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2014, Volume 30, Number 1, Page(s)
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DOI: 10.5146/tjpath.2013.01213 |
Atypical Teratoid Rhabdoid Tumor in Childhood, 15 Cases of a Single Institute Experience |
Maysa AL-HUSSAINI1, Noreen DISSI1, Usama AL-JUMAILY2, Maisa SWAIDAN3 |
1Departments of Pathology and Laboratory Medicine, King Hussein Cancer Center, AMMAN, JORDAN 2Departments of Pediatric Oncology, King Hussein Cancer Center, AMMAN, JORDAN 3Departments of Radiology, King Hussein Cancer Center, AMMAN, JORDAN |
Keywords:
Central nervous system neoplasms, Medulloblastoma, Immunohistochemistry |
Objective: Atypical teratoid rhabdoid tumor is a rare neoplasm with
a distinct cytogenetic profile that predominates in infancy. Many
cases show predominance of small cells with scanty rhabdoid cells,
making recognition of this tumor difficult. We aim at describing our
experience with atypical teratoid rhabdoid tumor cases diagnosed
over a 6-year period.
Material and Method: Clinicopathologic features and immunohistochemical
staining of atypical teratoid rhabdoid tumor cases diagnosed
between 2006 and 2011 are presented.
Results: Fifteen cases were identified including 9 males with a
median age of 26 months. The most common presenting symptom
was recurrent vomiting with a mean duration of 6 weeks. Nine cases
(60%) were infratentorial and cerebrospinal fluid was positive in 2
cases (13.3%) at time of diagnosis. The median overall survival of
the group was 9.5 months. All cases except one showed admixture of
rhabdoid and/or small round blue cells in variable proportions. Only
5 out of fourteen referred cases (35.7%) were correctly diagnosed.
Three cases showed unusual growth patterns. In 2 cases, nodular
medulloblastoma-like growth pattern predominated, with loss of
INI-1/BAF47 staining both within the nodules and the inter-nodular
areas. The third case contained scattered individual and small groups
of large cells with abundant acidophilic cytoplasm and eccentric
nuclei, reminiscent of rhabdomyoblasts that were positive for
GFAP and desmin, and retained nuclear staining for INI-1/BAF47,
consistent with reactive gemistocytes.
Conclusion: Pathologists should be aware of the various, and unusual
histopathologic patterns of atypical teratoid rhabdoid tumor. INI-1/
BAF47 immunostain should be performed on all central nervous
system embryonal tumors, especially in infants and young children.
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