Intracranial Myxoid Research Articles

The Exercise Intervention as Part of Rehabilitation in Intracranial Myxoid Chondrosarcoma: A Case Report

Malignant tumors after surgical trauma are frequently presented with unavoidable complications, such as physical dysfunction, decreased motor endurance, bowel disorders and cognitive impairment, and other symptoms. Due to the presence of malignant tumors, patients suffer from not only physical discomfort but also the presence of psychological and mental stress. Trauma often leads to poor emotional control and cannot cooperate with treatment. Exercise can not only change the function of the limbs but also have a positive effect on emotions. This case reports a 28-year-old male who experienced a decline in cognitive function and activities of daily living after undergoing tumor resection and radiotherapy. A year later, he was hospitalized and completed a four-week activity of daily living and exercise rehabilitation training. What’s more, his mood was managed along with the reintegration therapy. As the patient's mood improved, training coordination increased, quality of life and daily living ability gradually improved, and he recovered well during the follow-up period. Given this single case, multi-modal exercise rehabilitation can maintain or improve functional performance and QOL domains even during heavy treatments. When the patient is undergoing exercise rehabilitation, he also needs emotional management, and emotional intervention is required for the patient. Therefore, exercise and the emotional management of patients with malignancy are of great importance.

SWI/SNF deficient central nervous system neoplasms

The SWItch/Sucrose Non-Fermentable (SWI/SNF) complexes are ubiquitous ATP dependent chromatin remodeling complexes that provide epigenetic regulation of gene expressions across the genome. Different combination of SWI/SNF subunits allow tissue specific regulation of critical cellular processes. The identification of SMARCB1 inactivation in pediatric malignant rhabdoid tumors provided the first example that the SWI/SNF complex may act as a tumor suppressor. It is now estimated at least 20% of all human tumors contain mutations in the subunits of the SWI/SNF complex. This review summarizes the central nervous system tumors with alterations in the SWI/SNF complex genes. Atypical teratoid/rabdoid tumor (AT/RT) is a highly aggressive embryonal tumor genetically characterized by bi-allelic inactivation of SMARCB1, and immunohistochemically shows complete absence of nuclear expression of its protein product INI1. A small subset of AT/RT show retained INI1 expression but defects in another SWI/SNF complex gene SMARCA4. Embryonal tumors with medulloblastoma, pineoblastoma, or primitive neuroectodermal morphology but loss of INI1 expression are now classified as AT/RT. Cribriform neuroepithelial tumor (CRINET) is an intra or para-ventricular tumor that has similar SMARCB1 alterations as AT/RT but generally has a benign clinical course. Besides AT/RT and CRINET, compete loss of nuclear INI1 expression has also been reported in poorly differentiated chordoma and intracranial myxoid sarcoma within the central nervous system. Families with non-truncating SMARCB1 mutations are prone to develop schwannomatosis and a range of developmental syndromes. The schwannomas in these patients usually demonstrate a mosaic INI1 staining pattern suggestive of partial residual protein function. Finally, clear cell meningioma is a WHO grade II variant meningioma characterized by bi-allelic inactivation of the SMARCE1 gene and immunohistochemically show loss of its protein product BAF57 expression in tumor cell nuclei.

ESWR1-CREM Fusion in an Intracranial Myxoid Angiomatoid Fibrous Histiocytoma-Like Tumor: A Case Report and Literature Review

Gene fusions of EWSR1 with members of the CREB family of transcription factors (CREB1, ATF1, and CREM) have recently been described in exceptional intracranial myxoid mesenchymal tumors. Although this is a known gene fusion found in various mesenchymal tumors, EWSR1 fusion with CREM has only been observed in 3 intracranial myxoid tumors. In this paper, we present 1 such tumor with in-depth histopathological description and long-term follow-up. There is controversy regarding whether these tumors represent a novel entity or simply an intracranial localization of the myxoid variant of angiomatoid fibrous histiocytoma, a rare soft tissue tumor of the extremities. Out of 11 cases mentioned in the literature, the 3 isolated case reports by Dunham et al, Ochalski et al, and Alshareef et al are designated as angiomatoid fibrous histiocytoma, whereas the others are defined as a novel tumoral entity called intracranial myxoid mesenchymal tumor with EWSR1-CREB fusion. We believe the vast morphological and immunohistochemical spectrum of angiomatoid fibrous histiocytoma makes it difficult to dismiss this diagnosis.

Primary Intracranial Myxoid Chondrosarcoma: A case report

Objective: Intracranial extraskeletal myxoid chondrosarcoma is a rare tumor; up to date, only ten cases have been reported. Case report: We present a case report of a 65-year-old female admitted for right side hemiplegia of gradual onset and progressive course over three weeks. Magnetic resonance imaging (MRI) revealed left intra-axial parieto-occipital space occupying lesion, hypodense with ring enhancement surrounded by diffuse edema. The patient underwent subtotal resection of the mass using sonar guided craniotomy. The histopathological and immunohistochemical evaluation confirmed a diagnosis of extraskeletal myxoid chondrosarcoma. One month after initial discharge, the patient underwent radiotherapy. A follow-up examination 6 months after surgery revealed that the patient was deteriorated and died. Conclusion: intracranial extraskeletal myxoid chondrosarcoma is a rare malignant cartilaginous tumor. Pathological diagnosis is the gold standard and radical excision is the standard treatment

Primary Intracranial Myxoid Chondrosarcoma: A case report

Objective: Intracranial extraskeletal myxoid chondrosarcoma is a rare tumor; up to date, only ten cases have been reported. Case report: We present a case report of a 65-year-old female admitted for right side hemiplegia of gradual onset and progressive course over three weeks. Magnetic resonance imaging (MRI) revealed left intra-axial parieto-occipital space occupying lesion, hypodense with ring enhancement surrounded by diffuse edema. The patient underwent subtotal resection of the mass using sonar guided craniotomy. The histopathological and immunohistochemical evaluation confirmed a diagnosis of extraskeletal myxoid chondrosarcoma. One month after initial discharge, the patient underwent radiotherapy. A follow-up examination 6 months after surgery revealed that the patient was deteriorated and died. Conclusion: intracranial extraskeletal myxoid chondrosarcoma is a rare malignant cartilaginous tumor. Pathological diagnosis is the gold standard and radical excision is the standard treatment

Primary Intracranial Myxoid Chondrosarcoma

Primary Intracranial Myxoid Chondrosarcoma

Myxoid chondrosarcoma in the pineal region

The authors report a case of a myxoid chondrosarcoma of the pineal region in a 37-year-old woman who presented with an intratumoral hemorrhage. Partial removal of the tumor in an initial surgery resulted in failure to establish a definitive diagnosis. The residual tumor enlarged after a second intratumoral hemorrhage 14 months after the onset of the first symptoms, and gross-total resection of the tumor was achieved in a second surgery. Histological and immunohistochemical findings after the second surgery were consistent with a diagnosis of myxoid chondrosarcoma. Radical excision of a tumor was considered to play an important role in the management of intracranial myxoid chondrosarcoma.