[Diagnostic difficulties and therapeutic options of a giant chest paraganglioma]

Orv Hetil. 2022 Jul 31;163(31):1243-1249. doi: 10.1556/650.2022.32529. Print 2022 Jul 31.
[Article in Hungarian]

Abstract

Paragangliomas are mostly benign tumors originating from the sympathetic or parasympathetic ganglions, but malignant forms are also known. They are in the region of the head and neck, in the glomus caroticum, intra-abdominally as well as in the thorax. The investigation of the 39-year-old male patient began due to extremely high blood pressure, night sweats and a 10 kg weight loss. Chest CT scan described a huge mass in the right hilum, bronchoscopic sampling was inconclusive. Tumor biopsy was performed through right thoracotomy, but complete resection was not possible due to tissue adhesions and cardiac involvement. Histological examination verified paraganglioma, which was also confirmed by laboratory tests. Accordingly, somatostatin analog therapy was initiated, followed by I-131-MIBG treatment with good clinical effect. Coronary angiography confirmed that the right coronary artery contributed with two marginal branches to the blood supply of the thoracic mass. The tumor was successfully removed and after the cardio-thoracic surgery, the patient's antihypertensive therapy was stopped. There was no sign of relapse during follow-ups. During the medical investigation of severe blood pressure elevations, the possibility of paraganglioma should be considered. In these cases, invasive procedures, if not preceded by proper medication, can be fatal. By taking advantage of the ever-expanding therapeutic options and the cooperation between institutions, even patients with a giant paraganglioma can become tumor-free.

A paragangliomák a szimpatikus vagy paraszimpatikus ganglionokból kiinduló, többségükben jóindulatú daganatok, de rosszindulatú formájuk is ismert. A fej-nyak régióban, a glomus caroticumban, illetve a vegetatív idegrendszer dúcláncának megfelelően a hasüregben, illetve a mellüregben is előfordulhatnak. A 39 éves férfi beteg kivizsgálása extrém magas vérnyomásértékek, éjszakai izzadás és 10 kg-os fogyás miatt kezdődött. A mellkas-CT-vizsgálat a jobb oldali tüdőkapuban óriási térfoglalást írt le, a bronchoszkópos mintavétel sikertelen volt. Jobb oldali thoracotomiából mintavétel történt, reszekcióra a kifejezett adhéziók, illetve a szívérintettség miatt nem volt lehetőség. A szövettani vizsgálat eredménye paraganglioma lett, ezt a laborvizsgálatok is támogatták. Ennek megfelelőn szomatosztatinanalóg-terápiát kezdtünk, majd

Keywords: 131I-MIBG; case report; catecholamine; esetismertetés; katecholamin; paraganglioma; somatostatin analog; szomatosztatinanalóg.

Publication types

  • Case Reports

MeSH terms

  • 3-Iodobenzylguanidine
  • Adult
  • Humans
  • Iodine Radioisotopes* / therapeutic use
  • Male
  • Neoplasm Recurrence, Local / drug therapy
  • Paraganglioma* / diagnosis
  • Paraganglioma* / surgery

Substances

  • Iodine Radioisotopes
  • Iodine-131
  • 3-Iodobenzylguanidine