Episodes of prolonged “trance-like state” in an infant with hypothalamic hamartoma

Hypothalamic hamartoma (HH) is one of the most common causes of central precocious puberty (CPP) in first few years of life. It can present with either seizures or CPP, although both manifestations coexist in the majority of the children. Gelastic seizures (GS), or laughing spells, are usually the first type of seizures seen in patients with HH. Although a wide variety of seizure types are known to occur in children with HH, GS are most common and consistent seizure type. The clinical presentation of HH may vary with the size and position of the mass, although large tumours typically present with both CPP and seizures. Although CPP can be managed with medical therapy, seizures can be very difficult to treat, even with multiple antiepileptic drugs. Noninvasive gamma knife surgery has been used with some success for the treatment of refractory epilepsy. We present a case of HH with very early onset seizures and CPP. The patient had an atypical form of seizures described by the parents as a "trance-like state" in which the patient had prolonged episodes of unresponsiveness lasting for hours with normal feedings during the episodes. GS occurred late in the course and were refractory to various combinations of antiepileptic drugs. A brain magnetic resonance imaging showed a large sessile HH (>20 mm). Later in the course of the disease, the patient experienced cognitive and behavioural problems. The patient underwent gamma knife surgery at nearly 5 years of age and experienced a modest response in seizure frequency. This case highlights the presentation of HH as a previously unreported seizure morphology described as a prolonged "trance-like state."

Gamma knife surgery for pineal region tumors: an alternative strategy for negative pathology / Cirurgia com gamma knife como tratamento experimental para tumores da região da pineal: uma estratégia alternativa quando os dados anatomopatológicos são negativos

Objective : Pineal region tumors (PRTs) are uncommon, and treatments vary among neoplasm types. The authors report their experience with gamma knife surgery (GKS) as an initial treatment in a series of PRT patients with unclear pathological diagnoses. Method : Seventeen PRT patients with negative pathology who underwent GKS were retrospectively studied. Nine patients had further whole-brain and spinal cord radiotherapy and chemotherapy 6–9 months after GKS. Results : Sixteen of 17 cases were followed up over a mean of 33.3 months. The total response rate was 75%, and the control rate was 81.3%. No obvious neurological deficits or complications were attributable to GKS. Conclusion : The findings indicate that GKS may be an alternative strategy in selected PRT patients who have negative pathological diagnoses, and that good outcomes and quality of life can be obtained with few complications. .

Tumores da região da pineal (TRP) são pouco frequentes e as propostas de tratamento são bastante variadas. Os autores relatam sua experiência em cirurgias com uso gamma knife (CGK) como tratamento experimental inicial em séries de TRP que não têm diagnóstico anatomopatológico ou nos quais o diagnóstico não ficou claro. Foram estudados retrospectivamente 17 pacientes com TRP nestas condições e que foram submetidos a CGK. Destes, 9 pacientes foram submetidos posteriormente a radioterapia de todo o encéfalo e medula espinhal entre 6 e 9 meses depois da CGK. Dezesseis dos 17 pacientes foram acompanhados por um período médio de 33,3 meses. A taxa total de resposta nos pacientes foi de 75% e a taxa dos controles, 81,3%. Não houve nenhum déficit neurológico evidente que pudesse ser atribuído à CGK. A CGK como tratamento experimental pode ser uma estratégia alternativa no grupo específico de pacientes com TRP em que não há diagnóstico anatomopatológico, podendo ser obtida uma boa qualidade de vida com poucas complicações para esse grupo de pacientes.

Gamma Knife Surgery for Brain Metastases from Breast Carcinoma

PURPOSE: This study was performed to evaluate the feasibility of the Gamma Knife Surgery on the symptomatic control of brain metastasis from breast carcinoma. METHODS: We retrospectively reviewed patients with brain metastases from breast cancer who underwent Gamma Knife Surgery at our hospital, between May 2004 and November 2007. Total 82 metastases were treated with 26 cycles of Leksell gamma knife. Freedom from local recurrence and survival time were analyzed by the Kaplan-Meier method. RESULTS: 17 patients with 82 metastases were treated over 26 Gamma Knife Surgery sessions. The mean time to brain metastases was 41.7 months (8~84), median number was 2.0 (1~10), and median volume was 7.4 cm3 (0.6~25.4). Radiologic response occurred in 84.6%, and the rate of symptom relief was 73.1%. Local brain tumor recurrences were observed in 15.3% and intracranial distant recurrence occurred in 57.7% that occurred within 3.2 months. The median length of survival for all patients was 9.3 months (95% confidence interval, 4.23~9.37 months). CONCLUSION: Gamma Knife Surgery is an effective and feasible treatment for symptomatic control of brain metastases from breast cancer patients who have severe extracranial metastases and short life expectancy.

