Informação da revista
Vol. 33. Núm. 4.
Páginas 245.e1-245.e4 (Abril 2014)
Baixar PDF
Mais opções do artigo
Vol. 33. Núm. 4.
Páginas 245.e1-245.e4 (Abril 2014)
Case report
DOI: 10.1016/j.repc.2013.11.008
Open Access
Pheochromocytoma diagnosed after anticoagulation for atrial fibrillation ablation procedure: A giant in disguise
Feocromocitma diagnosticado após hipocoagulação para ablação de fibrilhação auricular – o gigante disfarçado
Carlos Galvão Bragaa,??
Autor para correspondência

Corresponding author.
, Sílvia Ribeiroa, Juliana Martinsa, Carina Arantesa, Vítor Ramosa, João Primob, Sónia Magalhãesa, Adelino Correiaa
a Serviço de Cardiologia, Hospital de Braga, Braga, Portugal
b Serviço de Cardiologia, Centro Hospitalar de Vila Nova de Gaia, Vila Nova de Gaia, Portugal
Informação do artigo
Texto Completo
Baixar PDF
Figuras (2)
Tabelas (1)
Table 1. Urinary and plasma metanephrines and catecholamines before and after surgery.

Pheochromocytoma is a rare catecholamine-producing tumor, discovered incidentally in 50% of cases. We present the case of a 44-year-old male with a history of paroxysmal palpitations. Baseline ECG, transthoracic echocardiogram and ECG stress test showed no relevant alterations. Paroxysmal atrial fibrillation was detected on 24-hour Holter ECG. After antiarrhythmic therapy, the patient remained symptomatic, and was accordingly referred for electrophysiological study and atrial fibrillation ablation. Anticoagulation was initiated before the procedure. After ablation and still anticoagulated, he complained of hematospermia. The abdominal and pelvic imaging study showed a 10-cm left adrenal mass, predominantly cystic, compatible with pheochromocytoma, which was confirmed after biochemical tests (increased urine metanephrines and plasma catecholamines). Metaiodobenzylguanidine scintigraphy scanning confirmed localized disease in the adrenal gland, excluding other uptake foci. Following appropriate preoperative management, surgical resection of the giant mass was performed successfully and without complications.

Atrial fibrillation

O feocromocitoma é um tumor raro produtor de catecolaminas, que se descobre incidentalmente em 50% dos casos. Os autores apresentam o caso clínico de um homem de 44 anos de idade, com história de palpitações paroxísticas. O ECG basal, o ecocardiograma transtorácico e a prova de esforço não demonstraram alterações de relevo. O Holter de 24 h revelou fibrilhação auricular paroxística. Após terapêutica antiarrítmica, o doente manteve-se sintomático, pelo que foi orientado para estudo eletrofisiológico e ablação da fibrilhação auricular, tendo iniciado hipocoagulação oral pré-procedimento. No período pós-ablação, ainda hipocoagulado, referiu hematoespermia. O estudo imagiológico abdomino-pélvico revelou uma massa de 10 cm na glândula suprarrenal esquerda, predominantemente cística, compatível com feocromocitoma, diagnóstico que foi confirmado após testes bioquímicos (elevação das metanefrinas urinárias e das catecolaminas plasmáticas). A cintigrafia corporal com metaiodobenzilguanidina confirmou doença localizada na glândula suprarrenal, excluindo outros focos de captação. Após preparação pré-operatória adequada, procedeu-se à resseção cirúrgica da massa gigante com sucesso e sem complicações.

Fibrilhação auricular
Texto Completo

Pheochromocytoma is a rare catecholamine-producing tumor, with an incidence of 2–8 cases per million per year,1 occurring in about 0.5% of patients with hypertension and suggestive symptoms.2 Typical symptoms include paroxysmal hypertension, pounding headache, palpitations, diaphoresis and pallor.3 The diagnosis is based on biochemical tests, with demonstration of catecholamine oversecretion by adrenal chromaffin cells, and imaging studies, which localize the tumor and guide surgical resection.1,3 Half of all pheochromocytomas are discovered incidentally.4 Cardiovascular complications include myocardial hypertrophy, heart failure, myocardial infarction and various arrhythmias, of which atrial fibrillation is one of the most common.5–7

We report a case of a giant pheochromocytoma diagnosed after imaging study for hematospermia subsequent to oral anticoagulation for an atrial fibrillation ablation procedure.

