Review Article

Optic Neuritis as Isolated Manifestation of Leptomeningeal Carcinomatosis: A Case Report and Systematic Review of Ocular Manifestations of Neoplastic Meningitis

Table 1

Clinical and instrumental findings in patients with meningeal carcinomatosis (review of literature).

Study referenceAge and genderLatency symptoms onset diagnosisOcular manifestationsAssociated clinical featuresImagingCSF examinationOriginal tumorTreatmentLife expectancy

[4]75, MNot specifiedDiplopia, left ptosis, anisocoriaHeadache, confusion, hearing loss, VII cn (cranial nerve) palsyCT scan (normal), MRI (GME)(1) Prot ↑
(2) not performed
Bladder and prostate cancerDeclined15 days

[5]49, F10 monthsBlurred vision and diplopia, horizontal nystagmusDizziness, ataxia, seizures, dysarthria, VII cn palsy, left lower limb weakness CT scan (normal), MRI + gad (FME, cauda equine, cerebellum)(1) MTC positive
(2) not performed
Ovarian cancerWBRT. IT: topotecan
(0.4 mg × 4)
4 months

[6]39, F6 monthsSudden bilateral visual loss, horizontal gaze palsyheadache, vertigo, seizuresMRI + gad (GME)(1) Prot ↑, glu ,
MTC +
(2) MTC negative
Gastric adenocarcinomaRT. IT: MTX
(12.5 mg × 2/we)

[7]40, F2 monthsVisual loss, bilateral sixth cn palsy, bilat papilledemaHeadache, neck pain, meningismContrast CT scan (GMEt); MRI + gad (GME)(1) Prot ↑, glu
(2) negative
MelanomaNot done1 year (after symptoms onset)

[8]33, F11 monthsVI cn palsyConfusion, seizures, increased intracranial pressureCT scan (FME, lateral ventricles)Not performedUterine cervical neuroendocrine tumorRT19 months (after cancer diagnosis)

[9]58, M2 months (symptoms),
12 months (carcinomatosis)
Left homonymous hemianopiaHeadache, ataxiaCT scan and MRI (right infarction of the caudate, internal capsule, and lentiform nucleus)(1) MTC positive
(2) not performed
Transitional cell carcinoma of the bladderNot done

[10]54, FPostmortem diagnosis of the original tumor, 4 months (symptoms-carcinomatosis)Ptosis, right III cn palsyV and XII cn palsy, dysgeusiaMRI + gad (GME)Not performedCollecting duct carcinomaIV: mannitol, dexamethasone, and morphinehydrochloride3 months

[11]65, M2 years (carcinomatosis), 6 weeks (symptoms)Right visual lossHearing loss, ataxiaMRI + gad (normal), repeated MRI + gad (FME. bilateral optic nerves, left V cn), bilateral cerebellopontine angle mass(1) Prot ↑, glu ,
MCT negative
(2) not performed
Colorectal cancerRT

[12]61, MPostmortem diagnosisIII cranial nerve palsyFlaccid paraparesis with bladder retention, dysarthria, VII cn palsy, right arm weakness MRI + gad (normal)(1) Normal
(2) Prot ↑, glu ,
cells ↑
Lung adenocarcinomaNot done

[13]51, F15 years (carcinomatosis),
2 months (symptoms)
Diplopia (VI cn palsy)Paraparesis with bladder retention, peripheral neuropathyMRI (normal)(1) Prot ↑, MTC positive
(2) MTC negative
Breast
Cancer
IT: MTX (15 mg), liposomal Ara-CLast followup: 3 years and 7 months after carcinomatosis diagnosis (clinically stable)

[14]67, M2 months (symptoms)Left ptosis, diplopia, and visual lossHeadache, hemifacial sensory loss, paraplegiaMRI (GME, left retrorbital mass) (1) Cells ↑, MTC negative
(2) MTC positive
Adrenal extranodal NK/T-cell lymphomaIV: dexamethasone2 months (after symptoms onset)

