TY - JOUR
T1 - Diabetes insipidus, panhypopituitarism, and severe menta l stat us deterioration in a patient with chordoid glioma
T2 - Case report and literat ure review
AU - Dziurzynski, Kristine
AU - Delashaw, Johnny B.
AU - Gultekin, S. Humayun
AU - Yedinak, Chris G.
AU - Fleseriu, Maria
PY - 2009/4
Y1 - 2009/4
N2 - Objective: To describe a rare progressive case of chordoid glioma clinically masquerading as idiopathic diabetes insipidus (DI). Methods: We describe the clinical, radiographic, and laboratory findings of the study patient and briefly review the relevant literature. Results: A 41-year-old woman was referred to our center for evaluation of worsening mental status changes, a newly diagnosed suprasellar mass, and possible endocrine dysfunction. Three years earlier, a physician at another institution diagnosed idiopathic DI and prescribed desmopressin. At that time, laboratory workup and magnetic resonance imaging (MRI) revealed no brain lesions or other hormonal irregularities. Slow, progressive symptomatology in the following 3 years included mental status changes, nonhealing skin lesions, recurrent infections, temperature dysregulation, and midsection weight gain. She became withdrawn and emotionally labile and developed a flat affect, short-term memory loss, poor concentration, and sleep disturbance. MRI revealed a 2.2 × 2.1 × 1.9-cm suprasellar region lesion. Biopsy samples from the third ventricular lesion revealed a circumscribed glial tumor. Chordoid glioma is a rare tumor, and the 50 previously reported cases have been located in the suprasellar region. This is the third reported case of a chordoid glioma positive for neurofilament protein, which brings into question the hypothesis of a single phenotype for glial tumors. Tumors in this region frequently result in endocrine dysfunction that prompts patients to seek medical attention. Conclusions: There is no formally recognized treatment protocol for chordoid glioma, and postoperative mortality and morbidity is high. Our report emphasizes the necessity of close follow-up of patients after a diagnosis of idiopathic DI. Early detection of any evolving occult hypothalamic-pituitary stalk lesion may improve outcome.
AB - Objective: To describe a rare progressive case of chordoid glioma clinically masquerading as idiopathic diabetes insipidus (DI). Methods: We describe the clinical, radiographic, and laboratory findings of the study patient and briefly review the relevant literature. Results: A 41-year-old woman was referred to our center for evaluation of worsening mental status changes, a newly diagnosed suprasellar mass, and possible endocrine dysfunction. Three years earlier, a physician at another institution diagnosed idiopathic DI and prescribed desmopressin. At that time, laboratory workup and magnetic resonance imaging (MRI) revealed no brain lesions or other hormonal irregularities. Slow, progressive symptomatology in the following 3 years included mental status changes, nonhealing skin lesions, recurrent infections, temperature dysregulation, and midsection weight gain. She became withdrawn and emotionally labile and developed a flat affect, short-term memory loss, poor concentration, and sleep disturbance. MRI revealed a 2.2 × 2.1 × 1.9-cm suprasellar region lesion. Biopsy samples from the third ventricular lesion revealed a circumscribed glial tumor. Chordoid glioma is a rare tumor, and the 50 previously reported cases have been located in the suprasellar region. This is the third reported case of a chordoid glioma positive for neurofilament protein, which brings into question the hypothesis of a single phenotype for glial tumors. Tumors in this region frequently result in endocrine dysfunction that prompts patients to seek medical attention. Conclusions: There is no formally recognized treatment protocol for chordoid glioma, and postoperative mortality and morbidity is high. Our report emphasizes the necessity of close follow-up of patients after a diagnosis of idiopathic DI. Early detection of any evolving occult hypothalamic-pituitary stalk lesion may improve outcome.
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U2 - 10.4158/EP.15.3.240
DO - 10.4158/EP.15.3.240
M3 - Article
C2 - 19364693
AN - SCOPUS:67649224769
SN - 1530-891X
VL - 15
SP - 240
EP - 245
JO - Endocrine Practice
JF - Endocrine Practice
IS - 3
ER -