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Atypical teratoid/rhabdoid tumor in sellar turcica in an adult: A case report and review of the literature.

Shitara S, Akiyama Y - Surg Neurol Int (2014)

Bottom Line: She died of respiratory failure 17 months after the initial treatment.AT/RT should be considered in the differential diagnosis of a sellar lesion in adult patients.However AT/RT is rare in adults, the appropriate immunohistochemical evaluation should be performed to diagnose this rare entity.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Tenri Hospital, Tenri, Nara, Japan.

ABSTRACT

Background: Atypical teratoid/rhabdoid tumor (AT/RT) is a rare central nervous system tumor composed of primitive rhabdoid cells that may differentiate along neuroectodermal, mesenchymal and epithelial lineages. AT/RT in adults is rare but not completely exceptional. It generally arises from the posterior fossa of infants, but the broad majority of the reported AT/RT in adults manifested supratentorially with the exception of four cases that arose in the cerebellum and two that arose in the spinal cord.

Case description: A 44-year-old female complained of visual disturbance. We performed craniotomies twice and removed partially for each time, but any malignant cells were not found in the specimens. Finally, we determined histological diagnosis from the extended lesion. She died of respiratory failure 17 months after the initial treatment.

Conclusion: AT/RT should be considered in the differential diagnosis of a sellar lesion in adult patients. However AT/RT is rare in adults, the appropriate immunohistochemical evaluation should be performed to diagnose this rare entity.

No MeSH data available.


Related in: MedlinePlus

T1-weighted MRI coronal (a) and sagittal (b) images clearly show inhomogeneously gadolinium enhancing mass in sellar turcica extending into suprasellar lesion
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Figure 1: T1-weighted MRI coronal (a) and sagittal (b) images clearly show inhomogeneously gadolinium enhancing mass in sellar turcica extending into suprasellar lesion

Mentions: A previously healthy 44-year-old female presented with a 2-month history of visual disturbance. Magnetic resonance imaging (MRI) revealed a heterogeneously enhancing mass within suprasellar and intrasellar lesions [Figure 1]. She underwent a partial resection, mainly caudal part of the tumor, via translabial transsphenoidal approach [Figure 2]. As the pathological examination could not find any malignant cells, histological diagnosis from this operation was lymphocytic hypophisitis.


Atypical teratoid/rhabdoid tumor in sellar turcica in an adult: A case report and review of the literature.

Shitara S, Akiyama Y - Surg Neurol Int (2014)

T1-weighted MRI coronal (a) and sagittal (b) images clearly show inhomogeneously gadolinium enhancing mass in sellar turcica extending into suprasellar lesion
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4061576&req=5

Figure 1: T1-weighted MRI coronal (a) and sagittal (b) images clearly show inhomogeneously gadolinium enhancing mass in sellar turcica extending into suprasellar lesion
Mentions: A previously healthy 44-year-old female presented with a 2-month history of visual disturbance. Magnetic resonance imaging (MRI) revealed a heterogeneously enhancing mass within suprasellar and intrasellar lesions [Figure 1]. She underwent a partial resection, mainly caudal part of the tumor, via translabial transsphenoidal approach [Figure 2]. As the pathological examination could not find any malignant cells, histological diagnosis from this operation was lymphocytic hypophisitis.

Bottom Line: She died of respiratory failure 17 months after the initial treatment.AT/RT should be considered in the differential diagnosis of a sellar lesion in adult patients.However AT/RT is rare in adults, the appropriate immunohistochemical evaluation should be performed to diagnose this rare entity.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Tenri Hospital, Tenri, Nara, Japan.

ABSTRACT

Background: Atypical teratoid/rhabdoid tumor (AT/RT) is a rare central nervous system tumor composed of primitive rhabdoid cells that may differentiate along neuroectodermal, mesenchymal and epithelial lineages. AT/RT in adults is rare but not completely exceptional. It generally arises from the posterior fossa of infants, but the broad majority of the reported AT/RT in adults manifested supratentorially with the exception of four cases that arose in the cerebellum and two that arose in the spinal cord.

Case description: A 44-year-old female complained of visual disturbance. We performed craniotomies twice and removed partially for each time, but any malignant cells were not found in the specimens. Finally, we determined histological diagnosis from the extended lesion. She died of respiratory failure 17 months after the initial treatment.

Conclusion: AT/RT should be considered in the differential diagnosis of a sellar lesion in adult patients. However AT/RT is rare in adults, the appropriate immunohistochemical evaluation should be performed to diagnose this rare entity.

No MeSH data available.


Related in: MedlinePlus