Glial choristoma of the tongue: report of a case and review of the literature

Abstract

Glial choristoma of the tongue is an extremely rare developmental malformation. The authors report a case of a 5-month-old male baby with a congenital glial choristoma located on the posterior part of midline of the left dorsal tongue. Histological examination of the resected specimen revealed a poorly demarcated submucosal mass containing neuroglial tissue, scattered neuron, choroids plexus and ependyma. In addition to neuroglial tissue, a sheet of leptomeningeal tissue was observed more rarely in the case. The clinical and pathological characteristics of previous cases and their probable embryogenesis were also reviewed.

Glial choristoma of the tongue. Clinicopathological analysis of a case and pathogenetic insights

“…In some cases, other brain tissue elements, in including neurons, ependymal-like epithelium, and choroid plexus-like structures were also detected in variable combination and proportion [5,6,7,8,11,13,15,17,19,20,21,25,26]. In one case [25], meningeal-like tissue was detected as well. Thus, the case reported here fulfils the clinicopathological criteria for the diagnosis of glial choristoma of the tongue.…”

“…Even though different terms have been used to define the lesion, including glioma [3,4,16], glial/gliomatous teratoma [5,10], heterotopic brain, or neural/glial/neuroglial tissue [6,7,8,9,11,24,28], the term glial choristoma is more appropriate because the lesion is a developmental anomaly and not a neoplasm and produces a tumour-like tissue mass [1,2]. Surgical excision with free margins is curative and, because the lesion may be only suspected at clinical examination, histology and immunohistochemistry are mandatory for the definitive diagnosis [13,15,17,18,20,21,22,23,24,25,26,27,28,29,30].…”

“…The pathogenetic mechanism leading to the development of the glial choristoma in the craniofacial region is not known, and different pathogenetic theories have been suggested [1,5,11,13,16,17,18,19,21,23,25,26,27,28,30,31]. Of note, a potential role of the cells normally emanating from the cranial neural crests (CNCs), which are involved in the development of the ectomesenchymal components of the human head including the tongue and express neuroglial differentiation as a normal developmental pathway [32,33,34,35,36,37,38,39,40,41,42,43,44], has been virtually never taken into account [1,18,30,31].…”

“…Glial choristoma, which is a choristoma composed by glial cells either in association or not with other normal brain tissue elements, is more commonly restricted to the craniofacial region where the tongue is rarely involved [2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29]. Even though different terms have been used to define the lesion, including glioma [3,4,16], glial/gliomatous teratoma [5,10], heterotopic brain, or neural/glial/neuroglial tissue [6,7,8,9,11,24,28], the term glial choristoma is more appropriate because the lesion is a developmental anomaly and not a neoplasm and produces a tumour-like tissue mass [1,2].…”

Congenital Neuroglial Heterotopia in a Neonatal Harbor Seal (Phoca vitulina richardsi ) with Evidence of Recent Exposure to Polycyclic Aromatic Hydrocarbons

“…This lesion typically occurs as a firm-to-cystic, solitary, space-occupying mass and may contain cerebrospinal fluid, presumably from autogenous production by choroid plexus tissue (Hendrickson et al, 1990). In humans, congenital extracranial heterotopic neuroglial tissue occurs most commonly in the sinonasal region on midline due to anatomic proximity to the central nervous system (Aanaes et al, 2008), but has also been associated with the nasopharynx (AlAmmar et al, 2006), hard and soft palate (Al-Nafussi et al, 1990;Ide et al, 1997;Anjaneyulu and Deka, 2004), tongue (Fan et al, 2008), lip (Pasyk et al, 1988), buccal region (Aanaes et al, 2008), middle ear (Gyure et al, 2000), face and neck (Hendrickson et al, 1990), chest wall (Shepherd et al, 1987), scalp (McDermott et al, 1996), and lung (Morgan et al, 2003). All existing reports describe a single mass, rather than a cluster of soft tissue masses; however, one facial tumor exhibited fibrous adherence to the periosteum of the facial bones, similar to this harbor seal (Hendrickson et al, 1990).…”