Surgical management of craniospinal axis malignant peripheral nerve sheath tumors: a single-institution experience and literature review

Table 1 Summary of the MPNST cases

N	Age / Sex	Clinical presentation	Symptom duration	Location	Size (cm)	EOR	Grade	Adjuvant therapy	Recurrence	Mets	KPS preop/postop	Follow-up (mo.)
1	67/M	Visual changes, CN VI palsy	4 mo	Rt orbital and superior orbital fissure/cavernous sinus	1.0	STR	High	RT	-	Lung	50/80	Alive at 69.5
2	58/M	Rt flank and LE pain, urinary retention^a	1 mo	Rt T10-sacrum paraspinal	20.2	GTR	High	RT	1	-	30/40	Dead at 5.4
3	58/F	Lt UE numbness and weakness	12 mo	Lt C7-T1 intradural extramedullary	2.5	STR	High	CHE, RT	-	-	70/50	Dead at 37.6
4	39/M	Lt head, ear, and neck numbness and paresthesia	3 mo	Lt C2-C3 extradural extramedullary	5.2	GTR	High	CHE, RT	3	-	90/100	Dead at 22.1
5	37/F	Rt side weakness, confusion, word finding difficulty	3 d	Lt frontal lobe	1.7	STR	High	CHE, RT	3	Lung	30/100	Dead at 26.7
6	35/F	Rt lower back pain^b	3 mo	Rt L2-L5 paraspinal (multiple other lesions non-malignant lesions present)	6.2	GTR	Low	-	-	-	100/100	Alive at 25.8
7	24/F	Rt LE paresthesia, chest pain	1 mo	Rt C7-T1 intradural extramedullary	3.0	GTR	Low	-	2	-	30/100	Alive at 105.2
8	15/M	Rt neck pain, Rt UE paresthesia^c	5 mo	C7 extradural	2.8	STR	High	CHE, RT	1	-	50/60	Dead at 25.1

Abbreviations: CHE chemotherapy, CN cranial nerve, d days, F female, GTR gross total resection, KPS Karnofsky performance score, LE lower extremity, Lt left, M male, Mets metastasis, mo months, RT radiotherapy, Rt right, STR subtotal resection, UE upper extremity, y year
^a Patient has NF-1 + removal of 3 peripheral tumors (3 MPNSTs)
^b History of NF-1 + multiple superficial neurofibromas
^c History of NF-1 + removal of 5 peripheral tumors (3 MPNSTs and 2 plexiform neurofibromas)

ISSN: 1477-7819