scholarly journals Malignant Cerebellar Neoplasm

2020 ◽  
Author(s):  
Keyword(s):  
2012 ◽  
Vol 30 (3) ◽  
pp. 180-184 ◽  
Author(s):  
Kenichiro Matsuda ◽  
Kaori Sakurada ◽  
Shinya Sato ◽  
Yoichi Nakazato ◽  
Takamasa Kayama

2001 ◽  
Vol 95 (5) ◽  
pp. 871-878 ◽  
Author(s):  
W. Jeffrey Elias ◽  
M. Beatriz S. Lopes ◽  
Wendy L. Golden ◽  
John A. Jane ◽  
Federico Gonzalez-Fernandez

✓ Results of recent studies have led investigators to suggest that the retinoblastoma tumor-suppressor (rb) gene plays an underappreciated role in the genesis of brain tumors. Such tumors cause significant rates of mortality in children suffering from hereditary retinoblastoma. It has been assumed that the pineal gland, which is ontogenetically related to the retina, accounts for the intracranial origin of these trilateral neoplasms. To address this issue, the authors describe an unusual trilateral retinoblastoma variant. The authors provide a detailed clinicopathological correlation by describing the case of a child with bilateral retinoblastoma who died of a medulloblastoma. The intraocular and intracranial neoplasms were characterized by performing detailed imaging, histopathological, and postmortem studies. Karyotype analysis and fluorescence in situ hybridization were used to define the chromosomal defect carried by the patient and members of her family. An insertion of the q12.3q21.3 segment of chromosome 13 into chromosome 18 at band q23 was identified in members of the patient's family. This translocation was unbalanced in the proband. The intraocular and cerebellar neoplasms were found to be separate primary neoplasms. Furthermore, the pineal gland was normal and the cerebellar neoplasm arose within the vermis as a medulloblastoma. Finally, the two neoplasms had different and characteristically identifiable cytolological and immunohistochemical profiles. The findings of the present study, taken together with those of recent molecular and transgenic studies, support the emerging concept that rb inactivation is not restricted to central nervous system regions of photoreceptor lineage and that inactivation of this tumor suppressor pathway may be relevant to the determination of etiological factors leading to medulloblastoma in humans.


Author(s):  
Jéssika de Oliveira Nascimento ◽  
Lara Caroline Anastacio Haro ◽  
Rafael da Silva Sá ◽  
Rebeca Espelho Storch

AbstractSeveral factors trigger the development of genetic mutations that are responsible for causing a neoplasm. Medulloblastoma is a malignant and invasive cerebellar neoplasm, that affects children and young adults. Mucinous carcinoma is a special type of breast cancer. Being a special atypical subtype of invasive carcinoma, it most frequently affects women of advanced age and represents 1 to 7% of all breast cancers. The reported case aims to show the rarity of the occurrence of desmoplastic medulloblastoma and mammary mucinous carcinoma in a young patient in a short period of time, in different sites, without direct anatomical attachment and without occurrence of metastasis. Initially, this patient had a desmoplastic medulloblastoma and was treated with lumpectomy and radiotherapy. After 13 months, the patient was diagnosed with a mucinous breast carcinoma, underwent mastectomy, adjuvant chemotherapy and is currently undergoing endocrinotherapy. We conclude, based on the metachronous characteristic of the neoplasia and clinical characteristics, that the patient is likely to have Li-Fraumeni syndrome, an autosomal dominant disease with mutation of the TP53 gene, which is the the main involved. Because the patient does not present all the characteristics of the phenotype of the syndrome, she can thus be classified as having Li-Fraumeni variant or Li-Fraumeni-like syndrome.


1983 ◽  
Vol 59 (2) ◽  
pp. 337-343 ◽  
Author(s):  
Samruay Shuangshoti ◽  
Supat O'Charoen

✓ A 3½-year-old boy had a neoplasm of mixed mesenchymal and neuroepithelial origin in the cerebellar vermis. The tumor was composed of rhabdomyosarcoma and medulloblastoma. There was additional differentiation within the neuroepithelium, including neoplastic neurons, neuroblasts, astroglia, oligodendroglia, ependymal cells as well as foci of glioblastomatous transformation. It is suggested that this combined tumor arose from the neural crest or combined neural crest and neural tube. The rhabdomyosarcoma could have arisen from the mesenchymal portion of the neural crest, and the medulloblastoma from the neuroectodermal part of the neural crest or from the neural tube itself. The two tumor types formed a neoplasm of mixed mesenchymal and neuroepithelial origin.


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