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Adult Primary Central Nervous System Atypical Teratoid/Rhabdoid Tumor Metastasizing to the Cervical Lymph Node
Nirvana Thangjam1, Biswajit Dey1, Vandana Raphael1
1Pathology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences (NEIGRIHMS), Shillong, IND.
Abstract:
Atypical teratoid/rhabdoid tumors (AT/RTs) of the central nervous system (CNS) are rare and aggressive, typically occurring in early childhood or infancy, with adult cases being extremely rare. These tumors are associated with the inactivation of the integrase interactor 1 (INI1) gene. The prognosis is poor, worsening significantly if metastasis is detected at diagnosis. While CNS tumors rarely metastasize to cervical lymph nodes, recent findings have shown that such dissemination is possible, challenging the previously held belief that CNS malignancies do not spread via the lymphatic system. Awareness of this potential pathway is crucial for early diagnosis and avoiding unnecessary treatments. We present a case of a young adult male patient with a primary CNS AT/RT who had presented with cervical lymph node metastasis.
Insights
Atypical teratoid/rhabdoid tumors (AT/RTs) are aggressive CNS cancers. This case highlights rare adult AT/RT with cervical lymph node metastasis, emphasizing the need to consider lymphatic spread in diagnosis.
Area of Science:
- Neuro-oncology
- Pathology
- Medical Oncology
Background:
- Atypical teratoid/rhabdoid tumors (AT/RTs) are rare, aggressive central nervous system (CNS) malignancies.
- AT/RTs predominantly affect infants and young children, with adult cases being exceptionally uncommon.
- These tumors are characterized by the inactivation of the integrase interactor 1 (INI1) gene, leading to a poor prognosis.
Observation:
- This report details an extremely rare case of a young adult male diagnosed with a primary CNS AT/RT.
- The patient presented with metastasis to the cervical lymph nodes, a highly unusual finding for this type of malignancy.
- This presentation challenges the established understanding of CNS tumor dissemination pathways.
Findings:
- The case confirms that central nervous system AT/RTs can, in rare instances, metastasize to cervical lymph nodes.
- This finding underscores the potential for lymphatic spread in CNS malignancies, contrary to previous assumptions.
- The presence of metastasis significantly worsens the prognosis for patients with AT/RT.
Implications:
- Increased awareness of potential lymphatic metastasis in CNS AT/RT is crucial for accurate diagnosis and staging.
- Early detection of metastasis can guide treatment strategies and potentially improve patient outcomes.
- This case necessitates a re-evaluation of diagnostic and therapeutic approaches for adult CNS AT/RT, considering the possibility of extranodal spread.
