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Biomedical subjects

B W Scheithauer

Publications and source records attributed to B W Scheithauer.

At least 217 records · Page 12Linked to original sources
PubMed1994

Dysembryoplastic neuroeptihelial tumor (DNT): an immunohistochemical and ultrastructural study.

To assess the range of differentiation of the cells comprising dysembryoplastic neuroepithelial tumor (DNT), particularly the oligodendrocyte-like cells (OLC), 14 DNT were immunochemically studied with a spectrum of neuronal and glial markers. Eight tumors were also studied ultrastructurally. Neurofilament protein, class III beta-tubulin, and synaptophysin preparations stained a few OLC in two, six and one lesion, respectively. In addition, many OLC within a single cortical nodule were reactive for class III beta-tubulin. The vast majority of OLC were strongly S-100 protein positive. Glial fibrillary acidic protein labeled a fair number of OLC in two cases and one nodule consisted almost entirely of immunoreactive astrocytes. Ultrastructurally, many OLC resembled oligodendrocytes in exhibiting microtubules, prominent Golgi and short cell processes; pericellular lamination of cell processes, a characteristic of oligodendroglia, was noted in only one tumor. In two cases, OLC with astrocytic features were seen to contain small numbers of intermediate filaments. In four cases, a few OLC resembled immature neurons with scant dense-core granules or synapses. This study confirms the glioneuronal nature of DNT, a lesion composed of heterogeneous cells, many resembling oligodendrocytes and a few showing early astrocytic and neuronal differentiation. Although their relation to OLC is unclear, the presence and peculiar distribution of mature neurons is nonetheless an integral diagnostic feature of the lesion.

Adolescent↗
PubMed1994

The significance of atypia and histologic malignancy in pilocytic astrocytoma of the cerebellum: a clinicopathologic and flow cytometric study.

Biologically malignant pilocytic astrocytomas are rare. Precise morphologic criteria permitting their identification and clinically meaningful distinction from more ordinary pilocytic astrocytomas have not been developed. The histologic grading schemes applied to fibrillary astrocytomas--ones based on nuclear atypia, increased cellularity, mitotic activity, endothelial proliferation, and necrosis--are thought not to correlate with prognosis in pilocytic astrocytomas. Nonetheless, these features were systematically sought in 107 cerebellar pilocytic astrocytomas, four of which were histologically malignant. The clinical, morphologic, and flow cytometric features of these cases were studied. The incidence of histologic malignancy occurring spontaneously in otherwise typical pilocytic astrocytomas was 0.9%; that occurring after radiation was 1.8%. The four histologically malignant tumors occurred in two males and two females, ages 6 to 18 years. All showed increased mitotic activity (three to five mitoses per high-power microscopic field [250x]). Endothelial proliferation and necrosis were present in three and two cases each. DNA ploidy analysis showed the spontaneously occurring malignant-appearing tumors to be aneuploid, whereas both previously radiated tumors were tetraploid; 5% to 11% of cells were in S phase. The appearance of histologic malignancy in pilocytic astrocytoma is rare and less reliably correlated with prognosis than in patients with fibrillary astrocytomas. Essential features of such tumors include a high mitotic index and perhaps a high percentage of cells in S phase.

Adolescent↗
PubMed1994

ras mutations in human prolactinomas and pituitary carcinomas.

Pituitary adenomas have been shown to be clonal in origin, indicating that one or more somatic mutations underlie tumor pathogenesis. Mutated oncogenic forms of ras protein have been identified in a number of human neoplasms, including thyroid adenomas and carcinomas. However, the potential role of activated ras in the development of specific human pituitary tumor phenotypes has not been determined. Although ras mutations were not found in glycoprotein hormone-secreting or somatotroph adenomas, we recently identified a mutation in the H-ras gene (Gly-Val) at codon 12 in a highly invasive prolactinoma. These data raise the possibility that ras mutations might play a role in the pathogenesis of PRL-secreting pituitary tumors and/or may be a marker for tumor invasiveness and malignant transformation. Therefore, we investigated 78 pituitary tumors (59 prolactinomas, 13 invasive prolactinomas, and 6 pituitary carcinomas) for activating point mutation in the three ras genes using oligonucleotide-specific hybridization. In contrast to the relatively high frequency of ras mutations in many different tumor types, no ras mutations were identified in either prolactinomas or pituitary carcinomas. Our data indicate that ras mutations are rare in prolactinomas and pituitary carcinomas.

