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Neurological deterioration after treatment for a brain tumor can be caused by various factors, such as tumor recurrence, progression, or metastases. Other pathology, such as spinal epidural lipomatosis (SEL), which can present similarly to neoplasms by causing neurological deficits secondary to mass effect, may pose a challenge for rapid identification. SEL is usually a benign disorder that is characterized by an accumulation of unencapsulated adipose tissue in the epidural space.1It is more commonly found in obese patients, those with chronic steroid exposure, as postsurgical changes, or for idiopathic reasons. In rare cases, it may cause compression of the spinal cord, causing symptoms of myelopathy or radiculopathy. This phenomenon has been well documented in adults, but there are limited reported cases in the pediatric population.2Here we present a case of progressive SEL in a pediatric patient recently treated for a malignant tumor of the brain, causing progressive paraparesis.
Illustrative Case
A 4-year-old White male with Phalen-McDermid syndrome and a history of a right frontal craniotomy for resection of an atypical teratoid rhabdoid tumor (ATRT) presented to the emergency room with 2 weeks of progressive paraparesis (Table 1). Prior to this admission, he had had some baseline developmental delay but was independently ambulatory and playful. On presentation, he appeared cushingoid, awake and alert, and able to move both arms equally with good strength, but with significant weakness of his lower extremities—unable to stand, 2–3/5 strength throughout—and absent lower extremity reflexes. There was no change in bowel or bladder function.
Cases of SEL in pediatric patients with a history of brain tumors.
| Authors & Year | Sex/Age (yrs) | Neuro-oncological Hx | Signs & Symptoms | Onset* | Steroid Dosage | Indication for Steroids | SEL Dx Since Starting Steroids (mos) | Extent of Lipomatosis | Management | Length of FU |
|---|---|---|---|---|---|---|---|---|---|---|
| Quint et al., 19883 | M/11 | Pineoblastoma s/p resection 1 yr prior | LE weakness, paresthesias at T2–3 sensory level, bowel/bladder deficit | 1 yr | 20 mg/day corticosteroid | NS | 12 | T3–9 | Thoracic laminectomies for resection of SEL | Worsening of symptoms w/ findings of disseminated disease (immediately postoperative) |
| Present case | M/4 | Rt frontal ATRT s/p resection 8 mos prior, & chemoradiation | LE weakness, marked paraparesis, unable to walk | 7 mos | 0.1–0.5 mg/kg/day of methylprednisolone, 15 mg/day total on admission | Respiratory failure, intrinsic pulmonary disease | 8 | L1–5 | L1–5 laminoplasty for resection of SEL, steroid wean | Mild residual weakness & ataxia present, able to walk unassisted (4 mos) |
Dx = diagnosis; FU = follow-up; Hx = history; LE = lower extremity; NS = not specified; s/p = status post.
Onset of symptoms after beginning steroid therapy.
Given his oncological history, there was concern for possible tumor recurrence or progression. Gross-total resection of his ATRT, 14 months earlier, was followed by chemotherapy with stem cell transplantation and craniospinal proton beam therapy. His course of radiation therapy (54 Gy) was completed 4 months prior to his presentation. There was no evidence of residual tumor or recurrence on recent postoperative surveillance magnetic resonance imaging (MRI) (Fig. 1).
Preoperative and serial postoperative axial T1-weighted fluid attenuated inversion recovery (FLAIR) postcontrast (left) and T2-weighted noncontrast (right) MRI of the brain.A:Preoperative MRI demonstrating a heterogeneously enhancing, T2-hyperintense right frontal paramedian lesion.B:Postoperative MRI demonstrating gross-total resection of the lesion.C:MRI of the patient’s brain on presentation, 13 months postoperatively, without evidence of recurrent lesion.
MRI of his brain and spine, obtained on presentation to the emergency room, confirmed no evidence of tumor progression or recurrence (Fig. 1C) but did demonstrate extensive lumbosacral SEL that had progressed over the last 8 months (Fig. 2). He was taken to surgery for L1 to L5 laminoplasty and decompression of his spinal cord and cauda equina via ultrasound aspiration of his SEL (Fig. 3). The patient demonstrated immediate improvements in strength postoperatively and was discharged to an acute inpatient rehabilitation center. Follow-up at 4 months postoperatively demonstrates expected postoperative changes (Fig. 3D)和神经系统进行持续改进:他是佤邦lking short distances independently with only mild persistent imbalance. A steroid wean was initiated shortly after diagnosis of symptomatic SEL and the patient was successfully weaned off of all steroids after 3 months without any deterioration of respiratory function.
Sagittal T1-weighted noncontrast MRI of the lumbar spine showing the progression of SEL in a 4-year-old boy on chronic steroids. Postoperative changes from a prior L5 laminotomy for release of a tethered cord and fatty filum resection are seen. Lumbar MRI performed 7 months prior (A), 3 months prior (B), and on the day of presentation (C). Note the progressive increasing volume of SEL both ventral and dorsal to the thecal sac, causing significant stenosis of the spinal canal.
