Figure 1
Teaching Case
SectionCardiovascular
Case TypeClinical Cases
Authors
Soler R, Méndez C, Rodríguez E, Flores X*.
Departments of Radiology and Cardiology*, Complexo Hospitalario Universitario A Coruña, A Coruña, Spain
Connected authors
37 years, female
A middle-aged woman presented with recurrent episodes of epigastric pain after social consumption of alcoholic beverages. Physical examination, complete blood count, routine serum chemistry test and serum tumour markers was unremarkable. The patient was normotensive and her medical history was negative for previous hypertension.
Synchronous pericardial paraganglioma and cystic adrenal pheochromocytoma
Cardiac haemangioma
Adrenal cyst
Adrenal pseudocyst
Adrenal carcinoma
Abdominal MRI showed a right cystic adrenal mass with a thin wall and multiple septa, as well as a mass in the mediastinum adjacent to the right atrium (Figures 1-3). Cardiac MRI (Figures 4, 5) revealed that the epicenter of the mass was located in the pericardial sac with no apparent fat plane between the lesion and the posterior wall of the right atrium. After contrast-enhancement, the mass showed intense heterogeneous enhancement (Figure 6).
Whole body iodine-131 metaiodobenzylguanidine (MIBG) scan revealed a focal uptake in the mediastinum and the right adrenal gland. Catecholamine determination revealed high plasma noradrenaline levels.
After preoperative α-blockade, the patient underwent thoracotomy and resection of the pericardial mass, which was densely adherent to the right atrium. The final pathologic diagnosis was pericardial paraganglioma. The patient was discharged 10 days later and a second stage operation for an elective right adrenalectomy was planned.
Whole body iodine-131 metaiodobenzylguanidine (MIBG) scan revealed a focal uptake in the mediastinum and the right adrenal gland. Catecholamine determination revealed high plasma noradrenaline levels.
After preoperative α-blockade, the patient underwent thoracotomy and resection of the pericardial mass, which was densely adherent to the right atrium. The final pathologic diagnosis was pericardial paraganglioma. The patient was discharged 10 days later and a second stage operation for an elective right adrenalectomy was planned.
Pheochromocytomas are rare primary neoplasms of the adrenal medulla that may produce catecholamines. All other tumours in this same family are referred to as paragangliomas [1].
These tumours may be either sporadic or familial. Familial, pheochromocytomas may be associated with type IIA and type IIB multiple endocrine neoplasia (MEN) syndromes, type I neurofibromatosis, von Hippel-Lindau disease, paraganglioma syndrome or Carney’s triad [Blake]. Genetic testing in apparently sporadic cases can be beneficial, especially in patients diagnosed before the age of 50 or presenting with bilateral, multifocal, malignant and/or extra-adrenal disease [2]. Our patient was not genetically screened.
The signs and symptoms of pheochromocytomas and paragangliomas are related to excess catecholamine secretion and include paroxysmal hypertension accompanied by headache, sweating and palpitations [3, 4]. Approximately 10 % of these tumours are discovered incidentally [4].
Cardiac paragangliomas are among the rarest primary cardiac tumours and are usually located in and around the left atrium [1]. Paragangliomas involving the pericardial sac are exceptional [1, 5]. On MRI, pericardial paragangliomas have been reported as hyperintense to the myocardium on T2-weighted images and iso or hyperintense on T1-weighted images. After gadolinium administration, these tumours show intense enhancement with non-enhanced areas at the core, denoting necrosis [5-7]. Our case exhibited a number of serpentine and punctate areas of signal void in the tumour matrix on T1 and TSE-T2 weighted SE-EPI. These MRI findings constitute the characteristic salt-and-pepper pattern of paragangliomas located in the neck and abdomen, attributed to the high flow velocity in the intratumoral vessel [4].
In our case, the pericardial paraganglioma presented with simultaneous massive cystic adrenal pheochromocytoma. To our knowledge, the combination seen here has not been described previously.
Most adrenal pheochromocytomas are highly vascularised tumours and show low signal intensity on T1-weighted imaging, high signal intensity on T2-weighted imaging and intense gadolinium enhancement. Areas of cystic degeneration due to necrosis and liquefaction are seen hyperintense on T2-weighted images with or without multiple septa, and wall enhancement after gadolinium administration [1, 3, 8]. Massive cystic degeneration, as in our case, is rare and these patients are more likely to be asymptomatic, complain of chronic abdominal pain or test negatively in biochemical evaluations [8].
