Management of Nerve Sheath Tumors Arising in the Sympathetic Chain

Surbhi Jain, MD; Kristin Houseknecht, MPH; Amyn M. Rojiani, MD, PhD; Matthias Setzer, MD; Frank D. Vrionis, MD, MPH, PhD

Disclosures

Cancer Control. 2008;15(4):352-357. 

In This Article

Illustrative Cases

All three patients were women, with a mean age of 44 years (range 41 to 46 years). Two patients were asymptomatic from their thoracic and lumbar tumors and 1 presented with Horner's syndrome and dysphagia from a cervical region tumor. The tumors were positive on positron-emission tomography (PET) scan and mimicked a spindle cell sarcoma. Complete excision of tumors was performed in all three patients without any added morbidity or mortality. Surgical observation, histopathology, and immunochemistry confirmed the tumors to be schwannomas of the sympathetic chain. The schwannomas had a mean diameter of 3.2 cm (range 3.1 to 3.5 cm) and all were benign. At a mean follow-up of 21 months (range 6 to 48 months) following resection, all patients remained free of disease recurrence. The Table summarizes preoperative symptoms, clinical findings, surgical approach, and outcome of all three patients.

Case 1

A 45-year-old woman presented with a right paraspinal mass that was demonstrated in a preoperative chest x-ray for scheduled discectomy and laminectomy of L4-L5. The patient presented with radicular pain and right foot drop presumably related to disc herniation at L4-L5. The patient had no symptoms or signs attributable to the T5-T6 mass and denied shortness of breath, cough, chest pain, or recent weight loss. Physical examination was significant only for findings related to disc herniation at L4-L5. Urinalysis showed normal catecholamine levels, decreasing the possibility of pheochromocytoma.

Chest computed tomography (CT) demonstrated a 3.2-cm right paraspinal tumor at T5-T6, and a subsequent PET scan demonstrated intense glucose hypermetabolism in the region of the lesion but in no other location. The lesion was enhancing on magnetic resonance imaging (MRI), and no evidence of spinal or intervertebral foramen invasion was present. Given the history and imaging findings, it was believed that the tumor was likely to be of neurogenic origin (Fig 1A-C).

Figure 1A-C.

MRI of coronal (A), sagittal (B), and axial (C) views of thoracic spine of a 45-year-old woman with an incidental large 5- to 6-cm mass involving posterior mediastinum and paraspinal region at T4 level. Preoperative PET scan showed hypermetabolism in the lesion. Tumor was excised by standard transthoracic approach. The patient had an uneventful postoperative course and is doing well 7 months following surgery.

The patient underwent resection of the tumor by means of right posterolateral thoracotomy through the bed of the fifth rib. The tumor was easily visualized as an extrapleural mass on the T5-T6 vertebral bodies that was not adherent to the lung. Grossly, it appeared as a bosselated mass with hyperemia that did not extend into the neuroforamen and appeared to be originating from the right sympathetic chain. The sympathetic chain was therefore cut both superiorly and inferiorly to the tumor and submitted with the tumor for pathologic evaluation. The patient recovered with no complications and has had no recurrence at 7 months of follow-up.

Case 2

A 46-year-old woman presented with a left-sided Horner's syndrome that retrospectively was determined to have been developing for 6 years prior to presentation. The patient was referred to our institution after discovery of a left-sided paracervical tumor. The patient described anhidrosis of the left forehead area, noticed drooping of her left eyelid, and also reported intermittent dysphagia. She denied neck pain and experienced no numbness or weakness in the left arm. On physical examination, a tender mass was evident deep to the left inferior sternocleidomastoid muscle. Miosis of the left pupil was also noted, although all other physical examination findings were within normal limits.

Magnetic resonance imaging (MRI) demonstrated a 3.5 cm × 2.5 cm homogenous and well-circumscribed left paracervical mass, the bottom edge of which was at the level of the clavicle. The tumor was at the level of C7-T1 and was located posterior to the carotid sheath and lateral to the trachea and esophagus, causing some esophageal compression. Prior to resection, it was felt that this tumor was likely arising from the cervical sympathetic ganglion.

The patient underwent resection of the tumor by means of an anterior approach involving an incision along the inferior medial border of the sternocleidomastoid muscle and the medial border of the clavicle. Because of the proximity of the tumor to the C8- T1 nerve, brachial plexus monitoring was performed during the surgery. After dissection and identification of surrounding structures, the tumor was visualized inferior to the internal jugular vein and appeared grossly as a well encapsulated mass with yellow necrotic contents. The tumor was specifically identified as arising from the left stellate ganglion of the cervical sympathetic chain, and parts of the tumor were sent for frozen section. The tumor was carefully dissected from the carotid artery and removal of the tumor and the left stellate ganglion was completed. The patient recovered with no complications other than persistent left Horner's syndrome.

Case 3

A 41-year-old woman presented with a 3-cm retroperitoneal mass that was found on routine abdominal ultrasound. After CT-guided biopsy revealed the mass to be a schwannoma, the patient was referred to our institution for further evaluation. The patient was asymptomatic at presentation, denying symptoms suggestive of a secreting tumor such as flushing, headaches, or hypertension. She was in good health with no personal or family history of malignancy or neurofibromatosis. Physical examination was unremarkable and within normal limits.

A PET scan demonstrated increased uptake in the area of the mass as well as in the left iliac node area. An MRI of the abdomen with contrast revealed a 3.1 × 2.3-cm enhancing mass located between the aorta and inferior vena cava just beneath the level of the left renal vein. The mass was visualized as external to the inferior vena cava and was causing compression due to its close proximity.

Surgical management involved joint efforts of the urological and neurological surgery teams. A midline incision was made from the xiphisternum to below the umbilicus and the abdomen was opened for exploration, with the intestines being retracted superiorly. Surrounding structures were dissected away from the tumor,which was located retroperitoneally just beneath the inferior vena cava and renal vein. The tumor was not vascular and was well encapsulated, appearing to arise from the sympathetic chain on the right side of the lumbar spine. After careful mobilization, the tumor was removed en bloc along with the associated ganglion and was sent for pathologic evaluation along with corresponding lymph nodes. The patient recovered from the procedure with no complications.

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