Apoplexy in an intradural clival chordoma causing intraventricular bleed

Mohindra, Sandeep; Kapoor, Ankur; Kursa, Gopi Krishna; Saikia, Uma Nahar

doi:10.4103/2152-7806.173562

Cited by 4 publications

(9 citation statements)

References 6 publications

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“…Intratumoral hemorrhage in chordomas may result from the rupture of thin-walled vessels, hemorrhagic necrosis due to rapid tumor growth, or destruction of the dural vessels by tumor invasion. 10,12 However, we could not identify any evidence of tumor bleeding during surgery in this case, and there was no histopathological evidence of intratumoral bleeding. Failure of the venous drainage of the right thalamus could reasonably be assumed to have been the cause of recurrent bleeding in this case considering the narrowing or collapse of the right ICV by the firm and hard tumor.…”

Section: Pineal Chordoma and Recurrent Bleedingmentioning

confidence: 64%

“…However, although rare, cases of bleeding from a chordoma abruptly exacerbating neurological status have been reported. [10][11][12] The cause of tumor hemorrhage in such cases remains to be elucidated. Intratumoral hemorrhage in chordomas may result from the rupture of thin-walled vessels, hemorrhagic necrosis due to rapid tumor growth, or destruction of the dural vessels by tumor invasion.…”

Section: Pineal Chordoma and Recurrent Bleedingmentioning

confidence: 99%

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Management challenges associated with a pineal region chordoma: illustrative case

Kwon

Hwang

et al. 2021

Journal of Neurosurgery: Case Lessons

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BACKGROUND Chordomas, which are rare malignant neoplasms arising from notochordal remnants, often cause gradually progressive clinical symptoms. Intradural cranial chordomas (ICCs) are extremely rare and generally have a favorable prognosis. However, the authors reported the case of a primary ICC originating in the pineal gland presenting with recurrent thalamic hemorrhage and displaying an aggressive postoperative clinical course. OBSERVATIONS A 41-year-old man arrived at the emergency department with morning headaches and recurrent syncope that had lasted several months. Computed tomography and magnetic resonance imaging (MRI) revealed a pineal gland mass causing obstructive hydrocephalus and a subacute hematoma in the right thalamus. Three weeks after an endoscopic third ventriculostomy was performed, recurrent hemorrhage was observed in the right thalamus. The tumor was promptly removed surgically. The yellowish-white tumor did not exhibit abundant bleeding. No evidence of intratumoral hemorrhage around the hematoma pocket was found. Histopathological examination revealed the characteristics of a chordoma with minimal vascularity. MRI performed 10 weeks postoperatively for worsening headaches revealed abnormal enhancement of multiple cranial nerves, suggesting leptomeningeal seeding (LMS) of the tumor. LESSONS Despite radiotherapy and intrathecal chemotherapy, the patient’s neurological status worsened; he died 2 years postoperatively. A pineal ICC may cause recurrent thalamic hemorrhage and potentially fatal LMS, even in the early postoperative period.

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Section: Pineal Chordoma and Recurrent Bleedingmentioning

confidence: 64%

Section: Pineal Chordoma and Recurrent Bleedingmentioning

confidence: 99%

Management challenges associated with a pineal region chordoma: illustrative case

Kwon

Hwang

et al. 2021

Journal of Neurosurgery: Case Lessons

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“…Gross consistency of chordoma is macroscopically described as semiliquid, soft, green, and transparent. 19,29,41,44 Chordomas frequently contain focal calcifications, ossifications, hemorrhagic areas, necrosis, and cyst formations. Microscopic examination shows the pathognomonic histological characteristics of these tumors, which is the presence of physaliferous cells (Fig.…”

Section: Tumor Growthmentioning

confidence: 99%

“…7,42 Clival chordomas tend to cluster in a younger population, whereas sacrococcygeal chordomas present more frequently in older adults. 16,29,50 The clinical presentation of patients suffering from chordoma is dependent on tumor location, and pain is the most common presenting symptom. 20 Patients with clival chordoma frequently present with cranial nerve palsies and headaches, whereas patients with sacral chordoma commonly present with sacral or flank pain and gait disturbances.…”

mentioning

confidence: 99%

“…20 Patients with clival chordoma frequently present with cranial nerve palsies and headaches, whereas patients with sacral chordoma commonly present with sacral or flank pain and gait disturbances. 29,50 Chordomas seldom metastasize, but when they do, the distal lesions occur late in the course of the disease (rates of chordoma metastasis reported in the literature range from 5% to 43%). 7,11,28,48,51 The current standard of care includes a combination of resection and radiotherapy.…”

