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PubMed2003

Ancillary FISH analysis for 1p and 19q status: preliminary observations in 287 gliomas and oligodendroglioma mimics.

Deletions of chromosomes 1p and 19q are associated with chemosensitivity and enhanced survival in oligodendrogliomas. Therefore, we have utilized FISH analysis as an ancillary tool for diffuse gliomas with suspected oligodendroglial features. To date, 246 gliomas have been analyzed in 131 male and 93 female patients, including 109 oligodendrogliomas (O), 109 mixed oligoastrocytomas/equivocal gliomas (MOA), and 28 astrocytomas (A). To address specificity, we also analyzed 41 oligodendroglioma mimics, including 12 central and 12 extraventricular neurocytomas (EVN), 12 dysembryoplastic neuroepithelial tumors, and 5 clear cell ependymomas. Aside from 2 EVNs, no mimics demonstrated codeletion. Three patterns were associated with glioma cell type (O vs. MOA/A): -1p/19q, -19q alone, and polysomies. Long-term survivals of >5-years (N=47) and >10-years (N=16) were associated with 1p/19q codeletion in 60% and 75% respectively, whereas solitary 19q deletion accounted for 11% and 6% respectively. Survivals<2-years (N=10) were associated with lack of deletions in 70%. A few older patients with high-grade, "genetically favorable" tumors did poorly, whereas prolonged survival was observed in several low-grade glioma patients despite a lack of the "genetically favorable" pattern. Our data suggests that: 1) FISH-detectable 1p/19q codeletion is relatively specific for oligodendrogliomas with long survival, 2) solitary 19q deletion may also portend a favorable prognosis in a smaller subset, and 3) combined clinicopathologic and genetic assessment likely provides a more accurate means of patient stratification than either one alone.

Adolescent↗
PubMed2003

Optic pathway tumors in children.

Tumors of the optic pathways are sub-divided in this review into those that arise in one or both optic nerves anterior to the chiasm (optic nerve tumors); those that arise within the chiasm and do not extend significantly into the hypothalamus (optic chiasmatic tumors) and the large exophytic tumors that involve both the optic chiasm and the hypothalamus to a lesser or greater degree (optic chiasmatic/hypothalamic tumors). The management of optic chiasmatic gliomas is controversial, partly related to failure to separate out chiasmatic tumors from the chiasmatic/hypothalamic tumors. The optic nerve tumors are reviewed briefly, since they rarely extend intracranially. Chiasmatic tumors tend to be associated with NF1 and to behave almost like hamartomas. Close observation is usually the most appropriate management. On the other hand, chiasmatic/hypothalamic tumors grow like typical neoplasms. The tumors are almost uniformly low grade astrocytomas, but growth rates may berapid, especially in infants. Modern management has trended away from radical surgical resection, which has significant morbidity, to chemotherapy as the first line of treatment. In this review, the clinical presentation and management of different types of optic pathway tumors are discussed.

Child↗
PubMedDate not supplied

Suprasellar monomorphous pilomyxoid neoplasm: an ultastructural analysis.

The authors report 3 patients, 2 children and 1 adult, each of whom presented with an unusual myxoid lesion reminiscent of pilocytic astrocytoma but with other features of myxopapillary ependymoma. The magnetic resonance imaging findings in all cases showed a diffusely contrast-enhancing suprasellar mass focally extending into the third ventricle. Involvement of adjacent structures was more extensive in both infants. By light microscopy, all were composed of a monotonous population of cells with delicate piloid-like processes, loosely arranged within a prominent myxoid background. Focally, the neoplastic cells converged upon small blood vessels in pseudorosette-like formations. These histomorphologic features are identical to those of the recently described astrocytoma with monomorphous pilomyxoid features. In addition, the individual tumor cells showed strong cytoplasmic immunoreactivity with antibodies to glial fibrillary acidic protein (GFAP) and vimentin, as well as nuclear and cytoplasmic staining with S-100. All stained positive for synaptophysin and negative for chromogranin. By electron microscopy, the tumor cells were bipolar with elongated processes and apical surfaces displaying microvilli, cytoplasmic blebs and rare cilia. Vesicles and coated pits were seen, as were occasional synaptoid complexes. The current study serves to expand our clincopathologic experience with this rare and enigmatic lesion, with particular attention given to the ultrastructural characteristics.

Adult↗
PubMed2004

Pilocytic and pilomyxoid hypothalamic/chiasmatic astrocytomas.

