Brain Tumor Res Treat.  2014 Oct;2(2):108-113. 10.14791/btrt.2014.2.2.108.

Adult-Onset Sellar and Suprasellar Atypical Teratoid Rhabdoid Tumor Treated with a Multimodal Approach: A Case Report

Affiliations
  • 1Department of Neurosurgery, Yonsei University College of Medicine, Seoul, Korea. euihyunkim@yuhs.ac
  • 2Center for Pediatric Oncology, National Cancer Center, Goyang, Korea.
  • 3Department of Pathology, Yonsei University College of Medicine, Seoul, Korea.
  • 4Center for Proton Therapy, National Cancer Center, Goyang, Korea.
  • 5Department of Brain Research Institute, Yonsei University College of Medicine, Seoul, Korea.

Abstract

We report a very rare case of sellar and suprasellar atypical teratoid rhabdoid tumor (ATRT) in a 42-year-old female patient. The tumor was removed subtotally with a transsphenoidal approach. Histopathologic study showed rhabdoid cells with prominent nucleoli and abundant cytoplasm. Immunohistochemistry for INI1 was completely negative in the tumor cells, consistent with ATRT. After surgery, she received radiotherapy including spinal irradiation with proton beam therapy and subsequent chemotherapy, with no evidence of recurrence for more than 2 years. Up to date, this is the 8th case of an adult-onset ATRT in the sellar or suprasellar region. Despite its rarity, ATRTs should be considered in the differential diagnosis of an unclear malignant sellar or suprasellar lesion in adult patients and the treatment strategies for adult ATRT patients could be differentiated from those of pediatric ATRT patients.

Keyword

Adult-onset; Atypical teratoid rhabdoid tumor; INI1; Sellar; Suprasellar

MeSH Terms

Adult
Cytoplasm
Diagnosis, Differential
Drug Therapy
Female
Humans
Immunohistochemistry
Proton Therapy
Radiotherapy
Recurrence
Rhabdoid Tumor*

Figure

  • Fig. 1 Preoperative magnetic resonance imaging shows a heterogeneously enhanced mass involving the sella in a T1-weighted gadolinium-enhanced coronal (A) and sagittal view (B). T2-weighted coronal (C) and sagittal (D) images reveals solid and cystic components of the tumor with compression of an optic apparatus.

  • Fig. 2 Low power views show relatively homogenous population of tumor cells with very high cellularity (A). At the inferior margin of the tumor mass, pituitary glandular structure (black arrows) is seen (B). High power views demonstrate a few tumor cells showing typical rhabdoid features with eccentric nuclei and prominent nucleoli (black arrowheads) (C). Abundant eosinophilic cytoplasm (black arrowheads) is noted (D). H&E, original magnification ×100 (A and B), ×400 (C and D).

  • Fig. 3 Immunohistochemistry (IHC) was negative for CD20 (A), cytokeratin (B), synaptophysin (C), and strong positive for CD99 (D). IHC stain for INI1 is negative in tumor cells whereas it is positive for endothelial cells (black arrowheads) (E). CD20-IHC, original magnification ×200 (A), cytokeratin-IHC, ×200 (B), synaptophysin-IHC, ×200 (C), CD99-IHC, ×200 (D), and INI1-IHC, ×400 (E).

  • Fig. 4 Patient's T1-weighted magnetic resonance imaging at the point of postoperative 2 years shows no evidence of a residual or remnant tumor.


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