Cervical intra and extramedullary hemangioblastoma with associated syringomyelia: A case report and review of the literature

Colamaria, Antonio; Fochi, Nicola Pio; Laguado, Yasser Andres Dallos; Blagia, Maria; Leone, Augusto; Carbone, Francesco

doi:10.25259/sni_814_2022

Cited by 7 publications

(8 citation statements)

References 11 publications

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“…Although intra- and extramedullary tumors and extradural metastases of different primary tumors are included in this analysis, no data on “multicompartmental” or rare tumors are presented that would not fall under this strict classification. Nevertheless, examples of previous reports on spinal oncologic paraparesis in the literature include an intradural extramedullary angiosarcoma ( 52 ), an intradural extramedullary tanycytic ependymoma (usually observed intradurally) ( 53 ), and a cervical intra- and extramedullary hemangioblastoma ( 54 ). Interestingly, in all those cases, the postoperative neurological condition of involved patients mimicked our extramedullary cohort, regardless of tumor histology.…”

Section: Discussionmentioning

confidence: 99%

Spinal oncologic paraparesis: Analysis of neurological and surgical outcomes in patients with intramedullary, extramedullary, and extradural tumors

et al. 2023

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ObjectivesParaparesis due to oncologic lesions of the spine warrants swift neurosurgical intervention to prevent permanent disability and hence maintain independence of affected patients. Clinical parameters that predict a favorable outcome after surgical intervention could aid decision-making in emergency situations.MethodsPatients who underwent surgical intervention for paraparesis (grade of muscle strength <5 according to the British Medical Research Council grading system) secondary to spinal neoplasms between 2006 and 2020 were included in a single-center retrospective analysis. Pre- and postoperative clinical data were collected. The neurological status was assessed using the modified McCormick Disability Scale (mMcC) Score. In a univariate analysis, patients with favorable (discharge mMcC improved or stable at <3) and non-favorable outcome (discharge mMcC deteriorated or stable at >2) and different tumor anatomical compartments were statistically compared.Results117 patients with oncologic paraparesis pertaining to intramedullary lesions (n=17, 15%), intradural extramedullary (n=24, 21%) and extradural lesions (n=76, 65%) with a mean age of 65.3 ± 14.6 years were included in the analysis. Thoracic tumors were the most common (77%), followed by lumbar and cervical tumors (13% and 12%, respectively). Surgery was performed within a mean of 36±60 hours of admission across all tumors and included decompression over a median of 2 segments (IQR:1-3) and mostly subtotal tumor resection (n=83, 71%). Surgical and medical complications were documented in 9% (n=11) and 7% (n=8) of cases, respectively. The median hospital length-of-stay was 9 (7-13) days. Upon discharge, the median mMcC score had improved from 3 to 2 (p<0.0001). At last follow-up (median 180; IQR 51-1080 days), patients showed an improvement in their mean Karnofsky Performance Score (KPS) from 51.7±18.8% to 65.3±20.4% (p<0.001). Localization in the intramedullary compartment, a high preoperative mMcC score, in addition to bladder and bowel dysfunction were associated with a non-favorable outcome (p<0.001).ConclusionThe data presented on patients with spinal oncologic paraparesis provide a risk-benefit narrative that favors surgical intervention across all etiologies. At the same time, they outline clinical factors that confer a less-favorable outcome like intramedullary tumor localization, a high McCormick score and/or bladder and bowel abnormalities at admission.

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Section: Discussionmentioning

confidence: 99%

Spinal oncologic paraparesis: Analysis of neurological and surgical outcomes in patients with intramedullary, extramedullary, and extradural tumors

et al. 2023

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“…Postoperatively, patients’ symptoms often regress within 1–2 postoperative weeks, while follow-up contrast MR studies help confirm complete tumor resection, along with documenting regression of cephalad/caudad cysts (i.e., syrinxes/hydromyelia over 3–6 months). [ 2 , 6 ]…”

Section: Discussionmentioning

confidence: 99%

“…Colamaria et al [2] 32 Female C2. Intramedullary -dorsal area Numbness in the upper extremities with gait instability Suboccipital craniectomy and total C1 laminectomy Gluf and Dailey.…”

Section: Article Patient's Age Sex Level Of the Lesion Symptoms Surgi...mentioning

confidence: 99%

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Intramedullary hemangioblastoma of the thoracic cord with a microsurgical approach: A case report and literature review

Piovesan

Silva

Mallmann

et al. 2023

Surgical Neurology International

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Background: Spinal cord hemangioblastomas (HBs) account for 2–15% of all spinal cord neoplasms. They are the third most common primary intramedullary tumor (1–5%). Here, 72-year-old female presented with a thoracic intramedullary spinal HB that responded well to surgery. Case Description: A 72-year-old female presented with a 3–4 years of progressive paresthesias and paraparesis. On examination, she exhibited diffuse distal weakness of the lower extremities. The magnetic resonance scan showed an intramedullary expansive lesion at the T1–T2 level that markedly enhanced with contrast with both proximal and distal hydromyelia. Surgery included a C7 partial and T1–T2 total laminectomies performed under microscope visualization with intraoperative monitoring. At surgery, there was a well-documented cleavage plane between the tumor and the cord; excision was facilitated using the cavitron ultrasonic surgical aspirator device. Conclusion: Surgery is the gold standard treatment for treating/resecting HBs and should include utilization of an operating microscope and intraoperative monitoring.

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“…Secondary holocord syringomyelia, in which the entire length of the spinal cord is affected, is an even rarer manifestation of this condition, with few cases reported in the literature [ 4 ]. Early diagnosis and appropriate management of patients with spinal hemangioblastomas and associated syringomyelia are crucial for preventing permanent neurological damage and improving patient outcomes [ 5 ]. However, the clinical presentation of these patients may be nonspecific and mimic other common pathological conditions, posing a diagnostic challenge for clinicians.…”

Section: Introductionmentioning

confidence: 99%

Secondary Holocord Syringomyelia Associated With Spinal Hemangioblastoma in a 29-Year-Old Female

Chu,

Sabourdy,

Cheong

2023

Cureus

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Syringomyelia is a neurological condition characterized by the presence of fluid-filled cavities within the spinal cord, resulting in progressive neurological deficits. Secondary holocord syringomyelia, a rare manifestation in the entire spinal cord, is associated with spinal hemangioblastomas. We report the case of a 29-year-old female who presented with neck and bilateral upper limb pain and numbness. She was diagnosed with secondary holocord syringomyelia associated with a spinal hemangioblastoma and underwent conservative management. Magnetic resonance imaging plays a critical role in diagnosing neurological conditions. The management of spinal hemangioblastomas and syringomyelia can be challenging and requires a multidisciplinary approach to patient care. In this report, we discuss the clinical presentation, diagnosis, and management of a patient with secondary holocord syringomyelia associated with spinal hemangioblastoma.

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Cervical intra and extramedullary hemangioblastoma with associated syringomyelia: A case report and review of the literature

Cited by 7 publications

References 11 publications

Spinal oncologic paraparesis: Analysis of neurological and surgical outcomes in patients with intramedullary, extramedullary, and extradural tumors

Spinal oncologic paraparesis: Analysis of neurological and surgical outcomes in patients with intramedullary, extramedullary, and extradural tumors

Intramedullary hemangioblastoma of the thoracic cord with a microsurgical approach: A case report and literature review

Secondary Holocord Syringomyelia Associated With Spinal Hemangioblastoma in a 29-Year-Old Female

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