AI Article Synopsis
- The study aimed to identify key clinical and imaging features of atypical teratoid rhabdoid tumor (ATRT) in both children and adults to improve diagnostic pathways.
- Researchers analyzed a group of 11 ATRT patients (10 children and 1 adult) and reviewed existing literature, focusing on distinctive clinical symptoms and imaging characteristics.
- Findings indicated that ATRT commonly occurs in children under 3 years, often presents as a mass near the brain's ventricles with cystic components, and that accurate diagnosis in adults heavily depends on neuropathological evaluation.
Article Abstract
Purpose: To assess the main imaging and clinical features in adult- and pediatric-onset atypical teratoid rhabdoid tumor (ATRT) in order to build a predefined pathway useful for the diagnosis.
Methods: We enrolled 11 ATRT patients (10 children, one adult) and we conducted a literature search on PubMed Central using the key terms "adult" or "pediatric" and "atypical teratoid/rhabdoid tumor". We collected clinical and neuroradiological data reported in previous studies and combined them with those from our case series. A three step process was built to reach diagnosis by identifying the main distinctive clinical and imaging features.
Results: Clinical evaluation: neurological symptoms were nonspecific. ATRT was more frequent in children under 3 years of age (7 out of 10 children) and infratentorial localization was reported more frequently in children under the age of 24 months. Midline/off-midline localization was influenced by the age.
Imaging Findings: Preferential location near the ventricles and liquor spaces and the presence of eccentric cysts were hallmark for ATRT; higher frequency of peripheral cysts was detected in children and in the supratentorial compartment (five out of eight patients with solid-cystic ATRT). Leptomeningeal dissemination at diagnosis was common (5 out of 10 children), while intratumoral hemorrhage, calcifications, and high cellularity were non-specific findings. Histopathological analysis: specific immunohistochemical markers were essential to confirm the diagnosis.
Conclusion: In younger children, a bulky, heterogeneous mass with eccentric cystic components and development near ventricles or cisternal spaces may be suggestive of ATRT. ATRT diagnosis is more challenging in adults and relies exclusively on neuropathological examination.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9914341 | PMC |
| http://dx.doi.org/10.3390/diagnostics13030475 | DOI Listing |
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