Medulloblastoma in childhood: an epidemiological study

1984 ◽  
Vol 61 (4) ◽  
pp. 657-664 ◽  
Author(s):  
Jacqueline R. Farwell ◽  
George J. Dohrmann ◽  
John T. Flannery
Keyword(s):  
Mortality Rate ◽  
Survival Rates ◽  
Female Ratio ◽  
Tumor Registry ◽  
Content Type ◽  
Probability Of Survival ◽  
Number Of Children ◽  
Male To Female ◽  
Fine Print

✓ The authors have reviewed 143 cases of medulloblastoma in children aged 19 years or younger who were treated in a 42-year period and reported in the Connecticut Tumor Registry. About 20 cases have occurred in each 5-year period since 1950, but 31 were seen between 1955 and 1959. Correspondingly, an excessive number of children born in the period 1954 to 1958 have developed medulloblastomas. A relationship to polio vaccine contaminated with SV40 virus may exist. Children with medulloblastomas had an increased number of immediate family members with brain tumors, leukemia, and childhood cancer when compared to controls. In this series, the male to female ratio was 1.33:1. Average age at diagnosis was 6½ years, with most children being diagnosed at 3 years old and fewer cases appearing in each successive hemidecade from birth to 20 years of age. Probability of survival at 6 months was 0.687; at 1 year, 0.444; at 2 years, 0.314; and at 5 years, 0.222. Survival probability was statistically significantly better in the years 1968 to 1977 than in previous decades, in part due to fewer autopsy diagnoses and lowered operative mortality, but also due to a decreased mortality rate in children several years after diagnosis. Fifty-one percent were treated with operation and irradiation, 17% with operation alone, 12% with irradiation alone, and 5% with operation, irradiation, and chemotherapy. Fifteen percent were not treated. One- and 5-year survival rates in patients with operation and irradiation were, respectively, 0.615 and 0.307; with operation, 0.125 and 0.042; with irradiation, 0.688 and 0.277; and with operation, irradiation, and chemotherapy, 0.857 and 0.643. All seven children who received chemotherapy were diagnosed after 1968, and five are still alive. Perhaps due to short follow-up time, the course and mortality rate of children treated with all three modalities were not statistically significantly different from those of children treated since 1968 with operation and radiation therapy.

Astrocytomas of the spinal cord in children and adolescents

1985 ◽  
Vol 63 (5) ◽  
pp. 669-675 ◽  
Author(s):  
Ronald Reimer ◽  
Burton M. Onofrio
Keyword(s):  
Spinal Cord ◽  
Histological Grade ◽  
Extended Period ◽  
Spinal Deformities ◽  
Duration Of Symptoms ◽  
Female Ratio ◽  
Content Type ◽  
Orthopedic Procedure ◽  
Male To Female ◽  
Fine Print

✓ The authors review 32 cases of spinal cord astrocytoma in patients under 20 years of age who were treated at the Mayo Clinic between 1955 and 1980. There was a 1.3:1 male to female ratio. Twenty patients were between 6 and 15 years of age at the time of diagnosis. The duration of symptoms prior to definitive diagnosis varied from 5 days to 9 years, with an average of 24 months. The most common symptoms were pain (62.5%), gait disturbance (43.7%), numbness (18.8%), and sphincteric dysfunction (18.8%). The most common neurological findings were a Babinski response (50.0%), posterior column sensory dysfunction (40.6%), and paraparesis (37.5%). A median follow-up period of 8.6 years (range 0.8 to 25.5 years) revealed that the survival time diminished with increased histological grade of the astrocytoma (p < 0.001). The development of postlaminectomy spinal deformities represented a serious postoperative complication. This occurred in 13 patients and was first recognized between 8 and 90 months postoperatively. Six deformities occurred following cervical laminectomy, and eight patients required at least one orthopedic procedure. It is crucial to follow these patients for an extended period of time to watch for postoperative spinal deformities.

