NEUROSURGICAL CASE PRESENTATION EXTRADURAL CERVICAL MYXOMATOUS CHORDOMA WITH EXTRA SPINAL EXTENSION AND ENCASEMENT OF THE VERTEBRAL ARTERY WALID S. MAANI PROFESSOR AND CHAIRMAN OF NEUROSURGERY JORDAN UNIVERSITY HOSPITAL AND MEDICAL SCHOOL 7/29/2018 FIRST SPECIALIST'S PROGRAM

CLINICAL PRESENTATION A 15 years old girl who was admitted to JUH complaining of: Neck pain of 3 months’ duration Numbness and parasthesias of the right upper limb of three months’ duration Right sided weakness of two weeks’ duration 7/29/2018 FIRST SPECIALIST'S PROGRAM

CLINICAL PRESENTATION Physical examination revealed: Weakness of the right upper limb predominant in the shoulder abduction, wrist extension & elbow flexion (1/5), with lost biceps and supinator reflexes There was weakness of the right lower limb (4/5), with exaggerated reflexes and a Babiniski sign . The left lower limb was normal in power but the reflexes were exaggerated and the planter was up. The rest of the examination was normal 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM NEURO-IMAGING PLAIN RADIOGRAPHS 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM NEURO-IMAGING PLAIN RADIOGRAPHS RIGHT LATERAL LEFT LATERAL 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM NEURO-IMAGING COMPUTERIZED TOMOGRAPHY TRANSVERSE CUTS WITH WIDEND RIGHT FORAMEN TRANSVERSARIUM 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM NEURO-IMAGING COMPUTERIZED TOMOGRAPHY 3D CT RECONSTRUCTION 7/29/2018 FIRST SPECIALIST'S PROGRAM

CT RECONSTRUCTION SHOWING WIDENING OF THE VERTEBRAL CANAL AND FORAMINA NEURO-IMAGING COMPUTERIZED TOMOGRAPHY CT RECONSTRUCTION SHOWING WIDENING OF THE VERTEBRAL CANAL AND FORAMINA 7/29/2018 FIRST SPECIALIST'S PROGRAM

T2 WEIGHTED IMAGES SHOWING EXTENSION THROUGH 2 INTERVERTEBRAL FORAMINA NEURO-IMAGING MAGNETIC RESONANCE (MRI) T2 WEIGHTED IMAGES SHOWING EXTENSION THROUGH 2 INTERVERTEBRAL FORAMINA C3/4 AND C4/5 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM NEURO-IMAGING MAGNETIC RESONANCE (MRI) T2 WIEGHTED SHOWING ENCASEMENT ON RIGHT T1 WIEGHTED WITH CONTRAST 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM NEURO-IMAGING MAGNETIC RESONANCE (MRI) LEFT T1 WIEGHTED WITH CONTRAST 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM MAGNETIC RESONANCE (MRI) TI WIEGHTED SAGITTAL WITH CONTRAST 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM NEURO-IMAGING VERTEBRAL ANGIOGRAPHY RIGHT VERTEBRAL ARTERY ENCASEMENT 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM NEURO-IMAGING VERTEBRAL ANGIOGRAPHY RIGHT VERTEBRAL ARTERY ENCASEMENT 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM SURGICAL MANAGEMENT FIRST STAGE OPERATION The patient underwent a C3 –C5 laminectomy in the sitting position on the 1st of July. The intra-spinal component of the tumor was excised completely. 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM SURGICAL MANAGEMENT 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM SURGICAL MANAGEMENT The tumour was grey blue in colour It was soft in nature Was removed completely by suction It passed through the intervertebral foramina on the right around the roots and was removed as far laterally as possible 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM HISTOPATHOLOGY The histopathology examination showed features of Myxomatous Chordoma. CHORDOMA 7/29/2018 FIRST SPECIALIST'S PROGRAM

PREPARATION FOR THE SECOND STAGE The patient had an occlusion test of the right vertebral artery two weeks later which proved that the artery could be ligated if needed without consequences. 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM SURGICAL MANAGEMENT SECOND STAGE OPERATION The Head and Neck surgery team went on the 28th of July and through a Mcaffey’s incision excised the extra-spinal part without sacrificing the vertebral artery, the foramina transversaria of C4-C6 were opened and the artery dissected and freed and the tumor was removed macroscopically 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM SURGICAL MANAGEMENT 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM SURGICAL MANAGEMENT 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM SURGICAL MANAGEMENT THE CERVICAL COMPONENT 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM HISTOPATHOLOGY CHORDOMA 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM HISTOPATHOLOGY A light microscopic view of a hematoxylin and eosin (H&E) stained section of a chordoma showing the characteristic physaliphorous cells and mucinous matrix. A higher magnification light microscopic view of a hematoxylin and eosin (H&E) stained section of a chordoma showing physaliphorous cells 7/29/2018 FIRST SPECIALIST'S PROGRAM