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confidence: 99%

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Effects of primary and recurrent sacral chordoma on the motor and nociceptive function of hindlimbs in rats: an orthotopic spine model

Sarabia-Estrada

Ruiz‐Valls²,

Shah

et al. 2017

Journal of Neurosurgery: Spine

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OBJECTIVEChordoma is a slow-growing, locally aggressive cancer that is minimally responsive to conventional chemotherapy and radiotherapy and has high local recurrence rates after resection. Currently, there are no rodent models of spinal chordoma. In the present study, the authors sought to develop and characterize an orthotopic model of human chordoma in an immunocompromised rat.METHODSThirty-four immunocompromised rats were randomly allocated to 4 study groups; 22 of the 34 rats were engrafted in the lumbar spine with human chordoma. The groups were as follows: UCH1 tumor–engrafted (n = 11), JHC7 tumor–engrafted (n = 11), sham surgery (n = 6), and intact control (n = 6) rats. Neurological impairment of rats due to tumor growth was evaluated using open field and locomotion gait analysis; pain response was evaluated using mechanical or thermal paw stimulation. Cone beam CT (CBCT), MRI, and nanoScan PET/CT were performed to evaluate bony changes due to tumor growth. On Day 550, rats were killed and spines were processed for H & E–based histological examination and immunohistochemistry for brachyury, S100β, and cytokeratin.RESULTSThe spine tumors displayed typical chordoma morphology, that is, physaliferous cells filled with vacuolated cytoplasm of mucoid matrix. Brachyury immunoreactivity was confirmed by immunostaining, in which samples from tumor-engrafted rats showed a strong nuclear signal. Sclerotic lesions in the vertebral body of rats in the UCH1 and JHC7 groups were observed on CBCT. Tumor growth was confirmed using contrast-enhanced MRI. In UCH1 rats, large tumors were observed growing from the vertebral body. JHC7 chordoma–engrafted rats showed smaller tumors confined to the bone periphery compared with UCH1 chordoma–engrafted rats. Locomotion analysis showed a disruption in the normal gait pattern, with an increase in the step length and duration of the gait in tumor-engrafted rats. The distance traveled and the speed of rats in the open field test was significantly reduced in the UCH1 and JHC7 tumor–engrafted rats compared with controls. Nociceptive response to a mechanical stimulus showed a significant (p < 0.001) increase in the paw withdrawal threshold (mechanical hypalgesia). In contrast, the paw withdrawal response to a thermal stimulus decreased significantly (p < 0.05) in tumor-engrafted rats.CONCLUSIONSThe authors developed an orthotopic human chordoma model in rats. Rats were followed for 550 days using imaging techniques, including MRI, CBCT, and nanoScan PET/CT, to evaluate lesion progression and bony integrity. Nociceptive evaluations and locomotion analysis were performed during follow-up. This model reproduces cardinal signs, such as locomotor and sensory deficits, similar to those observed clinically in human patients. To the authors’ knowledge, this is the first spine rodent model of human chordoma. Its use and further study will be essential for pathophysiology research and the development of new therapeutic strategies.

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Intrasellar hemorrhagic chordoma masquerading as pituitary apoplexy: case report and review of the literature

Kim

Dominguez

et al. 2021

British Journal of Neurosurgery

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Apoplexy in an intradural clival chordoma causing intraventricular bleed

Cited by 4 publications

References 6 publications

Management challenges associated with a pineal region chordoma: illustrative case

Management challenges associated with a pineal region chordoma: illustrative case

Effects of primary and recurrent sacral chordoma on the motor and nociceptive function of hindlimbs in rats: an orthotopic spine model

Intrasellar hemorrhagic chordoma masquerading as pituitary apoplexy: case report and review of the literature

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