Age at symptom onset and long-term survival in patients with neurofibromatosis Type 2

2003 ◽  
Vol 99 (3) ◽  
pp. 480-483 ◽  
Author(s):  
Goro Otsuka ◽  
Kiyoshi Saito ◽  
Tetsuya Nagatani ◽  
Jun Yoshida
Keyword(s):  
Symptom Onset ◽  
Survival Rates ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Vestibular Schwannomas ◽  
Content Type ◽  
Fine Print

Object. Neurofibromatosis Type 2 (NF2) is an intractable disorder predisposing to multiple, recurrent tumors of the central nervous system (CNS). To clarify the survival rate and characteristics that predict poor survival, we retrospectively reviewed clinical data in cases of NF2. Methods. From among 283 patients with neurofibromatosis who had been registered in a nationwide study in Japan between 1986 and 1987, 74 patients with bilateral vestibular schwannomas were analyzed. The mean duration of follow up after diagnosis was 121 months (range 2–287 months). Results of a Kaplan—Meier product-limit analysis indicated that overall 5-, 10-, and 20-year patient survival rates following diagnosis of NF2 were 85, 67, and 38%, respectively. Early onset of the initial symptom significantly compromised survival; 5-, 10-, and 20-year survival rates in patients with symptom onset at an age younger than 25 years were 80, 60, and 28%, respectively, whereas in patients with symptom onset at an age of 25 years or older the rates were 100, 87, and 62%, respectively. Patients with small vestibular schwannomas at diagnosis (< 2 cm in diameter) had better rates of survival. Other variables such as sex, additional tumors in the CNS, or dermal abnormalities did not significantly affect survival. Conclusions. This first report of long-term follow-up results concerning the survival of patients with NF2 indicates an adverse effect of early symptom onset.

Primary non-Hodgkin's lymphoma of the nose and nasopharynx: clinical features, tumor immunophenotype, and treatment outcome in 113 patients.

1998 ◽  
Vol 16 (1) ◽  
pp. 70-77 ◽  
Author(s):  
M M Cheung ◽  
J K Chan ◽  
W H Lau ◽  
W Foo ◽  
P T Chan ◽  
...  
Keyword(s):  
Clinical Features ◽  
Survival Rates ◽  
Chinese Patients ◽  
Female Ratio ◽  
Cd56 Expression ◽  
T Lymphomas ◽  
Hodgkin's Lymphomas ◽  
Male To Female ◽  
Disease Free

PURPOSE To study the clinical features and outcome for primary non-Hodgkin's lymphomas of the nose/nasopharynx (NNP-NHLs) according to immunophenotype. PATIENTS AND METHODS One hundred thirteen Chinese patients with primary NNP-NHLs that belonged to the categories E, F, G, or H according to the Working Formulation (WF), with full immunophenotypic data and complete clinical follow-up data, were analyzed in this retrospective study. RESULTS Ninety (79.6%) patients had localized (stage I or II) disease, while 23 (20.4%) had stage III or IV disease. The lymphomas in 51 (45.1%), 24 (21.3%), and 38 (33.6%) patients showed natural killer (NK)/T- (CD56-positive), T-cell, and B-cell immunophenotype, respectively. Seventy-three patients (65.8%) achieved a complete remission, of whom 34 (46.6%) subsequently relapsed. The median follow-up time for those alive was 88 months. The 5-year actuarial disease-free and overall survival rates were 34.4% and 37.9%, respectively. Multivariate analysis showed that only stage and immunophenotype were significant for survival. NK/T lymphomas were distinctive among the three immunophenotypes in the following aspects: the highest male-to-female ratio, more frequent involvement of the nasal cavity alone, higher risk of dissemination to the skin, more frequent development of hemophagocytic syndrome, and the worst prognosis (overall median survival, 12.5 months). CONCLUSION The three immunophenotypes studied are shown to exhibit different clinical patterns. Since the NK/T phenotype carries the worst prognosis, patients who present with NNP-NHL should have their tumors analyzed for CD56 expression.

Medulloblastoma: freedom from relapse longer than 8 years — a therapeutic cure?

