Pineal region tumors: Clinical symptoms and syndromes.
The present paper investigates the clinical picture and the different clinical signs that reveal pineal region tumors or appear during the course of the follow-up. Biological malignancy and tumor extension determine the semiology and its setting up mode. Typical endocrine signs, dominated by abnormal puberty development, are frequently a part of the clinical scene. Bifocal or ectopic localization in the hypothalamic-pituitary region is accompanied by other endocrine signs such as ante- or post-pituitary insufficiencies which occur several months or even years after the first neurological signs appear. Due to a mass syndrome and obstructive hydrocephalus, intracranial hypertension signs are frequent but unspecific. A careful ophthalmologic examination is essential to search upward gaze paralysis and other signs of the Parinaud's tetrad or pentad. Midbrain dysfunction, including extrinsic aqueduct stenosis, are also prevalent. Except for abnormal pubertal signs, hyper-melatoninemia (secretory tumors) or a-hypo-melatoninemia (tumors destructing pineal) generally remains dormant. Some patients present sleep problems such as narcolepsy or sleepiness during the daytime as well as behavioral problems. This suggests a hypothalamic extension rather than a true consequence of melatonin secretion anomalies. Similarly, some patients may present signs of a "pinealectomized" syndrome, including (cluster) headaches, tiredness, eventually responsive to melatonin.
Service de neurochirurgie pédiatrique, hôpital neurologique, groupe hospitalier Est, 59, boulevard Pinel, 69500 Bron, France; Service de neuropédiatrie, hôpital femme-mère-enfant, groupe hospitalier Est, 59, boulevard Pinel, 69500 Bron, France. Electronic address: christophe.rousselle@chu-lyon.fr.
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Rousselle
Service de neuropédiatrie, hôpital femme-mère-enfant, groupe hospitalier Est, 59, boulevard Pinel, 69500 Bron, France.
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des Portes
Service de neurochirurgie pédiatrique, hôpital neurologique, groupe hospitalier Est, 59, boulevard Pinel, 69500 Bron, France; Service d'endocrinologie pédiatrique, hôpital femme-mère-enfant, groupe hospitalier Est, 59, boulevard Pinel, 69500 Bron, France.
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Berlier
Service de neurochirurgie pédiatrique, hôpital neurologique, groupe hospitalier Est, 59, boulevard Pinel, 69500 Bron, France.
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Mottolese
D001932:Brain Neoplasms / Q000175:diagnosis / Q000473:pathology*; D005260:Female; D006801:Humans; D006849:Hydrocephalus / Q000150:complications / Q000473:pathology*; D019586:Intracranial Hypertension / Q000209:etiology / Q000473:pathology*; D008297:Male; D010870:Pineal Gland / Q000473:pathology*; D010871:Pinealoma / Q000378:metabolism*; D012890:Sleep / Q000502:physiology; D013577:Syndrome
Adultes; Clinical signs; Dysfonction endocrinienne; Endocrine dysfunction; Enfants; Hypertension intracrânienne; Intracranial hypertension; Melatonin; Mélatonine; Narcolepsie secondaire; Narcolepsy; Ophthalmological signs; Parinaud's syndrome; Pineal region tumors; Signes cliniques; Signes ophtalmologiques; Syndrome de Parinaud; Tumeurs de la région pinéale