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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

Postoperative MRI of the brain T1 postcontrast infusion sequences in axial, coronal, and sagittal planes demonstrating extent of surgical debulking
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Figure 0003: Postoperative MRI of the brain T1 postcontrast infusion sequences in axial, coronal, and sagittal planes demonstrating extent of surgical debulking

Mentions: The patient was taken to the operating room for a left far lateral approach for removal of the tumor. Upon opening of the dura and release of cerebrospinal fluid, the tumor could be seen under the arachnoid and appeared to originate intradurally. Several cranial nerves were adherent to the mass, eliminating the possibility of a total resection. During the operation, no connection between the intradural mass and the clivus could be appreciated. The tumor was carefully debulked within the limits of safety. A significant amount of cerebellar swelling was encountered during the case and ultimately led to early termination of the operation before subtotal resection could be completed. The patient was then transferred to the ICU. He remained intubated but was following commands with all four extremities. Postoperative MRI showed significant reduction of tumor burden from surgical debulking [Figure 3].


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)

Postoperative MRI of the brain T1 postcontrast infusion sequences in axial, coronal, and sagittal planes demonstrating extent of surgical debulking
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 0003: Postoperative MRI of the brain T1 postcontrast infusion sequences in axial, coronal, and sagittal planes demonstrating extent of surgical debulking
Mentions: The patient was taken to the operating room for a left far lateral approach for removal of the tumor. Upon opening of the dura and release of cerebrospinal fluid, the tumor could be seen under the arachnoid and appeared to originate intradurally. Several cranial nerves were adherent to the mass, eliminating the possibility of a total resection. During the operation, no connection between the intradural mass and the clivus could be appreciated. The tumor was carefully debulked within the limits of safety. A significant amount of cerebellar swelling was encountered during the case and ultimately led to early termination of the operation before subtotal resection could be completed. The patient was then transferred to the ICU. He remained intubated but was following commands with all four extremities. Postoperative MRI showed significant reduction of tumor burden from surgical debulking [Figure 3].

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