Tratamiento multimodal en los schwanomas vestibulares grandes / Multimodal Treatment for Large Vestibular Schwannomas

Introducción: Los schwanomas vestibulares son lesiones clasificadas como grado I por la Organización Mundial de la Salud. Las opciones conductuales incluyen la observación, la radiocirugía o la resección microquirúrgica endoscópica. Objetivo: Describir el rol del tratamiento multimodal en los schwanomas vestibulares. Métodos: Se realizó una búsqueda sistemática de literatura médica para la identificación e inclusión de artículos en las siguientes bases de datos: PubMed/Medline, SciELO y Google Scholar, además de los servidores de preprints BioRvix y MedRvix. Se usaron los siguientes descriptores: vestibular schwanoma OR acoustic neuroma AND nerve centered approach OR combined approach AND partial resection OR subtotal resection OR near total resection. Se excluyeron editoriales, cartas al editor, libros, revisiones, metaanálisis y aquellos artículos con método deficiente de más de 20 años de publicación o sin mención al tema de interés. Fueron incluidos 16 artículos. Desarrollo: Los artículos incluyeron un total de 699 pacientes, de los cuales solo 228 (32,6 porciento) recibieron radioterapia en el período posoperatorio. El período de seguimiento promedió 49,6 meses. La preservación de la función facial se logró en el 88,3 porciento de los casos y el control tumoral en el 80,7 porciento. El estado de la preservación auditiva solo fue informado en el 50 porciento de los estudios. El uso de la radioterapia adyuvante mostró gran variabilidad. Conclusiones: El tratamiento multimodal para los schwanomas vestibulares grandes -compuesto por la resección subtotal, vaciamiento intratumoral, seguido de radiocirugía- se ha convertido en una opción plausible. Se necesita la publicación de mayor cantidad de reportes para ofrecer recomendaciones y estratificar la conducta(AU)

Introduction: Vestibular schwannomas are lesions classified as grade I by the World Health Organization. Behavioral options include observation, radiosurgery or endoscopic microsurgical resection. Objective: To describe the role of multimodal treatment for vestibular schwannomas. Methods: A systematic search of medical literature, for the identification and inclusion of articles, was carried out in the databases PubMed/Medline, SciELO and Google Scholar, as well as in the preprint servers BioRvix and MedRvix. The following descriptors were used: vestibular schwannoma OR acoustic neuroma AND nerve centered approach OR combined approach AND partial resection OR subtotal resection OR near total resection. Editorials, letters to the editor, books, reviews and metaanalyses were excluded, as well as articles with deficient method of more than 20 years of publication or not mentioning the topic of interest. Sixteen articles were included. Development: The articles included a total of 699 patients, of which only 228 (32.6 ) received radiotherapy in the postoperative period. The average follow-up period was 49.6 months. Preservation of facial function was achieved in 88.3 percent of cases; and tumor control, in 80.7 percent. Hearing preservation status was reported in only 50 percent of the studies. The usage of adjuvant radiotherapy showed great variability. Conclusions: Multimodal treatment for large vestibular schwannomas -composed of subtotal resection and intratumoral draining, followed by radiosurgery- has become a plausible option. The publication of a greater amount of reports is necessary to provide recommendations and stratify the behavior(AU)

Resección microquirúrgica de schwannoma vestibular grado IVa con preservación de la función facial / Microsurgical resection of vestibular schwannoma grade IVa with preservation of facial function

