Diagnostic Dilemma: An Atypical Case of Astrocytoma in a Patient with Relapsing–Remitting Multiple Sclerosis
Abstract
:1. Introduction
2. Case Presentation
3. Discussion
4. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Patient’s Perspective on the Treatment
Acknowledgments
Conflicts of Interest
References
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Author, Year | Age/Sex | Age at MS Diagnosis | Age at Tumor Diagnosis | MS Phenotype | Tumor Location | Progressive Course or New Neurological Symptoms (Yes/No) | MRI Findings | Histopathology | Clinical Outcomes |
---|---|---|---|---|---|---|---|---|---|
Index Case (This Report) | 41 y/F | 34 y | 41 y | RRMS | Left Frontal | No | Large, Non-Enhancing Lesion, T1 Hypointense, FLAIR Hyperintense | Primary Diffuse Astrocytoma (WHO grade II) | Post-Operative Mild Expressive Dysphasia and a Seizure. MS Remained Stable by Clinical and MRI Criteria |
Turatti et al. [5], 2013 | 43 y/F | N.A. | 43 y | RRMS | Splenium of corpus callosum and the optic radiations | Yes (progressive visual field restriction) | Large tumefactive lesion (>3 cm) that involved the spleniumof corpus callosum and the optic radiations, with moderate perilesional edema and without gadolinium enhancement | High-grade astrocytoma | Fully ambulatory, hyperreflexia with impaired vibration sensation and pain in the left leg, main disability: blindness caused by radiotherapy |
Neil et al. [6], 2014 | 79 y/M | 45 y | 79 y | SPMS | Right parieto-occipital region | Yes (rapidly progressive cognitive decline) | Extensive, infiltrative mass which extended from the right parieto-occipital region across a significantly expanded corpus callosum | Anaplastic glioma | Family declined any further intervention and patient died in hospice |
Carvalho et al. [7], 2014 | 43 y/M | 23 y | 37 y | RRMS | Left frontal lobe | Yes (progressive right hemiparesis) | Extensive subcortical and deep white matter lesion in the left frontal lobe (non-contrast-enhancing) on routine brain MRI | Grade II oligodendroglioma | At 3-year follow-up: stable MRI lesions with minor right hemiparesis and dysarthria |
Mantero et al. [8], 2015 | 60 y/M | 42 y | 60 y | SPMS | Brainstem | Yes (rapidly progressing deterioration with tetraparesis, dysphagia, and dysarthria) | T2 hyperintense lesion in the brainstem, which increased in size on repeat MRI; thick enhancement surrounding a cyst-like cavity with involvement of left middle cerebellar peduncle | Glioblastoma | Patient died before the procedure (open brain biopsy); diagnosis was confirmed on autopsy |
Morales et al. [9], 2017 Case 1 | 67 y/F | 42 y | N.A | RRMS | Right frontoparietal lobe | Yes (had aggressive tumor and died 13 weeks after symptom onset) | Contrast-enhancing lesions in the right frontoparietal area which expanded in size and number over time | Glioblastoma multiforme with areas of necrosis and endothelial proliferation | Patient and family abstained from diagnostic work-up and patient died in hospice |
Case 2 | 26 y/M | 22 y | 25 y | RRMS | Right superior frontal lobe | No (lesion was discovered in the routine surveillance MRI) | Right superior frontal lobe mass at the cortical surface | Diffuse astrocytoma,IDH-mutant, WHO grade II | Developed focal seizures after surgery (treated with levetiracetam); treated with natalizumab, stable for 5 years |
Same patient as above | 22 y | 26 y | RRMS | Right frontal lobe | Yes (developed new left-sided weakness) | Recurrence of the right frontal lobe lesion was observed on subsequent brain MRI | Glioblastoma multiforme with primary neuroectodermal tumor (PNET)-like component, IDH-mutant, WHO grade IV | Showed no evidence of tumor recurrence; continued monthly natalizumab and daily levetiracetam treatment for seizure prophylaxis | |
Kantorova et al. [10], 2017 | 27 y/F | 19 y | 28 y | RRMS | Right frontal lobe | Yes (progression of expanded disability status scale (EDSS) from 2.0 to 5.0 and development of new symptoms) | Atypical hyperintense tumefactive demyelinating lesion | Anaplastic astrocytoma | Remained unstable (frequent epileptic seizures) and died during status epilepticus before starting oncological treatment |
Abrishamchi et al. [11], 2017 | 41 y/F | 26 y | N.A. | RRMS | Left frontal lobe involving corpus callosum | Yes (progressive ataxia and dizziness) | High signal lesions in the left frontal lobe involving corpus callosum on FLAIR | Grade II astrocytoma | Impaired tandem gait, Romberg sign, bilateral Babinski signs, quadriparesis |