[15]53, M4 months (carcinomatosis)Intermittent diplopiaHeadache, vertigoCT scan and MRI + gad (normal)(1) Normal
(2) MTC positive
Gastric cancerRT(45 Gy), betamethasone (16 mg/die)3 months

[16]43, F156 months (carcinomatosis)Progressive blurred vision and pain in the right eyeMRI + gad (FME, right optic nerve )(1) MCT positive
(2) not performed
Cervical cancerRT + triethylenethiophosphoramide2 months

[17]56, M4 days (symptoms)III cranial nerve palsyHeadache, nausea, and low back painMRI + gad (normal)(1) MTC positive
(2) not performed
Lung cancer

[18]31
(average);
3M, 4F
Average: 8 months (carcinomatosis)Ptosis (2),
diplopia (3),
visual loss (3)
Headache (4),
VII cn palsy (1)
MRI + gad (2 FME, cn, 2 GME, 3 normal)(1) MTC positive
(2) normal
NHL (3), ALL (1), AML (1), plasmablastic myeloma (1), breast cancer (1)NsAverage: 55 days

[19]50, F2 years (carcinomatosis)Bilateral loss of visionConfusion, headacheMRI + gad (thickening intraorbital optic nerves)(1) Prot ↑, MCT positive
(2) not performed
Ovarian serous cystadenocarcinomaNsNs

[20]44, M3 months (symptoms carcinomatosis)DiplopiaHeadache, confusion, meningeal signs, facial diplegia, ataxia, deafness MRI (normal)(1) Cells ↑, prot ↑, MTC positive
(2) MTC negative
Lung cancerIT: MTX (10 mg), Ara-C (39 mg). RT182 days (after carcinomatosis diagnosis), 272 days (after symptoms onset)

[21]67, F6 months (carcinomatosis)Blurred vision, diplopiaHeadaches, meningeal signs, and SIADHCT scan (normal)(1) MTC positive
(2) not performed
Gastric cancerRT7 months (after cancer diagnosis), 2 weeks (after carcinomatosis diagnosis)

[22]41, M2 weeks (symptoms)Visual loss, left ptosisHeadache, nauseaContrast CT scan (chiasmal thickening), MRI (hydrocephalus, increased CSF signal in the basal cisterns)(1) Prot ↑, MTC negative
(2) prot ↑, MTC negative
Rectal carcinomaOral: dexamethasone (4 mg)5 days (after carcinomatosis diagnosis)

[23]60, F2 weeks (symptoms)Diplopia, VI cn palsyAtaxia, VII cn palsy, and hearing lossCT scan (normal)(1) Glu , prot ↑, cells ↑, MTC positive
(2) MTC negative
Gallbladder carcinomaIT: MTX2 months and 2 weeks (after symptoms onset)

[24]5 months (carcinomatosis)Bilat visual loss papilledema, exotropia, ocular painHemiplegia, SAHCT scan (normal), MRI (normal)(1) MTC positive
(2) not performed
Non-Hodgkin lymphomaIT: MTX, corticosteroids2 weeks (after carcinomatosis diagnosis)

[25]49, MPostmortem diagnosis (2 months after symptoms onset)Visual loss, bilateral blindnessHeadache, confusion, leg weakness, dysphagia, seizures, ataxia, and intermittent paralysisCT scan (normal)(1) Normal
(2) not performed
Oesophageal adenocarcinomaNot done60 days (after symptoms onset)

[26]44, MDiplopiaHeadache, ataxia, meningeal signs, V, VII, IX, and X cn palsy(1) Cells ↑, MTC positive
(2) not performed
IT: Ara-C.RT