1991 ◽  
Vol 75 (4) ◽  
pp. 575-582 ◽  
Author(s):  
Mark G. Belza ◽  
Sarah S. Donaldson ◽  
Gary K. Steinberg ◽  
Richard S. Cox ◽  
Philip H. Cogen
Keyword(s):  
Medical Center ◽  
Survival Rates ◽  
Adjunctive Treatment ◽  
High Dose ◽  
Content Type ◽  
Significant Difference ◽  
Long Term Follow Up ◽  
Fine Print

✓ Seventy-seven patients presenting with medulloblastoma between 1958 and 1986 were treated at Stanford University Medical Center and studied retrospectively. Multimodality therapy utilized surgical extirpation followed by megavoltage irradiation. In 15 cases chemotherapy was used as adjunctive treatment. The 10- and 15-year actuarial survival rates were both 41% with an 18-year maximum follow-up period (median 4.75 years). There were no treatment failures after 8 years of tumor-free survival. Gross total removal of tumor was achieved in 22 patients (32%); the surgical mortality rate was 3.9%. No significant difference was noted in the incidence of metastatic disease between shunted and nonshunted patients. The classical form of medulloblastoma was present in 67% of cases while the desmoplastic subtype was found in 16%. Survival rates were best for patients presenting after 1970, for those with desmoplastic tumors, and for patients receiving high-dose irradiation (≥ 5000 cGy) to the posterior fossa. Although early data on freedom from relapse suggested a possible beneficial effect from chemotherapy, long-term follow-up results showed no advantage from this modality of treatment. The patterns of relapse and survival were examined; 64% of relapses occurred within the central nervous system, and Collins' rule was applicable in 83% of cases beyond the period of risk. Although patients treated for recurrent disease could be palliated, none were long-term survivors. The study data indicate that freedom from relapse beyond 8 years from diagnosis can be considered as a cure in this disease. Long-term follow-up monitoring is essential to determine efficacy of treatment and to assess survival patterns accurately.

The prognosis in subarachnoid hemorrhage of unknown etiology

1984 ◽  
Vol 61 (6) ◽  
pp. 1029-1031 ◽  
Author(s):  
Vagn Eskesen ◽  
Ebbe B. Sørensen ◽  
Jarl Rosenørn ◽  
Kaare Schmidt
Keyword(s):  
Subarachnoid Hemorrhage ◽  
Mortality Rate ◽  
Functional Capacity ◽  
Unknown Etiology ◽  
Vertebral Angiography ◽  
Content Type ◽  
Moderate Reduction ◽  
Bilateral Carotid ◽  
Fine Print

✓ The mortality rate, risk of rebleeding, relevant subjective and objective symptoms, and daily functional capacity after a verified subarachnoid hemorrhage (SAH) of unknown etiology were evaluated in 44 patients treated during a 5-year period (1978 to 1983). A vascular basis for the SAH had been excluded by bilateral carotid and vertebral angiography and computerized tomography. The patients were interviewed at a follow-up examination from 3 to 64 months (median 36 months) after the bleed. The results revealed a 5% mortality rate and a 7% risk of rebleeding. Persisting headache and fatigue were found in 40% of patients, 29% had mild demential symptoms, and 5% had persisting and severe objective neurological symptoms. None had developed epilepsy. A normal daily functional capacity was enjoyed by 84%, while 14% had a moderate reduction in these functions, but were independent of help from other persons. One patient (2%) was not fully assessed.

Reinforcement with gauze wrapping for ruptured aneurysms of the middle cerebral artery

1977 ◽  
Vol 47 (6) ◽  
pp. 828-832 ◽  
Author(s):  
Julien C. Taylor ◽  
Abdur R. Choudhury
Keyword(s):  
Mortality Rate ◽  
Middle Cerebral Artery ◽  
Cerebral Artery ◽  
Recurrent Hemorrhage ◽  
Content Type ◽  
Mortality And Morbidity ◽  
Ruptured Aneurysms ◽  
Follow Up Study ◽  
Fine Print

✓ A 2- to 15-year follow-up study is presented of a series of 35 consecutive patients treated for ruptured aneurysms of the middle cerebral artery by reinforcement with gauze wrapping. Only one patient suffered early recurrent hemorrhage in the postoperative period, and none had a late hemorrhage. The procedure carried a relatively low mortality rate (8.6%). Morbidity was the direct sequel of preoperative conditions. Wrapping is an effective and safe treatment of these aneurysms, with a low mortality and morbidity, and freedom from occlusive infarcts.