OBJECTIVE: Pilocytic astrocytoma (PA) is a common type of pediatric brain tumor that can arise within the hypothalamic/chiasmatic region and typically has an excellent outcome. We identified a group of tumors, previously classified as PAs, with unique histological features and aggressive behavior. This article describes the clinicopathological features of these unusual neoplasms, which are currently known as pilomyxoid astrocytomas (PMAs), to better differentiate them from typical PAs. METHODS: Medical information and surgical specimens were obtained for 42 PA cases and 21 PMA cases. Patient demographic features, treatment modalities, progression-free survival (PFS) times, overall survival (OS) times, and outcomes were compared between the groups with nonparametric tests. RESULTS: The PMA group included 12 male and 9 female patients. The PA group included 27 male and 15 female patients. The mean ages at diagnosis for the PMA and PA groups were 18 months (range, 2-84 mo) and 58 months (range, 4-189 mo), respectively (P < 0.01). The mean PFS times for the PMA and PA groups were 26 and 147 months, respectively (P < 0.001). The mean OS times for the PMA and PA groups were 63 and 213 months, respectively (P < 0.001). Sixteen patients with PMAs (76%) experienced local recurrence, and three of those patients demonstrated evidence of cerebrospinal fluid dissemination. Twenty-one patients with PAs (50%) experienced local recurrence, none with evidence of cerebrospinal fluid dissemination. Within the follow-up period, seven patients with PMAs (33%) and seven patients with PAs (17%) died as a result of their disease. In an age-matched set, the mean PFS times for the PMA and PA groups were 25 and 163 months, respectively (P < 0.01), and the mean OS times for the PMA and PA groups were 60 and 233 months, respectively (P < 0.001). CONCLUSION: Hypothalamic/chiasmatic PMAs occurred in a significantly younger population and were associated with substantially shorter PFS and OS times than were typical PAs. Increased recognition of these lesions could affect the prognosis and treatment of pediatric astrocytomas.

Adolescent↗
PubMed1983

Hypothalamic astrocytoma. Syndrome of hyperphagia, obesity, and disturbances of behavior and endocrine and autonomic function.

A 26-year-old woman had hyperphagia, obesity, aggressive behavior, visual hallucinations, reversal of wake-sleep patterns, hypothermia, hypothyroidism, and amenorrhea. She died of pancreatitis, probably secondary to hypothermia. Autopsy revealed a low-grade astrocytoma in the third ventricle and medial anterior and mid hypothalamus, primarily on the right. Although she exhibited thyroid and ovarian hypofunction, the patient had intact median eminence and pituitary function, suggesting end-organ failure, possibly of an autoimmune nature.

Adult↗
PubMed1990

Hypothalamic hamartomas and sexual precocity. Evaluation of treatment options.

We describe four male patients with hypothalamic hamartomas associated with sexual precocity. Our assessment of their management suggests that resection using current microsurgical techniques is a valid treatment option if the patient has a normal pubertal endocrine makeup, if the hamartoma is pedunculated, and if the patient is young enough to require years of parenteral medical treatment. Such surgical treatment can be curative, and subsequent growth and development can be normal (patients 1 and 2). However, if the patient is near to pubertal age (patient 3) or if neurosurgical or gonadotropin releasing hormone analogue treatment is not available, the natural history (patient 4) suggests that the only undesirable effects are accelerated growth, tall stature for age, and premature sexual development during childhood, as well as the psychosocial problems that may accompany them. Adult height may be compromised, although the two patients who did not undergo a surgical procedure and did not receive gonadotropin releasing hormone analogue therapy are above the lower limits of the normal range of adult male height. Therefore, if the hamartoma is pedunculated and cessation of pubertal development is desired, resection of the hamartoma is a reasonable therapeutic option.

Antineoplastic Agents↗
PubMed1993

Pituitary stalk and ectopic hyperintense T1 signal on magnetic resonance imaging. Implications for anterior pituitary dysfunction.

OBJECTIVE: To determine if improved delineation of hypothalamic-pituitary neuroanatomy by magnetic resonance imaging, especially the posterior pituitary hyperintense T1 signal, can be correlated with anterior and posterior pituitary endocrine function. DESIGN: Children with ectopic posterior pituitary tissue were identified at the Endocrine Clinic of the Children's Hospital of Pittsburgh (Pa) and their records were reviewed. PARTICIPANTS: Ten children with ectopic posterior pituitary tissue. MEASUREMENTS: Anterior pituitary hormone status, determined by standard testing, was correlated with the morphologic anomalies of the hypothalamic-pituitary region on magnetic resonance imaging. RESULTS: Patients were categorized by the appearance of the pituitary stalk based on the magnetic resonance image: attenuation of the stalk (group 1) or nonvisualization of the stalk (group 2). Patients in group 1 retained partial anterior pituitary function. Patients in group 2 had panhypopituitarism. CONCLUSION: Prospective evaluation of affected individuals may provide insight into the pathophysiologic mechanisms of idiopathic hypopituitarism.

Adolescent↗
PubMed2000

Late-onset central hypoventilation with hypothalamic dysfunction: a distinct clinical syndrome.

Idiopathic central hypoventilation has occasionally been reported in previously well children after infancy. The relationship between this late-onset central hypoventilation syndrome (LO-CHS) and congenital central hypoventilation syndrome (CCHS) has not been established. Both CCHS and LO-CHS have been associated with neural crest tumors, such as ganglioneuroblastoma and ganglioneuroma, and they generally occur in the presence of a histologically normal central nervous system. At least 10 case reports of idiopathic LO-CHS featured evidence of hypothalamic dysfunction (HD), including hyperphagia, hypersomnolence, thermal dysregulation, emotional lability, and endocrinopathies. We report on a case of LO-CHS/HD successfully treated by nasal intermittent positive pressure ventilation (NIPPV). Despite the commonalties with CCHS, we propose that LO-CHS/HD is a distinct clinical syndrome. In addition to the markedly different age at presentation, features of hypothalamic dysfunction are not seen in CCHS. Review of the literature was undertaken to further clarify the full spectrum of the disease.