Case report

A 44-year-old man with a history of tobacco use was referred for cardiological assessment in July 2009 due to a three-year history of paroxysmal palpitations lasting about five minutes, which were associated with a throbbing feeling in the head. No other accompanying symptoms were reported. Physical examination, blood pressure (110/70 mmHg), 12-lead electrocardiogram (ECG), transthoracic echocardiogram, ECG stress testing and standard blood analysis including blood glucose (97 mg/dl) were unremarkable. The 24-hour Holter ECG study revealed sinus rhythm with tachycardic activity during 45% of the record, alternating with several periods of ectopic atrial rhythm and one episode of atrial fibrillation lasting 1 hour and 15 minutes. The patient was initially prescribed antiarrhythmic therapy, but the symptoms did not resolve. Electrophysiological study with catheter ablation for atrial fibrillation was discussed and accepted by the patient. Oral anticoagulation was initiated and the pulmonary veins were isolated successfully in November 2010, although the procedure was complicated by a transient period of arterial hypotension and hypoxemia, requiring surveillance in the cardiac intensive care unit for a short period. The patient recovered well and was discharged with indication to maintain oral anticoagulation for three months. During that period, he returned for his periodic cardiological assessment and complained of hematospermia. After discussion with the urology department, antibiotic therapy with levofloxacin was started and abdominal and pelvic ultrasonography was performed, which revealed a 10-cm mass, predominantly cystic, adjacent to the left kidney upper pole. Abdominal magnetic resonance imaging (MRI) enabled better visualization of the mass and showed a nodular formation on the left adrenal gland with a major axis diameter of 9.8 cm, predominantly cystic, with thickened irregular walls and a solid component in the anterior, medial and inferior portions (maximum thickness 4 cm), with heterogeneous late enhancement after contrast administration and diffusion restriction (Figure 1), findings compatible with a giant cystic pheochromocytoma. Urinary metanephrines and plasma catecholamines (specifically adrenaline and noradrenaline) were markedly elevated (Table 1), confirming the diagnosis. A metaiodobenzylguanidine scintigraphy scan revealed localized disease with an intense uptake focus in the left adrenal gland (Figure 2) and no evidence of other uptake foci. Since the patient had no family history of pheochromocytoma, germline mutations that predispose to catecholamine-secreting tumors were not investigated. After appropriate preoperative treatment with phenoxybenzamine, beta-blockers and intravenous fluid expansion, surgical resection of the neoplasm by traditional open surgery was performed in May 2011. Histopathologic and immunohistochemical evaluation were consistent with pheochromocytoma. The immunohistochemical study was positive for chromogranin A, synaptophisin and S100, findings consistent with a diagnosis of cystic pheochromocytoma.8 There were no intraoperative or postoperative complications. Urinary and plasma metanephrines normalized after surgery (Table 1). During follow-up (more than two years) the patient remained asymptomatic without evidence of recurrence.

Figure 1.

Abdominal magnetic resonance imaging: T2-weighted transverse (A) and coronal (B) views showing a nodular formation on the left adrenal gland with major axis diameter of 9.8 cm, predominantly cystic, with thickened irregular walls and a solid component in the anterior, medial and inferior portions (maximum thickness 4 cm), with heterogeneous late enhancement after contrast administration and diffusion restriction.

Table 1.

Urinary and plasma metanephrines and catecholamines before and after surgery.

