Hypertension and Intrapericardial Paraganglioma: An Exceptional Presentation of Multiple Endocrine Neoplasia Type IIA Syndrome
2011; Lippincott Williams & Wilkins; Volume: 58; Issue: 5 Linguagem: Inglês
10.1161/hypertensionaha.111.180992
ISSN1524-4563
AutoresAndrea Imperatori, Lavinia De Monte, Nicola Rotolo, Gianlorenzo Dionigi, Silvia Uccella, Giovanni Mariscalco, Lorenzo Dominioni,
Tópico(s)Pituitary Gland Disorders and Treatments
ResumoHomeHypertensionVol. 58, No. 5Hypertension and Intrapericardial Paraganglioma: An Exceptional Presentation of Multiple Endocrine Neoplasia Type IIA Syndrome Free AccessLetterPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessLetterPDF/EPUBHypertension and Intrapericardial Paraganglioma: An Exceptional Presentation of Multiple Endocrine Neoplasia Type IIA Syndrome Andrea Imperatori, Lavinia De Monte, Nicola Rotolo, Gianlorenzo Dionigi, Silvia Uccella, Giovanni Mariscalco and Lorenzo Dominioni Andrea ImperatoriAndrea Imperatori Search for more papers by this author , Lavinia De MonteLavinia De Monte Search for more papers by this author , Nicola RotoloNicola Rotolo Search for more papers by this author , Gianlorenzo DionigiGianlorenzo Dionigi Search for more papers by this author , Silvia UccellaSilvia Uccella Search for more papers by this author , Giovanni MariscalcoGiovanni Mariscalco Search for more papers by this author and Lorenzo DominioniLorenzo Dominioni Search for more papers by this author Originally published10 Oct 2011https://doi.org/10.1161/HYPERTENSIONAHA.111.180992Hypertension. 2011;58:e189–e190Other version(s) of this articleYou are viewing the most recent version of this article. Previous versions: January 1, 2011: Previous Version 1 To the Editor:Paracardiac paragangliomas are very rare. A catecholamine-producing intrapericardial paraganglioma has never been described previously in the context of multiple endocrine neoplasia (MEN) IIA syndrome.1,2 We report the case of a patient with MEN IIA syndrome and secondary hypertension caused by functioning paraganglioma exceptionally located intrapericardially.A 34-year–old woman presented with recent episodes of headache, palpitation, and flushing. For several months she had arterial hypertension controlled by ramipril; however, she reported high blood pressure spikes (180/90 mm Hg). Moreover, her sister was diagnosed with medullary thyroid cancer. Examination of the patient's neck demonstrated a small lump in the left thyroid lobe. Aspiration cytology of the nodule (1 cm) revealed a medullary thyroid carcinoma cell pattern. Thyrocalcitonin serum level was high (217.6 pg/mL; normal: <18 pg/mL), whereas calcium, thyroid-stimulating hormone, T3, and T4 levels were normal. A MEN syndrome was suspected, and additional exams were performed. Very high 24-hour urine normetanephrine level (4132 μg/24 hours; normal: 162–527 μg/24 hours) and vanilmandelic acid level (16.2 mg/24 hours; normal: 1–6.7 mg/24 hours) were found. Imaging studies were done to localize the suspected catecholamine-secreting tumor. Computed tomography scan of the chest demonstrated a contrast-enhanced 55-mm intrapericardial mass below the left atrium roof (FigureA and B). Intense 123I-metaiodobenzylguanidine uptake concentrated solely in the thoracic mass (FigureC), supporting the diagnosis of intrapericardial functioning paraganglioma without metastases. Mutation of the proto-oncogene RET C618S was documented in the patient and in 5 of her family members (2 children, 1 sister, 1 uncle, and 1 cousin), confirming the diagnosis of MEN type IIA syndrome. Surgical resection of the intrapericardial tumor was carried out. At induction of general anesthesia, the patient had a hypertensive crisis (blood pressure: 210/100 mm Hg) and intraoperatively required multiple doses of phentolamine and labetalol. The tumor was approached by right thoracotomy and was resected without cardiopulmonary bypass. The resected specimen presented as a 45×55-mm pseudocapsulated oval mass, with cut surface showing multiple gray-tan nodules and hemorrhagic areas (FigureD). Pathology revealed a paraganglioma with a nesting pattern. Immunohistochemistry was positive for chromogranin A and protein S-100, as well as for tyrosine-hydroxylase, dopamine β-hydroxylase, and met-enkephalin. The tumor was negative for calcitonin and phenylethanole-mine N-methyl transferase.Download figureDownload PowerPointFigure. A and B, Chest computed tomography, showing a contrast-enhanced 55-mm mass located between the pulmonary artery and the roof of the left atrium. C, 