Enterogenous cyst of the orbital apex and superior orbital fissure.

BACKGROUND: Enterogenous cysts of the central nervous system are rare congenital tumors with a single layer of mucin-secreting epithelial cells resembling gastrointestinal epithelium. The tumor is located most commonly at lower cervical and cervicothoracic spinal levels; only 22 intracranial cases have been reported. To the authors knowledge, this entity has not been described in the orbit. METHODS: A 23-year-old woman with painful loss of vision and ophthalmoplegia in the left eye was treated with oral and intravenous corticosteroids for presumed orbital inflammation. After a cystic lesion in the left orbital apex was demonstrated on computed tomographic scan and magnetic resonance imaging, various diagnoses, including optic nerve tumor, granulomatous inflammation, lymphoma, vascular anomaly, and pseudotumor, were considered until transcranial biopsy established the correct diagnosis. The tumor subsequently recurred twice. RESULTS: More than 3 years after the last recurrence, the patient has no pain but had unilateral optic atrophy, significant visual field loss, limited motility, and an anesthetic cornea in the left eye. CONCLUSION: The diagnosis of enterogenous cyst is difficult without adequate biopsy because the radiologic and clinical presentation of this rare tumor may be confused with other lesions. Previous attempts to explain intracranially placed enterogenous cysts offer no explanation for an orbital occurrence nor do they adequately describe a mechanism for an intracranial location in general. An embryologically based explanation that takes into account the occurrence of this entity from the caudal to rostral extent of the neuraxis is described. This theory suggests that the orbit is the most rostral possible location for an enterogenous cyst.

Intratumor administration of beta-interferon in recurrent malignant gliomas. A phase I clinical and laboratory study.

We administered doses of 5 to 180 x 10(6) IU of beta-serine-interferon (IFN-beta ser17) twice weekly to 20 patients with recurrent malignant gliomas in a Phase I study. Interferon was given through an Ommaya reservoir connected by a catheter to the tumor cavity. Side effects of interferon therapy occurred in only one patient and consisted of nausea, vomiting, fever, and chills after each treatment, presumably due to rapid diffusion of interferon into ventricular cerebrospinal fluid (CSF). Problems with the Ommaya reservoir (obstruction in two patients and infection in four patients) led to six patients being terminated from the study, and represent the major difficulty with this form of therapy. Although this was primarily a study of interferon toxicity, of 12 evaluable patients, 3 had stable disease for 148, 192, and 539 days; 9 had progressive disease. In addition, we tested the effect of IFN-beta ser17 on the growth of early passage in vitro cultures of malignant gliomas established from patients. Growth inhibition varied from 0% to more than 50%. In all cultures evaluated, the combination of recombinant gamma-interferon plus IFN-beta ser17 enhanced growth inhibition. Further clinical and laboratory study is necessary to better define the therapeutic efficacy of IFN-beta ser17 and the role of combinations of interferons in the treatment of malignant gliomas.

Enhanced in vitro growth suppression of human glioblastoma cultures treated with the combination of recombinant fibroblast and immune interferons.

In the present study we have evaluated the effect of recombinant human fibroblast, IFN-beta ser, and immune, IFN-gamma, interferon, alone and in combination, on the proliferation of fifteen early passage human glioblastoma cell cultures. Explant cultures were established from glioblastoma tumor tissue obtained at the time of surgery. After sufficient outgrowth, cultures were dispersed with trypsin/versene and maintained as independent cell lines. IFN-beta ser induced a greater than or equal to 50% reduction in the 7 day growth of 6 of the 15 cultures. The majority of cultures, 9 of 15, displayed less than or equal to 50% growth suppression in comparison with control cultures after 7 days exposure to 2000 Units/ml of IFN-beta ser. When treated with 2000 Units/ml of IFN-gamma, only 1 of the 15 glioblastoma cultures exhibited a greater than or equal to 50% reduction in growth. In contrast, when treated with the combination of IFN-beta ser plus IFN-gamma, 1000 Units/ml of each interferon preparation, 12 of 15 cultures were inhibited by greater than or equal to 50% after 7 days growth. The combination of interferons was effective in suppressing glioblastoma growth both in cultures displaying relative sensitivity and those exhibiting innate resistance to either or both types of interferon when employed alone. One glioblastoma culture, G-7, was studied through 45 passages and displayed the same sensitivity at different passages to growth inhibition when exposed to IFN-beta ser, IFN-gamma or both interferons. Based on previous clinical studies indicating that IFN-beta or IFN-gamma when administered alone to patients do not generally alter the clinical progression of malignant gliomas, the present results suggest that the combination of IFN-beta plus IFN-gamma may prove more effective than either agent alone in the clinical treatment of patients with glioblastoma multiforme.

