioma (sPGL). Here, we describe the clinical, biochemical, and imaging features of sPGL compared with PCC.Methods: This retrospective study included 75 patients with sPGL and 150 patients with PCC. Diagnosis of PCC/PGL was based on surgical histopathology, and if histopathology was not available, on biochemistry and/or radiology.Results: sPGL was more frequently detected incidentally (P = .03), normetanephrine-secreting (P<.01), and metastatic compared with PCC (P≤.01). sPGL was most commonly located in the organ of Zuckerkandl (OOZ) (49%) and infradiaphragmatic area above the OOZ (27%). Patients with mediastinal sPGL were significantly older than those with sPGL in the OOZ (P = .03). Primary tumors of metastatic sPGL were significantly larger than those without metastasis (7.8 ± 4 cm vs. 5.6 ± 3.2 cm; P = .004). Percentage arterial enhancement (PAE) >100% was seen in 98% of sPGLs.Conclusion: Incidental presentation, normetanephrine-secreting phenotype, and metastatic disease were more frequent in patients with sPGL than those with PCC. sPGL arose most commonly in the OOZ. Tumor size is an independent predictor of malignancy among sPGL patients. PAE >100% is almost a universal finding in sPGL, and its absence is a sensitive parameter to differentiate sPGL from other abdominal masses.Abbreviations: AP = arterial phase; CECT = contrast-enhanced computed tomography; CT = computed tomography; DP = delayed phase; EVP = early venous phase; FDG = fluorodeoxyglucose; fPFMN = fractionated plasma free metanephrine; HU = Hounsfield units; MIBG = metaiodobenzylguanidine; MRI = magnetic resonance imaging; OOZ = organ of Zuckerkandl; PAE = percentage arterial enhancement; PCC = pheochromocytoma; PET = positron emission tomography; PFNMN = plasma free normetanephrine; PGL = paraganglioma; PRRT = peptide receptor radionuclide therapy; PVE = percentage venous enhancement; sPGL = sympathetic paraganglioma; UP = unenhanced phase; VMA = vanillyl mandelic acid |
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