Limits...
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

Histologically, the tumor tissue is mainly composed of hyperchromatic small cells with a relatively high nuclear/cytoplasm ratio and rhabdoid cells (H and E. Original magnification ×100 (a) and ×400 (b)). In immunohistochemical studies, Nestin (c), S-100 (d) and Vimentin (e) were positive and EMA (f) was focally positive. The proliferation index measured by Ki-67 was 85% in the areas of highest mitotic activity (g) and the tumor cells lack nuclear staining for the INI1 protein (h). Pathological findings were consistent with those of atypical teratoid/rhabdoid tumor
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC4061576&req=5

Figure 7: Histologically, the tumor tissue is mainly composed of hyperchromatic small cells with a relatively high nuclear/cytoplasm ratio and rhabdoid cells (H and E. Original magnification ×100 (a) and ×400 (b)). In immunohistochemical studies, Nestin (c), S-100 (d) and Vimentin (e) were positive and EMA (f) was focally positive. The proliferation index measured by Ki-67 was 85% in the areas of highest mitotic activity (g) and the tumor cells lack nuclear staining for the INI1 protein (h). Pathological findings were consistent with those of atypical teratoid/rhabdoid tumor

Mentions: The tumor was composed of polygonal cells with a relatively high nuclear/cytoplasm ratio and the rhabdoid cells had medium, round, single or double nuclei, and eosinophilic cytoplasmic inclusions. The tumor cells showed strong immunoreactivity for O-6 methylguanine DNA methyltransferase (MGMT), Nestin and S100 protein, and focal positivity for epithelial membrane antigen (EMA) and Glypican-3. Desmin, oligodendrocyte transcription factor 2 (oligo2), placentral alkaline phosphatase, myogenin and p63 were negative. Proliferative activity is high, and Ki-67/MIB-1 labeling index was more than 90%. Since the tumor cells lacked nuclear expression of INI1, the tumor was pathologically diagnosed as AT/RT [Figure 7].


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)

Histologically, the tumor tissue is mainly composed of hyperchromatic small cells with a relatively high nuclear/cytoplasm ratio and rhabdoid cells (H and E. Original magnification ×100 (a) and ×400 (b)). In immunohistochemical studies, Nestin (c), S-100 (d) and Vimentin (e) were positive and EMA (f) was focally positive. The proliferation index measured by Ki-67 was 85% in the areas of highest mitotic activity (g) and the tumor cells lack nuclear staining for the INI1 protein (h). Pathological findings were consistent with those of atypical teratoid/rhabdoid tumor
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 7: Histologically, the tumor tissue is mainly composed of hyperchromatic small cells with a relatively high nuclear/cytoplasm ratio and rhabdoid cells (H and E. Original magnification ×100 (a) and ×400 (b)). In immunohistochemical studies, Nestin (c), S-100 (d) and Vimentin (e) were positive and EMA (f) was focally positive. The proliferation index measured by Ki-67 was 85% in the areas of highest mitotic activity (g) and the tumor cells lack nuclear staining for the INI1 protein (h). Pathological findings were consistent with those of atypical teratoid/rhabdoid tumor
Mentions: The tumor was composed of polygonal cells with a relatively high nuclear/cytoplasm ratio and the rhabdoid cells had medium, round, single or double nuclei, and eosinophilic cytoplasmic inclusions. The tumor cells showed strong immunoreactivity for O-6 methylguanine DNA methyltransferase (MGMT), Nestin and S100 protein, and focal positivity for epithelial membrane antigen (EMA) and Glypican-3. Desmin, oligodendrocyte transcription factor 2 (oligo2), placentral alkaline phosphatase, myogenin and p63 were negative. Proliferative activity is high, and Ki-67/MIB-1 labeling index was more than 90%. Since the tumor cells lacked nuclear expression of INI1, the tumor was pathologically diagnosed as AT/RT [Figure 7].

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