Natural History and Management of Familial Paraganglioma Syndrome Type 1: Long-Term Data from a Large Family
Abstract
:1. Introduction
2. Patients and Methods
3. Results
3.1. Family Case Series
3.2. Clinical Presentation and Diagnosis
3.3. Imaging Work up
3.4. Treatment and Follow-up
4. Discussion
4.1. Natural History
4.2. Current Diagnostic Work-up
4.2.1. The Role of Serum and Urinary Markers
4.2.2. The Role of Magnetic Resonance Imaging
4.2.3. The Role of Ultrasonography
4.2.4. Functional Imaging: “Back to the Future”
4.3. Our Novel Insight for A Precision Diagnostic Algorithm
4.4. Therapy
5. Conclusions
Author Contributions
Funding
Conflicts of Interest
References
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Patient | Demographic Parameters: (1) Sex (2) Age at Clinical dx (yrs) (3) Age at Genetic dx (yrs) (4) Age at Last FU (yrs) | FU from Clinical Diagnosis (Months) | Tumor Lesions: (1) Number (2) Site | Size at Diagnosis (mm) | Basal Morphological Imaging | Urinary Fractionated Metanephrines | Histology | Surgery | Medical Therapy | Follow-up |
---|---|---|---|---|---|---|---|---|---|---|
Case 1 (T.D.) | (1) Female (2) 40 (3) 41 (4) 48 | 91 | (1) 3 (2) a: left carotid bifurcation b: right carotid bifurcation c: mediastinum | a: 18 b: 6 c: 38 | - echography - CT angiography - MR | M: 111 µg/24 h (50–340), N: 312 µg/24 (90–445) | a: PGL NSE+, synaptophysin +, CgA+, S100+; b:- c: PGL, synaptophysin +, CgA+, Ki67 < 1%, R1 | a: yes, 2013 b: no c: yes, 2013 after embolization and subsequent radiotherapy | yes, OCT LAR 30 mg every 28 days | - lesions operated (a,c): no persistence, no relapse - lesion not operated (b): 4 mm growth in 7 yrs at CT |
Case 2 (T.E.) | (1) M (2) 44 (3) 48 (4) 48 | 44 | (1) 4 (2) a: left laterocervical b: right carotid bifurcation c: right jugular foramen d: cervical | a: NA b: 6 c: NA d: NA | - echography - CT angiography (c and d lesion were detected exclusively by functional imaging) | M: 50 µg/24 h (50–340), N: 239 µg/24 (90–445) | NA | a: yes, 2015 b: no c: no d: no | no | - lesion operated (a): no persistence, no relapse - lesion not operated (b): echography, 6.5 mm (Nov 2018); MR angiography 20 mm (April 2019) |
Case 3 (T.I.) | (1) F (2) 19 (3) 16 (4) 19 | 15 | (1) 2 (2) a, b: left laterocervical | a: 30 b: 15 | - echography - MR angiography | M: 34 µg/24 h (50–340), N: 163 µg/24 (90–445) | NA | a, b: yes, 2019 | no | NA |
Case 4 (T.M.) | (1) F (2) 36 (3) 44 (4) 49 | 156 | (1) 3 (2) a: left laterocervical b: at 1.5 cm of the petrous canal between carotid and jugular c: right carotid glomus | a: NA b: 13 c: 16 | - echography - CT angiography - MR angiography | M: 57 µg/24 h (50–340), N: 104 µg/24 (90–445) | a: NA c: paraganglioma S100+; 1 mitosis/50 HPF | a: yes, 2006 b: no c: yes, 2007 | no | - lesions operated (a, c): no persistence, no relapse -lesion not operated (b): 11 mm growth in 7 yrs at CT |
Case 5 (T.R.) | (1) M (2) 50 (3) 47 (4) 52 | 26 | (1) 1 (2) a: left carotid bifurcation | a: 20 | - echography - MR angiography - CT angiography | M: 62 µg/24 h (50–340), N: 62 µg/24 (90–445) | a: Paraganglioma S100+; Synaptophysin +; CgA +/− | a: yes, 2018 | No | - lesion operated (a): no persistence, no relapse |
Patient | Lesions (Number) | 68Ga-DOTATOC PET-CT | 18F- FDG PET-CT | 18F-DOPA PET-CT |
---|---|---|---|---|
All | 13 | 9/9 (7 confirmed at morphological imaging and 2 not confirmed at morphological imaging) (4 NP) | 5/6 (mean SUV max 9.2) (7 NP) | 3/4 (confirmed at morphological imaging) (9 NP) |
Case 1 (T.D.) | 3 | 3/3 | 2/3 | 2/3 |
Case 2 (T.E.) | 4 | 3/3 (2 not confirmed at morphological imaging) (1 NP) * | NP | NP |
Case 3 (T.I.) | 2 | 2/2 | 2/2 | NP |
Case 4 (T.M.) | 3 | 1/1 (2 NP) * | 1/1 | NP |
Case 5 (T.R.) | 1 | NP | NP | 1/1 |
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Puliani, G.; Sesti, F.; Feola, T.; Di Leo, N.; Polti, G.; Verrico, M.; Modica, R.; Colao, A.; Lenzi, A.; Isidori, A.M.; et al. Natural History and Management of Familial Paraganglioma Syndrome Type 1: Long-Term Data from a Large Family. J. Clin. Med. 2020, 9, 588. https://doi.org/10.3390/jcm9020588
Puliani G, Sesti F, Feola T, Di Leo N, Polti G, Verrico M, Modica R, Colao A, Lenzi A, Isidori AM, et al. Natural History and Management of Familial Paraganglioma Syndrome Type 1: Long-Term Data from a Large Family. Journal of Clinical Medicine. 2020; 9(2):588. https://doi.org/10.3390/jcm9020588
Chicago/Turabian StylePuliani, Giulia, Franz Sesti, Tiziana Feola, Nicola Di Leo, Giorgia Polti, Monica Verrico, Roberta Modica, Annamaria Colao, Andrea Lenzi, Andrea M. Isidori, and et al. 2020. "Natural History and Management of Familial Paraganglioma Syndrome Type 1: Long-Term Data from a Large Family" Journal of Clinical Medicine 9, no. 2: 588. https://doi.org/10.3390/jcm9020588