Adolescent↗
PubMed1994

Pituitary adenomas in childhood and adolescence.

A clinicopathological study of 56 pediatric patients with non-ACTH-secreting pituitary adenomas removed by a transsphenoidal neurosurgical approach was undertaken to better define the clinical presentation, to assess demographic factors, to determine the immunohistochemical staining characteristics of the tumors, and to evaluate the outcome of transsphenoidal surgical treatment and other adjuvant therapies. A separate analysis of prolactinoma patients was performed. All tumors were confirmed histologically and immunophenotyped for pituitary hormones. Forty-one patients had tumors that stained for PRL alone, eight patients had tumors that stained for PRL and GH, six patients had plurihormonal adenomas, and one patient had a tumor that stained for glycoprotein hormones. No tumors contained GH alone. Macroadenomas exceeded microadenomas (1.4:1). There were no male patients with microadenomas of any type. Females outnumbered males (3.3:1). Patients presented most frequently with headache, menstrual dysfunction (in females), galactorrhea, and hypopituitarism. All but one of the patients with hypopituitarism at presentation had macroadenomas. Tumor staining characteristics did not always correlate well with clinical status, especially with regard to GH-containing tumors. Pediatric pituitary tumors did not appear to be more invasive or more aggressive than adult pituitary tumors, contrary to some previous reports. The patients with microadenomas had a 70% operative cure rate and a 65% long term cure rate; the recurrence rate for microadenoma patients was 25%. Macroadenoma patients had a 33% operative cure rate, a 55% long term cure rate, and a recurrence rate of 33%. Thus, microadenoma and macroadenoma patients had similar long term cure rates, but macroadenoma patients required more aggressive adjuvant therapy (second surgery, radiation, or bromocriptine) and had higher rates of hypopituitarism (52% of macroadenoma patients vs. 0% of microadenoma patients required long term hormone replacement).

Adenoma↗
PubMed1994

Mixed oligoastrocytomas: a survival and prognostic factor analysis.

Seventy-one patients with supratentorial mixed oligoastrocytomas underwent surgery only (5 patients) or surgery with postoperative radiation therapy (66 patients) between 1960 and 1982. The median survival for these 71 patients was 5.8 years and the 5-, 10-, and 15-year survival rates were 55, 29, and 17%, respectively--values significantly different from those of an age- and sex-matched normal reference population. Uni- and multivariate survival analyses were applied to 14 possible prognostic factors, including the following: patient factors--age, sex, and seizures; tumor factors--site, size, side, computed tomographic enhancement, and calcification; pathological factors--tumor grade and dominant cellular component; and treatment factors--extent of surgical resection, lobectomy, and radiation dose and field. Of these factors, tumor grade, as determined by the Kernohan method, was the most strongly associated with survival. The 60 patients with Grades 1 and 2 tumors had a median survival of approximately 6.3 years and 5- and 10-year survival rates of 58% and 32%, respectively, compared with 2.8 years (36 and 9%, respectively) for the 11 patients with Grades 3 and 4 tumors. Age < 37 years, gross total resection, partial brain radiation, and radiation dose > or = 5000 cGy were other factors significantly associated with improved survival in both uni- and multivariate models. Three of five patients not receiving postoperative radiation therapy experienced tumor recurrence and died. Seven of eight patients from whom tissue was obtained at the time of tumor progression demonstrated anaplastic transformation.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1994

Chronic meningitis: the role of meningeal or cortical biopsy.

