Analysis of the clinical characteristics of pheochromocytoma crisis / 中华内分泌外科杂志

Objective To analyze the clinical characteristics of pheochromocytoma crisis (PCC). Meth-ods Data of 123 cases of pheochromocytoma and paraganglioma (PPGL) admitted from Apr. 2011 to Feb. 2017 were retrospectively analyzed and they were divided into crisis group and noncrisis group according to the pa-tients with or without haemodynamic instability and end-organ damage. The differences of demographics charac-teristics, presentations, laboratory tests, imaging findings, perioperative clinical conditions and pathological fea-tures were compared between the two groups. Results ①16 cases were enrolled into crisis group, among whom 5 were misdiagnosed, while 107 cases were enrolled into noncrisis group. ②Compared with noncrisis group, the incidence of headache, palpitation, sweating, the classic triad, other presentations of PPGL, severe hypertension and hypotension were higher, and more patients had paroxysmal hypertension and admitted to our hospital for paroxysmal presentations in crisis group (P<0.05). ③Leukocyte, fasting blood glucose, liver transaminases, tro-ponin and D-dimmer were higher, while estimated glomerular filtration rate(eGFR) was lower, more tumors locat-ed in the left of adrenal in crisis group(P<0.05). ④ Patients in crisis group had higher plasma free metanephrines (MNs), larger maximal tumor diameter, higher enhanced CT value in each period, more benign tumors and hem-orrhage or necrosis in the tumors, but all the differences were not significant when compared with the noncrisis group. ⑤Patients in crisis group were more likely to undergo elective surgery. However, there was no difference in the preoperative time of α-blockade, type of surgery, intraoperative and postoperative complications, mortality a-mong the two groups. Conclusions PCC is a rare endocrinological emergency with a highly variable manifesta-tions, which commonly presents with typical triad, with higher incidence of hemodynamic instability and end-organ damage. Although biochemical and imaging examinations are relatively effective in the diagnosis of PCC, the mis-diagnosis remains inappropriate high. Once the diagnosis is established, clinicians should timely start drug prepa-ration while surgical resection is the key to the treatment of PCC.

Analysis of the clinical characteristics of pheochromocytoma crisis / 中华内分泌外科杂志

Objective@#To analyze the clinical characteristics of pheochromocytoma crisis (PCC) .@*Methods@#Data of 123 cases of pheochromocytoma and paraganglioma (PPGL) admitted from Apr. 2011 to Feb. 2017 were retrospectively analyzed and they were divided into crisis group and noncrisis group according to the patients with or without haemodynamic instability and end-organ damage. The differences of demographics characteristics, presentations, laboratory tests, imaging findings, perioperative clinical conditions and pathological features were compared between the two groups.@*Results@#①16 cases were enrolled into crisis group, among whom 5 were misdiagnosed, while 107 cases were enrolled into noncrisis group. ②Compared with noncrisis group, the incidence of headache, palpitation, sweating, the classic triad, other presentations of PPGL, severe hypertension and hypotension were higher, and more patients had paroxysmal hypertension and admitted to our hospital for paroxysmal presentations in crisis group (P<0.05) . ③Leukocyte, fasting blood glucose, liver transaminases, troponin and D-dimmer were higher, while estimated glomerular filtration rate (eGFR) was lower, more tumors located in the left of adrenal in crisis group (P<0.05) . ④ Patients in crisis group had higher plasma free metanephrines (MNs) , larger maximal tumor diameter, higher enhanced CT value in each period, more benign tumors and hemorrhage or necrosis in the tumors, but all the differences were not significant when compared with the noncrisis group. ⑤Patients in crisis group were more likely to undergo elective surgery. However, there was no difference in the preoperative time of α-blockade, type of surgery, intraoperative and postoperative complications, mortality among the two groups.@*Conclusions@#PCC is a rare endocrinological emergency with a highly variable manifestations, which commonly presents with typical triad, with higher incidence of hemodynamic instability and end-organ damage. Although biochemical and imaging examinations are relatively effective in the diagnosis of PCC, the misdiagnosis remains inappropriate high. Once the diagnosis is established, clinicians should timely start drug preparation while surgical resection is the key to the treatment of PCC.

Pheochromocytoma Crisis Triggered by Extra-Corporal Membrane Oxygenation Explantation.

Context: We report a 59 year old Chinese woman in pheochromocytoma multisystem crisis and was initially stabilised with ECMO; subsequently she manifested rapid cyclical fluctuation of blood pressure precipitated by explantation of ECMO. Case Description: Madam L presented with chest pain and giddiness. She developed cardiogenic shock refractory to double inotropes and intra-aortic balloon pump (IABP); but responded to Extra-Corporal Membrane Oxygenation (ECMO). Subsequently rapid cyclical blood pressure fluctuations from 230 mmHg to 50mmHg systolic occurred after explantation of ECMO. Biochemically urine metanephrines and normetanephrines were more than seven times above upper limit of normal. Imaging confirmed the presence of right adrenal mass likely pheochromocytoma. She was started on alpha blockade and underwent an uneventful laparotomy with removal of a 10 cm adrenal mass. Histology confirmed a large cystic pheochromocytoma with haemorrhagic components. Conclusions: Pheochromocytoma crisis should be suspected in cases of unexplained shock or Takutsubo cardiomyopathy; early recognition and ECMO support can be life-saving. In addition, ECMO explantation can trigger rapid cyclical blood pressure fluctuations and should be closely monitored for.