Introducción: La cirugía de los schwannoma vestibulares constituye un desafío para los neurocirujanos. Debido a que se trata de un tumor benigno la resección completa de la lesión implica la curación del paciente. Sin embargo, este objetivo no siempre es fácil de lograr preservando la función de los nervios facial y acústico, especialmente en tumores de gran tamaño. Objetivos: Presentar detalles técnicos de la cirugía de resección de un schwannoma vestibular de gran tamaño (IVa) en el que se pudo preservar la función facial. Materiales y métodos: Se presenta el caso de una paciente femenina de 36 años que consultó por hipoacusia izquierda. En la RM preoperatoria se evidenciaba una lesión ocupante de espacio del ángulo pontocerebeloso izquierdo compatible con schwannoma vestibular con compresión del tronco encefálico y sin efecto de masa sobre el IV ventrículo (grado IVa). Mediante un abordaje suboccipital retromastoideo en posición de decúbito lateral se realizó la resección de la lesión en forma completa asistida por monitoreo del nervio facial. En todo momento se pudo preservar el plano aracnoideo que separaba el tumor de los nervios adyacentes. Resultados: Se logró una resección macroscópicamente completa con preservación de la función del nervio facial. La paciente permaneció internada por 96 hs en el postoperatorio sin complicaciones derivadas del procedimiento. Conclusión: La preservación del plano aracnoideo es un detalle técnico de mucha importancia para disminuir las posibilidades de lesión de los nervios facial y auditivo en la cirugía de resección de los schwannoma vestibulares.

Introduction: The surgery of vestibular schwannomas is a challenge for neurosurgeons.Because it is a benign tumor, complete resection of the lesion involves healing the patient. However, this objective is not always easy to achieve, preserving the function of both the facial and acoustic nerves, especially when dealing with large tumors. Objective: The objective of the video is to present some technical details of a large vestibular schwannoma (IVa) surgery in which the facial function could be preserved. Materials and methods: We present the case of a 36-year-old female patient who consulted for left hearing loss. The preoperative MRI showed a space- occupying lesion of the left pontocerebellar angle, which was compatible with vestibular schwannoma, with compression of the brainstem but with no mass effect on the IV ventricle (grade IVa). By means of a retromastoid suboccipital approach in the lateral prone position, the lesion was completely resected assisted by neurophysiological monitoring of the facial nerve. At all times, the arachnoid plane separating the tumor from the adjacent nerves was preserved. Results: A macroscopically complete resection was achieved preserving the facial nerve function. The patient stayed hospitalized for 96 hours during the postoperative period without any complication from the procedure. Conclusion: Preserving the arachnoid plane is a very important technical detail to reduce the possibilities of injury of the facial and auditory nerves in the vestibular schwannoma resection surgery.

The extracisternal approach in vestibular schwannoma surgery and facial nerve preservation / A abordagem extracisternal na cirurgia de schwannoma vestibular e preservação do nervo facial

The classical surgical technique for the resection of vestibular schwannomas (VS) has emphasized the microsurgical anatomy of cranial nerves. We believe that the focus on preservation of the arachnoid membrane may serve as a safe guide for tumor removal. Method The extracisternal approach is described in detail. We reviewed charts from 120 patients treated with this technique between 2006 and 2012. Surgical results were evaluated based on the extension of resection, tumor relapse, and facial nerve function. Results Overall gross total resection was achieved in 81% of the patients. The overall postoperative facial nerve function House-Brackmann grades I-II at one year was 93%. There was no recurrence in 4.2 years mean follow up. Conclusion The extracisternal technique differs from other surgical descriptions on the treatment of VS by not requiring the identification of the facial nerve, as long as we preserve the arachnoid envelope in the total circumference of the tumor. .

A técnica cirúrgica clássica para ressecção de schwannomas vestibulares enfatiza a anatomia microcirúrgica dos nervos cranianos. Acreditamos que o foco na preservação da membrana aracnóide pode servir como parâmetro seguro para a remoção do tumor. Método A abordagem extracisternal é descrita em detalhe. Analisamos o prontuário de 120 pacientes tratados com esta técnica entre 2006 e 2012. Os resultados cirúrgicos foram baseados em extensão de ressecção, recorrência tumoral e função do nervo facial. Resultados Ressecção total foi obtida em 81% dos pacientes. O resultado global da função do nervo facial (House-Brackmann graus I-II) após um ano da cirurgia foi de 93%. Não houve recidiva em um seguimento médio de 4,2 anos. Conclusão A técnica extracisternal difere de outras descrições cirúrgicas no tratamento de schwannoma vestibular pois não requer a identificação do nervo facial, contanto que o plano de aracnóide seja preservado em toda circunferência do tumor. .