123I-metaiodobenzylguanidine chest scan, with evidence of high tracer uptake at middle mediastinum level. D, Cut surface of resected specimen (45×55 mm pseudocapsulated oval mass) showing multiple nodules and hemorrhagic areas.The patient became rapidly well after surgery and no longer had hypertension. On postoperative day 4 her urine normetanephrine level was normal (167 μg/24 hours). After recovery from thoracotomy, total thyroidectomy was performed, and histology documented medullary thyroid carcinoma positive for calcitonin and chromogranin A, confirming the MEN II A syndrome. At follow-up after 9 months from paraganglioma resection the patient is well, without symptoms.Extra-adrenal tumors producing catecholamines are called paragangliomas. Two histological types are described, chromaffin paragangliomas, usually producing catecholamines and causing hypertension, and nonchromaffin tumors, which are called chemodectomas.3Extra-adrenal pheochromocytomas most commonly originate in the abdominal superior para-aortic region, between the diaphragm and the lower renal poles, frequently in the organ of Zuckerkandl.4 Only ≈2% of paragangliomas are located within the chest, more frequently in the posterior mediastinum and less frequently within the lungs or in the esophageal wall. Intrapericardial and intracardiac paragangliomas are very rare, usually arising from the left atrial wall or from the interatrial septum,5 and approximately half of them have been associated with signs or symptoms of excess catecholamine secretion.Catecholamine-secreting tumors in the MEN IIA syndrome are usually located in the adrenal glands. They have never been described previously within the pericardium.Andrea ImperatoriLavinia De MonteNicola Rotolo Center for Thoracic Surgery Department of Surgical Sciences University of Insubria Ospedale di Circolo Varese, ItalyGianlorenzo Dionigi Endocrine Surgery Research Center Department of Surgical Sciences University of Insubria Varese, ItalySilvia Uccella Department of Human Morphology University of Insubria Varese, ItalyGiovanni Mariscalco Cardiac Surgery Unit Department of Surgical Sciences University of Insubria Ospedale di Circolo Varese, ItalyLorenzo Dominioni Center for Thoracic Surgery Department of Surgical Sciences University of Insubria Ospedale di Circolo Varese, ItalyDisclosuresNone.FootnotesLetters to the Editor will be published, if suitable, as space permits. They should not exceed 1000 words (typed double-spaced) in length and may be subject to editing or abridgment. References 1. Hamilton BH, Francis IR, Gross BH, Korobkin M, Shapiro B, Shulkin BL, Deeb CM, Orringer MB. Intrapericardial paragangliomas (pheochromocytomas): imaging features. AJR Am J Roentgenol. 1997; 168:109–113.CrossrefMedlineGoogle Scholar2. Brown ML, Zayas GE, Abel MD, Young WF, Schaff HV. Mediastinal paragangliomas: the Mayo Clinic experience. Ann Thorac Surg. 2008; 86:946–951.CrossrefMedlineGoogle Scholar3. Dunn GD, Brown MJ, Sapsford RN, Mansfield AO, Hemingway AP, Sever PS, Allison DJ. Functioning middle mediastinal paraganglioma (phaeochromocytoma) associated with intercarotid paragangliomas. Lancet. 1986; 1:1061–1064.CrossrefMedlineGoogle Scholar4. Disick GI, Palese MA. Extra-adrenal pheochromocytoma: diagnosis and management. Curr Urol Rep. 2007; 8:83–88.CrossrefMedlineGoogle Scholar5. Gopalakrishnan R, Ticzon AR, Cruz PA, Kennedy FB, Duffy FC, Barmada B, Giacobine JW. Cardiac paraganglioma (chemodectoma). J Thorac Cardiovasc Surg. 1978; 76:183–189.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Wang J, Han J, Jiang T and Li Y (2014) Cardiac Paragangliomas, Journal of Cardiac Surgery, 10.1111/jocs.12455, 30:1, (55-60), Online publication date: 1-Jan-2015. Yamamoto Y, Kodama K, Yamato H and Takeda M (2014) Successful Removal of Giant Intrapericardial Paraganglioma via Posterolateral Thoracotomy, Case Reports in Surgery, 10.1155/2014/308462, 2014, (1-4), . Rotolo N, Imperatori A, Bacuzzi A, Conti V, Castiglioni M and Dominioni L (2014) Management of Hypertension in Intrapericardial Paraganglioma, International Journal of Hypertension, 10.1155/2014/812598, 2014, (1-8), . November 2011Vol 58, Issue 5 Advertisement Article InformationMetrics © 2011 American Heart Association, Inc.https://doi.org/10.1161/HYPERTENSIONAHA.111.180992PMID: 21986504 Originally publishedOctober 10, 2011 PDF download Advertisement SubjectsCardiovascular SurgeryCatheter Ablation and Implantable Cardioverter-DefibrillatorEtiologyHypertension
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