SESSION TYPE: Miscellaneous Student/Resident Cases II
PRESENTED ON: Wednesday, October 24, 2012 at 11:15 AM - 12:30 PM
INTRODUCTION: Approximately 300 cases of pheochromocytomas have been reported during pregnancy. Mediastinal paraganglioma in pregnancy are exceptionally rare.
CASE PRESENTATION: A 28 year old woman had an emergency cesarean section for severe hypertension. Postoperatively, she suffered from dyspnea and chest tightness prompting an echocardiogram that demonstrated an abnormality involving the left atrium. A CT scan of the chest documented a large (7.4 cm), posterior mediastinal mass splaying the carina, compressing the left atrium and possibly involving the right main pulmonary artery. The subsequent metanephrine profile and MRI revealed a mediastinal paraganglioma. After appropriate α- and β-blockade, a team of cardiac and thoracic surgeons attempted to resect the tumor. Through a clamshell thoracotomy the tumor was mobilized off the spine and aorta posteriorly but it involved the superior aspect of the left atrium and infiltrated the right pulmonary and left superior pulmonary veins. Ultimately, the tumor was deemed unresectable. The tumour is not MIBG avid, thus she is currently undergoing treatment with Luteium-177 with signs of disease stabilization and abatement of hypertensive symptoms. Genetic testing is pending and she is also being assessed for cardio-pulmonary transplant. The infant is doing well.
DISCUSSION: Pheochromocytoma or functional paragangliomas during pregnancy present a significant risk to the health of both the mother and the fetus. Mortality rates are high and because this condition is found in less than 1 in 50000 pregnancies, a high index of suspicion is required for a timely diagnosis during pregnancy. Radioisotope therapy may be useful as a bridge to definitive surgical therapy or to control symptoms and growth for inoperative tumours. Patients with unresectable mediastinal paragangliomas, benign or malignant (with no distant metastasis), are also candidates for transplantation. Gowdamarajan et al, reports 28 cardiac transplantations. Median survival for benign disease was 48 months. 
CONCLUSIONS: Surgical resection ensures the best outcome for mediastinal paraganglioma, however, not all mediastinal tumors are resectable due to invasion of vital structures. Radioisotope therapy over the long term has significant toxicity and chemotherapeutic agents often have little impact on tumour growth. Thus surgical resection, or transplantation, continues to be the best option for patients with these tumours
1) Vorapong Phupong, Pairoj Witoonpanich, Thiti Snabboon, Chadin Tharavej, Pornthip Ultchaswadi. Bilateral pheochromocytoma during pregnancy. Arch. Gynecol. Obstet. 271, 276-279 (2005).
2) Arun Gowdamarajan and Robert E. Michler. Therapy for primary cardiac tumors: Is there a role for heart transplantation? Curr Opin Cardiol 2000, 15:121-126.
DISCLOSURE: The following authors have nothing to disclose: Sayf Gazala, Todd McMullen, David Ross, Eric Bedard
No Product/Research Disclosure InformationUniversity of Alberta, Edmonton, AB, Canada