POST-OPERATIVE COMPLICATIONS The patient developed a partial right Horner’s syndrome and some weakness of right shoulder abduction following this surgery. 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM ADJUVENT THERAPY The patient was discussed in the inter departmental meetings and agreement was reached on giving a dose of radio-therapy to this radio-resistant tumor. Radiotherapy was delivered at the King Hussein Cancer Center on Oct 5th. 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM 50.4 Gy 28 FRACTIONS 7/29/2018 FIRST SPECIALIST'S PROGRAM

POST OPERATIVE INSTABILITY ONE YEAR AFTER SURGERY PLAIN X-RAYS SHOWING SWAN NECK DEFORMITY FOLLOWING 3 LEVEL LAMINECTOMY 7/29/2018 FIRST SPECIALIST'S PROGRAM

POST OPERATIVE INSTABILITY FELXION & EXTENSION VIEWS OF THE INSTABILITY 7/29/2018 FIRST SPECIALIST'S PROGRAM

POST OPERATIVE INSTABILITY CT RECONSTRUCTION SHOWING SWAN NECK DEFORMITY (EXTENT OF LAMINECTOMY IS SEEN) 7/29/2018 FIRST SPECIALIST'S PROGRAM

POST OPERATIVE INSTABILITY MRI (T1 WEIGHTED) SHOWING SWAN NECK DEFORMITY 7/29/2018 FIRST SPECIALIST'S PROGRAM

POST OPERATIVE INSTABILITY MRI (T2 WEIGHTED) SHOWING SWAN NECK DEFORMITY 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM SURGICAL MANAGEMENT POST OPERATIVE FELXION AND EXTENSION VIEWS 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM SURGICAL MANAGEMENT ANTERIOR CERVICAL FUSION WITH 2 CAGES , PLATE AND 6 SCREWS 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM FOLLOW-UP CLINICAL ALL FUNCTIONS RETURNED TO NORMAL THE PATIENT RETURNED TO SCHOOL SHE IS ATTENDING REGULAR OPD CLINICS RADIOLOGICAL MRI: NO INTRASPINAL COMPONENTS MRI: SMALL EXTRASPINAL NODULE 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM CHORDOMAS Rare tumors (histologically benign) forming <1% of CNS tumors, but around 3% of bone tumors. First described by Virchow in 1857 and called them ecchondrosis physaliphora, believing they were cartilaginous in origin In 1895, Ribbert pierced a nucleus pulposus and found similar tumors. From this bit of evidence, he correctly surmised the notochordal origin of chordomas 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM CHORDOMAS Arise from remnants of the notochord. Occur anywhere from sphenoid to coccyx. Common at basi-occiput (30%)and sacrococcygeal regions (50%), spinal axis tumors are very rare. Vertebral chordomas form 15% and occur in lumbar, cervical and thoracic regions in that order. They invade and destroy bone (locally invasive and rarely metastasize). Occur at any age but mainly 4th. decade. Most patients die within 10 years of presentation 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM CHORDOMAS A light microscopic view of a hematoxylin and eosin (H&E) stained section of a chordoma showing the characteristic physaliphorous cells and mucinous matrix. A higher magnification light microscopic view of a hematoxylin and eosin (H&E) stained section of a chordoma showing physaliphorous cells 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM CHORDOMAS Diagnosis is by: Imaging Exclusion FNA of tumor Treatment: Surgical Radiation Medical: Clinical trials are underway to study the effectiveness of imatinib mesylate in the treatment of chordoma. Imatinib mesylate is a tyrosine kinase inhibitor targeting several enzymes including platelet-derived growth factor receptor--b (PDGFRB), which can be expressed in chordomas. This drug has been shown to have antitumor activity in chordomas 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM CHORDOMAS Frequent follow-up is required because of the high rate of recurrence of these tumors. Tumor recurrence identified early is easier to treat. The average interval to recurrence is 3.8 years for radically resected tumors, 2.1 years for subtotal resection followed by radiation therapy, and 8 months for subtotal excision without adjuvant therapy. The interval of follow-up, including repeat MRI or CT scans, depends on the completeness of the resection Because residual tumor drastically shortens the recurrence time, patients with known or suspected residual tumor need to be evaluated more frequently. 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM CHORDOMAS The 5-year survival rate is estimated to be 51%, and the 10-year survival is estimated to be 35%. Factors that may improve prognosis are young age, complete resection, and the addition of radiation therapy in incompletely resected tumors 7/29/2018 FIRST SPECIALIST'S PROGRAM

FIRST SPECIALIST'S PROGRAM THANK YOU 7/29/2018 FIRST SPECIALIST'S PROGRAM