İzole Abducens Paralizisi ve Akut Koroner Sendrom ile prezente olan Paraganglioma olgusu

Paragangliomalar, adrenal bez dışındaki otonomik sinir sisteminin ganglion zincir kromaffin hücrelerinden köken alırlar ve katekolamin salgılayan tümörlerin %10-15'ini oluştururlar. Yetişkinlerde en sık dördüncü ve beşinci dekadda gözlenirler. Klinikte hipertansiyon, çarpıntı ve terleme ataklarının eşlik ettiği baş ağrısına vurgu yapılmaktadır. Bununla birlikte, hastaların %5-15'i normotensif olup ortostatik hipotansiyon atakları dahi yaşayabilir. Kardiyovasküler komplikasyonlar arasında miyokardit, kardiyomiyopati, atriyal veya ventriküler aritmiler, akut koroner sendrom bulunmaktadır. Özellikle hipovolemi varlığında vasküler ton kaybı eklenmesiyle birlikte şok tablosu gözlenebilir. Nörolojik bulgular olarak ise mental durum değişiklikleri, hipertansif ensefalopati ve inme sıralanabilir. Bu makalemizde iç hastalıkları polikliniğine başvurusu sonrası paraganglioma tanısı konulan hasta sunulmaya çalışılmıştır.

Anahtar Kelimeler:

Paraganglioma, Akut koroner sendrom, İzole Abducens paralizisi

A case of paraganglioma presenting with isolated Abducens Paralysis and Acute Coronary Syndrome

Paragangliomas originate from ganglion chain chromaffin cells of the autonomic nervous system other than the adrenal gland and constitute 10-15% of catecholamine-secreting tumors. They are most frequently observed in the fourth and fifth decade in adults. In the clinic, headache associated with hypertension, palpitation and sweating attacks is emphasized. However, 5-15% of patients are normotensive and may experience orthostatic hypotension attacks. Cardiovascular complications include myocarditis, cardiomyopathy, atrial or ventricular arrhythmias, and acute coronary syndrome. Shocks may be observed with the addition of vascular tone loss especially in the presence of hypovolemia. As neurological findings mental status changes, hypertensive encephalopathy and stroke can be listed. In this article, we present a patient who was diagnosed with paraganglioma after admission to the internal medicine outpatient clinic.

Keywords:

Paraganglioma, Acute coronary syndrome, Abducens paralysis,

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Bolu E et al. Feokromasitoma ve Paraganglioma. Adrenal ve Gonadal Hastalıklar Kılavuzu, Adrenal ve Gonadal Hastalıklar Çalışma Grubu. Türkiye Endokrinoloji ve Metabolizma Derneği, 12. Baskı Mayıs 2018; pages 47-63
Çetinkalp Ş. Endokrinoloji. Feokromasitoa 1. Baskı. Türkiye : Türkiye Klinikleri Inc 2017; pages 251-266
Kantorovich V, Koch CA, Pacak K. Pheochromocytoma and Paraganglioma Emergencies. Endotext [Internet]. South Dartmouth (MA): MDText.com, Inc.; 2000-2015; Apr 12
Petri BJ, Van Eijck CHJ, De Herder WW, Wagner A, De Krijger RR. Phaeochromocytomas and sympathetic paragangliomas, British Journal of Surgery 2009; 96: 1381-1392
Plouin PF, Gimenez-Roqueplo AP. Pheochromocytomas and secreting paragangliomas. Orphanet Journal of Rare Diseases 2006; 1:49
Erdoğan G. Adrenal Medulla ve Hastalıkları. Endokrinoloji Temel ve Klinik. 2. Baskı. Türkiye : MN Medikal & Nobel Inc 2005; pages 561-571
Özgen G. Adrenal Medulla ve Hastalıkları Kavramları. Kabalak T(ed), Yılmaz C(ed), Tüzün M(ed). Endokrinoloji El Kitabı. 3. Baskı. Türkiye : İzmir Güven Kitabevi Inc 2004; pages 473-480
Pavai Z, Orosz Z, Horvath E, Seres-Sturm L, Jung J. Immunohistochemical features of paragangliomas. J.Cell.Mol.Med. 2001; Vol 5, No 2, 311-316
Ayala-Ramirez M, Feng L, Johnson MM, Ejaz S, Habra MA, Rich T, Busaidy N, Cote GJ et al. Clinical Risk Factors for Malignancy and Overall Survival in Patients with Pheochromocytomas and Sympathetic Paragangliomas: Primary Tumor Size and Primary Tumor Location as Prognostic Indicators. J Clin Endocrinol Metab, March 2011; 96(3):717-725
Laird AM, Gauger PG, Doherty GM, Miller BS. Paraganglioma: not just an extra-adrenal pheochromocytoma Langenbecks Arch Surg 2012; 397:247-253
Fitzgerald SC, Gingell Littlejohn M, Parnaby CN, Connell JM, O’Dwyer PJ. Abdominal Paragangliomas: Analysis of Surgeon’s Experience. WorldJournal ofEndocrine Surgery, May-August 2011; 3(2):55-58
Honnorat J, Antoine JC. Paraneoplastic neurological syndromes. Orphanet J. Rare Dis. 2007; 2:22
Kaltsas G, Dimitriadis GK, Androulakis II, Grossman A. Paraneoplastic Syndromes related to Neuroendorine Tumours. Endotext [Internet]. MDText.com, Inc, 2017 Feb 16
Eisenhofer G, Tischler AS, De Krijger RR. Diagnostic Tests and Biomarkers for Pheochromocytoma and Extra-adrenal Paraganglioma: From Routine Laboratory Methods to Disease Stratification Endocr Pathol 2012; 23:4-14
Parenti G, Zampetti B, Rapizzi E, Ercolino T, Giach V, Mannelli M. Updated and New Perspectives on Diagnosis, Prognosis, and Therapy of Malignant Pheochromocytoma/ Paraganglioma Journal of Oncology Volume 2012; 10 pages
Ayala-Ramirez M, Palmer JL, Hoffman MC, de la Cruz M, Moon BS, Waguespack SG, Habra MA, Jimenez C: Bone Metastases and Skeletal-Related Events in Patients with Malignant Pheochromocytoma and Sympathetic Paraganglioma. J Clin Endocrinol Metab 2013
Corssmit EP, Romijn JA. Management of endocrine disease : Clinical management of paragangliomas. European Journal of Endocrinology 2014; 171, R231–R243
Jimenez C, Rohren E, Habra MA, Rich T, Jimenez P, Ayala Ramirez M and Baudin E: Current and future treatments for malignant pheochromocytoma and sympathetic paragan¬glioma. Curr Oncol Rep 2013; 15: 356-371