Primary intracranial malignant ectomesenchymoma in an adult: Report of a rare case and review of the literature.
Swati MahajanVaishali SuriMehar Chand SharmaShweta KediaHardik SardanaTripti NakraPublished in: Neuropathology : official journal of the Japanese Society of Neuropathology (2019)
Malignant ectomesenchymoma (MEM) is an exceedingly rare rapidly progressing tumor of soft tissues of the central nervous system, believed to be derived from neural crest cells. The majority of cases have been observed in young children or adolescents. So far only 11 patients with intracranial manifestations (with confirmed clinicopathological data) have been documented. We report the first case of adult intracranial MEM in a 54-year-old man who presented with a 4 months history of headache and weakness of right side of the body. Magnetic resonance imaging showed a homogenously enhanced dural-based lesion in the left fronto-temporo-parietal lobe with significant perilesional edema and mass effect. No metastatic disease was identified and the lesion was grossly resected. Histopathological and immunohistochemical examination revealed mature and immature neurons and bizarre astrocytes admixed with a mesenchymal spindle cell (rhabdomyoblastic) component. Specific risk factors that contribute toward the development of MEM are unknown. Due to the scarcity of reported cases the role of adjuvant therapy is unclear.
Keyphrases
- rare case
- magnetic resonance imaging
- risk factors
- optic nerve
- single cell
- induced apoptosis
- young adults
- squamous cell carcinoma
- small cell lung cancer
- lymph node
- stem cells
- cell therapy
- physical activity
- spinal cord
- cell cycle arrest
- computed tomography
- bone marrow
- machine learning
- cell death
- oxidative stress
- big data
- cerebrospinal fluid
- mesenchymal stem cells
- endoplasmic reticulum stress
- spinal cord injury
- contrast enhanced
- signaling pathway
- diffusion weighted imaging
- myasthenia gravis