Gamma knife surgery for hemangioblastomas

2005 ◽  
Vol 102 (Special_Supplement) ◽  
pp. 171-174 ◽  
Author(s):  
Masao Tago ◽  
Atsuro Terahara ◽  
Masahiro Shin ◽  
Keisuke Maruyama ◽  
Hiroki Kurita ◽  
...  
Keyword(s):  
Contrast Enhancement ◽  
Gamma Knife ◽  
Survival Rates ◽  
Gamma Knife Surgery ◽  
Tumor Control ◽  
Repeated Treatment ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Fine Print

Object. The authors reviewed their 14-year experience using stereotactic radiosurgery for the treatment of hemangioblastomas and define the role and the proper strategy for radiosurgery of this condition. Methods. This is a retrospective study of 38 hemangioblastomas in 13 patients. Seven patients had von Hippel—Lindau disease. All patients have undergone at least one follow-up visit. The median and mean tumor volumes were 0.23 cm3 and 0.72 cm3 respectively (range 0.004:4.84 cm3). Twenty-eight tumors received 20 Gy to the margin, and the remainder received 18 Gy. The median clinical follow-up period was 36 months (range 3–159 months). No patient died. The survival rate was 84.6% (11 of 13 patients). The actuarial 5- and 10-year survival rates were both 80.8%. The median radiological follow-up period was 35 months (range 7–147 months). Only one tumor increased in volume 24 months after treatment in association with an intratumoral hemorrhage. The tumor control rate was 97.4% (37 of 38 tumors). Actuarial 5- and 10-year control rates were both 96.2%. New lesions and/or those increasing in size outside the irradiated area were discovered in five patients (38.5%). Nine tumors revealed peritumoral contrast enhancement which was seen more frequently in larger tumors with a volume greater than 0.5 cm3 (p = 0.0034). Conclusions. Gamma knife surgery is a safe and effective method to control hemangioblastomas for as many as 10 years. Higher doses and smaller tumors probably contribute to good outcomes. Recurrence outside the original irradiated area is common. Peritumoral contrast enhancement may be seen in larger tumors. The authors recommend regular imaging follow up and early repeated treatment in the face of new or growing tumors.

Fusion rate: a time-to-event phenomenon

2004 ◽  
Vol 1 (1) ◽  
pp. 47-51 ◽  
Author(s):  
Sagun K. Tuli ◽  
Jayshree Tuli ◽  
Peng Chen ◽  
Eric J. Woodard
Keyword(s):  
Median Time ◽  
Fusion Rate ◽  
Smoking History ◽  
Time To Event ◽  
Female Ratio ◽  
Content Type ◽  
Kaplan Meier ◽  
Significant Difference ◽  
Fine Print

Object. The term “fusion rate” is generally denoted in the literature as the percentage of patients with successful fusion over a specific range of follow up. Because the time to fusion is a time-to-event phenomenon a more accurate method of representation may be made using the Kaplan—Meier method of estimation. Methods. The current study was performed to illustrate that fusion rate is more accurately represented by median times as calculated using survival analysis. Patients undergoing a cervical decompressive corpectomy and reconstruction formed the basis of the primary analysis. A secondary analysis was made to evaluate the difference in the fusion times for one- compared with multilevel corpectomy cases. Data were collected at a tertiary care institution over a 5-year period with 6-month follow up after the last recruitment. Descriptive statistics of baseline patient characteristics, the extent of disease, and the surgical intervention were obtained. Fusion was the final outcome, and it was defined as the “event.” The presence of any trabeculae bridging between the vertebral body and allograft signified the occurrence of an event. Postoperative static radiographs were evaluated by independent neuroradiologists to assess the presence of fusion. Fusion rate was determined using the Kaplan—Meier estimate. The median time to fusion was calculated, as were the 95% confidence intervals (CIs). These were stratified for patients who underwent one- and two-level vertebrectomy. The log-rank test was used to differentiate between one-level and multilevel corpectomy. Multivariate analysis was performed using Cox regression for further evaluation, by adjusting for covariates (age, sex, smoking history). Fifty-seven patients underwent single- or multilevel corpectomy and fusion. The male/female ratio was similar, with a median age of 53 years. Fourteen patients had a history of cigarette smoking. Thirty-six patients underwent a one-level corpectomy, 20 a two-level corpectomy, and one patient underwent a three-level corpectomy. The analysis was restricted to one- and two-level cases. The median time to fusion for the cephalad and caudad aspect of the graft—host interface was 88 days (95% CI 82–94 days) and 85 days (95% CI 77–93 days), respectively. As generally reported in the literature, this translates to a 92% (by 2.1 years) and 93% (by 1.5 years) fusion rate, for the cephalad and caudad, respectively. The median time to fusion for the cephalad aspect of the graft for one-level vertebrectomy was 87 days (95% CI 83–91 days), whereas for two-level vertebrectomy was 90 days (95% CI 59–121 days). The median time to fusion for the caudal aspect of the graft—host interface was 85 days (95% CI 80–90 days) for one-level corpectomy and 90 days (95% CI 83–97 days) for the two-level cases. There was no statistically significant difference in the median time to fusion for one- and two-level corpectomy at either the superior or inferior aspect of the graft (p = 0.19 and 0.84, respectively). This held true even after adjusting for covariates. Conclusions. Fusion rate is a time-to-event phenomenon and is more accurately represented using the Kaplan—Meier method of estimation.