  Before surgery  After surgery  Reference value 
Plasma metanephrines
Metanephrine  –  <17  <90 pg/ml 
Normetanephrine  –  82  <200 pg/ml 
Plasma catecholamines
Adrenaline  >698  <20  <150 pg/ml 
Noradrenaline  >823  334  <370 pg/ml 
Dopamine  <20  <20  <200 pg/ml 
Urinary metanephrines
Metanephrine  6372  49  74–297 μg/24 h 
Normetanephrine  3913  156  105–354 μg/24 h 
Urinary catecholamines
Adrenaline  –  <2  <18 μg/24 h 
Noradrenaline  –  36  <80 μg/24 h 
Dopamine  –  209  209 μg/24 h 
Figure 2.

Body metaiodobenzylguanidine scintigraphy scan 24 hours after intravenous contrast administration in anterior (A) and posterior (B) views showing a pathological accumulation in the left adrenal gland compatible with pheochromocytoma (note the preferential distribution corresponding to the tumor's solid part); no other uptake foci are evident.


A “rule of 10s” has been attributed to pheochromocytoma, with approximately 10% extra-adrenal location (i.e., secretory paraganglioma), childhood onset, bilateral tumor formation, recurrence after resection, malignant phenotype and familial occurrence.3 Recently, however, inherited germ line mutations have been described in up to 25% of patients.4,9

The catecholamines secreted by these tumors (primarily norepinephrine and epinephrine) are responsible for the clinical manifestations, which can be highly diverse, producing signs and symptoms similar to many other clinical conditions.6 Our patient presented initially with symptoms that were attributed to paroxysmal atrial fibrillation, but these manifestations could have represented hyperadrenergic spells.

Hematospermia is defined as the presence of blood in the ejaculated seminal fluid. Although it is a painless, benign and self-limiting symptom in most cases, its advent may be frightening and alarming to the patient.10 In the case presented, the most likely mechanism for hematospermia was the association between oral anticoagulation and probable episodes of paroxysmal hypertension.11

Cystic pheochromocytomas are less common than the solid variant and are more often asymptomatic (or associated with upper abdominal discomfort only) and have negative biochemical tests, since the tumor burden is smaller.12,13 The cystic component reflects necrosis and liquefaction within the tumor, which can be visualized as a hyperintense signal on T2-weighted MRI. Morphologically, the main characteristic is the relatively thick wall (with or without septa within) and persistent wall enhancement after administration of contrast media,13 as in our patient.

Generally, tumors larger than 6 cm are malignant.12,14 Nevertheless, in this case, the giant cystic mass was probably a benign pheochromocytoma, since no metastatic disease has been detected. Although a variety of immunohistochemical and other prognostic markers have been assessed for association with malignancy in pheochromocytoma,15 currently the only criterion for diagnosis of malignancy is the presence of metastatic spread.16

Once an adrenal neoplasm is diagnosed, early resection is indicated to prevent complications. Before surgery, combined alpha- and beta-adrenergic blockade is recommended to control blood pressure and to prevent intraoperative hypertensive crisis.1,3

Some reports have concluded that laparoscopic adrenalectomy is safe, effective, and minimally invasive and, as such, it is considered the gold standard in the surgical management of small benign adrenal tumors.17 In our case, considering the large size of the tumor (about 10 cm) and possible malignancy, traditional open surgery was performed.


This case illustrates the importance of a high clinical index of suspicion to diagnose pheochromocytoma, since it can have multiple and unusual presentations. In particular, the cystic variant of pheochromocytoma can grow unnoticed due to the lesser tumor burden and give rise to a large mass that in most cases is detected incidentally.

Ethical disclosuresProtection of human and animal subjects

The authors declare that no experiments were performed on humans or animals for this study.

Confidentiality of data

The authors declare that they have followed the protocols of their work center on the publication of patient data and that all the patients included in the study received sufficient information and gave their written informed consent to participate in the study.

Right to privacy and informed consent

The authors have obtained the written informed consent of the patients or subjects mentioned in the article. The corresponding author is in possession of this document.

Conflicts of interest

The authors have no conflicts of interest to declare.