Myserlis et al. [12], 2017 | 37 y/M | 23 y | 37 y | RRMS | Frontoparietal | Yes (gradual loss sensory and motor symptoms with cognitive decline) | A 5 cm circular, ring-enhancing lesion | Glioblastoma multiforme (grade IV) | Recurrence of the tumor with gradual loss of motor and sensory functions, ataxia, speech difficulties, cognitive decline and EDSS = 9 |
Preziosa et al. [13], 2017Case 1 | 59 y/M | 43 y | 59 y | SPMS | Right post-rolandic regions | Yes (gradual progression of spastic hypertonia, hyposthenia and EDSS) | T2-hyperintense and T1-hypointense pseudotumoral lesion in the right post-rolandic region with irregular and poorly defined margins | Glioblastoma with leptomeningeal infiltration (grade IV) | Deterioration of locomotor functions and inability to walk (possible consequences of radiotherapy). Patient refused diagnostic procedure |
Case 2 | 55 y/M | 38 y | 53 y | RRMS | Left supero-anterior parietal regions | Yes (subtle difficulty in reading and fatigue) | Heterogeneous T2-hyperintense and T1-hypointense lesion in the left superior anterior parietal regions with internal cystic areas and irregular enhancement | Grade IV glioblastoma | Deterioration of cognitive function, fatigue, reading difficulty and acalculia (only radiotherapy and temozolomide were used; no surgical removal of lesion) |
Shirani et al. [14], 2018 | 44 y/M | 44 y | 9 months after MS ds | RRMS | Right frontal lobe | Not evident (only the initial presentation of sudden onset right-sided optic neuritis was described) | Multiple FLAIR white matter lesions in periventricular, juxtacortical and subcortical areas | Grade II oligodendroglioma | An early postoperative brain MRI revealed a new demyelinating lesion in the right posterior periventricular white matter and the patient remained on glatiramer acetate at the time of the report. |
Sinclair et al. [15], 2019 | 41 y/F | N.A. | 30 y | RRMS | Left frontal lobe | Yes (gradually developed the clinical signs of mass-effect) | Suspected glioma in the left frontal lobe and multiple supratentorial MS-like lesions in both hemispheres | Oligodendroglioma; WHO grade 2–3 | During the next 5 years after microsurgery, patient underwent systemic treatment due to a series of tumor- or MS-like relapses (signs of focal recurrence on postoperative MRI, scattered MS-suspected lesions, right-sided hemiparesis) |
Same patient as above | N.A. | 39 y | RRMS | Left frontal lobe | Yes (right-sided hemiparesis) | Tumor regrowth was observed next to surgical site (deemed unsuitable for microsurgery) | Anaplastic astrocytoma | Patient suffered from right foot palsy from the second surgical resection and fatigue (due to chemotherapy) | |
Sirko et al. [16], 2020 | 30 y/M | 31 y | 32 y | RRMS | Right temporal lobe | Yes (a negative trend in the neurological examination) | T2 hyperintense lesion in the right temporal lobe and insula with irregular edges and blurred outlines involving both gray and white matter | Anaplastic oligoastrocytoma | Patient was waiting for surgery and was under the active supervision of a neurologist and a neurosurgeon |
Algahtani et al. [17], 2020 | 23 y/F | 23 y | 23 y | RRMS | Left superior and part of the middle frontal gyri | Yes (gradually progressive walking difficulty and imbalance) | 5.6 cm cortical-based tumor which originated from the left superior and part of the middle frontal gyri (surrounded by vasogenic edema) | Anaplastic oligodendroglioma (WHO grade III) | Six months post-surgery, new white matter demyelinating lesions consistent with MS were detected and diagnosis of RRMS was confirmed. Targeted sequencing revealed a mutation in the GBA2 gene consistent with the diagnosis of autosomal-recessive cerebellar ataxia with spasticity (positive family history) |
London et al. [18], 2020 | 55 y/F | 26 y | 55 y | RRMS | Left frontal lobe | Yes (developed mild gait ataxia, right hypoesthesia, and nystagmus) | Closed-ring contrast-enhancing lesion in the left frontal lobe with surrounding edema | Glioblastoma (WHO grade IV) | Follow-up MRI 3 months post-operation showed tumor progression. Despite treatment, the patient worsened and she died 5 months after diagnosis |
