Extradural Dorsal Spinal Paraganglioma: A Rare Case Report and Literature Review

Paragangliomas are neuroendocrine tumors originating from specialized cells of neural crest derivative. They are mostly found in the carotid and jugulotympanic region. Paragangliomas are extremely rare in the spine, and in literature, most cases are described as intradural extramedullary lesion in the cauda equine or filum terminale region. Extradural dorsal spine paragangliomas are even rarer, and till date, only 11 cases are reported in available literature. We describe a case of an adult woman who presented with mid dorsal region pain and sensory-motor spastic paraplegia. Imaging features were suggestive of D5 Pott's spine with epidural granulation tissue. Perioperatively, the tumor was moderately vascular and complete excision done. On HPE and IHC, diagnosis of paraganglioma was made. There was no evidence of clinical recurrence of the lesion at 6 months follow-up.

SoxE Proteins Are Differentially Required in Mouse Adrenal Gland Development

Sry-box (Sox)8, Sox9, and Sox10 are all strongly expressed in the neural crest. Here, we studied the influence of these closely related transcription factors on the developing adrenal medulla as one prominent neural crest derivative. Whereas Sox9 was not expressed, both Sox8 and Sox10 occurred widely in neural crest cells migrating to the adrenal gland and in the gland itself, and they were down-regulated in cells expressing catecholaminergic traits. Sox10-deficient mice lacked an adrenal medulla. The adrenal anlage was never colonized by neural crest cells, which failed to specify properly at the dorsal aorta and died apoptotically during migration. Furthermore, mutant neural crest cells did not express Sox8. Strong adrenal phenotypes were also observed when the Sox10 dimerization domain was inactivated or when a transactivation domain in the central portion was deleted. Sox8 in contrast had only minimal influence on adrenal gland development. Phenotypic consequences became only visible in Sox8-deficient mice upon additional deletion of one Sox10 allele. Replacement of Sox10 by Sox8, however, led to significant rescue of the adrenal medulla, indicating that functional differences between the two related Sox proteins contribute less to the different adrenal phenotypes of the null mutants than dependence of Sox8 expression on Sox10.

Delta signaling mediates segregation of neural crest and spinal sensory neurons from zebrafish lateral neural plate

We examined the role of Delta signaling in specification of two derivatives in zebrafish neural plate: Rohon-Beard spinal sensory neurons and neural crest. deltaA-expressing Rohon-Beard neurons are intermingled with premigratory neural crest cells in the trunk lateral neural plate. Embryos homozygous for a point mutation in deltaA, or with experimentally reduced delta signalling, have supernumerary Rohon-Beard neurons, reduced trunk-level expression of neural crest markers and lack trunk neural crest derivatives. Fin mesenchyme, a putative trunk neural crest derivative, is present in deltaA mutants, suggesting it segregates from other neural crest derivatives as early as the neural plate stage. Cranial neural crest derivatives are also present in deltaA mutants, revealing a genetic difference in regulation of trunk and cranial neural crest development.