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Chordoma in the lateral medullary cistern in a patient with tuberous sclerosis: A case report and review of the literature.

Kimmell KT, Dayoub H, Stolzenberg ED, Sincoff EH - Surg Neurol Int (2010)

Bottom Line: A 38-year-old man with the diagnosis of TSC presented with the complaint of dizziness and near syncope.Imaging revealed a mass in the lateral medullary cistern that was found at the time of surgery to be a chordoma.This study presents an unusual presentation and location for a chordoma and contributes to the growing literature associating chordomas with TSC.

View Article: PubMed Central - HTML - PubMed

Affiliation: College of Medicine, The University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA.

ABSTRACT

Background: Chordomas are rare intracranial tumors. There are several reported cases of these tumors arising in patients with tuberous sclerosis (TSC), a neurocutaneous disorder inherited in autosomal dominant fashion that predisposes patients to hamartomatous and neoplastic lesions.

Case description: A 38-year-old man with the diagnosis of TSC presented with the complaint of dizziness and near syncope. Imaging revealed a mass in the lateral medullary cistern that was found at the time of surgery to be a chordoma. The patient underwent a left far lateral approach for removal of the tumor. Upon opening of the dura, the tumor could be seen under the arachnoid. The tumor was carefully debulked within the limits of safety. The patient did well postoperatively and was referred to the radiation oncology department at our institution for follow-up radiotherapy of the tumor bed.

Conclusion: This study presents an unusual presentation and location for a chordoma and contributes to the growing literature associating chordomas with TSC.

No MeSH data available.


Related in: MedlinePlus

(a) CT brain showing periventricular calcifications and (b) MRI FLAIR sequence showing subcortical and periventricular white matter changes. Both of these findings are common in patients with a diagnosis of tuberous sclerosis
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Figure 0001: (a) CT brain showing periventricular calcifications and (b) MRI FLAIR sequence showing subcortical and periventricular white matter changes. Both of these findings are common in patients with a diagnosis of tuberous sclerosis

Mentions: A 38-year-old man presented to the emergency room at our institution with the complaints of dizziness and a near-syncopal episode. This episode was preceded by a 1-week history of nausea, vomiting, and dysarthria. He also reported a 2-month history of new headache. The patient denied any visual disturbances, hearing changes, recent seizures, loss of consciousness, weakness, or sensory changes. Medical history was significant for a diagnosis of TSC with mild mental retardation and seizures in the distant past as well as hypertension. The patient had had a cataract removed remotely. Physical examination revealed adenoma sebaceum on the patient's malar surfaces. Complete neurological examination revealed no focal deficits. The results of laboratory tests were within normal ranges. A computed tomography (CT) of the head from an outside hospital demonstrated a brain mass with high attenuation. The mass appeared adjacent to the left cerebellar hemisphere near the Foramen of Lushka and extending into the foramen magnum. The patient had mild hydrocephalus evidenced by the prominence of his ventricular system. Periventricular calcifications were consistent with his diagnosis of TSC [Figure 1a]. The patient was admitted for work-up of this brainstem mass. Magnetic resonance imaging (MRI) of the brain with contrast demonstrated deep cortical white matter changes typical of patients with TSC [Figure 1b]. MRI also showed an extra-axial, irregularly shaped, well-marginated mass with heterogeneous enhancement in the left aspect of the medullary cistern with displacement of the brainstem and cerebellar peduncle to the right [Figure 2]. Further careful review of the imaging showed that the majority of the clivus also demonstrated uptake and enhancement of contrast. Increased T2 signal in the brainstem and cerebellum was consistent with edema. The patient was administered steroids and discharged with a plan to return to the hospital for elective removal of the brain mass.


Chordoma in the lateral medullary cistern in a patient with tuberous sclerosis: A case report and review of the literature.

Kimmell KT, Dayoub H, Stolzenberg ED, Sincoff EH - Surg Neurol Int (2010)

(a) CT brain showing periventricular calcifications and (b) MRI FLAIR sequence showing subcortical and periventricular white matter changes. Both of these findings are common in patients with a diagnosis of tuberous sclerosis
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2908358&req=5

Figure 0001: (a) CT brain showing periventricular calcifications and (b) MRI FLAIR sequence showing subcortical and periventricular white matter changes. Both of these findings are common in patients with a diagnosis of tuberous sclerosis
Mentions: A 38-year-old man presented to the emergency room at our institution with the complaints of dizziness and a near-syncopal episode. This episode was preceded by a 1-week history of nausea, vomiting, and dysarthria. He also reported a 2-month history of new headache. The patient denied any visual disturbances, hearing changes, recent seizures, loss of consciousness, weakness, or sensory changes. Medical history was significant for a diagnosis of TSC with mild mental retardation and seizures in the distant past as well as hypertension. The patient had had a cataract removed remotely. Physical examination revealed adenoma sebaceum on the patient's malar surfaces. Complete neurological examination revealed no focal deficits. The results of laboratory tests were within normal ranges. A computed tomography (CT) of the head from an outside hospital demonstrated a brain mass with high attenuation. The mass appeared adjacent to the left cerebellar hemisphere near the Foramen of Lushka and extending into the foramen magnum. The patient had mild hydrocephalus evidenced by the prominence of his ventricular system. Periventricular calcifications were consistent with his diagnosis of TSC [Figure 1a]. The patient was admitted for work-up of this brainstem mass. Magnetic resonance imaging (MRI) of the brain with contrast demonstrated deep cortical white matter changes typical of patients with TSC [Figure 1b]. MRI also showed an extra-axial, irregularly shaped, well-marginated mass with heterogeneous enhancement in the left aspect of the medullary cistern with displacement of the brainstem and cerebellar peduncle to the right [Figure 2]. Further careful review of the imaging showed that the majority of the clivus also demonstrated uptake and enhancement of contrast. Increased T2 signal in the brainstem and cerebellum was consistent with edema. The patient was administered steroids and discharged with a plan to return to the hospital for elective removal of the brain mass.

Bottom Line: A 38-year-old man with the diagnosis of TSC presented with the complaint of dizziness and near syncope.Imaging revealed a mass in the lateral medullary cistern that was found at the time of surgery to be a chordoma.This study presents an unusual presentation and location for a chordoma and contributes to the growing literature associating chordomas with TSC.

View Article: PubMed Central - HTML - PubMed

Affiliation: College of Medicine, The University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA.

ABSTRACT

Background: Chordomas are rare intracranial tumors. There are several reported cases of these tumors arising in patients with tuberous sclerosis (TSC), a neurocutaneous disorder inherited in autosomal dominant fashion that predisposes patients to hamartomatous and neoplastic lesions.

Case description: A 38-year-old man with the diagnosis of TSC presented with the complaint of dizziness and near syncope. Imaging revealed a mass in the lateral medullary cistern that was found at the time of surgery to be a chordoma. The patient underwent a left far lateral approach for removal of the tumor. Upon opening of the dura, the tumor could be seen under the arachnoid. The tumor was carefully debulked within the limits of safety. The patient did well postoperatively and was referred to the radiation oncology department at our institution for follow-up radiotherapy of the tumor bed.

Conclusion: This study presents an unusual presentation and location for a chordoma and contributes to the growing literature associating chordomas with TSC.

No MeSH data available.


Related in: MedlinePlus