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Atypical teratoid/rhabdoid tumor in adults: a systematic review of the literature with meta-analysis and additional reports of 4 cases

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Abstract

Introduction

Atypical teratoid/rhabdoid tumor (AT/RT) is a highly aggressive embryonal CNS neoplasm, characterized by inactivation of SMARCB1 (INI1) or rarely of SMARCA4 (BRG1). While it is predominantly a childhood tumor, AT/RT is rare in adults.

Methods

We provide a comprehensive systematic review of literature with meta-analysis; 92 adult cases were found from 74 articles. We additionally present 4 cases of adult AT/RTs (age ranging from 19 to 29 years), located to cerebellum in 2 cases, to ponto-cerebellar angle in 1 case and to spinal cord in the remaining case.

Results

Microscopic features of our 4 cases showed a highly cellular tumor with rhabdoid morphology and high mitotic activity. All tumor cells lacked nuclear SMARCB1/INI1 protein expression. In case no. 3 we also performed methylation profiling which clustered the tumor with pediatric AT/RT-MYC subgroup. Prognosis remains poor in both pediatric and adult population with a median overall survival of 11 months. Our review demonstrated median overall survival of 15 months among the adult populations. However, consistent with a recent review, adult AT/RT seems to have highly variable prognosis and some patients reach long term survival with 22.9% of 5-year survival without evidence of disease and mean follow up time of 35.9 months (SD = 36.5). 27.1% of dissemination was also reported among the adult population.

Conclusions

Adult AT/RTs predominantly arise in female patients and in supratentorial location. Midline structures, including the sellar region, are the most affected sites, especially among females aged > 40 years. Male gender is more prevalent between the age of 18 and 40 years and more frequently associated with non-midline tumors. Factors significantly associated with better prognosis are patient’s age (< 40 years), combined radio-chemotherapy adjuvant approach and Ki-67 score < 40%.

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Data availability

The data of this study are available from the corresponding author upon reasonable request.

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Authors and Affiliations

  1. Department of Medical, Surgical Sciences and Advanced Technologies “G.F. Ingrassia”, Anatomic Pathology, University of Catania, 95123, Catania, Italy

    Giuseppe Broggi

  2. Department of Radiological, Oncological and Anatomo-Pathological Sciences, University Sapienza, Viale Regina Elena 324, 00161, Rome, Italy

    Francesca Gianno, Doron Theodore Shemy, Felice Giangaspero & Manila Antonelli

  3. Fondazione IRCCS-Istituto Nazionale dei Tumori, Milan, Italy

    Maura Massimino

  4. Department of Haematology/Oncology, Scientific Directorate, Giannina Gaslini Children’s Hospital, Genova, Italy

    Claudia Milanaccio

  5. Department of Hematology/Oncology, Cell and Gene Therapy, IRCCS Bambino Gesù Children’s Hospital, 00165, Rome, Italy

    Angela Mastronuzzi

  6. Pathology Unit, Department of Laboratories, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy

    Sabrina Rossi

  7. IRCCS Neuromed, Pozzilli, IS, Italy

    Antonietta Arcella & Felice Giangaspero

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  1. Giuseppe Broggi
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  2. Francesca Gianno
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  8. Antonietta Arcella
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  9. Felice Giangaspero
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  10. Manila Antonelli
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Contributions

Conceptualization: GB, MA and FG; formal analysis: DTS; investigation: DTS; writing—original draft: DTS; writing—review and editing: GB, MA and FG; resources: MM, CM, AM, SR and AA; visualisation: MA; supervision: FG. All authors accepted the final version of the manuscript.

Corresponding author

Correspondence to Manila Antonelli.

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The authors declare no conflict of interest.

Informed consent

The study complied with the Ethical Principles for Medical Research Involving Human Subjects according to the World Medical Association Declaration of Helsinki; the non-interventional, retrospective nature of our study did not require any informed consent, even if a written informed consent had been obtained from each patient before surgical procedures. The clinical information had been retrieved from the patients’ medical records and pathology reports. Patients’ initials or other personal identifiers did not appear in any image. Finally, all samples were anonymized before histology and immunohistochemistry; therefore, no further ethical approval was necessary to perform the retrospective study.

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Broggi, G., Gianno, F., Shemy, D.T. et al. Atypical teratoid/rhabdoid tumor in adults: a systematic review of the literature with meta-analysis and additional reports of 4 cases. J Neurooncol 157, 1–14 (2022). https://doi.org/10.1007/s11060-022-03959-z

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