Clinical research on 46 cases of microoperation for acoustic neurinomas under the F wave assisted electrophysiological monitoring intraoperatively / 肿瘤研究与临床

ObjectiveTo study the methods of how to protect facial nerve function following complete resection of acoustic neurinomas and the value of the techniques of F wave assisted electrophysiological monitoring intraoperatively.Methods Retrospectivelysummarizing theresultsof combining three electrophysiological monitoring techniques such as nasal muscle F wave recording,online EMG and triggered EMG to monitor 46 cases of microoperations for acoustic neurinomas intraoperatively during the period of Feb.2004 to Dec. 2008. Correlating every intraoperative monitoring index with their follow-up results of facial nerve function 1 day and 6 months after their operations.The tendency of the two continuous monitoring techniques between nasal F wave recording and online EMG of facial muscles has also been studied in this paper. Results Among 46 cases of acoustic neurinomas, 45(97.83 %) tumors have been totally resected, and 1 (2.17 %) tumor subtotally resected,lcase (2.17 %)died after operation,and 2ases occurred the leakage of cerebrospinal fluid(CSF) which have been cured through conservative treatment. The whole anatomic protection rate of facial nerve is 97.83 %,and their functional protection rates 6 months after operation are:HB Ⅰ - Ⅱ,75.56 %;Ⅲ-Ⅳ,22.22 % and Ⅴ-Ⅵ,2.22 %.The completely accordant rate between the intraoperative findings of nasal F wave recording and online EMG is 52.17 %, partially accordant rate is 45.65 %, and totally opposite rate is 2.17 % (x2 趋势= 6.113, P ＜0.05). The intraoperative monitoring indexes in nasal muscle F wave recording are correlated well with the facial nerve function in the 6th month' s follow-up (κ=0.429, P ＜0.001).In triggered EMG monitoring after tumors being resected,the stimulus threshold ratio and maximum amplitude ratio of facial nerve between leaving brain stem part and inner acoustic porus part are also correlated well with the facial nerve function 6 months after operation(κ=0.576, P ＜0.001; κ=0.595, P ＜0.001). ConclusionNasal muscle F wa recording cooperated well with online EMG and triggered EMG intraoperatively and correlates well with the postoperative facial nerve function, so they should be routinely applied together intraoperatively.

Neurinoma del acústico diagnosticado durante el embarazo: reporte de un caso / Acoustic neurinoma during pregnancy: a case report

Los tumores cerebrales son infrecuentes durante el embarazo. Los neurinomas del acústico pueden ser sintomáticos por primera vez durante la gestación y representan el 6-8 por ciento de las neoplasias intracraneales. El objetivo de esta comunicación es presentar el caso de una primigesta adolescente que presentó sintomatología neurológica de afectación del VIII par y de fosa posterior, característica de la neurofibromatosis tipo 2.

Brain tumors are infrequent during pregnancy. Neurinomas of auditive nerve can get to be symptomatic during gestation, representing 6-8 percent of the intracranial neoplasias. The objective is to report a case of a pregnant adolescent who present a neurological symptoms of VIII pair affectation and posterior cranial fossa, characteristic of the neurofibromatosis type 2.

Microsurgical resection of large acoustic neurinoma through suboccipital retrosigmoid approach / 中国微创外科杂志

Objective To evaluate the safety and efficacy of microsurgical resection of large acoustic neurinoma via the suboccipital retrosigmoid approach.Methods Forty-nine patients with large acoustic neurinoma(≥4 cm) underwent microsurgical resection through suboccipital retrosigmoid approach.The craniotomy was performed by way of an unilateral S-shaped suboccipital incision.With microsurgical techniques the outmost layer of the arachnoid membrane was preserved in order to avoid damaging to the surrounding vital structures.The tumor was resected intracapsularly from the superior pole and the internal auditory meatus was finally opened.The last pieces of tumor were removed by sharp dissection from the facial nerve bidirectionally,and were resected cautiously in a piecemeal fashion.Results Of the 49 patients,45 patients(92%) received a total resection of the tumors,and 4 patients(8%) subtotal resection.No patients died.The facial nerve was preserved anatomically in 42 patients(86%) and functionally in 36 patients(73%).The acoustic nerve was preserved anatomically in 7 patients(14%) and functionally in 3 patients(6%).One patient experienced a postoperative haematoma,and a re-operation was required.No recurrence was seen in 37 patients during a follow-up for 6 months ～ 5 years(mean,2.8 years).These patients had recovered for normal work and daily activities.Conclusions Microsurgical operation through suboccipital retrosigmoid approach is a favorable treatment for large acoustic neurinomas.The procedure can improve the rate of total resection,decrease the morbidity and mortality,and effectively protect the function of the acoustic and facial nerves.