Meningeal and cortical biopsies were evaluated in 37 patients (25 men and 12 women; mean age, 54 yr) who had chronic meningitis of an unknown cause between 1985 and 1993 (the era of magnetic resonance imaging). Magnetic resonance imaging with gadolinium contrast was the most useful diagnostic imaging technique, demonstrating meningeal enhancement in 15 of 32 patients (47%). Only 2 of 32 (6%) computed tomographic scans revealed enhancement. A definitive diagnosis was made in 16 of 41 biopsies (39%), but in cases where enhancement was present on either magnetic resonance imaging or computed tomography, a diagnosis was obtained in 80% (12 of 15 cases). Only 2 of 22 biopsies (9%) from nonenhancing regions were diagnostic. Although the locations of enhancement were distributed evenly, biopsies through suboccipital and pterional craniotomies gave the highest diagnostic yields (50%). Furthermore, if the biopsies were obtained from enhancing regions, the yield of these two approaches increased to 84 and 100%, respectively. Of 18 cases in which biopsy samples were taken from both the meninges and cortex, only 1 had cortical involvement alone. The meninges were therefore diagnostic in 15 of the 16 definitive diagnostic cases (94%). Second biopsies were necessary in four cases, of which the three biopsies from enhancing regions were diagnostic. The most frequent causes of chronic meningitis were sarcoid (31%) and metastatic adenocarcinoma (25%).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1994

Angiographically occult vascular malformations: a correlative study of features on magnetic resonance imaging and histological examination.

With reference to vascular malformations, the term cavernous has architectural as well as histologic connotations. It refers to a compact pattern of growth wherein no intervening brain parenchyma is evident, as well as to the histological nature of the vessels, which are hyaline and collagenous in appearance, lacking the microscopic features of arteries or veins. Historically, cavernous angioma has been defined as exhibiting both features. Twenty-five patients with neurological symptoms and neuroimaging abnormalities who underwent surgery for cerebral vascular malformations between 1987 and 1990 satisfied the following study criteria: their lesions were angiographically occult and both magnetic resonance imaging (MRI) and histological sections were available for review. The patients' ages ranged from 4 to 49 years (mean, 30 years), the male to female ratio being 1:2. Two thirds of the lesions were supratentorial in location and all were intraparenchymal. All patients had clinical improvement after resection. In 24 of the 25 lesions, the vascular channels were histologically cavernous in nature; one inadequate specimen precluded classification. Three demonstrated a purely compact or cavernous pattern, 20 a mixed cavernous and racemose pattern, and one a purely racemose pattern. The authors conclude that 1) histologically cavernous lesions are the commonest form of occult vascular malformation; 2) a purely compact or cavernous architectural pattern is uncommon, most lesions showing a partially racemose architecture; 3) some histologically cavernous malformations possess a capillary component; 4) clinical growth of cavernous malformations may have its basis in intraluminal thrombosis and subsequent recanalization; 5) the T2-weighted MRI pattern of cavernous malformations varies, the most common being a multifocal hyperintense center surrounded by a hypointense ring; 6) the MRI pattern reflects the histological appearance; 7) since no thrombosed arteriovenous malformations were encountered, such lesions must be rare; 8) in that the pathophysiological hallmark of a cavernous lesion is recurrent thrombosis and hemorrhage, a resolving hematoma cannot always be distinguished from a cavernous lesion; 9) MRI is the examination of choice in evaluating occult vascular malformations; and 10) microsurgical excision is a satisfactory method of treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Actins↗
PubMed1994

Cerebellar pleomorphic xanthoastrocytoma: case report.

We report a case of pleomorphic xanthoastrocytoma occurring in the midline cerebellum of a 48-year-old woman. Radiological, histological, immunohistochemical, and flow cytometric findings are discussed. Pleomorphic xanthoastrocytoma typically occurs in the superficial cerebral hemispheres of young patients. To our knowledge, this is the first report of the occurrence of this neoplasm in the cerebellum. The English literature is reviewed with regard to the clinicopathological features of this uncommon form of astrocytoma.

Astrocytoma↗
PubMed1994

Estrogen receptor gene expression in craniopharyngiomas: an in situ hybridization study.

