Paraganglioma Tanılı Hastada Postoperatif ARDS: Olgu Sunumu

Yıl 2025, Cilt: 47 Sayı: 5, 850 - 855, 04.09.2025

Orkhan Ulfanov , Yavuz Selim Angın , Mehmet Kılıç

https://doi.org/10.20515/otd.1623118

Öz

Paraganglioma, genellikle retroperitoneal yerleşimli ve kromaffin hücrelerden köken alan nadir bir nöroendokrin tümördür. Feokromositoma gibi katekolamin salgılayarak kardiyovasküler sistem üzerinde ciddi etkiler oluşturabilir. Özellikle cerrahi girişim, travma veya anestezi gibi stres faktörleri, ani ve yoğun katekolamin salınımını tetikleyerek feokromositoma krizine yol açabilir. Bu klinik tablo, kardiyovasküler instabiliteye ek olarak nadir ancak ciddi bir komplikasyon olan akut respiratuvar distres sendromu (ARDS) ile birlikte seyredebilir. ARDS, alveol-kapiller bariyerin bozulması sonucu gelişen yaygın pulmoner ödemle karakterize olup, zamanında müdahale edilmediğinde mortal seyredebilir. Bu olgu sunumunda, paraganglioma nedeniyle opere edilen ve postoperatif dönemde feokromositoma krizine sekonder olarak ARDS gelişen nadir bir vaka sunulmuştur. 53 yaşındaki kadın hasta, mevcut hipertansiyon, diyabetes mellitus ve pankreatit öyküsüne ek olarak ani gelişen hipertansif ataklar nedeniyle ileri tetkiklerle değerlendirilmiş ve retroperitoneal yerleşimli paraganglioma tanısı almıştır. Preoperatif dönemde uygulanan alfa ve beta bloker tedaviye rağmen cerrahi sonrası erken dönemde hemodinamik instabilite ve hipoksemik solunum yetmezliği gelişmiş; toraks bilgisayarlı tomografi (BT) ile ARDS tanısı doğrulanmıştır. Yoğun bakım ünitesinde invaziv mekanik ventilasyon desteğiyle takip edilen hasta, klinik iyileşme sonrası ekstübe edilerek şifa ile taburcu edilmiştir. Sonuç olarak, paraganglioma cerrahisi öncesinde feokromositoma krizi ve ARDS gibi hayatı tehdit eden komplikasyonların öngörülmesi büyük önem taşımaktadır. Tanı ve tedavi sürecinde multidisipliner yaklaşımın etkinliği kritik rol oynamaktadır.

Anahtar Kelimeler

PARAGANGLİOMA , ARDS , FEOKROMASİTOMA KRİZİ

Postoperative ARDS in a Patient with Paraganglioma: A Case Report

Öz

Paraganglioma is a rare neuroendocrine tumor that typically originates from chromaffin cells and is most commonly located in the retroperitoneal region. Similar to pheochromocytoma, it can secrete catecholamines and cause significant cardiovascular effects. Surgical intervention, trauma, or anesthesia may act as stressors, triggering a pheochromocytoma crisis through sudden and excessive catecholamine release. This clinical condition may lead not only to cardiovascular instability but also to a rare yet life-threatening complication: acute respiratory distress syndrome (ARDS). ARDS is characterized by widespread pulmonary edema due to disruption of the alveolar-capillary barrier and may become fatal if not managed promptly.In this case report, we present a rare case of ARDS secondary to a pheochromocytoma crisis in the postoperative period following paraganglioma surgery. A 53-year-old female patient with a history of hypertension, diabetes mellitus, and pancreatitis was evaluated for sudden hypertensive episodes. Advanced imaging revealed a retroperitoneal paraganglioma. Despite preoperative treatment with alpha- and beta-blockers, the patient developed hemodynamic instability and hypoxemic respiratory failure in the early postoperative period. The diagnosis of ARDS was confirmed by thoracic computed tomography. The patient was managed in the intensive care unit with invasive mechanical ventilation and was successfully extubated and discharged following clinical improvement.In conclusion, potentially life-threatening complications such as pheochromocytoma crisis and ARDS should be considered before paraganglioma surgery. A multidisciplinary approach plays a crucial role in the effective management of diagnosis and treatment processes.