Presigmoid Transpetrosal Approach for Recurrent Acoustic Neurinomas: Indications and Surgical Results

OBJECTIVE: In order to facilitate total removal with preservation of the facial nerve, the authors applied presigmoid(PS) approach in some patients with recurrent acoustic neurinoma(AN) patients who had undergone previous retrosigmoid(RS) approaches. The surgical outcomes of PS approach were retrospectively analyzed and compared to those of RS approaches and we suggest the indication of PS approach for recurrent AN. METHODS: From 1989 to 1999, twenty-one of 183 operated AN patients underwent re-operation due to regrowth of the residual tumors. Nine of the 21 recurrent tumors were removed by PS approach and 12 cases underwent RS approach. The surgical extent of removal and the facial nerve preservation rate were compared between the two different approaches. RESULTS: In PS approaches, the total removal was achieved in four patients(44%) and the facial nerve could be identified and preserved anatomically in all patients. Among 12 cases who underwent RS approaches, the rate of total removal was 44% and the facial nerve identified and preserved in only 5 cases (42%). The rate of facial nerve preservation was significantly different between two modes of approaches (p=0.0007). CONCLUSION: PS transpetrosal approach is recommended in recurrent AN patients who had underwent RS approach previously and already lost the hearing. Early identification of the facial nerve and easy removal of the tumors can be achieved using the PS approach.

Some opinions of diagnosis of acoustic neurinomas by CT scanner and magnetic resonance image

Exact diagnosis of the acoustic neurinomas depends on CT Scanner and Magnetic Resonance Imaging 41 of these 47 cases were done CT Scanner and MRI with 12 cases revealed the wonderful positive results in diagnosis. So it is very easy in order to choose the procedure in the treatment via CT Scanner and MRI.

Microsurgical treatment of large acoustic neurinoma and facial nerve preservation / 中国综合临床

Objective To explore the microsurgical technique and results of the large acoustic neurinoma and the facial nerve preservation.Methods 35 cases of large acoustic neurinoma treated microsurgically were analyzed retrospectively.Results Total resection was achieved in 33 patients,subtotal in one patient and partial in one patient.The facial nerve was preserved completely in 32 cases (91.4%).One died postsurgery.Conclusion The application of microsurgical techniques and rational selection of operational approach can remarkably increase the total removal rate and facial nerve preservation rate.

Microsurgical treatment of large acoustic neurinoma / 中华显微外科杂志

Objective To discuss microsurgical experience for transsuboccipital retrosigmoid sinus approach of large acoustic neuronomas,and improve the rate of tumor removing,and facial nerve and auditory nerve preserving Methods A retrospective analysis of 39 patients with acoustic neuronomas which were more than 4 cm in diameter were treated by microsurgery between January 1994 and December 2000 Tumor's excision was performed by transsuboccipital retrosigmoid sinus approach The surgical main points of transsuboccipital retrosigmoid sinus approach,and their experience of total removal,and rate of facial nerve and auditory nerve preservation were described Results The tumors were totally removed microsurgically in 34 patients,the total removal rate was 87 2%,and subtotal removal in 5 patients Facial nerve was anatomically preserved in 31 cases,the rate of facial nerve preservation was 79 5%,but the function of facial nerve was recovered in 22 cases (56 4%) The auditory nerve was preserved anatomically in 15 cases (38 5%) and functionally in 7 cases (17 95%) Conclusions An minimally invasive microsurgical treatment are crucial for increasing a satisfactory living quality of the patients It is considered that the total removal rate of tumor,the preservative rate of facial nerve and auditory depend upon microsurgical technique and intraoperative facial nerve monitoring in acoustic neuromas surgery