Craniopharyngiomas are histologically benign epithelial neoplasms of the sellar region that frequently exhibit invasive and aggressive local growth. In this study, we have investigated the presence and cellular distribution of estrogen receptor messenger ribonucleic acid by in situ hybridization in 23 surgically removed craniopharyngiomas. All craniopharyngiomas studied, including 19 adamantinomatous and 4 papillary variants, uniformly expressed the estrogen receptor gene. In all cases, an intense estrogen receptor messenger ribonucleic acid hybridization signal was demonstrated; one localized exclusively to the epithelial cells of the tumor. Connective tissue and vascular elements were devoid of hybridization signal. Coexpression of the estrogen receptor protein was also studied by immunohistochemistry. Despite the relative abundance of estrogen receptor message in all cases studied, the estrogen receptor protein was focally but conclusively detected in only two tumors. The basis of this discrepancy is unclear. Progesterone receptor protein was also studied in all cases; however, its definitive presence was noted in only one instance and, in that case, in only occasional nuclei. The expression of the estrogen receptor gene by the proliferative epithelial elements of craniopharyngiomas raises the questions of a possible hormonal component to the genesis and/or progression of the craniopharyngiomas and a potential responsiveness to therapeutic hormonal manipulation.

Cell Division↗
PubMed1994

Changes in proliferating cell nuclear antigen expression in glioblastoma multiforme cells along a stereotactic biopsy trajectory.

Proliferating cell nuclear antigen, an auxiliary protein of deoxyribonucleic acid polymerase-delta, has been shown to be a reliable marker of nuclear deoxyribonucleic acid synthetic activity. We applied a monoclonal antibody to proliferating cell nuclear antigen to a series of serial stereotactic biopsies from patients with glioblastoma multiforme and found the proliferative activity to vary relative to biopsy location within or surrounding the solid tissue component of the tumor. Twenty-seven trajectories in 26 patients were analyzed, each consisting of two to five sequential 10 x 1.5 mm core biopsies (mean = 3). The proliferative index (PI) was greatest in those cells located at the solid tumor-infiltrated parenchyma interface. PI values were significantly lower in those biopsy cores located proximal (within infiltrated parenchyma) and distal (within solid tumor tissue) to the solid tumor-infiltrated parenchyma interface (median PI values, proximal to distal: 0.38, 0.66, 5.45 solid tumor-infiltrated parenchyma interface], 0.39, 0.09%). The mean PI values were significantly lower in neoplastic cells samples from regions of peripheral hypodensity on computed tomographic scans compared with those sampled from contrast-enhancing regions (0.9 and 3.91%, respectively). There was no significant difference in the mean PI values of neoplastic cells sampled from regions of contrast enhancement or central hypodensity (3.91 and 4.31%, respectively).

Biomarkers, Tumor↗
PubMed1994

Pituitary adenomas in childhood and adolescence. Results of transsphenoidal surgery.

Pituitary adenomas are uncommon in childhood. Between 1975 and 1988, 36 patients underwent transsphenoidal resection at the Mayo Clinic before their 17th birthday. Fifteen (41.7%) had prolactin (PRL)-secreting tumors, 16 (44.4%) had adrenocorticotropic hormone (ACTH)-secreting tumors (including two patients with Nelson-Salassa syndrome), and three (8.3%) had tumors secreting growth hormone (GH); two patients (5.6%) had clinically nonfunctioning tumors. Of the 36 patients, 21 (58.3%) were girls, and girls predominated in every group except that with ACTH-secreting tumors, where they accounted for only 37.5% of the patients. The average age at the time of surgery was 14.7 +/- 2.2 years, the youngest patient being 7.3 years old. The ACTH-secreting tumors were associated with an earlier onset of symptoms than the other tumor types: six (37.5%) of 16 patients became symptomatic before 10 years of age, compared with two (10.0%) of the 20 other patients. Presenting symptoms primarily reflected endocrine dysfunction, with neurological presentation (visual field deficits) occurring in only two patients. There were no deaths in this patient group. Significant operative morbidity included steroid-induced psychosis in one patient and diabetes insipidus in three, which resolved in two. Plurihormonal tumors, broadly defined as tumors producing more than one hormone, were common, comprising five (36%) of 14 PRL-secreting tumors, all three GH-secreting tumors, and one of two clinically nonfunctioning tumors, or 25.7% overall. Macroadenomas accounted for 89% of plurihormonal tumors. Long-term follow-up review (median > 5 years) revealed good control of PRL-secreting tumors (although five of 15 patients had received postoperative radiotherapy), contrasted with a 25% late recurrence rate for ACTH-secreting tumors, which had an 80% initial remission rate. The majority of patients had good endocrine function, including reproductive function. It is concluded that: 1) although pediatric pituitary adenomas occur primarily in adolescence, Cushing's disease is found at any age; 2) transsphenoidal surgery is feasible and safe in this age group; 3) plurihormonal tumors occur more frequently in the pediatric age group than in adults; and 4) long-term control rates in PRL- and ACTH-secreting tumors are probably similar to those seen in adults.

