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Published on: January 17, 2018
High-Grade Atypical Teratoid/Rhabdoid Tumor in the Pituitary Region
Vincent S Alexander1,2, Michael Ernst1, Wang L Cheung3
1Department of Research, Alabama College of Osteopathic Medicine, Dothan, USA.
Insights
A rare adult case of atypical teratoid/rhabdoid tumors (AT/RT) in the pituitary region is presented. This central nervous system embryonal tumor highlights challenges in adult treatment protocols.
Area of Science:
- Neuro-oncology
- Pediatric Oncology
- Neuropathology
Background:
- Atypical teratoid/rhabdoid tumors (AT/RT) are rare embryonal tumors of the central nervous system (CNS).
- AT/RT predominantly affect infants and young children, with adult presentations being exceptionally uncommon.
- Pituitary region AT/RT is exceedingly rare, particularly in adult patients.
Observation:
- A 37-year-old female presented with intractable headache, sudden monocular diplopia, and left cranial nerve VI palsy.
- Imaging revealed a pituitary region mass.
- Transsphenoidal resection confirmed the diagnosis of AT/RT.
Findings:
- The patient received adjuvant chemotherapy and radiation therapy following surgical resection.
- The diagnosis of AT/RT in an adult pituitary region mass is a rare occurrence.
- The patient died 14 months post-treatment from unrelated causes.
Implications:
- This case underscores the diagnostic and therapeutic challenges posed by adult AT/RT.
- The rarity of adult AT/RT complicates the establishment of standardized treatment protocols.
- Further research into adult AT/RT is needed to improve clinical management and outcomes.
Abstract:
Atypical teratoid/rhabdoid tumors (AT/RTs) are embryological tumors of the central nervous system (CNS). They are typically found in children, with rare presentations in adults. We describe the presentation of an AT/RT in the pituitary region of a 37-year-old female. The patient presented with a two-week history of intractable cephalgia with sudden onset of monocular diplopia and left-sided cranial nerve VI palsy. The patient underwent transsphenoidal resection of their mass, which revealed the diagnosis. She then underwent systemic therapy with chemotherapy as well as radiation. She ultimately died 14 months after treatment completion due to unrelated events. The case highlights the rarity of AT/RT in adults, emphasizing the challenge of establishing standardized treatment protocols due to its rarity in adult presentations.
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