In summary, when adrenal pheochromocytoma and paraganglioma is suspected, a combination of biochemical tests, MIBG scintigraphy and MRI may be useful for detecting and characterising these lesions.
.
These tumours may be either sporadic or familial. Familial, pheochromocytomas may be associated with type IIA and type IIB multiple endocrine neoplasia (MEN) syndromes, type I neurofibromatosis, von Hippel-Lindau disease, paraganglioma syndrome or Carney’s triad [Blake]. Genetic testing in apparently sporadic cases can be beneficial, especially in patients diagnosed before the age of 50 or presenting with bilateral, multifocal, malignant and/or extra-adrenal disease [2]. Our patient was not genetically screened.
The signs and symptoms of pheochromocytomas and paragangliomas are related to excess catecholamine secretion and include paroxysmal hypertension accompanied by headache, sweating and palpitations [3, 4]. Approximately 10 % of these tumours are discovered incidentally [4].
Cardiac paragangliomas are among the rarest primary cardiac tumours and are usually located in and around the left atrium [1]. Paragangliomas involving the pericardial sac are exceptional [1, 5]. On MRI, pericardial paragangliomas have been reported as hyperintense to the myocardium on T2-weighted images and iso or hyperintense on T1-weighted images. After gadolinium administration, these tumours show intense enhancement with non-enhanced areas at the core, denoting necrosis [5-7]. Our case exhibited a number of serpentine and punctate areas of signal void in the tumour matrix on T1 and TSE-T2 weighted SE-EPI. These MRI findings constitute the characteristic salt-and-pepper pattern of paragangliomas located in the neck and abdomen, attributed to the high flow velocity in the intratumoral vessel [4].
In our case, the pericardial paraganglioma presented with simultaneous massive cystic adrenal pheochromocytoma. To our knowledge, the combination seen here has not been described previously.
Most adrenal pheochromocytomas are highly vascularised tumours and show low signal intensity on T1-weighted imaging, high signal intensity on T2-weighted imaging and intense gadolinium enhancement. Areas of cystic degeneration due to necrosis and liquefaction are seen hyperintense on T2-weighted images with or without multiple septa, and wall enhancement after gadolinium administration [1, 3, 8]. Massive cystic degeneration, as in our case, is rare and these patients are more likely to be asymptomatic, complain of chronic abdominal pain or test negatively in biochemical evaluations [8].
In summary, when adrenal pheochromocytoma and paraganglioma is suspected, a combination of biochemical tests, MIBG scintigraphy and MRI may be useful for detecting and characterising these lesions.
.
References
[1] Araoz PA, Mulvagh SL, Tazelaar HD, et al (2000) CT and MR imaging of benign primary cardiac neoplasms with echocardiographic correlation. Radiographics 20:1303-1319 (PMID: 10992020)
[2] Neumann HP, Eng C (2009) The approach to the patient with paraganglioma. J Clin Endocrinol Metab 94:2677-2683 (PMID: 19657044)
[3] Blake MA, Kalra MK, Maher MM, et al (2004) Pheochromocytoma: an imaging chameleon. Radiographics 24 Suppl 1:S87-599 (PMID: 15486252)
[4] Lee KY, Oh YW, Noh HJ, et al (2006) Extraadrenal paragangliomas of the body: imaging features. AJR 187:492-504 (PMID: 16861555)
[5] Hamilton BH, Francis IR, Gross BH, et al (1997) Intrapericardial paragangliomas (pheochromocytomas): imaging features. AJR 168:109-113 (PMID: 8976931)
[6] Fisher MR, Higgins CB, Andereck W (1985) MR imaging of an intrapericardial pheochromocytoma. J Comput Assist Tomogr 9:1103-1105 (PMID: 4056145)
[7] Alghamdi AA, Sheth T, Manowski Z, et al (2009) Utility of cardiac CT and MRI for the diagnosis and preoperative assessment of cardiac paraganglioma. J Card Surg 24:700-701 (PMID: 19682163)
[8] Alghamdi AA, Sheth T, Manowski Z, et al (2008) Cystic phaeochromocytoma is a distinctive subgroup with special clinical, imaging and histological features that might mislead the diagnosis. BJU Int 101:345-350 (PMID: 18070168)
Case information
| URL: | https://www.eurorad.org/case/9214 |
| DOI: | 10.1594/EURORAD/CASE.9214 |
| ISSN: | 1563-4086 |
Figure 2
Figure 3
Figure 4
Figure 5
Figure 6
Figure 1
Figure 2
Figure 3
Figure 4
Figure 5
Figure 6
