Anesthetic Management of a Large Retroperitoneal Paraganglioma: A Rare Case Report

R Arun Kumar^1*, S Vishnu Priyangan², B Kavin Kumar^3
1Associate Professor, Department of Anaesthesiology, PSG Institute of Medical Sciences and Research, Coimbatore, Tamilnadu, India
²Junior Resident, Department of Anaesthesiology, PSG Institute of Medical Sciences and Research, Coimbatore, Tamilnadu, India
³Junior Resident, Department of General Surgery, PSG Institute of Medical Sciences and Research, Coimbatore, Tamilnadu, India

Correspondence to: R Arun Kumar, Associate Professor, Department of Anaesthesiology, PSG Institute of Medical Sciences and Research, Coimbatore, Tamilnadu, India; Email: shivaaniarun76@gmail.com
Received date: November 21, 2020; Accepted date: December 04, 2020; Published date: December 11, 2020
Citation: Kumar RA, Priyangan SV, Kumar BK, et al. (2020) Anesthetic Management of a Large Retroperitoneal Paraganglioma: A Rare Case Report. J Clin Anesthes Res 1(1): pp. 1-4. doi: 10.52916/jcar204002
Copyright: ©2020 Kumar RA, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution and reproduction in any medium, provided the original author and source are credited.

Abstract

Paragangliomas are rare tumors derived from the neural crest cells. Most of the paragangliomas occur as sporadic tumors. The commonest incidence occurs in the second and third decade of life with a slight male preponderance. Clinically patients with a retroperitoneal paraganglioma often present with back pain or a palpable mass. There is a 5% incidence of turning into malignancy and these tumors are associated with a high risk of morbidity and mortality from cardiovascular complications. Management for paragangliomas typically involves complete surgical excision. A multi-disciplinary approach is suggested for a better outcome of the procedure

Keywords

Retroperitoneal paraganglioma, Anesthesia concerns, Sporadic tumor, Neural crest.

Introduction

Phaeochromocytoma is an endocrine tumor arising from catecholamine producing chromaffin cells in the adrenal medulla by 2004 WHO classification. Closely related tumors found in the extra-adrenal sympathetic and parasympathetic paraganglia were classified as extra-adrenal paragangliomas [1]. Paraganglioma are rare tumors derived from the neural crest cells. Extra adrenal paragangliomas may develop anywhere from chromaffin tissue along the autonomic nervous system usually found with many of the major blood vessels over the head, neck, thorax, abdomen, and pelvis [2]. In this context, we present the anesthetic management of such rare tumors.

29 year aged male, Engineer by profession working in a private concern was referred to a general surgical department in our institute with a history of abdominal fullness and discomfort for the past 2 years on and off. No significant past medical or surgical history of any systemic illness and neither a smoker nor an alcoholic. On examination, he was fairly nourished and found to be anemic. His weight was 52 kg, height was 160 cm with a BMI of 20.3 Vitals findings showed a heart rate of 110 beats per minute and blood pressure was 124 over 90 mm/Hg. Airway and spine examination was normal. Abdominal examination revealed a firm mass of size 15 × 20 cm occupying the whole of the abdomen (Figure 1). No tenderness was present over the abdomen. Another systemic examination was normal.

Routine blood investigations revealed anemia with hemoglobin of 7.1 g/dl with all other blood parameters within normal limits. Urine metanephrines was 166.2 μg/24 hours. Chest X-ray, electrocardiography, Renal, and liver functions were within normal limits. Ultrasound abdomen revealed a large extensively vascular heterogeneous mass lesion in the centre of the abdomen (Figure 2). Computed tomography with contrast, revealed a large well defined solid mass involving mesentery and retroperitoneum occupying the whole of the abdomen, displacing right iliac vessels posteriorly and laterally with no evidence of encasements along with right ureter displacement. Anteriorly mass extended till abdominal wall without any infiltration and posteriorly it was compressing both psoas muscle. Bowel loops were displaced laterally and all features were suggestive towards the diagnosis of the gastrointestinal stromal tumor.