Adenoma↗
PubMed1994

Symptomatic glial cysts of the pineal gland.

Small asymptomatic cysts of the pineal gland represent a common incidental finding in adults undergoing computerized tomography or magnetic resonance (MR) imaging or at postmortem examination. In contrast, large symptomatic pineal cysts are rare, being limited to individual case reports or small series. The authors have reviewed 24 cases of large pineal cysts. The mean patient age at presentation was 28.7 years (range 15 to 46 years); 18 were female and six male. Presenting features in 20 symptomatic cases included: headache in 19; nausea and/or vomiting in seven; papilledema in five; visual disturbances in five (diplopia in three, "blurred vision" in two, and unilateral partial oculomotor nerve palsy in one); Parinaud's syndrome in two; hemiparesis in one; hemisensory aberration in one; and seizures in one. Four lesions were discovered incidentally. Magnetic resonance imaging typically demonstrated a 0.8- to 3.0-cm diameter mass (mean 1.7 cm) with homogeneous decreased signal intensity on T1-weighted images, increased signal intensity on T2-weighted images, and a distinct margin. Hydrocephalus was present in eight cases. The cysts were surgically excised via an infratentorial/supracerebellar approach (23 cases) or stereotactically biopsied (one case). Histological examination revealed a cyst wall 0.5 to 2.0 mm thick comprised of three layers: an outer fibrous layer, a middle layer of pineal parenchymal cells with variable calcification, and an inner layer of hypocellular glial tissue often exhibiting Rosenthal fibers and/or granular bodies. Evidence of prior hemorrhage, mild astrocytic degenerative atypia, and disorganization of pineal parenchyma were often present. Postoperative follow-up review in all 24 cases (range 3 months to 10 years) revealed no complications in 21, mild ocular movement deficit in one, gradually resolving Parinaud's syndrome in one, and radiographic evidence of a postoperative venous infarct of the superior cerebellum with ataxia of 1 week's duration in one. Of the patients referred for study, the cysts were most often initially misdiagnosed as a pineocytoma in eight and a pilocytic astrocytoma in three. Only two patients were correctly diagnosed as having pineal cysts. This stresses the importance of recognizing the histopathological spectrum of pineal cysts, as well as correlation with radiographic findings, if a correct diagnosis is to be attained.

Adolescent↗
PubMed1994

Benign glial cysts of the pineal gland: unusual imaging characteristics with histologic correlation.

PURPOSE: To describe the spectrum of MR and CT findings in clinically symptomatic pineal cysts and to determine whether there are certain diagnostic imaging features that allow one to distinguish a benign pineal cyst from other neoplasms of the pineal region. METHODS: MR and CT scans of 19 patients with clinically symptomatic pineal cysts were retrospectively reviewed. Age range was 15 to 46 years with a mean age of 28 years. There were five male and 14 female patients. RESULTS: Presenting features included headache (15 patients), diplopia (four), nausea and vomiting (four), papilledema (four), seizure (three), Parinaud syndrome (two), ataxia (one), and hemiparesis (one). All cysts were resected or biopsied to provide histopathologic confirmation of the diagnosis. Preoperative diagnoses included pineal neoplasm (14 of 19), pineal cyst (3 of 19), and dermoid cyst (2 of 19). The lesions ranged from 0.8 to 3.0 cm, with a mean diameter of 1.6 cm. Three cysts showed fluid/fluid levels consistent with hemorrhage. Slightly less than half (9 of 19) had evidence of hydrocephalus. The MR signal changes were variable but typically demonstrated low signal on T1-weighted images and high signal on T2-weighted images. More than half (7 of 12) demonstrated enhancement with gadolinium. Calcification of the cyst wall was observed in only four of nine patients who had CT studies but identified histologically in all cases. CONCLUSION: The MR appearance of benign pineal cysts is variable, ranging from that of an uncomplicated cystic mass to a mass associated with hemorrhage, enhancement, or hydrocephalus. This variability may make them indistinguishable from other pineal-region tumors.

