Rare extracranial localization of primary intracranial neoplasm

Abstract

Meningioma, craniopharyngeoma and glioma are mainly intracranial lesions. Nevertheless, in rare cases these entities may occur solely as extracranial lesions that may present as intranasal/sinusoidal masses, with headaches and nasal obstruction. We present three cases of common intracranial tumors, with purely extracranial extension. The three described cases demonstrate, that preoperative MRI and CT imaging is important for differential diagnosis to exclude intracranial connections of the tumors. A definitive diagnosis requires specialized immunohistochemical examinations. In all cases of intranasal or pharyngeal neoplasm the diagnosis of meningioma, craniopharyngeoma and glioma should be considered as differential diagnosis to optimize the surgical procedure.

Multicentric paraspinal neuroglial heterotopia with Müllerian and renal agenesis: a variant of Mayer-Rokitansky-Küster-Hauser syndrome?

“…It is often obvious at birth but may remain asymptomatic until late childhood or even adulthood. Complete surgical excision is curative in most cases [ 1 , 3 , 10 , 11 ]. On the other hand, paraneuraxial neuroglial heterotopia is rare and may involve the paracranial or paraspinal spaces, such as the occipital bone, retroperitoneum, and deep neck.…”

Retroperitoneal neuroglial heterotopia: a case report and literature review

“…While the preoperative serum NSE level was elevated in our case, this phenomenon was not observed in other cases (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15). We explored the potential correlation between this elevation in NSE and the size of the mass, considering our case is the largest documented to date.…”

“…The etiopathogenesis of retroperitoneal neuroglial heterotopia remains unclear. Several theories have been proposed, including (1) encephalocele and myelomeningocele occurring through a primary osseous defect followed by partial or complete secondary closure, resulting in sequestration of the herniated lesion ( 8 – 10 ); (2) retention of vestigial neural crest ( 3 ); (3) aberrant migration and differentiation of neuroectodermal remnants ( 14 ); and (4) hamartoma ( 6 ). However, none of these theories alone provide a satisfactory explanation for the pathogenesis.…”

“…Immunohistochemical staining for GFAP, S100, and NSE corroborated the presence of glial tissue in the current case. Additional markers such as synaptophysin ( 2 , 5 , 6 , 10 , 12 , 14 ), NeuN ( 7 ), vimentin ( 5 , 9 , 14 ), chromogranin A (CGA) ( 2 ), CD56 ( 11 ), and neurofilament (NF) ( 5 – 7 ) contribute to confirming the diagnosis. It is imperative to further validate serum NSE or alternative biomarkers as diagnostic and prognostic tools for neuroglial heterotopia, necessitating a larger sample size to bolster their significance.…”