Simultaneous occurrence of melanotic neuroectodermal tumor and brain heterotopia in the oropharynx

Abstract

A case is described of heterotopic brain tissue with simultaneous occurrence of melanotic neuroectodermal tumor in the oropharynx of a 6‐week‐old infant. The melanotic neuroectodermal tumor was embedded within the heterotopic glial tissue. This coexistence leads us to speculate that a defect causing a pinching‐off of both neural crest cap and medullary epithelium of neural tube might have taken place at, or before, the 25–30 somite stage. The displaced embryonic structures subsequently differentiated into glial tissue, choroid plexus, and melanotic neuroectodermal tumor. This observation may be interpreted as further support in favor of a neural crest origin of juvenile melanotic neuroectodermal tumor.

Nasopharyngeal glioma: A unique presentation in infants

“…Absence of invasive pattern and lack of mitosis rules out the possibility of any glial neoplasm. Though Lee et al (16) described a case of 6 weeks old infant in which oropharyngeal melanotic ectodermal tumor coexisted with heterotopic brain tissue.…”

Heterotopic Brain in the Pterygopalatine Fossa

“…The neural elements are typically embedded in fibrous mesenchymal tissue and may exhibit calcification. Rare instances of neoplasm occurring within excised lesions have been reported, including oligodendroglioma (19) and neuroectodermal tumor (20). The absence of many components-particularly neurons, choroid plexus, and ependyma-from nasal heterotopic Iesions has led some authors to view them as separate entities (19).…”

Neurogenic Tumors of Soft Tissue

“…In addition, the possibility of a true encephalocele or meningoencephalocele should be investigated by thorough radiographic and intraoperative evaluation for possible connections between the neuroglial lesion and the central nervous system [58,98,101,110,120]. Malignant degeneration is exceptional [111,114,121].…”