Adolescent↗
PubMed1993

Chordoma and chondroid neoplasms of the spheno-occiput. An immunohistochemical study of 41 cases with prognostic and nosologic implications.

BACKGROUND: Chordomas are rare neoplasms that show a proclivity for the spheno-occiput and sacral regions. A "chondroid" variant involving the spheno-occiput has been associated with improved survival. "Classic" or nonchondroid chordomas are uniformly immunoreactive for keratins. Chondroid chordomas are said to be immunonegative for epithelial markers, a feature used to support the concept that they represent chondrosarcomas. METHODS: The authors performed immunohistochemical studies on 25 patients with chondroid chordoma (mean age, 40.0 years) and on 16 patients with classic chordomas (mean age, 44.2 years) to establish tumor subsets based upon immunophenotype, specifically reactivity for epithelial markers. Kaplan-Meier survival curves were then constructed for each group with age as an added variable. RESULTS: All classic chordomas reacted for keratins as did 8 (32%) of the 25 chondroid chordomas. Forty-four percent of classic and 85% of chondroid chordomas were positive for S-100 protein. At 5 years, all patients younger than 40 years of age were alive in both the classic and chondroid groups. In contrast, of patients older than 40 years of age, only 22% with classic chordomas and 38% with chondroid chordomas were alive. CONCLUSIONS: Regardless of tumor subtype, age is the single most important variable in determining survival; patients younger than 40 years of age do better than older patients. There are no significant survival differences between patients with cartilage-containing tumors that are keratin immunopositive ("true" chondroid chordoma) or negative (chondrosarcoma). Immunostaining for keratins is of no prognostic value in assessing chondroid lesions of the spheno-occiput.

Adult↗
PubMed1993

Supratentorial pilocytic astrocytomas. A clinicopathologic, prognostic, and flow cytometric study of 51 patients.

BACKGROUND: Supratentorial pilocytic astrocytomas are uncommon tumors, the prognosis and management of which have been controversial. METHODS: The authors retrospectively studied the clinical and pathologic aspects of 51 patients with supratentorial pilocytic astrocytomas, with particular reference to their response to treatment. RESULTS: The study group consisted of 25 male and 26 female patients (median age, 15 years). The extent of surgical removal was gross total or radical subtotal in 16 patients and subtotal removal or biopsy in 35. At a median follow-up of 14.9 years, 41 patients were alive. Overall survival was 82% at 10 and 20 years; 89% of surviving patients were fully active. Survival at 10 years was 100% for the 16 patients who underwent gross total or radical subtotal removal and 74% for the 35 who had subtotal tumor removal or biopsy. No association was found between pathologic features such as histologic grade or flow cytometric characteristics and patient survival. CONCLUSIONS: Supratentorial pilocytic astrocytomas behave differently from low-grade astrocytomas of the diffuse or fibrillary type in that they have a favorable prognosis. Radiation therapy appears not to be needed for patients undergoing gross total removal. In patients undergoing subtotal resection, close follow-up with additional surgery or radiation therapy at the time of tumor progression is recommended. Patients who have biopsy only should receive radiation therapy. Histologic grading and flow cytometric analysis add little to clinical decision making with regard to patients with pilocytic astrocytoma.

Adolescent↗
PubMed1993

Pineal parenchymal tumors. Clinical, pathologic, and therapeutic aspects.

