title: Mature salivary gland rests within sonic hedgehog-positive medulloblastoma: case report and insights into the molecular genetics and embryopathology of ectopic intracranial salivary gland analogs.
abstract: Intracranial ectopic salivary gland rests within dural-based lesions are reported very infrequently in the literature. The authors report the unique case of a 12-year-old boy with a cerebellar medulloblastoma positive for sonic hedgehog (Shh) that contained intraaxial mature ectopic salivary gland rests. The patient underwent clinical and radiological monitoring postoperatively, until he died of disseminated disease. An autopsy showed no evidence of salivary glands within disseminated lesions. The intraaxial presence of salivary gland rests and concomitant Shh positivity of the described tumor point to a disorder in differentiation as opposed to ectopic developmental foci, which are uniformly dural based in the described literature. The authors demonstrate the characteristic "papilionaceous" appearance of the salivary glands with mucicarmine stain and highlight the role of Shh signaling in explaining the intraaxial presence of seromucous gland analogs. This article reports the first intraaxial posterior fossa tumor with heterotopic salivary gland rests, and it provides molecular and embryopathological insights into the development of these lesions.
authors.affiliation: Department of Neurological Surgery, The Neurological Institute, and.
authors.forename: Berje
authors.initials: B
authors.lastname: Shammassian
authors.affiliation: Department of Neurological Surgery, The Neurological Institute, and.
authors.forename: Sunil
authors.initials: S
authors.lastname: Manjila
authors.affiliation: Department of Neurological Surgery, The Neurological Institute, and.
authors.forename: Efrem
authors.initials: E
authors.lastname: Cox
authors.affiliation: Department of Neurological Surgery, The Neurological Institute, and.
authors.forename: Kaine
authors.initials: K
authors.lastname: Onwuzulike
authors.affiliation: Department of Pathology, University of Cincinnati, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
authors.forename: Dehua
authors.initials: D
authors.lastname: Wang
authors.affiliation: Department of Pathology, University Hospitals Case Medical Center, Cleveland.
authors.forename: Mark
authors.initials: M
authors.lastname: Rodgers
authors.affiliation: Division of Pediatric Neuro-Oncology, Department of Pediatrics, Rainbow Babies and Children's Hospital, University Hospitals Case Medical Center, Cleveland; and.
authors.forename: Duncan
authors.initials: D
authors.lastname: Stearns
authors.affiliation: Department of Neurological Surgery, The Neurological Institute, and.
authors.forename: Warren R
authors.initials: WR
authors.lastname: Selman
mesh_terms: D002528:Cerebellar Neoplasms / Q000000981:diagnostic imaging* / Q000235:genetics / Q000601:surgery; D002648:Child; D002828:Choristoma / Q000000981:diagnostic imaging* / Q000235:genetics / Q000601:surgery; D017809:Fatal Outcome; D053823:Hedgehog Proteins / Q000235:genetics; D006801:Humans; D008297:Male; D008527:Medulloblastoma / Q000000981:diagnostic imaging* / Q000235:genetics / Q000601:surgery; D012469:Salivary Glands
chemical_list: D053823:Hedgehog Proteins
keywords: CN = cranial nerve; HSGT = heterotopic salivary gland tissue; Shh = sonic hedgehog; differentiation; dissemination; ectopic salivary gland; medulloblastoma; oncology; salivary gland rests; sonic hedgehog