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Hypothalamic hamartoma secreting corticotropin-releasing hormone. Case report. 2004

Jean-Marc Voyadzis, and Ines Guttman-Bauman, and Mariarita Santi, and Philip Cogen
Department of Neurosurgery, Georgetown University Medical Center, Washington DC 20007, USA. voyadzij@georgetown.edu

The authors describe a unique case of a 2-year-old boy with a hypothalamic hamartoma secreting corticotropin-releasing hormone (CRH). The patient presented with a history of behavioral disturbances progressing over 12 months. His neurological status was intact. Magnetic resonance imaging demonstrated a 1.8 x 1.6 x 1.2-cm isointense, nonenhancing hypothalamic lesion. Endocrinological workup revealed elevated serum CRH and adrenocorticotropic hormone levels, nonsuppression with low-dose dexamethasone, and partial suppression with high-dose dexamethasone. He underwent tumor resection via a right frontotemporal craniotomy. Pathological examination of the tissue confirmed a hypothalamic hamartoma with CRH immunostaining. Postoperatively, his hormone levels normalized and his behavioral disturbances abated. The radiographic and clinical characteristics of hypothalamic hamartomas are reviewed and therapeutic considerations discussed.

UI MeSH Term Description Entries
D007027 Hypothalamic Diseases Neoplastic, inflammatory, infectious, and other diseases of the hypothalamus. Clinical manifestations include appetite disorders; AUTONOMIC NERVOUS SYSTEM DISEASES; SLEEP DISORDERS; behavioral symptoms related to dysfunction of the LIMBIC SYSTEM; and neuroendocrine disorders. Froehlich's Syndrome,Hypothalamic-Neurohypophyseal Disorders,Pituitary Diencephalic Syndrome,Hypothalamic Dysfunction Syndromes,Hypothalamic Dysinhibition Syndrome,Hypothalamic Overactivity Syndrome,Hypothalamic Pseudopuberty,Hypothalamic-Adenohypophyseal Disorders,Diencephalic Syndrome, Pituitary,Diencephalic Syndromes, Pituitary,Disease, Hypothalamic,Diseases, Hypothalamic,Disorder, Hypothalamic-Adenohypophyseal,Disorder, Hypothalamic-Neurohypophyseal,Disorders, Hypothalamic-Adenohypophyseal,Disorders, Hypothalamic-Neurohypophyseal,Dysfunction Syndrome, Hypothalamic,Dysfunction Syndromes, Hypothalamic,Dysinhibition Syndrome, Hypothalamic,Dysinhibition Syndromes, Hypothalamic,Froehlich Syndrome,Froehlichs Syndrome,Hypothalamic Adenohypophyseal Disorders,Hypothalamic Disease,Hypothalamic Dysfunction Syndrome,Hypothalamic Dysinhibition Syndromes,Hypothalamic Neurohypophyseal Disorders,Hypothalamic Overactivity Syndromes,Hypothalamic Pseudopuberties,Hypothalamic-Adenohypophyseal Disorder,Hypothalamic-Neurohypophyseal Disorder,Overactivity Syndrome, Hypothalamic,Overactivity Syndromes, Hypothalamic,Pituitary Diencephalic Syndromes,Pseudopuberties, Hypothalamic,Pseudopuberty, Hypothalamic,Syndrome, Froehlich's,Syndrome, Hypothalamic Dysfunction,Syndrome, Hypothalamic Dysinhibition,Syndrome, Hypothalamic Overactivity,Syndromes, Hypothalamic Dysfunction,Syndromes, Hypothalamic Dysinhibition,Syndromes, Hypothalamic Overactivity,Syndromes, Pituitary Diencephalic
D007031 Hypothalamus Ventral part of the DIENCEPHALON extending from the region of the OPTIC CHIASM to the caudal border of the MAMMILLARY BODIES and forming the inferior and lateral walls of the THIRD VENTRICLE. Lamina Terminalis,Preoptico-Hypothalamic Area,Area, Preoptico-Hypothalamic,Areas, Preoptico-Hypothalamic,Preoptico Hypothalamic Area,Preoptico-Hypothalamic Areas
D008279 Magnetic Resonance Imaging Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques. Chemical Shift Imaging,MR Tomography,MRI Scans,MRI, Functional,Magnetic Resonance Image,Magnetic Resonance Imaging, Functional,Magnetization Transfer Contrast Imaging,NMR Imaging,NMR Tomography,Tomography, NMR,Tomography, Proton Spin,fMRI,Functional Magnetic Resonance Imaging,Imaging, Chemical Shift,Proton Spin Tomography,Spin Echo Imaging,Steady-State Free Precession MRI,Tomography, MR,Zeugmatography,Chemical Shift Imagings,Echo Imaging, Spin,Echo Imagings, Spin,Functional MRI,Functional MRIs,Image, Magnetic Resonance,Imaging, Magnetic Resonance,Imaging, NMR,Imaging, Spin Echo,Imagings, Chemical Shift,Imagings, Spin Echo,MRI Scan,MRIs, Functional,Magnetic Resonance Images,Resonance Image, Magnetic,Scan, MRI,Scans, MRI,Shift Imaging, Chemical,Shift Imagings, Chemical,Spin Echo Imagings,Steady State Free Precession MRI
D008297 Male Males
D002653 Child Behavior Disorders Disturbances considered to be pathological based on age and stage appropriateness, e.g., conduct disturbances and anaclitic depression. This concept does not include psychoneuroses, psychoses, or personality disorders with fixed patterns. Childhood Behavior Disorders,Behavior Disorder, Child,Behavior Disorder, Childhood,Child Behavior Disorder,Childhood Behavior Disorder,Disorder, Childhood Behavior
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D003346 Corticotropin-Releasing Hormone A peptide of about 41 amino acids that stimulates the release of ADRENOCORTICOTROPIC HORMONE. CRH is synthesized by neurons in the PARAVENTRICULAR NUCLEUS of the HYPOTHALAMUS. After being released into the pituitary portal circulation, CRH stimulates the release of ACTH from the PITUITARY GLAND. CRH can also be synthesized in other tissues, such as PLACENTA; ADRENAL MEDULLA; and TESTIS. ACTH-Releasing Hormone,CRF-41,Corticotropin-Releasing Factor,Corticotropin-Releasing Hormone-41,ACTH-Releasing Factor,CRF (ACTH),Corticoliberin,Corticotropin-Releasing Factor-41,ACTH Releasing Factor,ACTH Releasing Hormone,Corticotropin Releasing Factor,Corticotropin Releasing Factor 41,Corticotropin Releasing Hormone,Corticotropin Releasing Hormone 41
D003907 Dexamethasone An anti-inflammatory 9-fluoro-glucocorticoid. Hexadecadrol,Decaject,Decaject-L.A.,Decameth,Decaspray,Dexasone,Dexpak,Hexadrol,Maxidex,Methylfluorprednisolone,Millicorten,Oradexon,Decaject L.A.
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D006222 Hamartoma A focal malformation resembling a neoplasm, composed of an overgrowth of mature cells and tissues that normally occur in the affected area. Hamartomas

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