Combined clinical and computed tomographic diagnosis of orbital glioma and meningioma.

The clinical information on 22 patients with orbital optic nerve gliomas and 47 patients with meningiomas was correlated with computed tomographic findings obtained in both axial and coronal studies. Most of the gliomas occurred in children, although 7 patients presented after 20 years of age. Among the patients with meningiomas, the majority were women in early middle age, although two tumors occurred in children less than 20 years of age. Low grades of proptosis (median, 2 mm for both tumors), frequent significant visual field obscurations with eye movements, and opto-ciliary shunt vessels pointed toward the diagnosis of an optic nerve tumor. Patients with gliomas generally manifested massively swollen fusiform optic nerves with clear-cut margins due to circumscription by an intact dura. Kinks and bucklings of the optic nerve as well as infarctive cysts distinguished the glioma CT-scan patterns from the meningiomas. Distinctive axial CT-scan features of the meningiomas not shared by the gliomas were narrowly and diffusely enlarged nerves with polar expansions either at the orbital apex or immediately behind the globe; calcification; irregular excrescent margins signifying extradural invasion into the orbital soft tissues; a negative optic nerve shadow running down the center of the lesion; and bone erosion near the orbital apex. Coronal studies often revealed irregular margins signifying transgression of the dura. A diffusely and narrowly enlarged optic nerve shadow with regular margins (intrasheath lesions) was the one morphologically overlapping pattern displayed by 11 meningiomas and three gliomas. In these cases there tended to be more profound visual loss in the gliomas compared with the meningiomas, as well as the more frequent presence of opto-ciliary vessels in the meningiomas. Arteriography may be helpful in this particular category by demonstrating a tumor blush for the meningiomas, whereas this finding is typically absent with optic nerve gliomas. Meningiomas may be very closely simulated by dural or intraneural inflammations.

The architecture of optic nerve gliomas with and without neurofibromatosis.

A retrospective clinicopathologic study of 34 patients with optic nerve glioma disclosed two important architectural tumor forms: circumferential-perineural pattern featuring tumor eruption and proliferation in the subarachnoid space correlating with the presence of neurofibromatosis, and an expansile-intraneural pattern correlating with the absence of neurofibromatosis. The 18 patients with neurofibromatosis had a mean age of 4.9 years at presentation and tended to be younger than the 16 patients without neurfibromatosis (mean age, 12 years). Instead of "arachnoidal hyperplasia," electron microscopic studies in the electron microscopic studies in the circumferential-perineural pattern showed tumor astrocytes admixed with reactive meningothelial cells, fibroblasts, and collagen in distended subarachnoid space. Florid invasion of the leptomeninges (arachnoidal gliomatosis) exhibited by many optic nerve gliomas in neurofibromatosis suggests that they are true neoplasms rather than hamartomas, but their frequent location in the distal anterior visual pathway often confers a good clinical prognosis.

The specificity of serum prolactin as a diagnostic indicator of pituitary adenoma.

Serum prolactin levels were determined in 205 patients with a variety of intracranial diseases, including 70 cases with pituitary tumors. It is concluded that although the absence of elevated prolactin levels does not help to rule out pituitary pathology, the presence of hyperprolactinemia is highly specific for diseases of the pituitary and hypothalamus, and prolactin determination should be part of the regular work-up of pituitary tumor suspects. Excluding known causes of hyperprolactinemia, such as tranquilizing drug ingestion, the presence of neurological disease outside the hypothalamic-pituitary area was not associated with increased serum prolactin concentrations.

Neurofibromatosis and optic glioma: clinical and morphological correlations.

Although an association between optic glioma and neurofibromatosis is well recognized, there has been no previous analysis of cases relating the locus of tumor to the presence or absence of von Recklinghausen's disease. This paper presents the results of such an analysis as well as a description of the comparative histology of optic gliomas in patients with and without neurofibromatosis. We conclude that optic gliomas in patients with neurofibromatosis present preferentially as multicentric lesions or as lesions affecting the optic nerve alone without invasion of the chiasm, that these tumors frequently differ morphologically, and that patients with neurofibromatosis and optic glioma may have a clinical course different from that of patients with optic glioma unassociated with neurofibromatosis. Previously published reports are reviewed in light of these observations.

Luteinizing hormone-releasing hormone in human pituitary blood.

Luteinizing hormone (LH) and LH-releasing hormone (LHRH) were measured by radioimmunoassay in blood samples collected from the pituitary gland during transsphenoidal surgery in 19 patients. Detectable levels of LHRH were present in 12 patients. Wide fluctuations of LHRH were seen in sequential samples collected at 10-minute intervals, suggesting a pulsatile mode of release. This technique may yield useful data on hypothalamic control of pituitary secretion.

