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Chromosomas and sub-neurothyroid tumors are rare neurogenic neuroendocrine tumors that originate from adrenal myelin or by-neural sections, respectively.
neuroblastomas are most common in the abdomen, pelvis, or chest, while adiopathic neurosurgioblastomas are most common in the head and neck (e.g. cervical aneurysms).
, especially the sensory neurotype, is usually hormone-active and secretes cerium phenolamines, similar to chromoblastoma.
up to 40% of all sub-neurothymas occur in the context of genetic syndrome, most commonly due to mutations in the sDHx gene.
from histological point of view, the sub-neurothroid tumor often manifests it as the nest-like or ertermic "zellbalen" growth pattern of the primary cell and surrounding supporting cells.
Immune histification characteristics of parasurgiothroma and chromoblastoma are cell keratin-negative, with synaptic, chromoglobulin, GATA3 and tyrosine hydroxyase-positive primary cells, S100-positive supporting cells and smaller primary cells, and in most genetic cases SDHB expression is missing.
Contrary, rare side neurothroid tumors that occur in the horsetail (CEP) or end-filament region have been shown to be hormone-silent, clinically inert, and have variable keratin expression, suggesting that these tumors may represent a single pathological entity.
we reviewed 17 CEPs in 11 male and 6 female patients, with an average age of 38 years (range 21-82) and no family history of neuroendocrine tumors.
17 tumors showed significant differentiation of nerve cells or neurocytoblastomas.
immune grouping showed that all CEPs did not show GATA3-positive or SDHB stain loss, and 17 CEPs were cytochlorin-positive.
conducted genome-wide DNA methylation analysis of 12 tumors and compared them with publicly available genome-wide DNA methylation data.
clustering showed that CEPs formed a unique overt genetic group, separated from the external bits of the spinal cord by subsurgery, chromosoma and other neuroendocrine tumors.
copy number analysis showed that the vast majority of CEPs had a double genome, while the extra-spinal secondary neuroblastomas were mostly non-wholesomes with 1q triples and 1p, 3 and 11 monosomes, which did not exist in the CAP queue.
method: 17 horsetail subneural tumors, defined as endometrial spinal spinal subsurgery tumors in the lumbar vertebrae, horsetail, endospinal or tima regions, obtained from surgical (8) and consulted (9) UCSF local IRB approved neuropathological documents.
all tumor specimens were fixed in a 10% neutral buffer of Formarin and encased in paraffin.
used a 2.0 mm one-time biopsy punch to selectively perforated tumor tissue from a fixed paraffin envelist of 12 horsetail parasiotic tumors.
then used QIAamp-DNA-FFPE tissue kits to extract genomic DNA.
250 ng genome DNA into sulphate using the EZ DNA methylated kit, as recommended by the manufacturer.
, these findings suggest that CEPs may represent a different entity.
future genomics research needs to further clarify the molecular pathogenesis of these tumors.
Ramani, B., Gupta, R., Wu, J. et al. The immunohistochemical, DNA methylation, and chromosomal copy number profile of cauda equina paraganglioma is distinct from extra-spinal paraganglioma. Acta Neuropathol (2020). MedSci Original Source: MedSci Original Copyright Notice: All text, images and audio and video materials on this website that indicate "Source: Mets Medicine" or "Source: MedSci Originals" are owned by Mets Medicine and are not authorized to be reproduced by any media, website or individual, and are authorized to be reproduced with the words "Source: Mets Medicine".
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