Atypical teratoid rhabdoid tumor in adulthood

Unusual clear cell, lymphoplasmacyte-rich, dural-based tumor with divergent differentiation: a tricky case mimicking a meningioma

“…Polyphenotypic differentiation is characteristic of the infrequent atypical teratoid/rhabdoid tumor (AT/RT), a pediatric neoplasm that can also occur in adults [8]. Similar to our case, AT/RT contains clear cells with divergent differentiation, indicated by variable positivity for cytokeratins, EMA, smooth muscle actin, synaptophysin, neurofilaments, and CD34.…”

Atypical teratoid rhabdoid tumor involving the nasal cavities and anterior skull base

“…[8][9][10][11]17 Based on the present and previous cases, [8][9][10]17 ATRT in adolescents seems to be preferentially supratentorial, as it is in adults. 12 To the best of our knowledge, this is the third extra-axial case of ATRT and the first infiltrating the nasal tract. Of the two previously described extra-axial cases, 11,18 one was located in the terminal phylum in an adolescent, 11 while the other was a temporal mass in a 22-month-old child.…”

“…ATRT in adolescents is extremely rare and only six cases have been reported up to now . Based on the present and previous cases, ATRT in adolescents seems to be preferentially supratentorial, as it is in adults . To the best of our knowledge, this is the third extra‐axial case of ATRT and the first infiltrating the nasal tract.…”

“…It is mainly a pediatric neoplasia which shows the highest incidence in children under the age of 3 years . It may rarely occur in children older than 6 years, and very rarely in adolescents or in adults . Localization in the CNS varies according to the age of patients; indeed ATRT may be supratentorial or infratentorial in children, while it is mainly supratentorial in the cerebral hemispheres in adults …”

“…[7][8][9][10][11][12] Localization in the CNS varies according to the age of patients; indeed ATRT may be supratentorial or infratentorial in children, 7 while it is mainly supratentorial in the cerebral hemispheres in adults. 12 The pathogenesis of ATRT is related to the inactivation of the INI1/SMARCB1 gene, which is located in chromosome band 22q11.2 13 and which encodes for INI1 protein, a tumor suppressor of the ATP-dependent SWI-SNF chromatin remodeling complex. Inactivating mutations in SMARCB1 gene result in the absence of INI1 protein expression, which may be evidenced by immunohistochemistry in formalin-fixed and paraffin-embedded samples of ATRT.…”