J. Izzo, D. Sica, H. Black.
Management of pheochromocytoma.
Hypertension primer, 4th ed., pp. 571-573
K. Pacak, W.M. Linehan, G. Eisenhofer, et al.
Recent advances in genetics, diagnosis, localization, and treatment of pheochromocytoma.
Ann Intern Med, 134 (2001), pp. 315-329
N. Kaplan, R. Victor.
Pheochromocytoma. Kaplan's clinical hypertension.
10th ed., Lippincott Williams & Wilkins, (2010), pp. 358-377
P.-F. Plouin, A.-P. Gimenez-Roqueplo.
Pheochromocytomas and secreting paragangliomas.
Orphanet J Rare Dis, 1 (2006), pp. 49
T. Zelinka, O. Petrák, H. Turková, et al.
High incidence of cardiovascular complications in pheochromocytoma.
Horm Metab Res, 44 (2012), pp. 379-384
F. Galetta, F. Franzoni, G. Bernini, et al.
Cardiovascular complications in patients with pheochromocytoma: a mini-review.
Biomed Pharmacother, 64 (2010), pp. 505-509
A. Prejbisz, J.W.M. Lenders, G. Eisenhofer, et al.
Cardiovascular manifestations of phaeochromocytoma.
J Hypertens, 29 (2011), pp. 2049-2060
E. Antedomenico, R.A. Wascher.
A case of mistaken identity: giant cystic pheochromocytoma.
Curr Surg, 62 (2005), pp. 193-198
A.-P. Gimenez-Roqueplo.
Hereditary paragangliomas and pheochromocytomas.
Néphrol Thérapeutique, 2 (2006), pp. S137-S142
P. Kumar, S. Kapoor, V. Nargund.
Haematospermia – a systematic review.
Ann Roy Coll Surg Engl, 88 (2006), pp. 339-342
S. Bhaduri, V.C. Riley.
Haematospermia associated with malignant hypertension.
Sex Transm Infect, 75 (1999), pp. 200
C. Li, Y. Chen, W. Wang, et al.
A case of clinically silent giant right pheochromocytoma and review of literature.
Can Urol Assoc J, 6 (2012), pp. E267-E269
C. Andreoni, R.K. Krebs, P.C. Bruna, et al.
Cystic phaeochromocytoma is a distinctive subgroup with special clinical, imaging and histological features that might mislead the diagnosis.
J. Park, C. Song, M. Park, et al.
Predictive characteristics of malignant pheochromocytoma.
Korean J Urol, 52 (2011), pp. 241-246
M.R. Clarke, R.J. Weyant, C.G. Watson, et al.
Prognostic markers in pheochromocytoma.
Hum Pathol, 29 (1998), pp. 522-526
R. DeLellis, R. Lloyd, P. Heitz, et al.
Tumours of endocrine organs (WHO Classification of Tumours Volume 8). Pathology and genetics.
3rd ed., IARC, (2004),
H.-S. Wang, C.-C. Li, Y.-H. Chou, et al.
Comparison of laparoscopic adrenalectomy with open surgery for adrenal tumors.
Kaohsiung J Med Sci, 25 (2009), pp. 438-444
Copyright © 2013. Sociedade Portuguesa de Cardiologia
Revista Portuguesa de Cardiologia

Receba a nossa Newsletter

Opções de artigo
en pt

Are you a health professional able to prescribe or dispense drugs?

Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos

Ao assinalar que é «Profissional de Saúde», declara conhecer e aceitar que a responsável pelo tratamento dos dados pessoais dos utilizadores da página de internet da Revista Portuguesa de Cardiologia (RPC), é esta entidade, com sede no Campo Grande, n.º 28, 13.º, 1700-093 Lisboa, com os telefones 217 970 685 e 217 817 630, fax 217 931 095 e com o endereço de correio eletrónico Declaro para todos os fins, que assumo inteira responsabilidade pela veracidade e exatidão da afirmação aqui fornecida.