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Objective To analyze the clinical characteristics of pheochromocytoma/paraganglioma (PPGL) patients with normal level of plasma free metanephrines (MNs), and offer a proposal for the diagnosis and treatment of such patients. Methods A retrospective study was conducted of 99 patients hospitalized from January 2019 to April 2021 and diagnosed as PPGL, of which 98 patients were pathologically confirmed as PPGL and 1 patient was clinically diagnosed as Von Hippel-Lindau (VHL) syndrome.Plasma free MNs were detected, and according to the MNs level, all the patients were divided into PPGL with normal MNs group(n=10) and PPGL with elevated MNs group (n=89). The differences were contrasted and analyzed between the two groups in clinical manifestation, imaging characteristics, preoperative preparation and intraoperative blood pressure fluctuation. Results No significant difference was detected in the incidence of typical triad between the two groups, but the incidence of nausea and vomiting in PPGL with normal MNs group was higher (40.0% vs. 13.5%, P=0.031). The proportion of retroperitoneal paraganglioma was obviously higher in PPGL with normal MNs group than in PPGL with elevated MNs group (60.0% vs. 20.2%, P=0.027), and the proportion of patients possessing typical PPGL imaging manifestations (70.0% vs. 92.1%, P=0.028) and tumor necrosis (40.0% vs.71.9%, P=0.039) was lower in PPGL with normal MNs group than in PPGL with elevated MNs group. Among 10 patients in PPGL with normal MNs group, 2 cases had slightly increased urinary metanephrine (MN) or 3-MT, and the maximum diameter of tumor in 8 cases was ≥3 cm. CT or MRI showed that tumors with typical PPGL imaging features in 7 cases, and functional imaging was positive in 6 cases. Conclusions Normal plasma MNs can not be used as sufficient exclusion of PPGL diagnosis. For patients suspected with PPGL, when normal MNs are obtained, it is recommended to measure extra indexes such as 24 h urine MNs and 3-MT.In addition, functional imaging is of great value in this condition.
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| 科 Family | 属数 Number of genus | 种数 Number of species | 占总种数比例 Percentage of total species (%) | 属 Genus | 种数 Number of species | 占总种数比例 Percentage of total species (%) |
|---|---|---|---|---|---|---|
| 鹅膏菌科Amanitaceae | 2 | 11 | 5.26 | 鹅膏菌属 Amanita | 10 | 4.78 |
| 小菇科 Mycenaceae | 2 | 12 | 5.74 | 丝盖伞属 Inocybe | 5 | 2.39 |
| 多孔菌科 Polyporaceae | 8 | 14 | 6.70 | 蜡蘑属 Laccaria | 5 | 2.39 |
| 红菇科 Russulaceae | 3 | 23 | 11.00 | 小皮伞属 Marasmius | 6 | 2.87 |
| 小菇属 Mycena | 11 | 5.26 | ||||
| 光柄菇属 Pluteus | 5 | 2.39 | ||||
| 红菇属 Russula | 17 | 8.13 | ||||
| 栓菌属 Trametes | 5 | 2.39 |
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