2018 年 65 巻 12 号 p. 1155-1159
A 45-year-old male suddenly experienced left-flank abdominal pain. Echocardiography revealed akinesis of the ‘takotsubo cardiomyopathy’ type. He experienced a sudden haemodynamic collapse (blood pressure, 324/154 mmHg; pulse rate, 180 beats/min) during emergency cardiac catheterisation. An abdominal computed tomography (CT) revealed expansion of a soft tissue mass 64 × 33 mm in dimension in the left adrenal region, with accumulation of fluid surrounding the left pararenal space. Three days after the attack, his urinary catecholamine concentrations were slightly elevated. We suspected the patient as having a pheochromocytoma followed by acute haemorrhagic rupture, based on signatures of adrenal mass, ‘takotsubo cardiomyopathy’, and the hypertensive crisis. Over the next few weeks, he recovered well as an outpatient, and his blood pressure remained around 110/60 mmHg without medication. Three weeks after the attack, an abdominal CT showed shrinkage of the ruptured adrenal mass (to a diameter of 30 mm) and absorption of the retroperitoneal hematoma. On day 190 after the attack, abdominal CT did not detect any left adrenal mass. This is the first report of the case showing a complete vanishing of ruptured adrenal mass with takotsubo cardiomyopathy. Although surgical approaches for ruptured adrenal mass involve either emergency or elective surgery, the patients did not need even the elective surgery. Accumulation of the similar cases may unravel clinical factors predicting self-limiting of the ruptured adrenal mass to avoid unnecessary risky surgery.
THIS is the first case of a completely vanishing of ruptured adrenal mass with takotsubo cardiomyopathy.
A 45-year-old Japanese male suddenly experienced left-flank abdominal pain. He did not have any medical history of hypertension and was asymptomatic before. His blood pressure (BP) was 130/60 mm Hg and pulse rate (PR) was 88 beats/min on arrival. His electrocardiogram (ECG) demonstrated a 2-mm ST-segment depression in the precordial leads (Fig. 1A). Chest X-ray findings indicated no hilar congestion with a cardiothoracic ratio of 50% and no lung abnormalities. A blood cell count revealed elevated counts of white blood cells (12,900/μL). Blood chemistry examination showed elevated levels of myocardial enzymes including creatinine kinase (the myocardial isoenzyme, CK 582 IU/L, and CK-MB 29.4 ng/mL), troponin T (6.8 ng/mL) aspartate aminotransferase (54 IU/L), and lactate dehydrogenase (231 IU/L) (Table 1). The echocardiography revealed the apical areas with akinesis of the ‘takotsubo cardiomyopathy’ type. He was tentatively diagnosed with acute coronary syndrome and admitted to our intensive care unit. During emergency cardiac catheterization, he experienced a sudden hemodynamic collapse (BP 324/154 mmHg and PR 180 beats/min). Although coronary angiography revealed no critical obstruction, pulmonary congestion and effusion followed to occur. Treatment with antihypertension agents and nitroglycerin was initiated with non-invasive positive pressure ventilation (Fig. 2). An abdominal computed tomography (CT) revealed expansion of a soft tissue mass 64 × 33 mm in dimension in the left adrenal region, with accumulation of fluid surrounding the left pararenal space (Fig. 3A). Three days after the attack, the endocrinological examination included plasma adrenaline 25 pg/mL, plasma noradrenaline 380 pg/mL, plasma dopamine 5 pg/mL, urinary adrenaline 31.6 mg/day (reference range, 3.4–26.9 mg/day), urinary noradrenaline 213.4 mg/day (reference range, 48.6–168.4 mg/day), urinary dopamine 462.4 mg/day (reference range, 365–961.5 mg/day), urinary metanephrine 0.06 mg/day (reference range, 0.04–0.19 mg/day), urinary normetanephrine 0.17 mg/day (reference range, 0.09–0.33 mg/day) (Table 2). Over the next few weeks, he recovered well as an outpatient, and his BP remained around 110/60 mmHg with normal limits of the ECG (Fig. 1B) without medication. Three weeks after the attack, an abdominal CT showed shrinkage of the ruptured adrenal mass (to a diameter of 30 mm) and absorption of the retroperitoneal hematoma. In addition, magnetic resonance imaging (MRI) showed 30 mm-diameter left adrenal mass highly intense in T1- and moderately intense in T2-weighted images, which seems compatible findings with post-hemorrhagic state (Fig. 4). Iodine-123 metaiodobenzylguanidine scintigraphy (Fig. 5) and F-18-fluorodeoxyglucose positron-emission tomography did not reveal label accumulation, suggestive of coagulation necrosis of the mass. Four weeks after the attack, the levels of catecholamine-related metabolites were not highly elevated to pathological levels (Table 2). Unexpectedly, on day 190 after the attack, abdominal CT did not detect any left adrenal mass (Fig. 3B).