Echocardiography showed features of sinus tachycardia with left ventricular hypertrophy with an ejection fraction of 39%. Cardiac magnetic resonance imaging with contrast showed moderate systolic dysfunction due to left ventricular moderate hypokinesia, thickening of the interventricular septum, left ventricular myocardial risk index was in upper normal limits with diffuse thin subendocardial enhancement in the left ventricle suggesting amyloidosis. Pre-operatively blood transfusion was done to improve hemoglobin to 10 g/dl along with haematinics. USG guided trucut biopsy was taken from the mass under conscious sedation. There was no hemorrhage or any hemodynamic instability following the biopsy and the histopathology report was suggestive of paraganglioma/phaeochromocytoma.

Endocrinology opinion was sought and advised for urine metanephrines, serum prolactin, and ANA profile. All of these test reports were within normal limits. The cardiologist diagnosed as catecholamine-induced cardiomyopathy provisionally and started on alpha (Tablet Prazosin 2.5 mg OD) and beta-blockers (Tablet Inderal 10 mg BD). Specialist opinion including pre-anesthetic evaluation and fitness for surgery was obtained from all concerned specialties before planning for wide excision of the mass with prophylactic bilateral ureteric stenting. He was categorized as American Society of Anesthesiologist ASA Grade II and proceeded to surgery with adequate reservation of blood and blood products, starvation of solids 6 hours and clear fluids up to 2 hours before surgery and obtained consent for epidural anesthesia, invasive lines, general anesthesia including post-operative ventilation considering the major surgery. Normal saline was started at a rate of 50ml/Hr as maintenance from midnight.

The plan of anesthesia executed was combined epidural and general anesthesia with controlled ventilation and invasive lines. Emergency cardiac drugs were kept ready. Datex ohmeda anesthesia Boyle’s machine was used; ASA standard monitoring was done and baseline readings noted. Under local anesthetic infiltration, Epidural anesthesia was instituted at the level of T8-T9 with 18G Tuohy needle and invasive lines were secured under USG guidance in the right internal jugular vein for CVP monitoring and fluid administration. Right radial artery cannulated for continuous arterial pressure monitoring and blood sample analysis when needed.

The patient was premedicated with Injection Glycopyrrolate 0.1 mg and Injection Midazolam 2 mg intravenously (IV) in the pre-operative room half an hour before shifting the patient inside the OT. Analgesia was supplemented with Injection Fentanyl 100 μg IV and stress response was attenuated using preservative-free 2% lignocaine 40 mg IV 90 seconds before intubation. Anesthesia was induced with Injection Etomidate 10mg and the patient was intubated using 4 MAC blade 8.5 mm portex cuffed endotracheal tube with Injection Vecuronium 5mg IV. Laryngoscopic Grade 1 was noted and the endotracheal tube was fixed at 22 cm at the angle of the mouth, bilateral air entry checked and confirmed by waveform capnography. Anaesthesia maintained with controlled ventilation using N₂O:O₂ (50:50) and Sevoflurane achieving a MAC of 1 to 1.3. Epidural anesthesia initiated with 0.2% Ropivacaine with Fentanyl 2μg/ml at a titrated dose. BIS monitoring is used to assess the depth of anesthesia.

Bilateral ureteric stenting was done before laparotomy. The abdomen was opened in layers and a solid encapsulated tumor of size 20 × 15 cm noted, occupying the mesentry of small bowel and retroperitoneum displacing the large bowel and small bowel to the left. No metastasis or ascites noted. On mobilization of the tumor, it was found to be adherent to right common iliac artery and internal iliac artery. The vascular surgeon was sought for dissection and the tumor was excised partially leaving behind the adhered part and primary repair of the common iliac artery was done with prolene sutures. Nitroglycerin infusion was titrated to blood pressure response at the rate of 0.5 to 1.5 μg/kg/min. Retroperitoneum closed in layer and good hemostasis was attained before abdominal closure.

During the surgery, the patient had hypotension during tumor dissection and following excision, requiring Dopamine about 8 to 10 μg/kg/min and Noradrenaline support up to 0.08 μg/kg/ min and a single dose of Hydrocortisone also supplemented. Intraoperative blood loss was around 1500 ml and required 2 packed cells, 2 fresh frozen plasma transfusions. 10% of Calcium gluconate 10cc was given. No evidence of hypoxia, acidosis or hypothermia was noted intraoperatively. Arterial blood gases were satisfactory after elective ventilation for two hours in Surgical ICU. The patient had a good clinical recovery, normothermic, weaned off vasopressors, no acidosis or alkalosis and was extubated in the surgical ICU on the same day of surgery (Figure 3).