Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A retrospective study was undertaken of 1005 normal contrast-enhanced head computed tomographic (CT) scans in children to determine normal standards for pituitary stalk diameter, basilar artery diameter, and their ratio for age and gender. The pituitary stalk enlarges with age, and girls have larger stalks than do boys after age 8. The pituitary stalk-to-basilar artery ratio is easily estimated visually. Ratios greater than or equal to 1 are unusual in normal children. A ratio greater than or equal to 1 should prompt direct measurement of the stalk and comparison with age-matched normal values. If the stalk measurement is greater than 2 SD above the age-matched mean, it is presumably abnormal and deserves further evaluation for numerous possible pathologic conditions including histiocytosis X, pituitary adenoma, hypothalamic lesions, Rathke cleft cysts, metastases, sarcoidosis, hypothyroidism, and infection.
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PMID:Normal pituitary stalk size in children: CT study. 387 41

CT and MR imaging findings of 57 sphenoidal masses were retrospectively reviewed to assess the possibility of differential diagnosis between them. Various kinds of masses such as pituitary adenoma, epipharyngeal cancer, mucocele, chordoma, chondroma, chondrosarcoma, distant metastasis, multiple myeloma, fibrous dysplasia, craniopharyngioma, hemangiopericytoma, giant cell tumor, primary sphenoidal cancer, malignant melanoma, leukemia, histiocytosis X, and giant cell tumor were included in this series. CT scanning was performed in all cases, while MR images were obtained in 48 cases using a spin-echo pulse sequence. The relative density of the masses, bony changes and calcification were evaluated on CT, and on MR images, signal intensity of the masses relative to the normal gray matter, contrast enhancement and extension/contour were evaluated. Although no single feature appeared to be specific to the masses, detection of calcification on CT, identification of the normal pituitary gland as deformed or displaced on T1-weighted images, signal intensity on T2-weighted images, and extension of the masses seemed to be useful and should be examined in terms of their ability to assist in differential diagnosis. Finally, accommodative classification of sphenoidal masses primarily based on presumed origin or mode of extension was attempted.
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PMID:[CT and MR imaging findings of sphenoidal masses]. 807 65

The most common indication for the use of radiation therapy in the treatment of benign central nervous system disease is for the treatment of benign brain tumors, such as meningioma, pituitary adenoma, acoustic neuroma, arteriovenous malformation, and craniopharyngioma. Other less common benign intracranial tumors treated with radiation include chordoma, pilocytic astrocytoma, pineocytoma, choroid-plexus papilloma, hemangioblastoma, and temporal bone chemodectomas. Benign conditions, such as histiocytosis X, trigeminal neuralgia, and epilepsy, are also amenable to radiation treatment. There have also been reports of radiosurgery being used for the treatment of movement disorders and psychiatric disturbances, such as obsessive-compulsive and anxiety disorders. For benign brain tumors, radiation therapy as either primary or adjuvant therapy plays an integral role in improving local control. In the treatment of trigeminal neuralgia, epilepsy, tremor, and some psychiatric disturbances, radiosurgery may help ameliorate or eliminate some symptoms. Patients with benign central nervous system disease are expected to live a long time. As such, treatment should be highly conformal and based on three-dimensional planning using magnetic resonance imaging, computed tomography, or both. It is critical that damage to normal brain be minimized.
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PMID:Radiation therapy for benign central nervous system disease. 1009 4

The natural course and optimal treatment for isolated hypothalamic Langerhans cell histiocytosis (LCH) are unknown. We describe an adult female in whom total resection of a hypothalamic LCH granuloma was performed 12 years after transphenoidal resection of a pituitary adenoma. A retrospective review of the histological specimen of the first operation revealed CD1a positive cells characteristic of LCH along with a plurihormonal adenoma 12 years earlier. No other manifestations of LCH were found and MRI of the brain at the last follow-up 4 years after surgery did not show any recurrent or additional lesion. The diagnosis of isolated hypothalamic LCH is only possible by biopsy and our case demonstrates the feasability of a gross total resection in certain cases.
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PMID:Resection of a Langerhans cell histiocytosis granuloma of the hypothalamus: case report. 1061 92

Central diabetes insipidus (DI) can be the outcome of a number of diseases that affect the hypothalamic-neurohypophyseal axis. The causes of the condition can be classified as traumatic, inflammatory, or neoplastic. Traumatic causes include postoperative sella or transection of the pituitary stalk, while infectious or inflammatory causes include meningitis, lymphocytic hypophysitis, and granulomatous inflammations such as sarcoidosis and Wegener's granulomatosis. Various neoplastic conditions such as germinoma, Langerhans cell histiocytosis, metastasis, leukemic infiltration, lymphoma, teratoma, pituitary adenoma, craniopharyngioma, Rathke cleft cyst, hypothalamic glioma, and meningioma are also causes of central DI. In affected patients, careful analysis of these MR imaging features and correlation with the clinical manifestations can allow a more specific diagnosis, which is essential for treatment.
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PMID:MR imaging of central diabetes insipidus: a pictorial essay. 1175 30

The homeodomain transcription factor, NANOG, along with OCT3/4 (POU5F1) and SOX2, is part of the core set of transcription factors that maintain embryonic stem cell self-renewal and pluripotency. Expression of NANOG has been detected in fetal germ cells and in gonadal germ cell tumors. To assess the diagnostic utility of NANOG in central nervous system (CNS) germ cell tumors, we analyzed its expression by immunohistochemistry in a series of 12 CNS germinomas and compared its expression with other stem cell markers. Strong nuclear expression of NANOG was demonstrated in >90% of the tumor cells in all cases. In contrast, expression of OCT3/4 and placental alkaline phosphatase was inconsistent and SOX2 was expressed in only rare cells. NANOG was not detected in tumor types frequently considered in the differential diagnosis of CNS germinoma: pineoblastoma, primitive neuroectodermal tumors, medulloblastoma, lymphoma, pituitary adenoma, atypical teratoid/rhabdoid tumor, Langerhans cell histiocytosis, and gliomas. These findings demonstrate that NANOG is a sensitive and specific marker of CNS germinoma. Compared with other currently used markers, NANOG may have superior diagnostic characteristics and can facilitate identification of germinomas in minute surgical biopsies commonly obtained from these tumors. These findings also suggest a potential biologic role for NANOG in maintenance of CNS germinoma.
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PMID:Comparative analysis of germ cell transcription factors in CNS germinoma reveals diagnostic utility of NANOG. 1712 19