Neurofibrosarcoma of the Cervical Sympathetic Trunk: Report of a Clinical Case

The aim of this work is to review the management of compressive cervical neurofibrosarcoma. We report the case of a 45-year-old female housewife patient (DT) with no medical or surgical history admitted to the department for left laterocervical swelling. The onset of the disease dates to 6 months after admission, marked by a progressive left laterocervical swelling, with permanent evolution associated with left neck pain, torticollis, and dysphagia to solids. The cervical examination noted a left laterocervical swelling of approximately 8 cm/6 cm, hard, fixed, painful to palpation, with regular contours, non-blowing, non-beating, skin with an inflamed appearance, a bulge of the left lateral wall of the oropharynx pushing the left tonsil inward. A cervical CT scan revealed a weakly enhanced laterocervical tissue hypodensity pushing outward the jugulocarotid axis communicating with the conjugation foramina of C3, C4 and C5 with mass effect on the left vascular-nervous axis of the neck and the pharynx. The standard preoperative biological assessment noted a hemoglobin level of 11.04 g/dl with a hematocrit of 41.06%, and the chest X-ray, frontal and profile, was unremarkable. A transfusion of two bags of isogroup rhesus blood pre- and post-operatively was performed. Our approach used a wide cervicotomy by Paul Andre’s “J” incision removing the skin, the subcutaneous cellular tissue and the platysma. The reclination of the anterior flap gave us access to the omohyoid and sternocleidomastoid muscles which we sectioned. The jugulocarotid and vagal neurovascular bundle were dissected from the capsule of the mass, and the spinal nerve was preserved. The tumor was freed from the cervical vertebrae and pharynx. The resection was complete with some ipsilateral cellulolymphatic flows. We established a 2 mm margin from the edges of the lesion. This required us to perform a resection of the transverse processes and the superior articular processes of C3, C4 and C5. We performed a layer-by-layer closure on a drain. Histology revealed bundles of spindle cells with large and hyperchromatic nuclei; the nucleoli are visible and abnormal mitoses which are in favor of a neurofibrosarcoma. Immunohistochemistry allowed us to confirm the diagnosis of neurofibrosarcoma. The wound healed around the 15th day. The patient was referred for radiotherapy after a multidisciplinary consultation meeting. The clinical evolution after 6 months of post-radiotherapy monitoring was favorable. No functional discomfort was noted. No functional discomfort was noted.