A. The patient’s electrocardiogram on arrival; B. The patient’s electrocardiogram after the patient was stable.
WBC | 12.9 × 103 /μL | PT | 11.6 s |
RBC | 4.57 × 106 /μL | APTT | 27.2 s |
Hb | 14.1 g/dL | TC | 139 mg/dL |
Ht | 42.6 % | TG | 22 mg/dL |
Plt | 201 × 103 /μL | HDL-C | 45 mg/dL |
BUN | 17.4 mg/dL | Plasma glucose | 148 mg/dL |
Cr | 0.76 mg/dL | HbA1c | 5.9 % |
UA | 6.5 mg/dL | Amylase | 63 IU/L |
Na | 144 mEq/L | CRP | 1.3 mg/dL |
K | 4.0 mEq/L | CK | 582 IU/L |
Cl | 106 mEq/L | CK-MB | 29.4 ng/mL |
AST | 54 IU/L | Troponin T | 6.8 ng/mL |
ALT | 24 IU/L | BNP | 162.7 pg/mL |
ALP | 152 IU/L | FT3 | 1.6 pg/mL |
γ-GTP | 31 IU/L | FT4 | 0.92 ng/mL |
LDH | 231 IU/L | TSH | 0.4 μU/mL |
AST, aspartate aminotransferase; ALT, alanine aminotransferase; ALP, alkaline phosphatase; γ-GTP, γ-glutamyl transpeptidase; LDH, lactate dehydrogenase; PT, prothrombin time; APTT, activated partial thromboplastin time; TC, total cholesterol; TG, triglycerides; HDL-C, high-density lipoprotein cholesterol; CRP, C-reactive protein; CK-MB, creatine phosphokinase MB isoform; BNP, brain natriuretic peptide
The patient’s clinical course.
A. Abdominal CT on the sudden hemodynamic collapse showed rupture of the adrenal mass. The adrenal mass was enlarged to 64 × 33 mm in diameter, and surrounded by a retroperitoneal hematoma; B. Abdominal CT on the 190 days after attack did not detect a left adrenal mass and absorption of the retroperitoneal hematoma.
Endocrinological Test | Three days after the attack |
normal range | 4 weeks after the attack |
normal range |
---|---|---|---|---|
ACTH | ≦2.0 | 7.2–63.3 (pg/mL) | 21.4 | <46 (pg/mL) |
Cortisol | 19.9 | 6.2–19.4 (μg/dL) | 10.7 | 6.2–19.4 (μg/dL) |
PRA | 3.0 | — (ng/mL/hr) | 5.6 | 0.2–2.7 (ng/mL/hr) |
PAC | 37.1 | — (mg/dL) | 207 | 20–130 (mg/dL) |
Plasma adrenaline | 25 | 0–100 (pg/mL) | 13 | 0–100 (pg/mL) |
Plasma noradrenaline | 380 | 100–450 (pg/mL) | 321 | 100–450 (pg/mL) |
Plasma dopamine | <5 | 0–20 (pg/mL) | <5 | 0–20 (pg/mL) |
Urinary adrenaline | 31.6 | 3.4–26.9 (μg/day) | 7.0 | 1.0–23.0 (μg/day) |
Urinary noradrenaline | 213.4 | 48.6–168.4 (μg/day) | 152.0 | 29–100 (μg/day) |
Urinary dopamine | 462.4 | 365.0–961.5 (mg/day) | 1,300 | 100–1,000 (mg/day) |
Urinary metanephrine | 0.06 | 0.04–0.19 (mg/day) | 0.09 | 0.05–0.20 (mg/day) |
Urinary normetanephrine | 0.17 | 0.09–0.33 (mg/day) | 0.19 | 0.10–0.28 (mg/day) |
PRA, plasma renin activity; PAC, plasma aldosterone concentrations
Magnetic resonance imaging showed 30 mm-diameter left adrenal mass.