Gamma Knife Surgery for Brain Metastasis from Renal Cell Carcinoma: Relationship Between Radiological Characteristics and Initial Tumor Response

OBJECTIVE: The authors have speculated that metastatic brain lesions from renal cell carcinoma (RCC) show diverse radiological patterns and tumor responses after Gamma knife surgery (GKS), and have hypothesized that these can be predicted from tumor radiological characteristics. The goal of the current study was to identify the radiological characteristics of RCC brain metastases and the predictors of initial radiosurgical response after GKS. METHODS: A retrospective analysis was performed on 48 lesions in 18 patients with RCC brain metastasis treated by GKS. The radiological characteristics of these lesions in magnetic resonance images (MRI) were classified into 3 categories according to enhancement patterns in T1-weighted images and signal intensity characteristics in T2-weighted images. Responses to GKS were analyzed according to these categories, and in addition, other potential predictive factors were also evaluated. RESULTS: MRI findings in the three categories were diverse, though numbers of the lesion were comparable. At 2-month MRI follow-ups after GKS, response rate was 54% and the local tumor control rate 83%. T2 signal intensity was found to be the principal predictive factor of response to GKS, namely negative predictive factor. Other variables such as age, sex, tumor volume, dose, duration from initial diagnosis to GKS, and previous systemic therapies failed to show significant relationships with treatment response by multivariate analysis. CONCLUSION: Careful evaluation of the radiological characteristics of brain metastases from RCC is important prior to GKS because MRI heterogeneity has predictive value in terms of determining initial tumor response.

Gamma Knife Surgery for the Pineal Region Tumors

OBJECTIVE: Gamma Knife Surgery(GKS) for the management of pineal region tumors is challengeable strategy as direct access to this area is not easy. The experiences of pineal region tumor patients treated with GKS were analyzed to evaluate the effectiveness. METHODS: Seven patients with tumors in the pineal region were treated with GKS between September 1998 and May 2005. The histological diagnosis were pineal parenchymal tumor (2 patients), low-grade astrocytoma (2 patients), immature teratoma (1 patient), and choriocarninoma (1 patient). One patient was diagnosed as metastatic brain tumor based on histological diagnosis for primary site and brain imaging study. The median marginal dose was 15Gy (range; 11~20) at the 50% isodose line. The median target volume was 2.5cm3 (range; 0.8~12.5). The median clinical follow up period was 29 months (range; 13~93) and the median radiological follow up period was 18 months (range; 6~73). RESULTS: Tumor volume measured in follow-up images showed reduction in six patients, disappearance in one. No adverse effect due to GKS was found during the follow-up period . The performance status was preserved in all patients except one who died due to progression of primary cancer in spite of controlled metastatic brain lesion. CONCLUSION: Gamma Knife Surgery can be applied to pineal region tumors irrespective of their histology whenever surgery is not indicated.

Quality of Life in Patients with Vestibular Schwannoma after Treatment: Short Form-36 Based Study

OBJECTIVE: To evaluate the quality of life(QOL) of patients with vestibular schwannoma(VS) and to compare QOL among subtotal resection(STR) group, radical resection(RR) group, and gamma knife surgery only (GKS) group, we report a retrospective analysis of consecutive 104 patients with VS who were treated between 1997 and 2000. METHODS: The STR group included 31 patients, RR group did 26, and GKS only did 47. Various approaches such as retrosigmoid transmeatal approach(45), translabyrinthine approach(4), transotic approach(2), middle fossa approach(3), and combined approach(3) were used. A questionnaire concerning postoperative symptoms and the Short Form 36(SF-36) QOL instrument were mailed to 104 patients with VS. Follow-up imaging studies, Karnofsky performance scale(KPS) scores, and SF-36 scores at the last follow-up were assessed. The mean duration of follow-up was 33.4 months. RESULTS: The survey response rate was 51%(53 patients). The postoperative QOL in VS patients, as quantified by the eight SF-36 health scales was less than the appropriate matched healthy standard. Furthermore there were differences between the doctor's assessment(KPS) and the patient's assessment(SF-36). Facial nerve outcome had little influence on QOL in VS patients. CONCLUSION: Patients with VS have a significant impairment of their QOL. In the selection of treatment options for VS, the QOL of patient should be considered seriously.

