title: Resolution of precocious puberty following resection of fourth ventricular medulloblastoma: case report.
abstract: Medulloblastoma is a malignant embryonal tumor that arises in the cerebellum and invades the fourth ventricle, often resulting in obstructive hydrocephalus. Patients typically present with symptoms related to increased intracranial pressure and cerebellar dysfunction. The authors report a rare case of classic medulloblastoma with central precocious puberty (CPP) as its only presenting symptom. A 7-year-old boy with no prior history of medulloblastoma presented with Tanner Stage IV testicular enlargement and a 4-month history of acne and pubic hair. Laboratory tests of blood samples demonstrated highly elevated luteinizing hormone (LH), follicle-stimulating hormone (FSH), and testosterone. Admission MRI of the brain revealed a mass in the posterior fossa, which bordered and compressed the fourth ventricle. The patient also exhibited mild lateral and third ventriculomegaly. Surgical options were discussed with the neurosurgical department. A suboccipital craniotomy and C-1 laminectomy were performed. A large mass was seen arising from the inferior surface of the vermis, and lying within the fourth ventricle. Gross-total microsurgical resection of the mass was performed. Histopathological investigation characterized the tumor as classic medulloblastoma. Follow-up laboratory tests of blood samples demonstrated a reduction of LH, FSH, and testosterone back to prepubertal levels. The patient then began radiation and chemotherapy. This report demonstrates that mild obstructive hydrocephalus due to a posterior fossa tumor may present with unexpected symptoms, such as CPP. To the authors' knowledge, precocious puberty has not yet been associated with medulloblastoma, although it has been found with other posterior fossa tumors. Extensive imaging of the CNS for patients presenting with CPP is recommended.
authors.affiliation: School of Medicine, Saint Louis University;
authors.forename: Renata G
authors.initials: RG
authors.lastname: Medina
authors.affiliation: Department of Pediatrics, Division of Endocrinology;
authors.forename: David P
authors.initials: DP
authors.lastname: Dempsher
authors.affiliation: Department of Pediatrics, Division of Hematology-Oncology, Washington University in St. Louis School of Medicine, Saint Louis, Missouri.
authors.forename: Karen M
authors.initials: KM
authors.lastname: Gauvain
authors.affiliation: Department of Neurology and Psychiatry, Division of Child Neurology; and.
authors.forename: Thomas J
authors.initials: TJ
authors.lastname: Geller
authors.affiliation: Department of Neurosurgery, Division of Pediatric Neurosurgery, Saint Louis University School of Medicine; and.
authors.forename: Samer K
authors.initials: SK
authors.lastname: Elbabaa
mesh_terms: D015415:Biomarkers / Q000097:blood; D002551:Cerebral Ventricle Neoplasms / Q000097:blood / Q000150:complications* / Q000601:surgery*; D002552:Cerebral Ventricles / Q000473:pathology; D002648:Child; D003399:Craniotomy / Q000379:methods; D020546:Fourth Ventricle; D006801:Humans; D007986:Luteinizing Hormone / Q000097:blood; D008279:Magnetic Resonance Imaging; D008297:Male; D008527:Medulloblastoma / Q000097:blood / Q000150:complications* / Q000601:surgery*; D009777:Occipital Bone; D011629:Puberty, Precocious / Q000097:blood / Q000209:etiology*; D013739:Testosterone / Q000097:blood; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; D013739:Testosterone; D007986:Luteinizing Hormone
keywords: CPP = central precocious puberty; FSH = follicle-stimulating hormone; GnRH = gonadotropin-releasing hormone; HPA = hypothalamic-pituitary axis; LH = luteinizing hormone; MMC = myelomeningocele; TS = Tanner Stage; hydrocephalus; medulloblastoma; oncology; pediatric neurosurgery; posterior fossa tumor; precocious puberty