BACKGROUND: Pineal parenchymal tumors are rare; therefore, only limited clinical data regarding their behavior is available. This study was performed to provide further information regarding the pathologic features, clinical behavior, and response to therapy of these tumors. METHODS: This study includes data concerning 30 patients (15 male and 15 female patients) with pineal parenchymal tumors (PPT) diagnosed between 1939 and 1991. Based on gross and microscopic features, tumors were divided into four groups: pineocytomas (9); PPT with intermediate differentiation (4); mixed PPT exhibiting elements of both pineocytoma and pineoblastoma (2); and pineoblastomas (15). At the time of diagnosis, four patients had evidence of spinal seeding (two with pineoblastoma, two with PPT with intermediate differentiation). Twenty-two patients received radiation therapy (RT): 6 were treated to local fields, 7 to the whole brain, and 9 to the craniospinal axis. RESULTS: For patients who received RT and had a minimum follow-up of 6 months, local failure occurred in one of four patients with pineocytomas, zero of four patients with PPT with intermediate differentiation, one of two with mixed PPT, and four of nine (44%) with pineoblastomas. In patients receiving > or = 50 Gy to the primary tumor, 0 of 12 had local failure compared with 6 of 7 (86%) patients receiving lesser doses. Leptomeningeal failure occurred in zero of four patients with pineocytomas, zero of four patients with PPT with intermediate differentiation, one of two with mixed PPT, and four of nine with pineoblastomas. All leptomeningeal failures occurred in patients with persistent primary tumor. Of the patients with seeding tumors (PPT other than pineocytomas) one of seven (14%) developed leptomeningeal failure when treated with craniospinal irradiation, compared with four of eight (50%) treated to lesser volumes. The projected 1-year, 3-year, and 5-year survival rates of patients with pineocytomas were 100%, 100%, and 67%, and were 88%, 78%, and 58% for those with the other forms of PPT, respectively. CONCLUSIONS: RT recommendations are described in detail and include the use of doses of > or = 50 Gy to areas of gross disease and the administration of craniospinal irradiation in patients with tumors prone to seeding. Surgical, chemotherapeutic, and pathologic considerations are discussed.

Adolescent↗
PubMed1993

Midline cerebral dysgenesis, dysfunction of the hypothalamic-pituitary axis, and fetal alcohol effects.

OBJECTIVE: Neuropathologic evaluation was performed on an infant with fetal alcohol effects. DESIGN: Coronal brain sections and representative tissue blocks stained with hematoxylin-eosin, silver stain, and immunocytochemical stains for hypothalamic and pituitary hormones were evaluated for neuropathologic abnormalities. PATIENT: A 2.5-month-old American Indian girl who had been exposed to first-trimester maternal binge alcohol abuse died after persistent problems of growth failure, sodium imbalance, aberrant temperature regulation, respiratory distress, and seizures. RESULTS: Autopsy revealed severe microcephaly, hypertelorism, midfacial hypoplasia, a high-arched palate, shortened palpebral fissures, and a small brain. The frontal lobes were fused anteriorly; olfactory bulbs and tracts were absent; and optic nerves were hypoplastic. An enlarged and bulbous hypothalamus obscured the pituitary gland. The thalamus and caudate nuclei were fused across the midline. Posteriorly, the single ventricle split to form rudimentary lateral horns. The anterior corpus callosum, septum pellucidum, fimbria, and fornices could not be identified. The anterior commissure and supraoptic nuclei were microscopically present. Many Purkinje cells were horizontally positioned, with abnormal dendritic structure. The posterior pituitary lobe was absent, and the infundibulum was flanked by a hypoplastic adenohypophysis and a large subarachnoid heterotopia. Immunocytochemical studies identified only vasopressin and neurophysin in the hypothalamus and only growth hormone and prolactin in the pituitary gland. CONCLUSION: To our knowledge, an association between fetal alcohol effects and a complex cerebral anomaly with features of incomplete holoprosencephaly and septo-optic dysplasia has not previously been reported and suggests a possible common pathogenesis needing further study.

Brain↗