Shanik J. Fernando1, Kelly L. Groom2, James. L. Netterville2

1Vanderbilt University School of Medicine, 2Department of Otolaryngology-Head and Neck Surgery, Vanderbilt University Medical Center, Nashville, TN USA

Primary Tumors of the Cervical and Brachial Plexus

2. Introduction Primary tumors of the cervical and brachial plexus are rare, with their description in the literature being limited in nature1,2 These infrequently seen tumors primarily involving the cervical and brachial plexus commonly present to Head & Neck Surgeons. In our experience, utilization of multidisciplinary teams working in collaboration has resulted in excellent outcomes patients Presentation Most commonly asymptomatic with incidental mass found on imaging3 Subcutaneous enlarging mass which may or may not be tender to palpation In some cases, may present with a motor and/or sensory deficit4 Diagnosis Symptoms as reviewed above Imaging: CT or MRI to localize mass and determine extent Biopsy: Ultrasound guided FNA/Core biopsy/Excisional biopsy Management Resection of the tumor via the transcervical approach with modification dependent on tumor location and presentation

o In the case of a schwannoma pathology, enucleation of the tumor in an attempt to preserve neurologic function of the nerve may be pursued5

1. Abstract Objectives: The infrequently seen tumors primarily involving the cervical and brachial plexus commonly present to the head and neck surgeon.

We will review the various pathologies, presenting symptoms, surgical approaches, and patient outcomes in our series treated over the last 15

years.

Study Design: Case series of patients having cervical or brachial plexus tumors requiring surgical intervention at a tertiary care referral center

from 2000-2014.

Methods: Chart review from EMR.

Results: Fourteen patient charts met inclusion criteria (12 women, 2 men, age 14-73 years.) Neurofibroma (28.5%, 1/4 malignant) and

schwannoma (28.5%, 1/4 malignant) were the most commonly presenting pathologies. The remainder of pathologies included vascular lesions,

chordoma, Castlemans disease, and desmoid tumor. Most present with a tender or asymptomatic mass. With no intracordal extension, the

lesions of the cervical plexus were treated solely by our team via a transcervical approach. In one patient with intraforaminal extension we

partnered with the neurosurgical spine team. For treatment of brachial plexus lesions we partnered with the orthopedic hand surgery team.

Brachial plexus function was initially preserved in all patients, however one patient with malignant schwannoma died of recurrent disease

following radiation therapy.

Conclusions: This case series examines our surgical management of patients presenting with primary tumors of the cervical and brachial

plexus. In all cases, MRI was the preferred imaging modality for preoperative evaluation of the soft tissue involvement of the tumors. In lesions

of the brachial plexus or cervical plexus lesions with intraforaminal extension, it is important to form appropriate surgical teams to achieve the

best outcome.

3. Methods & Materials An IRB approved retrospective review of patients presenting to the Department of Otolaryngology at Vanderbilt University Medical Center

between 2000-2015 with primary cervical or brachial plexus pathology ultimately receiving surgical intervention.

4. Results A total of 14 patients received surgical treatment for either cervical or brachial plexus lesions between January 2000 and April 2015. The ages of these patients ranges from 14 to 73, with the average age being 41.3. There was a strong female preponderance with males only comprising 14.3% of study subjects (2/14.) Neurofibroma and schwannoma were the most common pathologies (4/14 for each, 1/4 malignant for each, 28.5% for each.) The remaining observed pathologies included vascular lesions, chordoma, Castlemans disease, and desmoid tumor. Presentations varied but an asymptomatic presentation (5/14) was the most common presentation. Symptoms of swelling, pain, paresthesias were found in various combinations. MRI represented the most common pre operative imaging technique (12/14 patients.) Biopsy was performed in 64.2% patients (9/14) patients and identified the correct pathology in 44.4% of patients (4/9.)The length of follow up ranged from 1 to 64 months, with the average length of follow up being 23.2 months. All patients received gross total resection but specific approach and surgical team were dependent on the patient/pathology.