A. highly intense in T1-weighted images; B. moderately intense in T2-weighted images.
Iodine-123 metaiodobenzylguanidine scintigraphy did not reveal label accumulation, suggestive of coagulation necrosis of the mass.
We suspected the patient as having a pheochromocytoma followed by acute haemorrhagic rupture, based on signatures of adrenal mass, ‘takotsubo cardiomyopathy’, and the hypertensive crisis. Acute haemorrhagic rupture is extremely rare as the initial manifestation of a pheochromocytoma. Other clinical features and symptoms such as sudden-onset haemodynamic collapse or flank pain as with our case had also been reported [1-7]. In general, it is difficult to diagnose pheochromocytoma after rupture due to the following reasons. First, Iodine-123 metaiodobenzylguanidine (MIBG) and F-18-fluorodeoxyglucose do not accumulate to the mass with total coagulation necrosis. Second, previous case reports for ruptured pheochromocytoma often failed in proving elevated levels of catecholamines because of difficulty in hormonal examination under emergency situation [2] and early normalization in plasma and urinary levels of catecholamines [1, 2]. Third, in the diagnosis process of the ruptured pheochromocytoma, there is a strong correlation between hemodynamic instability and rate of misdiagnosis [2, 3]. Indeed, the rate of preoperative misdiagnosis of the ruptured pheochromocytoma was as high as 55% [3]. Among the adrenal mass including adenoma, cancer and pheochromocytoma is prone to hemorrhage and spontaneous rupture because of its high intracapsular pressure in association with vasoconstriction in the nodule and subsequent necrosis. In addition, pheochromocytoma is known as the major cause for the takotsubo cardiomyopathy. Therefore, we reminded a pheochromocytoma as the most possible diagnosis when we encountered the present case with adrenal mass rupture and the takotsubo cardiomyopathy in emergency. Surgical treatment (emergency surgery or elective surgery) is considered even if ruptured adrenal mass is shrunken and absorbed to the retroperitoneal hematoma [1]. Surgical approaches for all ruptured pheochromocytoma involve either emergency or elective surgery. The emergency surgery of pheochromocytoma is associated with a high mortality rate, whereas no mortality has been reported in patients undergoing elective surgery with good control of total body water using alpha-adrenergic blocker and fluid infusion therapy [2, 3, 8]. Even the elective surgery was not necessary because of complete vanishing of adrenal mass after rupture in our patient.
The limitation of this case report includes insufficient evidence of pheochromocytoma. Evidence of elevated urinary excretion of metanephrine or normetanephrine, the MRI and MIBG images, and histological confirmation indicative of pheochromocytoma are lacking. Other differential diagnosis includes adrenal infarction and haemorrhage revealing pseudopheochromocytoma followed by adrenal failure as reported in a patient with antiphospholipid syndrome [9]. However, our patient is unlikely to have the lupus anticoagulant (Table 1), signs of microthrombi, and adrenal failure. Other differential diagnosis may be the takotsubo cardiomyopathy secondary to Cushing’s syndrome as reported in a patient with cortisol-producing adrenal adenocarcinoma [10]. Indeed, our patient showed suppressed ACTH level and mild elevation of cortisol level on 3 days after the attack, which were normalized on 4 weeks after the attack. Therefore, we cannot rule out the possibility that our patient experienced rupture of the cortisol-secreting adrenal adenoma. Anyway, there is no reported case of the vanishing adrenal mass in association with adrenal infarction, adrenal haemorrhage, and Cushing’s syndrome [9-11].
In summary, this is the first report of the case showing a complete vanishing of ruptured adrenal mass with takotsubo cardiomyopathy. To date, we have believed that possible ruptured pheochromocytoma should be surgically removed either in emergency or elective. However, pheochromocytoma-like symptoms in our patient were self-limited due to the vanishing of the ruptured adrenal mass and did not require surgery. Accumulation of the similar cases may unravel clinical factors predicting self-limiting of the ruptured adrenal mass to avoid unnecessary risky surgery.
YT was the lead clinician in management of the patient and wrote the manuscript. CT was the clinician in management of the patient. TT were responsible for care of the patient and edited the manuscript. All authors approved the final submitted version.
We declare no competing interests.