Period of risk for recurrence in medulloblastoma.

Ninety-nine patients with medulloblastoma who received surgery and radiotherapy, and had a statistically sufficient follow-up period were analyzed for factors influencing survival and the relevance of the "period of risk for recurrence" hypothesis. This postulate states that the period of risk for recurrence of a congenital tumor is equal to the age at presentation of illness plus 9 months' gestational time. The assumption is made that a tumor of embryonic origin will become manifest after a period of time determined by its inherent rate of growth and that tumor cells surviving treatment will multiply and present with recurrence in an equal period of time. Ten of 43 patients survived the period of risk, a presumed cure rate of 23%. None of these patients has subsequently developed evidence of tumor recurrence. Older patients at initial surgery had a somewhat greater survival rate for the first 5 years after treatment (10 of 26 older patients (38%) versus 15 of 54 younger patients (28%)), but by 10 years there was no appreciable difference in survival rates between those over 16 years of age and those younger. The beneficial effect of total neuraxis megavoltage radiotherapy is indicated by the improved 5-year survival rate from 9 of 41 patients (22%) to 16 of 39 patients (41%) with the newer techniques.

Microsurgical anatomy of the orbital apex and principles of transcranial orbital exploration.

Although many problems may present with unilateral proptosis, a careful clinical investigation can narrow the differential diagnosis to those problems which can be treated best by the neurosurgeon. The primary indication for transcranial exploration is when the pathological process is found to involve both the orbit and the cranial cavity as in all cases of optic nerve glioma, orbital meningioma, encephalocele, and some mucoceles, hemangiomas, aneurysmal bone cysts, and ossifying fibromas. Transcranial exploration may also be indicated for some tumors which crowd the orbital apex, such as, neurofibromas and osteomas; tumors in this location can be more widely exposed and safely dealt with by this route. A prime objective in these cases, however, must be preservation of function with a good cosmetic result. It is of equal importance to be aware that many conditions producing exophthalmos can be cured by direct orbital exploration, radiotherapy, or medical management. Malignant orbital tumors should never be exposed to the cranial cavity by transcranial exploration, whereas, radical exenteration may be curative in some carefully studied cases. Accuracy in clinical diagnosis and the proper selection of treatment modality for conditions in the orbit requires a clear understanding of this regional anatomy. I hope that this brief review of the pertinent microsurgical anatomy of the orbital apical region has helped to substantiate a strong neurosurgical claim to all primary optic nerve tumors; and, incidentally, I hope it provides an explanation for why I sent so many other patients back to Dr. Algernon Reese with the recommendation that transcranial surgery did not seem indicated. The success of this attitude may be attested to by the fact that we performed no unnecessary craniotomies nor did we pass by problems which would have best been treated by transcranial orbital exploration.

Adjuvant nitrosourea therapy for glioblastoma.

An attempt was made to evaluate the potential advantages of chemotherapy in the treatment of 62 patients with glioblastoma. Twenty-four of the 62 patients received adjuvant nitrosourea chemotherapy with carmustine (BCNU), lomustine (CCNU), or semustine (methyl CCCNU) in addition to surgery and radiotherapy. Thirty-three of the 62 patients were involved in a controlled, prospective, randomly allocated study. Quality or quantity of survival was not prolonged in patients who received chemotherapy. Age greater than 64 years, a severe postoperative neurological deficit, or the onset of symptoms less than 12 months prior to surgery were associated with a worse prognosis. The valid evaluation of the effect of a form of treatment on survival in patients with glioblastoma is contingent on the regorous avoidance of preselected factors that may predispose the treated group to a more favorable prognosis.

Empty sella syndrome as complication of benign intracranial hypertension.

An empty sella was demonstrated on air study in five patients with the benign intracranial hypertension (BIH) syndrome. All patients had a protracted course and very high cerebrospinal fluid pressure; two required a shunt procedure. No patient had any endocrine symptoms or visual field defects but an air study was done to exclude a mass lesion in the sella region. Among the last 50 patients seen with the BIH syndrome, there were five cases of an associated empty sella (10%). In these cases, the empty sella is a probable consequence of the long-standing intracranial hypertension associated with a congenital deficiency of the diaphragma sellae.

Experience with videotape monitoring of microscopic neurosurgical procedures.

This report describes technical details of a low-cost color videotape system for monitoring microneurosurgical procedures. The participation of audiovisual specialists was proven essential in insuring maximum effectiveness of these projects.