Pt IDCervical or

Brachial Plexus

Age

(years)Sex Presenting Symptom Pre-op Imaging

Structures

involvedCollaboration* Procedure Pathology F/u (months) Complications

1 Cervical 17 F Fullness in neck CT/MRI C1-C2 Resection Neurofibroma 7

2 Cervical 73 F

Discomfort and pain in L

shoulder, associated

paresthesias CT/MRI C4-C5 Ortho Resection Desmoid 64

Pain in post-

operative period

requiring PT

3 Cervical 64 F Asymptomatic MRI C2 (I) NSGY Resection Neurofibroma 58 CSF leak

4 Cervical 30 F

Neck mass, mild pain,

numbness of the

overlying skin. Horner's CT/MRI C2 Resection Neurofibroma 30

First bite

syndrome and

hoarseness

5 Cervical 60 F

Fullness in neck with

pain in trapezius and MRI C3-C5 (I) NSGY Resection Chordoma 6

Neck tightness

noted 2 months

6 Cervical 34 F Asymptomatic CT/MRI C6 Resection Schwannoma 1

7 Cervical 53 F

Swelling and tender to

palpation U/S, MRI C1-C2 Resection Schwannoma 3

Seroma

requiring

drainage

8 Brachial 33 F Asymptomatic CT/MRI C5-C6 Ortho Enucleation Schwannoma 41

Numbness in

the

supraclavicular

area

9 Brachial 14 F

Left supraclavicular

adenopathy and

tenderness CT/MRI C5-C6 Ortho Enucleation Schwannoma 30

Reduced

function L arm

and recurrence

leading to death

10 Brachial 27 F Headaches CT/MRI C4-C6 Ortho Resection

Intramuscular

hemangioma 30

11 Brachial 56 F Asymptomatic MRI C7 roots Enucleation Schwannoma 6

12 Brachial 55 M

Weakness and

numbness in L neck and

supraclavicular region MRI C8-T1 Ortho Resection Neurofibroma 1

13 Both 22 F Asymptomatic CT/MRI Resection

Castleman's

disease 39

Temporary

weakness of arm

14 Both 41 M

Pain and pressure in L

neck. Dysphagia CT Resection Hemangioma 9

9. Correspondence Shanik Fernando; School of Medicine, Vanderbilt University, Light Hall 2215 Gartland Avenue

Nashville, TN 37212; email Shanik.Fernando@Vanderbilt.edu; telephone: 615-322-5000

5. Discussion The presentation of patients with either cervical or brachial plexus tumors is often asymptomatic but may be accompanied by symptoms

such as a painless mass, fullness, pain, paresthesias, or shoulder weakness. Imaging is key to planning surgical approach, often using MRI for optimal soft tissue visualization6 In consideration of primary tumors being a small percentage of lesions appearing in the cervical and brachial plexus, it is advisable to

perform biopsy despite the equivocal role of biopsy in this case series The most common pathologies were neurofibromas and schwannomas.

o Of note, schwannomas may be addressed using enucleation in order to spare the patient of the post-operative morbidity that accompanies nerve resection5

Collaboration with appropriate surgical teams is important in addressing surgically challenging pathologies7 o In the case of a brachial plexus tumor, collaboration with the Orthopedics service may be considered o In the case of a cervical plexus tumor, collaboration with the Neurosurgery service may be considered

o Collaboration will be likely necessary in the case of intraforaminal extension Consider consultation of the intraoperative Nerve Monitoring service

6. Conclusion This case series adds to the limited existing literature regarding primary tumors of the cervical and brachial plexus, uniquely highlighting the

role of collaboration between specialists when addressing primary tumors of the cervical and brachial plexus Creation of appropriate collaborative surgical teams may assist in achieving optimal patient outcomes

7. References 1. Binder, Devin K., Justin S. Smith, and Nicholas M. Barbaro. "Primary Brachial Plexus Tumors: Imaging, Surgical, and Pathological Findings in 25 Patients." Neurosurgical FOCUS 16.5 (2004): 1-6. Web. 2. Desai, Ketan I. "Primary Benign Brachial Plexus Tumors." Neurosurgery 70.1 (2012): 220-33. Web. 3. Ganju, Aruna, Norbert Roosen, David G. Kline, and Robert L. Tiel. "Outcomes in a Consecutive Series of 111 Surgically Treated Plexal Tumors: A Review of the Experience at the Louisiana State University Health Sciences Center." Journal of Neurosurgery 95.1 (2001): 51-60. Web. 4. Go, Myeong Hoon, Sang Hyun Kim, and Ki Hong Cho. "Brachial Plexus Tumors in a Consecutive Series of Twenty One Patients." Journal of Korean Neurosurgical Society. The Korean Neurosurgical Society, n.d. Web. 02 Apr. 2015. 5. Netterville JL, Groom K. “Function sparing intracapsular enucleation of cervical schwannomas..” Curr Opin Otolaryngology Head Neck Surg. 2015 Apr;23(2):176-9. 6. Rettenbacher T, Sögner P, Springer P, Fiegl M, Hussl H, zur Nedden D. Schwannoma of the brachial plexus: cross-sectional imaging diagnosis using CT, sonography, and MR imaging. Eur Radiol. 2003 Aug;13(8):1872-5 7. Komisar A, Blaugrund SM, Camins M, Mangiardi J. “Combined approach for excision of cervical nerve tumors with dural extension.” Head Neck. 1993 Mar-Apr;15(2):153-7.

8. Acknowledgements Funding provided by the Medical Scholars Research Fellowship at Vanderbilt University School of Medicine, and the Department of Otolaryngology-Head and Neck Surgery, Vanderbilt University Medical Center

Table 1: Summary of the clinical characteristics of tumors, pre-operative imaging, location/structures involved, collaborating operative service, procedure, tumor pathology, follow-up in months, and complications (*Ortho : Orthopedic Surgery, NSGY : Neurosurgery, (I): Intraforaminal.)

Figure 1: Preoperative and intraoperative imaging of cervical (Pt 7; ABC) and brachial plexus (Pt 11; DEF) tumor cases. A. Gadolinium-contrasted T1 MRI axial cut of C1-C2 cervical plexus schwannoma. B. Intraoperative photo demonstrating approach and dissection for cervical plexus tumor. C. Intraoperative photo of vagus lying on carotid artery and fascial edge of sympathetic trunk retracted; demonstrating structures are intact and isolated from cervical plexus schwannoma. D. Gadolinium-contrasted STIR MRI coronal cut of C7 root brachial plexus schwannoma. E. Intraoperative photo demonstrating approach and dissection for enucleation of brachial plexus schwannoma. F. Intraoperative photo demonstrating successful enucleation of schwannoma.

A

D

B

E

C

F