Case Reports in Neurological Medicine

Case Report

Ominous Occurrence of Spinal Intradural Primary Malignant Peripheral Nerve Sheath Tumor Four Decades following Radiation Therapy for Testicular Seminoma

Table 1

Primary spinal malignant peripheral nerve sheath tumors (MPNST) reported in the literature.
Author & yearAge (yrs), sexLocationPresentationNF historyRadiation historyExtent of resectionRadiation therapyChemotherapyRecurrenceMetastasisOutcome
Thomeer et al., 1981 [12]42, MCauda equinaLow back pain for 9 yrs with occasional L radiculopathy, impotence for 4 wks, leg weakness for 3 wksNot specifiedTotalT11-S4 2.5 Gy 4x/wk for 6 wksYes, 3 yrs T9-11NoAlive at 3 yrs
Valdueza et al., 1991 [13]40, FThoracicThoracic pain, R leg weaknessNot specifiedPartialTotal 12 Gy after initial operation, total 40 Gy after spinal metastasisNoYes, 2 moChest, pelvis, spineAlive at 4 yrs
43, FThoracicLow back pain for 1 mo, leg weakness for 2 wksNot specifiedPartialTotal 24 Gy after first operation, total 32 Gy after second operation for recurrenceNoYes, 8 yrsNoAlive at 10 yrs
47, MCervicalNeck pain radiating to R shoulder for 9 mo, R arm weakness for 1 moNF1Not specifiedTotalTotal 10 GyNoYes, 3 mosBrain, lumbarDead at 18 mos
18, MCervicalL shoulder and arm pain for 4 mosNot specifiedTotalNoNoNoNoAlive at 8 mos
70, FCervicalNeck pain radiating to R shoulder for 6 mosNot specifiedTotalNoNoNoNoAlive at 7 mos
Seppälä et al., 199313, MLumbarParaparesisNF112 yrs ago for Wilm’s tumorPartialNot specifiedNot specifiedYesBrain, systemicDead at 2 mos
13, MLumbarParaparesisNot specifiedTotalNot specifiedNot specifiedYesSystemicDead at 7 mos
45, FSacralNot specifiedNF1Not specifiedPartialNot specifiedNot specifiedYesSystemicDead at 2 mos
35, MLumbarRadiculopathyNF2Not specifiedTotalNot specifiedNot specifiedYesSystemicDead at 18 mos
23, FThoracicNot specifiedNot specifiedTotalNot specifiedNot specifiedYesSystemicDead at 8 mos
37, FCervicalRadiculopathyNot specifiedTotalYesNoYes, 2 yrsSystemicDead at 6 yrs
Celli et al., 1995 [15]52, FThoracicPain for 8 mos, weaknessNot specifiedTotalNoNoNoNoAlive at 6 yrs
68, FLumbarPain for 9 mos, weaknessNot specifiedTotalNoNoNoNoAlive at 2 yrs
43, MLumbarPain for 3 mosNot specifiedTotalNoNoNoNoAlive at 6 yrs
36, FThoracicPain for 5 mosNot specifiedTotalYesNoYesNoAlive at 4 yrs
22, FCervicalPain for 2 yrs, weakess, incontinenceNF1Not specifiedTotalYesNoYesLungDead at 6 mos
30, MThoracicPain for 3 yrs, weaknessNot specifiedTotalYesNoYesLungDead at 14 mos
West et al., 1997 [7]40, MSacralRadiculopathy8 yrs ago for testicular seminoma, 30.6 GyTotalNoNoYes, 7 mosNoNot specified
Kourea et al., 1998 [8]11, FLumbar, sacralNot specifiedNF1Not specifiedPartialYesYesYes, 0.5 mosYes, location not specifiedDead at 5 mos
25, FLumbarNot specifiedNF1Not specifiedPartialYesYesNoNoAlive at 18 yrs
33, FLumbarNot specifiedNF1Not specifiedPartialNoNoNoYes, location not specifiedDead at 2 mos
33, MLumbarNot specifiedNF1Not specifiedPartialYesYesYes, 3 mosYes, location not specifiedDead at 22 mos
31, FLumbarNot specifiedNF1Not specifiedpartialyesYesyes, 6 mosyes, location not specifieddead at 10 mos
37, FLumbarIncidentalNot specifiedPartialNoYesYes, 3 mosNoDead at 4 mos
40, MSacralNot specifiedFor testicular seminomaTotalNoNoYes, 9 mosYes, location not specifiedAlive at 14 mos
