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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) On medium-magnification, eosinophilic neoplastic cells can be seen in the midst of a mucoid matrix; Inset: high-powered magnification shows markedly foamy cytoplasm in some neoplastic cells (the so-called physaliphorous cell). Immunohistochemical staining for (b) epithelial membrane antigen, (c) Pan-cytokeratin, and (d) S-100 demonstrate positive staining for all three in the neoplastic cells. This pattern of immunohistochemical staining, coupled with the histologic characteristics of the tumor, are consistent with a chordoma of the skull base.
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Figure 0004: (a) On medium-magnification, eosinophilic neoplastic cells can be seen in the midst of a mucoid matrix; Inset: high-powered magnification shows markedly foamy cytoplasm in some neoplastic cells (the so-called physaliphorous cell). Immunohistochemical staining for (b) epithelial membrane antigen, (c) Pan-cytokeratin, and (d) S-100 demonstrate positive staining for all three in the neoplastic cells. This pattern of immunohistochemical staining, coupled with the histologic characteristics of the tumor, are consistent with a chordoma of the skull base.

Mentions: Grossly, the tumor was gray-tan in appearance. Microscopic examination of the tumor revealed plump, vacuolated cells within a mucinous matrix [Figure 4]. Tumor cells were positive for pan-cytokeratin (pan-CK) and epithelial membrane antigen (EMA), focally positive for S-100, and negative for glial fibrillary acidic protein (GFAP) and neurofilament (NF). Less than 1% of nuclei labeled positively for Ki-67. These histologic and immunohistochemical staining characteristics were consistent with a diagnosis of chordoma. This diagnosis was unexpected given the location of the tumor both within the lateral medullary cistern as well as under the arachnoid.


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) On medium-magnification, eosinophilic neoplastic cells can be seen in the midst of a mucoid matrix; Inset: high-powered magnification shows markedly foamy cytoplasm in some neoplastic cells (the so-called physaliphorous cell). Immunohistochemical staining for (b) epithelial membrane antigen, (c) Pan-cytokeratin, and (d) S-100 demonstrate positive staining for all three in the neoplastic cells. This pattern of immunohistochemical staining, coupled with the histologic characteristics of the tumor, are consistent with a chordoma of the skull base.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 0004: (a) On medium-magnification, eosinophilic neoplastic cells can be seen in the midst of a mucoid matrix; Inset: high-powered magnification shows markedly foamy cytoplasm in some neoplastic cells (the so-called physaliphorous cell). Immunohistochemical staining for (b) epithelial membrane antigen, (c) Pan-cytokeratin, and (d) S-100 demonstrate positive staining for all three in the neoplastic cells. This pattern of immunohistochemical staining, coupled with the histologic characteristics of the tumor, are consistent with a chordoma of the skull base.
Mentions: Grossly, the tumor was gray-tan in appearance. Microscopic examination of the tumor revealed plump, vacuolated cells within a mucinous matrix [Figure 4]. Tumor cells were positive for pan-cytokeratin (pan-CK) and epithelial membrane antigen (EMA), focally positive for S-100, and negative for glial fibrillary acidic protein (GFAP) and neurofilament (NF). Less than 1% of nuclei labeled positively for Ki-67. These histologic and immunohistochemical staining characteristics were consistent with a diagnosis of chordoma. This diagnosis was unexpected given the location of the tumor both within the lateral medullary cistern as well as under the arachnoid.

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