Intraoperative photographs and postoperative imaging.A:L1–5 laminotomies were performed bilaterally. The laminae were detached caudally at L5–S1 and retracted rostrally, exposing the extensive SEL.B:Post-ultrasound aspiration of the adipose tissue showing the underlying dura.C:Replacement and reattachment of the laminoplasty segment via interrupted sutures to adjoining interlaminar lateral ligaments.D:Postoperative T1-weighted noncontrast MRI of the lumbar spine at 3 months postoperatively demonstrating expected postsurgical changes and resection of the patient’s extensive SEL.
Discussion
Literature Review
A PubMed search through October 2022 was conducted using the search terms “(pediatric OR infant OR child) AND (spinal OR epidural) AND (lipomatosis)” (Fig. 4). Papers were included only if full-text articles were available in English and described pediatric patients (age ≤21 years who had SEL with a past or current brain tumor.
PRISMA flowchart of citations identified and evaluated. Format provided by Moher D, Liberati A, Tetzlaff J, Altman DG; The PRISMA Group. Preferred Reporting Items for Systematic Reviews and Meta-Analyses: the PRISMA statement.PLoS Med. 2009;6(7):e1000097.
One paper by Quint et al.3met the criteria (Table 1). The paper reported on an 11-year-old male patient with a pineoblastoma treated with 20 mg/day of corticosteroids for 1 year who was diagnosed with symptomatic thoracic SEL requiring surgical intervention.
Observations
In children, SEL is most common in the thoracic region but can also present in the lumbosacral and rarely the cervical region.4Long-term corticosteroid treatment has been implicated in the development of SEL in adults and pediatric patients. Other associated disorders include, but are not limited to, Cushing’s syndrome, obesity, and Scheuermann kyphosis.1–5Nonverbal pediatric patients may pose a particular challenge for identifying SEL because a common early complaint is back pain.6Once the severity of fat accumulation is enough to compress the spinal cord, symptoms such as weakness, numbness, urinary incontinence, fecal incontinence, and cauda equina syndrome can occur depending on the spinal region.1–4The diagnostic and most sensitive test is a T1-weighted MRI; however, computed tomography can be diagnostic as well.7Treatment varies based on etiology. Conservative measures usually include weaning of steroid use and replacement with more targeted therapies for existing disorders.4Other conservative treatments for noniatrogenic etiologies include weight reduction, dietary modification, and correction of endocrine dysfunction.1,2,4,6Based on the provider’s discretion and severity of symptoms, surgical decompression may be considered.7
We describe a case of SEL in a developmentally delayed 4-year-old male with a history of a brain tumor who presented with lower extremity weakness and irritability (Table 1). Having completed chemoradiation therapy for his brain tumor 4 months prior, our patient’s irritability and difficulty walking could have been secondary to tumor recurrence or metastatic spread. Other conditions, such as iatrogenic Cushing’s syndrome due to his chronic steroid use, can cause symptoms such as muscle weakness, depression, anxiety, irritability, and cognitive difficulties.8此外,我们的病人基线张力减退nd developmental delay due to his chromosomal deletion syndrome. The combination of his comorbidities could easily distract clinicians from SEL as the primary diagnosis.
Quint et al.3presented another unique case of symptomatic SEL in a young male with a history of pineoblastoma (Table 1), the only other reported case of this nature in the literature. Initial MRI evaluation led to his diagnosis of symptomatic thoracic SEL. However, this patient did not improve after decompressive surgery and actually experienced worsening of symptoms. Subsequent myelography and cytological examination of cerebral spinal fluid diagnosed extensive seeding of his thecal sac with metastatic disease. This case is unique in that it highlights how SEL and tumor can mimic each other and, unfortunately, even coexist.
In conclusion, SEL can be an uncommon cause of neurological decline and should be considered in the differential diagnosis when assessing neuro-oncological patients with an acute decline in function.
Lessons
Limitations of this paper include the retrospective nature and small sample size. However, this is reflected in the paucity of available literature on this clinical scenario. As such, we aim to highlight this rare, but potentially surgical, pathology. Careful examination of radiographic imaging and review of patient comorbidities and medical history is always required in evaluating a patient with new neurological deficits. Recurrence or progression of central nervous system tumors is often at the forefront of the differential diagnosis when considering neurological deterioration in patients with a history of central nervous system tumors. However, neurological decline due to symptomatic SEL in pediatric patients treated with steroids should be considered in the differential.
Disclosures
The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.
Author Contributions
Conception and design: Ruge. Acquisition of data: Berlet, Li. Analysis and interpretation of data: all authors. Drafting of the article: all authors. Critically revising the article: all authors. Reviewed submitted version of the manuscript: all authors. Approved the final version of the manuscript on behalf of all authors: Ruge. Administrative/technical/material support: Ruge.
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