P042: MALIGNANT PERIPHERAL NERVE SHEATH TUMOR OF THE BRACHIAL PLEXUS IN A JEHOVAH'S WITNESS PATIENT
Shahrukh Bengali; Asad Bashir; Barys Ihnatsenka; University of Florida
Introduction / Background: Malignant peripheral nerve sheath tumors (MPNST) are uncommon, aggressive tumors representing 5 to 10% of all soft-tissue sarcomas. The primary treatment for MPNSTs remains wide local excision although prognosis is poor. Compared to other soft tissue sarcomas, MPNST has the highest risk of sarcoma-specific death with a 5-year overall survival rate of 49%. Resection of these tumors can be challenging depending on anatomic location and tumor size, and there may be significant risk for blood loss intraoperatively. Here we present a unique case of MPNST of the brachial plexus in a Jehovah’s Witness patient undergoing surgical resection involving complex spine surgery and forequarter amputation.
Methods: A 51 year old female Jehovah’s Witness patient initially developed paresthesias of her right arm which progressed to weakness prompting further imaging. MRI of brachial plexus showed a necrotic mass involving the brachial plexus roots measuring 5.3cm in greatest diameter with spread medially to C6 - T1 nerve roots, anterior chest wall, and subclavian vessels and biopsy confirmed a diagnosis of malignant spindle cell neoplasm. Preoperative hemoglobin (Hgb) level was 11.7 g/dL and the patient would not accept packed red blood cells, fresh frozen plasma, platelets, or cryoprecipitate due to religious beliefs. She underwent balloon test occlusion and vertebral artery sacrifice to minimize blood loss for her posterior instrumentation and fusion from C3 - T2 with facetectomy, laminectomy, and ligation of C5 - T1 nerve roots. Postoperative Hgb was 7.9 g/dL, and a decision was made to postpone amputation to allow for Hgb recovery with administration of intravenous iron and epoetin alfa (EPO). She underwent forequarter amputation one week later with preoperative Hgb of 9.6 g/dL. Our team implemented a variety of blood conservation techniques including acute normovolemic hemodilution (ANH), permissive hypotension, administration of tranexamic acid (TXA), and utilization of an elastic bandage exsanguination technique to the operative arm. A preoperative thoracic epidural was placed at the level of T4 along with superficial cervical plexus and interscalene block to assist with hypotensive anesthesia and minimize surgical stress.
Results: Forequarter amputation surgery was uneventful with an estimated blood loss of 150mL. She was extubated in the operating room and the epidural was discontinued to avoid postoperative hypotension. Postoperative Hgb was 9.0 g/dL and her postoperative course was complicated by deep venous thrombosis. She was discharged home on postoperative day 9 from amputation.
Discussion / Conclusion: In conclusion, surgical resection of MPNST in a Jehovah’s Witness patient with preoperative anemia can be a challenging case requiring a large interdisciplinary team. Preoperative supplementation with intravenous iron and EPO, ANH, and intraoperative TXA have all been shown to significantly reduce allogeneic transfusion requirements. Furthermore, studies suggest epidural and regional anesthesia is an effective technique for hypotensive anesthesia which reduces blood loss and transfusion requirements in orthopedic surgery patients.