A Study on the Selection Criteria of Surgical Approaches to Vestibular Schwannomas

This study was carried out in order to clarify the selection criteria of the surgical approaches and validate their efficacy in patients with vestibular schwannoma. The evolution and modification of the transpetrosal approach was evolved into combined approaches to vestibular schwannomas. These approaches have been advocated to reduce the mortality and morbidity as well as to improve the surgical results especially in large acoustic neurinoma. Having been stimulated with the fascinating preliminary surgical results of the suboccipital and combined transpetrosal approaches, we have builded up a decision making policy based on the tumor size and the preoperative hearing status for the surgical approaches to vestibular schwannomas. Between May 1996 and September 1997, 21 patients with vestibular schwannoma including one case of NF II underwent either suboccipital or various transpetrosal approaches. There was no surgical mortality. Gross total removal was achieved in 20 patients(95%). Facial nerve function was preserved in 19(90%) and hearing function in 6(46%) among the 13 patients who underwent hearing preservation surgery. The authors confirmed the validity of various transpetrosal approaches in this study and conclude that our policy on the surgical approaches to vestibular schwannomas is quite reasonable.

Supratentorial Epidural Hematoma as a Complication of Acoustic Neurinoma Surgery

The authors report a case of postoperative supratentorial epidural hematoma as a complication of acoustic neurinoma surgery. The 43-year-old man presented with hearing difficulty on the left side and decreased facial sensation. MR imaging demonstrated a huge, cystic left acoustic neurinoma and the retromastoid suboccipital approach was used for gross-total resection of the tumor. Postoperatively, the patient was drowsy and showed right hemiparesis. Computed tomographic scanning revealed a huge epidural hematoma in the left parietooccipital convexity. The intraoperative course was uneventful. Immediate craniotomy was performed and the epidural hematoma was removed. Bleeding from the superior sagittal sinus occurred, but was completely controlled. Postoperatively, the patient was neurologically normal, except for mild left facial weakness. Remote postoperative intracranial hemorrhage is a rare complication of craniotomy with significant morbidity and mortality. Such hemo-rrhages are likely to develop at or soon after surgery and can be related to the craniotomy site, operative positioning, and nonspecific mechanical factors. In this case, mechanical displacement of the brain secondary to excessive dehydration and CSF drainage was thought to be the cause of postoperative epidural hematoma. Clinical awareness of this rare but potential complication is essential to its early diagnosis and treatment. Difficulty in awakening from anesthesia and the development of new neurological deficits not attributable to the operative site are the most important keys to early diagnosis. Computed tomography is the diagnostic method of choice.

Contralateral Trigeminal Neuralgia Caused by Acoustic Neurinoma

Acase of trigeminal neuralgia caused by a contralateral acoustic neurinoma is presented. After removal of the tumor, the neuralgic pain has completely disappeared. The pathophysiology of this entity is briefly reviewed. The neuralgic pain may be caused by the compression of the contralateral trigeminal nerve by the mass effect in this case.

Surgical Results of Large Acoustic Neurinomas: Preoperative Prediction of Facial Nerve Displacement by MRI

A total of 22 patients(13 males and 9 females;mean age 49 years) with large acoustic neurinoma who underwent the retrosigmoid transmeatal approach from May 1993 to May 199 at the Chonnam University Hospital was reviewed retrospectively. The authors investigated the surgical results and accuracy in predicting the direction of displaced facial nerve which was determined by preoperative magnetic resonance(MRI) findings. In all cases the preferred method was the suboccipital transmeatal approach. The direction of displacement of the facial nerve could be predicted by preoperative axial and coronal MRI scans and verified intraoperatively. Gross total removal was performed in 82%, the accuracy rate for facial nerve displacement was 77% and anatomical preservation was accomplished in 82.3% of the totally removed cases. During follow-up, good functional outcomes were achieved in 91% and fair in 4.5%. The authors conclude that such good surgical results can be attributed to advances in microsurgical technique and intr aoperrative facial monitoring. In addition, preoperative prediction of the direction of displaced facial nerves has significantly reducd the incidence of severe facial nerve weakness.