The natural history of symptomatic arteriovenous malformations of the brain: a 24-year follow-up assessment

1990 ◽  
Vol 73 (3) ◽  
pp. 387-391 ◽  
Author(s):  
Stephen L. Ondra ◽  
Henry Troupp ◽  
Eugene D. George ◽  
Karen Schwab
Keyword(s):  
Mortality Rate ◽  
Arteriovenous Malformations ◽  
Original Population ◽  
Major Morbidity ◽  
Content Type ◽  
History Of ◽  
The Mean ◽  
The Brain ◽  
Fine Print

✓ The authors have updated a series of 166 prospectively followed unoperated symptomatic patients with arteriovenous malformations (AVM's) of the brain. Follow-up data were obtained for 160 (96%) of the original population, with a mean follow-up period of 23.7 years. The rate of major rebleeding was 4.0% per year, and the mortality rate was 1.0% per year. At follow-up review, 23% of the series were dead from AVM hemorrhage. The combined rate of major morbidity and mortality was 2.7% per year. These annual rates remained essentially constant over the entire period of the study. There was no difference in the incidence of rebleeding or death regardless of presentation with or without evidence of hemorrhage. The mean interval between initial presentation and subsequent hemorrhage was 7.7 years.

Incidence and clinical features of asymptomatic meningiomas

2000 ◽  
Vol 92 (5) ◽  
pp. 766-770 ◽  
Author(s):  
Jun-ichi Kuratsu ◽  
Masato Kochi ◽  
Yukitaka Ushio
Keyword(s):  
Clinical Features ◽  
Tumor Size ◽  
Morbidity Rate ◽  
Intracranial Tumors ◽  
Period Range ◽  
Female Ratio ◽  
Content Type ◽  
Significant Difference ◽  
Fine Print

Object. The increased use of computerized tomography (CT) and magnetic resonance (MR) technology has led to an increase in the detection of asymptomatic meningiomas, although the surgical indication for these tumors remains undetermined. The authors investigated the incidence of asymptomatic meningiomas and their clinical features.Methods. An epidemiological survey was conducted of primary intracranial tumors diagnosed in Kumamoto Prefecture between 1989 and 1996. Follow-up neuroradiological imaging and clinical studies for asymptomatic meningiomas were performed.Primary intracranial tumors were diagnosed in 1563 residents. Of these lesions, 504 (32.2%) were meningiomas, and of these meningiomas 196 (38.9%) were asymptomatic. The incidence of asymptomatic meningiomas was significantly higher in individuals older than 70 years of age. Furthermore, the incidence of asymptomatic meningiomas was significantly higher in female than in male patients. Of the asymptomatic meningiomas in 196 patients, 87 (44.4%) were surgically removed, whereas 109 (55.6%) were treated conservatively. Of these conservatively treated patients, 63 received follow-up care for more than 1 year. In 20 of these 63 cases, the tumors increased in size over the 27.8-month average follow-up period (range 12–87 months), whereas in the other 43 cases, the tumor size did not increase during a 36.6-month average follow-up period (range 12–96 months). There was no significant difference with respect to age, tumor size, and male/female ratio between the patient group in which the tumor size increased and the group in which it did not increase during the follow-up period. Asymptomatic meningiomas that evidenced calcification on CT scans and/or hypointensity on T2-weighted MR images appear to have a slower growth rate.Conclusions. Among patients older than age 70 years who underwent operation for asymptomatic meningioma, the neurological morbidity rate was 23.3%; it was 3.5% among younger patients. This indicates that the advisability of surgery in elderly patients with asymptomatic meningiomas must be considered very carefully.

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