Child↗
PubMed1982

Bronchogenic carcinoma and diabetes insipidus: case report and review.

Tumor metastasis to the posterior pituitary-hypothalamic area rarely is expressed by either clinical or radiographic evidence. A case is reported of widespread metastic ACTH secreting bronchogenic carcinoma presenting to the clinician as diabetes insipidus. This type of tumor associated with diabetes insipidus is apparently rare, as is antemortem radiographic documentation of metastasis to the pituitary--hypothalamic region. A review of the ten-year experience of Loyola University Medical Center and Hines VA Hospital with the association of bronchogenic carcinoma and diabetes insipidus is presented with a brief review of metastic disease to the pituitary.

ACTH Syndrome, Ectopic↗
PubMed1983

Systemic cancer presenting as diabetes insipidus. Clinical and radiographic features of 11 patients with a review of metastatic-induced diabetes insipidus.

Since computerized transaxial tomography (CTT) scanning at the Mayo Clinic (1974-1981), 25 cases of metastatic diabetes insipidus (DI) have been identified. Of 100 consecutive cases of DI of any cause, 14 were due to metastatic cancer. Diabetes insipidus was the initial presentation in 11 patients with systemic cancer. In the 11 patients, the most common sources metastatic to the posterior pituitary-hypothalamic region were lung (3) and the leukemia/lymphoma group (4). Although skull x-ray results were usually normal (9 of 11), CTT scanning results were abnormal in 5 of 11, including demonstration of pituitary stalk enlargement, suprasellar masses, or both. Metastases elsewhere in the nervous system were apparent in four patients. Anterior pituitary and visual system involvement occur in a minority group of patients.

Adult↗
PubMed1989

Chronic lymphocytic leukemia with hypothalamic invasion.

Headaches and a state of confusion developed in a patient with chronic lymphocytic leukemia (CLL). A computed tomography (CT) scan of the head showed a hyperdense lesion in the hypothalamus with obstruction of the foramina of Monro. Leukemic cells with monoclonal lambda B-cell markers were identified in the ventricular fluid at the time of surgical decompression. Similar cells were identified in the peripheral blood. This lesion has not been described as a complication of CLL, a disease that, unlike other leukemias, rarely has central nervous system manifestations.

Aged↗
PubMed1980

Congenital hypothalamic hamartoblastoma, hypopituitarism, imperforate anus and postaxial polydactyly--a new syndrome? Part I: clinical, causal, and pathogenetic considerations.

We report on six infants with a neonatally lethal malformation syndrome of hypothalamic hamartoblastoma, postaxial polydactyly, and imperforate anus. Some, but not all, patients had laryngeal cleft, abnormal lung lobulation, renal agenesis and/or renal dysplasia, short 4th metacarpals, nail dysplasia, multiple buccal frenula, hypoadrenalism, microphallus, congenital heart defect, and intrauterine growth retardation. The infants also had hypopituitarism and hypoadrenalism. All were sporadic cases, parents were not consanguineous, chromosomes were apparently normal. Family histories were unremarkable. There was insecticide and/or herbicide exposure in several of the cases, but no exposures were common to all 6 mothers. Five of the patients were born within an 8-month period, but all in different geographic locations. It is postulated that this is a previously apparently unreported syndrome of presently unknown cause.

Anus, Imperforate↗
PubMed1980

Congenital hypothalamic hamartoblastoma, hypopituitarism, imperforate anus, and postaxial polydactyly--a new syndrome? Part II: Neuropathological considerations.

Unusual and virtually identical hypothalamic tumors were recently studied in three unrelated neonates with a similar complex malformation syndrome. Previous reports of either the hypothalamic tumors or the syndrome as a whole have not been found. Each tumor was apparent on the inferior surface of the cerebrum and extended from the optic chiasma to the interpeduncular fossa. The tumor replaced the hypothalamus and other nuclei which originate in the embryonic hypothalamic plate; it was principally composed of cells resembling primitive, undifferentiated germinal cells. The term "hamartoblastoma" is used to designate these tumors in order to emphasize the malformational and neoplastic aspects. In addition, short olfactory tracts suggest a relation to the arrhinencephaly field defect.

Anus, Imperforate↗
PubMed1981

Kaufman syndrome.

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Abnormalities, Multiple↗
PubMedDate not supplied

Partial fra(X) phenotype with megalotestes in fra (X)-negative patients with acquired lesions of the central nervous system.

We describe 3 men with acquired lesions of the central nervous system (one male with tumor of the third ventricle, two males with peri- or postnatal accidents). In all three patients macroorchidism was associated with facial characteristics similar to those found in fra(X) males, but they were fra(X)-negative. The finding of megalotestes associated with a partial fra(X) phenotype in the present patients and, more particularly, the documentation of a hypothalamic tumor in the first patient, suggests that a specific hypothalamic lesion in fra(X) males is responsible for some of their manifestations.

Adolescent↗