Diagnosis and Management of Patients with Neurofibromatosis Type 2 : Experience of 16 Cases

Neurofibromatosis type 2(NF-2) is a dominantly inherited disorder characterized by the occurrence of bilateral acoustic neurinomas and the frequent association of other central nervous system tumors. We present a retrospective review of 16 patients with NF-2 who were treated at our hospital from 1984 to 1995. In 13 cases, the diagnoses of NF-2 were based on the criteria developed at the Consensus Development Conference of National Institute of Health in the United States, and in another 3 cases, the criterias of NF-2 were not fully satisfied, but the diagnoses of NF-2 were highly suspected. The average age of the patients was 27.6 years, ranging from 13 years to 56 years. The most common symptom was hearing difficulty;intervals between symptom onset and deafness ranged from 8 months to 6 years(mean:2.9 years.) One family of NF-2 was documented, consisting of a sister, a brother and their mother. Nine patients underwent operations on unilateral acoustic neurinomas;these were subtotally removed in eight patient s and totally in one patient. Among these patients, five were deaf on the ipsilateral side at surgery. Among the other four patients with useful hearing before surgery, hearing was preserved to preoperative status in two patients. Four patients with diagnoses of meningioma, received operations to relieve mass effect with subtotal removal in two patients and total removal in the other two. Early diagnosis and treatment are the most important in the management planning of patients with NF-2 for reasons of early manifestation and rapid progression of the disease.

Clinical Experience of Gamma Knife Rediosurgery for Acoustic Neurinomas

Microsurgical tumor removal is the treatment of choice to relieve the mass effect for the treatment of acoustic neurinoma patients. Gamma knife radiosurgery is another treatment option for patients with tumor size of less than approximately 4cm. Between May, 1990 and March, 1994, 55 tumor lesions from 50 patients were treated with gamma knife radiosurgery at Asan Medical Center. Following microsurgery, 20 patients underwent gamma knife radiosurgery for tumors not removed surgically. The remaining 30 patients underwent gamma knife radiosurgery alone. For an average follow-up period of 26 months(ranging from 6 to 45 months), 37 lesions out of 39 lesions responded, giving a tumor growth control rate of 94.9%. Facial neuropathy and trigeminal neuropathy were noted in 8% and 6%, respectively. According to these results, it is suggested that gamma knife radiosurgery in an effective altrnative therapeutic modality for the management of small to moderate sized acoustic neurinomas.

The Translabyrinthine Approach for Removal of the Acoustic Neurinomas: Comparison with the Suboccipital Approach

The result of a series of thirteen translabyrinthine removals of acoustic neurinomas are reported. From 1990 March to 1993 May, thirteen patients with acoustic neuromas underwent translabyrinthine removal at Seoul National University Hospital with cooperation of Department of Neurosurgery and Otolaryngology. In four patients with the medium-sized(11-20 mm) tumors, complete removal with preservation of the facial nerve was possible. In nine patients with the large(21-40 mm) tumors, complete removal of the tumor was possible in seven. Preservation of the facial nerve was possible in all but one, whose facial nerve was incidentally transected at the distal intracanalicular portion and was anastomosed end-to-endly. In one, postoperative leakage of cerebrospinal fluid required reoperation. The results of translabyrinthine approaches were compared with those of suboccipital approaches performed during the same period. In medium-sized or large acoustic tumors, translabyrinthine approach was comparable with suboccipital approach in the rate of total tumors removal, preservation of the facial nerves, and complication.