Classification | Age (years) | Sex | MS Phenotype | Tumor Type [Case Reference] | Tumor Site |
---|---|---|---|---|---|
Glioblastoma/Astrocytoma (n = 10)
| 43 | F | RRMS | Astrocytoma [5] | Splenium of corpus callosum and the optic radiations |
26 | M | RRMS | Diffuse astrocytoma [9] Glioblastoma multiforme [9] | Right superior frontal Right frontal | |
27 | F | RRMS | Anaplastic astrocytoma [10] | Right frontal | |
41 | F | RRMS | Astrocytoma [11] | Left frontal lobe involving corpus callosum | |
60 | M | SPMS | Glioblastoma [8] | Brainstem | |
67 | F | RRMS | Glioblastoma multiforme [9] | Right frontoparietal | |
37 | M | RRMS | Glioblastoma multiforme [12] | Frontoparietal | |
59 | M | SPMS | Glioblastoma [13] | Right post-rolandic | |
55 | M | RMMS | Glioblastoma [13] | Left supero-anterior parietal | |
55 | F | RRMS | Glioblastoma [18] | Left frontal | |
Oligodendroglioma (n = 5)
| 43 | M | RRMS | Oligodendroglioma [7] | Left frontal |
44 | M | Oligodendroglioma [14] | Right frontal | ||
41 | F | Oligodendroglioma [15] Anaplastic astrocytoma [15] | Left frontal Left frontal | ||
30 | M | Anaplastic oligodendroglioma [16] | Left superior and part of the middle frontal gyri | ||
23 | F | Anaplastic oligodendroglioma [17] | Left superior and part of the middle frontal gyri | ||
Undifferentiated (n = 1) | 79 | M | SPMS | Anaplastic glioma [6] | Right parieto-occipital |
MS | TDLs | Brain Tumors in MS | |
---|---|---|---|
Demographic features | Females > males; can occur at any age (usually between 20 and 40 years); relatively subacute onset [22] | Females > males; usually in young adults (middle age); relatively slow onset [23] | Brain tumors are more likely in males (this may vary with type of tumor); usually in older adults; gradual onset [24] |
Clinical presentation | Variable, but typical syndromes include monocular loss of vision, double vision, ataxia, sensory loss, or limb weakness [25] | Polysymptomatic (usually sensory, motor, and cognitive symptoms), but may include focal neurological deficits, seizure, or aphasia [23] | Atypical manifestations may include (but are not limited to) headaches, tumor location-specific symptoms, or behavioral changes [26] |
Clinical course | Relapsing–remitting (most common), secondary progressive, primary progressive | Could be monophasic or recurrent [23] | Usually progressive |
Size and site of lesion(s) | 3–5 mm or larger, typical white matter lesions in MS are periventricular, juxtacortical, and callososeptal, or at cerebellar peduncles in the infratentorial region [27] | >2 cm, often found in the supratentorial region (mostly in the frontal and parietal lobes) [28] | Large and variable size, usual distribution at frontal and temporal lobes [29] |
MRI | Typical white matter lesions are round to ovoid in shape, should be at least 3 mm in size (long axis), and appear hyperintense on T2 and FLAIR sequences [30] | Large lesion (>2 cm) but with relatively little mass effect or surrounding edema, incomplete (open-ring pattern) contrast enhancement [28] | Mass effect, perilesional edema, necrosis, and continued enlargement of lesion [10] |
H-MRS | Acute MS lesions: increased Cho, reduced NAA, and presence of lipids in acute MS lesions Chronic MS lesions: reduced NAA levels [31] | Increased Cho and lactate are supportive, but non-specific Typically demonstrates increased Cho/NAA ratio, reduced NAA/Cr ratio, and increased Cho/Cr ratio [23,32,33] | Persistently elevated Cho is more suggestive of tumor, could exhibit decreased NAA/Cr ratio, increased Cho/Cr ratio, and variable lactate and lipid peaks [13] |
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Kahovec, C.; Saini, A.; Levin, M.C. Diagnostic Dilemma: An Atypical Case of Astrocytoma in a Patient with Relapsing–Remitting Multiple Sclerosis. Neurol. Int. 2021, 13, 240-251. https://doi.org/10.3390/neurolint13020025
Kahovec C, Saini A, Levin MC. Diagnostic Dilemma: An Atypical Case of Astrocytoma in a Patient with Relapsing–Remitting Multiple Sclerosis. Neurology International. 2021; 13(2):240-251. https://doi.org/10.3390/neurolint13020025
Chicago/Turabian StyleKahovec, Chantal, Aman Saini, and Michael C. Levin. 2021. "Diagnostic Dilemma: An Atypical Case of Astrocytoma in a Patient with Relapsing–Remitting Multiple Sclerosis" Neurology International 13, no. 2: 240-251. https://doi.org/10.3390/neurolint13020025