[27]35, F20 weeks (carcinomatosis), 1 week (symptoms)Bilat visual loss to blindnessHeadache, vomiting, meningeal signs, and loss of consciousnessCT scan (normal)(1) Prot ↑, glu , cells ↑, MTC positive
(2) prot ↑, MTC positive
Breast cancerIT: MTX (70 mg tot), Ara-C (80 mg tot)21 days (after symptoms onset)

[28]36, FNSBlindnessHeadache, loss of consciousnessCT scan (normal)(1) MTC negative
(2) MTC positive
Lung cancerIT: MTX, Ara-C, ACNU, IL-22 years (after carcinomatosis diagnosis)

[29]60, F3 months (symptoms carcinomatosis)Visual lossHeadaches, paraparesis CT scan (normal), repeated CT scan of brain and orbits (right globe soft mass)(1) Prot ↑, cells ↑
(2) prot ↑, cells ↑, MTC positive
Gastric cancerPrednisone (80 mg)

[30]37, FDiplopiaHeadache, nausea, vomiting, and meningeal signs(1) MTC positive
(2) MTC negative
Gastric cancerIT: MTX, Ara-C, prednisolone
IV: adriamycin, ftorafur
365 days (after symptoms onset)

[31]58, MPostmortem diagnosisVisual lossVertigo, loss of consciousness(1) Cells ↑
(2) not performed
Colon cancerNot done10 days (after symptoms onset)

[32]11, MVisual lossBurkitt's lymphoma

[33]49, M1 month (symptoms-carcinomatosis)Scotomas, diplopiaHeadache, nausea and vomiting, and vertigoCT scan (normal)(1) Prot ↑, cell ↑
(2) MTC positive
Gastric cancerIT: MTX 20 mg60 days (after symptoms onset)

[34]59, F39 months (carcinomatosis)Bilateral blindnessDizziness, tinnitus, and confusionCT scan (normal)(1) Prot ↑, MTC positive
(2) Pprot ↑, MTC negative.
(3) prot ↑, MTC negative.
(4) glu , prot ↑, MTC positive.
Breast cancerRT. IT: Ara-C (70 mg), dexamethasone. IV: 5-FU, vincristine, MTX. Oral: cyclophosphamide, prednisone4 years and 2 months (after cancer diagnosis), 9 months (after carcinomatosis diagnosis)
49, F5 years and 3 months (carcinomatosis)Bilateral blindnessCT scan (normal)(1) Glu , prot ↑, MTC positive
(2) normal
Breast cancerRT. IT: Ara-C, corticosteroids5 years and 6 months (after cancer diagnosis), 3 months (after carcinomatosis diagnosis)

[35]53, FPostmortem diagnosis
(1 month after symptoms onset)
Anisocoria, papilledemaHeadache, hemiparesis, meningeal signs, and loss of consciousness Subdural hematomaNot performedGastric cancer26 days (after symptoms onset)

[36]53, MPostmortem diagnosis
(3 months after symptoms onset)
BlindnessNausea, vomiting, confusion, ataxia, meningeal signs, and seizuresCT scan (normal)(1) Cells ↑, prot ↑,
glu
(2) prot ↑
Lung cancerNot done3 months (after symptoms onset)

[37]59, F46 months (symptoms carcinomatosis); 10 months (carcinomatosis)Scotomas, visual loss to blindnessCerebellar ataxiaSkull radiography (normal)(1) cells ↑, MTC positive
(2) normal
Anaplastic endometrioid sarcomaRT. IT: MTX (20 mg × 4),
IM: citrovorum factor
60 days (after carcinomatosis diagnosis), 365 days (after cancer diagnosis)

Cn: Cranial nerve; SIADH: syndrome of inappropriate antidiuretic hormone secretion; SAH: subarachnoid Haemorrhage; GME: generalised meningeal enhancement; FME: focal enhancement; NHL: non Hodgkin lymphoma; ALL: acute lymphoblastic leukemia; AML: acute myelocytic leukemia; IT: intrathecal; O: oral; IM: intramuscular.