Surgical Treatment of Facial Nerve Injury Following Removal of Acoustic Neurinoma

To evaluation effectiveness of internal anastomosis and facial-hypoglossal anastomosis the medical records of 29 patients with facial nerve injury in the course of operation for acoustic neurinoma were reviewed. In 8 patients, the interrupted facial nerves were anastomosed at the end of tumor operation, and facial-hypoglossal anastomoses were performed in 20 patients. One patient underwent facial-hypoglossal anastomosis 6 months after internal anastomosis. All the patients had been treated from January 1983 to March 1993 and were followed up for more than 1 year. After primary internal anastomoses, 7 patients resulted in symmetric face at rest and only one failed to show improvement of facial muscle tone. The mean time to the first evidence of facial reinnervation was 5.3 months. In 20 patients with facial-hypoglossal anastomosis, improvement of facial muscle tone was demonstrated in 19 patients and in one patient there was no improvement. Thirteen cases(68%) showed facial symmetry at rest and 5 of them could close their eyes completely. The first evidence of reinnervation was noted between 3 and 12 months after anastomosis with a mean of 6.3 months. The anastomoses were performed between 8 days and 22 months after injury of the facial nerve, and the time to reinnervation after anastomosis was significantly influenced by the duration of nerve injury(P=0.0439). Primary internal anastomosis revealed better recovery of facial muscle function compared with facial-hypoglossal anastomosis(P=0.0299).

A Case of Neurofibromatosis 2 with Multiple Intracranial and Intraspinal Tumors:Neurofibromatosis 2(NF2)

Neurofibromatosis 1(NF-1, Von Recklinghausen disease) and Neurofibromatosis 2(NF-2, bilateral acoustic neurinoma) have been established as distinct disorders by the National Institutes of Health(NIH) Consensus Development Conference in 1988. We recently have encountered a patient of NF-2 who develop multiple intracranial and spinal tumors 12 years after the surgical treatment of Schwannoma of lumbar nerve roots. The tumors were right acoustic neurinoma, left cerebellopontine angle(CPA) meningioma, multiple intracranial meningiomas, intraorbital glioma, multiple ependymomas of the brain stem and cervical spinal cord, and meningiomas of cervical nerve roots. We present this case with a review of reported cases of neurofibromatosis.

Clinical Report of 46 Intracranial Tumors with LINAC Based Stereotactic Radiosurgery / 대한치료방사선과학회지

Between July 1988 and December 1992, we treated 45 patients who had deep seated inoperable or residual and/or recurrent intracranial tumors using LINAC based stereotactic radiosurgery at the Department of Therapeutic Radiology, Kangnam St. Mary's Hospital, Catholic University Medical College. Treated intracranial tumors included pituitary tumors(n=15), acoustic neurinomas(n=8), meningiomas(n=7), gliomas(n=6), craniopharyngiomas(n=4), pinealomas(n=3), hemangioblastomas(n=2), and solitary metastatic tumor from lung cancer (n=1). The dimension of treatment field varied from 0.23 to 42.88 cm3(mean ; 7.26 cm3). The maximum tumor doses ranging from 5 to 35.5 Gy (mean; 29.9 Gy) were given, and depended on patients' age, target volume, location of lesion and previous history of irradiation. There were 22 male and 23 female patients. The age was varied from 5 to 74 years of age(a median age; 43 years). The mean duration of follow-up was 35 months (2~55 months). To date, 18(35.1%) of 46 intracranial tumors treated with SRS showed absent or decrease of the tumor by serial follow-up CT and/or MRI and 16(34.8%) were stationary, e.g. growth arrest. From the view point of the clinical aspects, 34(73.9%) of 46 tumors were considered improved status, that is, alive with no evidence of active tumor and 8(17.4%) of them were stable, alive with disease but no deterioration as compared with before SRS. Although there showed slight increase of the tumor in size according to follow-up imagings of 4 cases(pituitary tumor 1, acoustic neurinomas 2, pinealoma 1), they still represented clinically stable status. Clinically, two(4.4%) patients who were anaplastic astrocytoma(n=1) and metastatic brain tumor(n=1) were worsened following SRS treatment. So far, no serious complications were found after treatment. The minor degree headache which could be relieved by steroid or analgesics and transient focal hair loss were observed in a few cases. There should be meticulous long term follow-up in all cases.