Pilocytic Astrocytoma Occuring in a Patient Treated with Gamma Knife Surgery: Case Report

We report a case of pilocytic astrocytoma and atypical meningioma occurred within the field of gamma knife surgery for the management of preexisting meningioma. A 76-year old woman received gamma knife surgery for the management of meningioma in the right parietal convexity 9 years ago. Three weeks before admission, left hemiparesis and speech disturbance were developed and magnetic resonance image showed cystic mass with nodular enhancement in the right parietal area. Craniotomy and total removal of mass was performed and the histological diagnosis of atypical meningioma and pilocytic asrtocytoma were done.

Gamma-Knife Radiosurgery for Vestibular Schwannoma

OBJECT: The goals of radiosurgery include preservation of neurological function and prevention of tumor growth. We document the results of gamma-knife radio-surgery for vestibular schwannoma. METHOD AND OBJECT: Eighty-two patients underwent stereotactic radiosurgery for an vestibular schwannoma from October, 1994 to December, 2000. Sixty-five of these patients were followed up for radiological and clinical evaluation. As pregamma-knife modality, surgical resection were done in 23 patients,and V-P shunt in 2 patients. Initial symptoms were headache(n=45), dizziness(n=16), tinnitus(n=17). While normal facial function(House-Brackmann grade 1) was present in 48 patients(73.8%), other patients showed grade 2 function in 8, grade 3 function in 7,and grade 4 function in 2. The Gardner/Robertson scale was used to code hearing function. Male to female ratio was 1:3. Mean tumor volume was 7.98cm3. Mean dose delivered to the tumor margin was 14.2Gy,and mean maximal dose was 28.3Gy. RESULTS: Mean follow-up duration of 19.9 months. Thirty-five showed decrease(53.8%) in size, 19 patients(29.2%) stationary, 3(4.6%) initial decrease follow up increase, 5(7.6%) initial increase follow up decrease,and 59 patients (90.8%) were well controlled. Two patients experienced transient facial neuropathy, one transient trigeminal neuropathy, and one transient hearing deterioration. After gamma-knife radiosurgery, ventriculoperitoneal shunt was done in 4 patients. CONCLUSIONS: Gamma-knife radiosurgery can be used to treat postoperative residual tumors as well as in patients with concomitant medical problems in patients with preserved hearing function. Gamma-knife radiosurgery is safe and effective method to treat small, medium sized(less than 3cm in extracanalicular diameter), intracanalicular vestibular schwannoma, associated with low rate of cranial neuropathy.

Treatment Strategy of Growth Hormone Secreting Pituitary Adenoma

The goals of the pituitary tumor surgery are restoration of the normal pituitary function and relieving the mass effect on the adjacent normal brain structures. The main purpose of this study is to analyze our surgical results of growth hormone(GH) secreting pituitary adenomas which were treated extensive and aggressive surgical removal. The surgical results were reviewed in 36 patients treated between 1993 and 1997. The criteria of clinical remission are postoperative basal GH less than 5ng/ml and suppression of GH less than 2ng/ml in oral glucose tolerance test. As the results, 25 of 36 patients(69.4%) achieved surgical remission. Eleven patients who could not achieve remission by surgery were treated with medical treatment(octreotide, bromocriptine) and Gamma-knife surgery. In conclusion, the tumor morphology and extensiveness of tumor removal were well correlated with surgical outcome, and the aggressive resection of tumor seems to be the most important factor achieving remission in GH secreting pituitary adenomas.

The Effect of Gamma Knife Surgery on Uveal Melanoma

The optimal management of uveal melanoma is still a matter of controversy. To determine the effect of Gamma Knife surgery on patients with uveal melanoma, the authors reviewed the outcome of five operations performed between September 1993 and August 1996. The mean age of the patients was 60.7(range 42 to 76) years; the median follow-up period was 10 months, and four patients were followed up for more than 6 months. The mean tumor volume was 3442mm3(mean diameter 15.3mm) and all patients were irradiated with a mean maximum dose of 74Gy (range 60-80Gy), using a 50% isodose on the tumor margin. In one patient, the tumor disappeared completely 32 months after Gamma Knife surgery; because the tumor did not regress, one patient subsequently required enucleation, and two remained stable. During a mean follow-up period of 10 months, vision was preserved in two patients, but one went blind; in one, enucleation was performed because the tumor did not regress. These results suggest that in cases of uveal melanoma Gamma Knife surgery can effectively control local tumors, can spare the eyeball, and may prevent loss of vision.