Anahtar Kelimeler

PARAGANGLİOMA , Pheochromocytoma crisis , Acute Respiratory Distress Syndrome

Kaynakça

• 1. Emuze, Martins Ehizode, et al. "Extra-adrenal phaeochromocytoma in a resource poor setting: A case report." Endocrine Regulations 56.1 (2022): 48-54
• 2. Henri J L M Timmers, David Taïeb, Karel Pacak, Jacques W M Lenders, Imaging of Pheochromocytomas and Paragangliomas, Endocrine Reviews, Volume 45, Issue 3, June 2024, Pages 414–434
• 3. Nölting, Svenja, et al. "Personalized management of pheochromocytoma and paraganglioma." Endocrine reviews 43.2 (2022): 199-239
• 4. Lee, J.A., Duh, QY. Sporadic Paraganglioma. World J Surg 32, 683–687 (2008).
• 5. Pacak, Karel ve Sunil J. Wimalawansa. "Feokromasitoma ve paraganglioma." Endokrin Uygulaması 21.4 (2015): 406-412
• 6. Pacak, K., & Eisenhofer, G. (2007). Clinical Management of Pheochromocytoma. Endocrine Practice, 13(4), 422-429
• 7. Lenders, J. W. M., Duh, Q. Y., Eisenhofer, G., Gimenez-Roqueplo, A. P., Grebe, S. K. G., Murad, M. H., & Young, W. F. (2014). Pheochromocytoma and paraganglioma: An Endocrine Society Clinical Practice Guideline. The Journal of Clinical Endocrinology & Metabolism, 99(6), 1915-1942.
• 8. Xie, Y., Zhang, A., Qi, M. et al. Pheochromocytoma crisis with refractory Acute Respiratory Distress Syndrome (ARDS), Takotsubo syndrome, emergency adrenalectomy, and need for Extracorporeal Membrane Oxygenation (ECMO) in a previously undiagnosed and asymptomatic patient, due to the use of metoclopramide. BMC Endocr Disord 23, 145 (2023). https://doi.org/10.1186/s12902-023-01404-4
• 9. The American-European Consensus Conference on ARDS. (1994). Definition of Acute Respiratory Distress Syndrome (ARDS). American Journal of Respiratory and Critical Care Medicine, 149(3), 818-824.
• 10. Ranieri, V. M., Rubenfeld, G. D., & Thompson, B. T. (2012). Acute Respiratory Distress Syndrome: The Berlin Definition. JAMA, 307(23), 2526-2533.
• 11. Ware, L. B., & Matthay, M. A. (2000). The Acute Respiratory Distress Syndrome. New England Journal of Medicine, 342(18), 1334-1349.
• 12. Tong M, Wang S, Bai Y, Wang H. Paraganglioma-induced pheochromocytoma crisis successfully treated by veno-arterial extracorporeal membrane oxygenation: a case report. Journal of International Medical Research. 2024;52(9).
• 13. Lee JA, Zarnegar R, Shen WT, Kebebew E, Clark OH, Duh Q. Adrenal Incidentaloma, Borderline Elevations of Urine or Plasma Metanephrine Levels, and the “Subclinical” Pheochromocytoma. Arch, Surg. 2007;142(9):870–874. doi:10.1001/archsurg.142.9.870
• 14. Manger, W.M., Eisenhofer, G. Pheochromocytoma: Diagnosis and management update. Current Science Inc 6, 477–484 (2004).
• 15. Friedman, Lindsay R., Bhavishya Ramamoorthy, and Naris Nilubol. "Progress in surgical approaches and outcomes of patients with pheochromocytoma and paraganglioma." Best Practice & Research Clinical Endocrinology & Metabolism (2024): 101954.
• 16. Aygun, Nurcihan, and Mehmet Uludag. "Pheochromocytoma and paraganglioma: from treatment to follow-up." Şişli Etfal Hastanesi Tip Bülteni 54.4 (2020): 391-398
• 17. Julian Naranjo, Sarah Dodd, Yvette N. Martin, Perioperative Management of Pheochromocytoma,Journal of Cardiothoracic and Vascular Anesthesia,Volume 31, Issue 4,2017,Pages 1427-1439,ISSN 1053-0770,
• 18. Jacques W. M. Lenders, Quan-Yang Duh, Graeme Eisenhofer, Anne-Paule Gimenez-Roqueplo, Stefan K. G. Grebe, Mohammad Hassan Murad, Mitsuhide Naruse, Karel Pacak, William F. Young, Pheochromocytoma and Paraganglioma: An Endocrine Society Clinical Practice Guideline, The Journal of Clinical Endocrinology & Metabolism, Volume 99, Issue 6, 1 June 2014, Pages 1915–1942,
• 19. Dai, J., Chen, Sj., Yang, Bs. et al. Recurrence of non-cardiogenic pulmonary edema and sustained hypotension shock in cystic pheochromocytoma. J. Zhejiang Univ. Sci. B 18, 449–452 (2017).
• 20. Manita Choudhary, Yufei Chen, Oren Friedman, Natasha Cuk, Anat Ben-Shlomo, Pheochromocytoma Crisis Presenting With ARDS Successfully Treated With ECMO-Assisted Adrenalectomy, AACE Clinical Case Reports, Volume 7, Issue 5, 2021,Pages 310-314, ISSN 2376-0605,