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

• Park HG
• Yoon JH
• Kim SH
• Cho KH
• Park HJ
• Kim SH
• Kim EH

• KMID: 2134281
• DOI: http://doi.org/10.14791/btrt.2014.2.2.108

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

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  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.

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  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).

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  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).

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  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.

Reference

1. Rorke LB, Packer RJ, Biegel JA. Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood: definition of an entity. J Neurosurg. 1996; 85:56–65. PMID: 8683283.

2. Bruch LA, Hill DA, Cai DX, Levy BK, Dehner LP, Perry A. A role for fluorescence in situ hybridization detection of chromosome 22q dosage in distinguishing atypical teratoid/rhabdoid tumors from medulloblastoma/central primitive neuroectodermal tumors. Hum Pathol. 2001; 32:156–162. PMID: 11230702.

3. Arrazola J, Pedrosa I, Méndez R, Saldaña C, Scheithauer BW, Martínez A. Primary malignant rhabdoid tumour of the brain in an adult. Neuroradiology. 2000; 42:363–367. PMID: 10872158.

4. Haberler C, Laggner U, Slavc I, et al. Immunohistochemical analysis of INI1 protein in malignant pediatric CNS tumors: Lack of INI1 in atypical teratoid/rhabdoid tumors and in a fraction of primitive neuroectodermal tumors without rhabdoid phenotype. Am J Surg Pathol. 2006; 30:1462–1468. PMID: 17063089.

5. Biegel JA, Kalpana G, Knudsen ES, et al. The role of INI1 and the SWI/SNF complex in the development of rhabdoid tumors: meeting summary from the workshop on childhood atypical teratoid/rhabdoid tumors. Cancer Res. 2002; 62:323–328. PMID: 11782395.

6. Horn M, Schlote W, Lerch KD, Steudel WI, Harms D, Thomas E. Malignant rhabdoid tumor: primary intracranial manifestation in an adult. Acta Neuropathol. 1992; 83:445–448. PMID: 1575023.

7. Kawaguchi T, Kumabe T, Watanabe M, Tominaga T. Atypical teratoid/rhabdoid tumour with leptomeningeal dissemination in an adult. Acta Neurochir (Wien). 2004; 146:1033–1038. discussion 1038. PMID: 15340816.

8. Lutterbach J, Liegibel J, Koch D, Madlinger A, Frommhold H, Pagenstecher A. Atypical teratoid/rhabdoid tumors in adult patients: case report and review of the literature. J Neurooncol. 2001; 52:49–56. PMID: 11451202.

9. Arita K, Sugiyama K, Sano T, Oka H. Atypical teratoid/rhabdoid tumour in sella turcica in an adult. Acta Neurochir (Wien). 2008; 150:491–495. discussion 496. PMID: 18309453.

10. Kuge A, Kayama T, Tsuchiya D, et al. [Suprasellar primary malignant rhabdoid tumor in an adult: a case report]. No Shinkei Geka. 2000; 28:351–358. PMID: 10769834.

11. Las Heras F, Pritzker KP. Adult variant of atypical teratoid/rhabdoid tumor: immunohistochemical and ultrastructural confirmation of a rare tumor in the sella tursica. Pathol Res Pract. 2010; 206:788–791. PMID: 20705400.

12. Raisanen J, Biegel JA, Hatanpaa KJ, Judkins A, White CL, Perry A. Chromosome 22q deletions in atypical teratoid/rhabdoid tumors in adults. Brain Pathol. 2005; 15:23–28. PMID: 15779233.

13. Schneiderhan TM, Beseoglu K, Bergmann M, et al. Sellar atypical teratoid/rhabdoid tumours in adults. Neuropathol Appl Neurobiol. 2011; 37:326–329. PMID: 20663057.

14. Cho JM, Ahn JY, Chang JH, Kim SH. Prevention of cerebrospinal fluid rhinorrhea after transsphenoidal surgery by collagen fleece coated with fibrin sealant without autologous tissue graft or postoperative lumbar drainage. Neurosurgery. 2011; 68(1 Suppl Operative):130–136. discussion 136-7. PMID: 21206312.

15. Biegel JA, Tan L, Zhang F, Wainwright L, Russo P, Rorke LB. Alterations of the hSNF5/INI1 gene in central nervous system atypical teratoid/rhabdoid tumors and renal and extrarenal rhabdoid tumors. Clin Cancer Res. 2002; 8:3461–3467. PMID: 12429635.

16. Lualdi E, Modena P, Debiec-Rychter M, et al. Molecular cytogenetic characterization of proximal-type epithelioid sarcoma. Genes Chromosomes Cancer. 2004; 41:283–290. PMID: 15334553.

17. Judkins AR, Mauger J, Ht A, Rorke LB, Biegel JA. Immunohistochemical analysis of hSNF5/INI1 in pediatric CNS neoplasms. Am J Surg Pathol. 2004; 28:644–650. PMID: 15105654.

18. Packer RJ, Biegel JA, Blaney S, et al. Atypical teratoid/rhabdoid tumor of the central nervous system: report on workshop. J Pediatr Hematol Oncol. 2002; 24:337–342. PMID: 12142780.

19. Makuria AT, Rushing EJ, McGrail KM, Hartmann DP, Azumi N, Ozdemirli M. Atypical teratoid rhabdoid tumor (AT/RT) in adults: review of four cases. J Neurooncol. 2008; 88:321–330. PMID: 18369529.

20. Shonka NA, Armstrong TS, Prabhu SS, et al. Atypical teratoid/rhabdoid tumors in adults: a case report and treatment-focused review. J Clin Med Res. 2011; 3:85–92. PMID: 21811535.

21. Takahashi K, Nishihara H, Katoh M, et al. Case of atypical teratoid/rhabdoid tumor in an adult, with long survival. Brain Tumor Pathol. 2011; 28:71–76. PMID: 21181449.

22. Buscariollo DL, Park HS, Roberts KB, Yu JB. Survival outcomes in atypical teratoid rhabdoid tumor for patients undergoing radiotherapy in a Surveillance, Epidemiology, and End Results analysis. Cancer. 2012; 118:4212–4219. PMID: 22213196.

23. Gardner SL, Asgharzadeh S, Green A, Horn B, McCowage G, Finlay J. Intensive induction chemotherapy followed by high dose chemotherapy with autologous hematopoietic progenitor cell rescue in young children newly diagnosed with central nervous system atypical teratoid rhabdoid tumors. Pediatr Blood Cancer. 2008; 51:235–240. PMID: 18381756.

24. Park ES, Sung KW, Baek HJ, et al. Tandem high-dose chemotherapy and autologous stem cell transplantation in young children with atypical teratoid/rhabdoid tumor of the central nervous system. J Korean Med Sci. 2012; 27:135–140. PMID: 22323859.

25. Chi SN, Zimmerman MA, Yao X, et al. Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor. J Clin Oncol. 2009; 27:385–389. PMID: 19064966.