17, MThoracicNot specifiedNF1Not specifiedPartialNoYesYesYes, location not specifiedDead at 11 mos
19, FThoracicIncidentalNF1Not specifiedTotalNoNoNoNoAlive at 35 mos
53, FThoracicNot specifiedFor breast carcinomaPartialNoNoYes, 6 mosYes, location not specifiedDead at 7 mo
26, MThoracicNot specifiedFor Hodgkin’s lymphomaPartialYesNoYes, 13 mosYes, location not specifiedDead at 27 mo
Acharya et al., 2001 [20]32, MCauda equinaBack pain, leg weakness, bowel and bladder dysfunctionNot specifiedPartialYesNoNoNoAlive at 18 mos
Yone et al., 2004 [16]4, MCauda equinaLow back pain, radiculopathy, bladder dysfunctionNot specifiedTotalYesYesYesBrain, spineDead at 21 mos
Adamson et al., 2004 [17]37, MCervicalL C6 radiculopathy6 yrs ago for Hodgkin’s lymphomaPartialYesNoNot specifiedNoDead after 1 yr
30, FCervicalR Horner syndrome, R C6 radiculopathy, R tricep and wrist extensor weakness5 yrs ago for Hodgkin’s lymphomaPartialNoNoNot specifiedNoDead after 1 yr
Amin et al., 2004 [9]38, MCauda equinaBack pain, leg weakness, bowel and bladder dysfunction10 yrs ago for testicular seminoma, 30 Gy/15 fractions over 3 wksBiopsyNoYes, palliativeYes, 7 mosNot specifiedNot specified
Albayrak et al., 2006 [18]25, MThoracicParaparesis, bladder dysfunctionNF1Not specifiedTotalNoNoYes, 7 wksLungAlive at 7 wks
Chamoun et al., 2009 [14]5, FCervicalPain, gait disturbanceNot specifiedPartialYesYesYesBrain, thoracic and lumbar spineAlive at 4 mos
Xu et al., 20128, MLumbarPainNot specifiedTotalYesNoYesBrainDead at 16 mos
Mitsuhara et al., 201347, FCauda equinaBack pain, R leg weakness, bowel and bladder dysfunction, altered mental statusNF215 yrs ago for uterine and cervical cancer, 22 GyPartialYes, 36 Gy brain and spine, additional 14.4 Gy to lumbosacral lesion/28 fractionsNoNot specifiedNoNot specified
Stark et al., 201356, FSacralL leg radiculopathy, L foot paresis15 yrs ago for non-Hodgkin’s lymphomaNot specifiedNoYesYesBrainstem, spineDead at 24 mos
Wu et al., 20149, FThoracic, lumbar, sacralR hip pain, bilateral leg weaknessNF2Not specifiedPartialNoNoYesBrainDead at 9 mos
Li et al., 201433, FLow thoracic, upper lumbarLow back pain, R leg radiculopathy, hydrocephalusNot specifiedPartialYes, 28 Gy/19 fractionsNoYesBrain, diffuse spineDead at 29 mos
Lau et al., 2014 [10]43, MCauda equinaLow back pain, L leg radiculopathy for 5 mos10 yrs ago for testicular seminomaTotalNoYes, alternating between ifosfamide/doxorubicin and ifosfamide/etoposideYes, about 60 mosBrainstem, cervical spine, renalDead at 5 yrs
Thomas et al., 201449, MCauda equinaLow back pain, paraparesisNot specifiedPartialNoNoNoBrain and spineNot specified
Baharvahdat et al., 20163, FCervical, upper thoracicParaplegia, bladder and bowel dysfunctionNot specifiedPartialNoNoNoBrain and spineDead shortly after surgery
Chou et al., 2017a5–74 (mean 40)76% had adjuvant therapy38% over 2 yrs59% alive at 2 yrs
Samancia et al., 201727, MCervical, upper thoracicHydrocephalusNot specifiedPartialYesYesNoNoNot specified
Present study77, MLumbarLeft hip and buttock pain for 3 mosAbout 45 yrs ago for testicular cancerPartialNoNoNoIntracranial leptomeningeal diseaseDead at 2 mos
F: Female, Gy: gray, L: left, M: male, mos: months, NF1: neurofibromatosis type 1, NF2: neurofibromatosis type 2, R: right, wks: weeks, yrs: years.a Multicenter series (), individual patient data not available.