Cerebellar medulloblastoma in a 73-year-old woman

A case of a 73-year-old woman with cerebellar medulloblastoma is described. The patient presented with the classical symptoms and signs of a medulloblastoma, and radiological findings were also consistent with this entity. Nevertheless, because of the patient's advanced age, the possibilities of metastatic tumor involving the cerebellum or a primary cerebellar lymphoma were considered before operation. Pathological examination of the operative specimen showed a classical medulloblastoma with occasional areas of early neuroblastic differentiation. Immunoperoxidase strains for neurofilaments were of help in confirming the neurogenic origin of the neoplastic cells. Although medulloblastomas in adults are not rare, onset after the age of 50 is exceptional, with only two cases on record in patients 65 or older. The present patient seems to be the oldest individual with a cerebellar medulloblastoma thus far reported.     

Cerebellar medulloblastomas in adults

Between 1981 and 1991, 11 adults over 16 years of age were treated for medulloblastoma at the authors' institutions. These patients were studied retrospectively. The patients were managed uniformly, and the treatment included extensive surgical resections and radiation therapy. Chemotherapy was used on only three patients with recurrence. Probable prognostic factors, including tumor location, extent of surgical resection, dose and extent of radiation therapy, and histological characteristies of the tumor such as neuronal or glial differentiation and desmoplasia were investigated. The classical form of medulloblastoma was present in seven cases while the desmoblastic subtype was found in four cases. All patients with the desmoplastic form had the tumor in cerebellar hemisphere. Gross total removal of the tumor was achieved in seven patients and subtotal excision in four patients. There was no surgical mortality in our series. The extent of surgical resection and location of the tumor had an important effect on longterm survival. The extent and dose of radiation therapy had a major effect on recurrence-free survival. Survival rates were best for patients receiving high-dose irradiation to the entire neuroaxis. Other factors such as age and sex had no major effect on prognosis.     

Cerebellar medulloblastomas in adults

A retrospective analysis of 32 patients older than 16 years of age treated at Neurosurgical Department of Hacettepe University within the last 30 years (1959–1988) for cerebellar medullobastoma was considered. The clinical features, treatment modalities and outcome are discussed. The survival rates for 5 and 10 years were 14% and 7% respectively. The results are compared with that of literature.     

The prognosis of medulloblastoma in adults

We studied retrospectively the series of 24 patients (17 men, 7 women), median age 25.5 years (range: 16-57), operated upon for a cerebellar medulloblastoma from March 1979 to June 1988. The tumors were diagnosed for all the patients by C.T. scan and by M.R. imaging for the six last patients. Seven tumors (29.2%) were located in the fourth ventricle, 2 in the vermis (8.3%), 9 in the cerebellar hemisphere (37.5%), 5 in the vermis and cerebellar hemisphere (20.8%). One patient had a diffuse infiltration of the cerebellum. All patients have been operated on (complete removal: 15 patients (62.5%), subtotal removal: 8 patients (33.3%), biopsy: 1 patient). Operative mortality was 8.3% (2/24). One patient died at two months from septicemia. The twenty-one surviving patients received radiotherapy. Twelve patients received both radiotherapy and chemotherapy. Six patients (25%) died during the follow up at 17, 22, 24, 60, 84 and 85 months. One patient is lost to follow up at 45 months and 13 patients are living at the closing date (June 1989). For the total group (n = 24) the probability of survival at 5 years was 64.8% (C.I. 95%: 42.8%-86.8%). Among the 21 patients who received radiotherapy alone or radiotherapy and chemotherapy the probability of survival at 5 years was 74.4% (C.I. 95%: 52.2%-96.7%). We studied the following predictors of poor outcome: age, sex, prognosis subgroups as defined by Chang et Coll., extension of tumor removal, adjunction of chemotherapy, histology. None of these factors was statistically related to the survival duration.(ABSTRACT TRUNCATED AT 250 WORDS)     

Cerebellar mutism after removal of a vermian medulloblastoma in an adult

Cerebellar mutism is a rare postoperative phenomenon that generally occurs in children after resection of a cerebellar mass lesion. A 22-year-old man developed cerebellar mutism after posterior fossa surgery for a mass lesion in the vermis. Histological examination showed a medulloblastoma. The tumor was totally removed via a suboccipital approach. The patient developed mutism on the first postoperative day, which persisted for two weeks. Thereafter, this condition gradually improved. Although pathophysiology and the anatomical substrate of cerebellar mutism still remain unclear, it is important that neurosurgeons be aware of this syndrome after posterior fossa surgery in adults. This phenomenon usually has a self-limiting course and a favorable prognosis for resolution within the first three months.     

Cerebellar medulloblastoma: results of multidisciplinary treatment. Report of 120 cases

The authors report a series of 120 cerebellar medulloblastomas. All patients were operated on between 1953 and 1982. Among them, 88 completed the treatment with radiotherapy, and 32 had additional chemotherapy. The operative mortality was 22.5%. The 5-year survival rate was 30% in the whole series. Recurrences occurred in 35 patients, associated with supratentorial or spinal metastasis in 60% of cases.     

Cerebellar hemorrhage in adults. Diagnosis by computerized tomography

A series of 10 adult patients with cerebellar hemorrhage diagnosed by computerized tomography (CT) is described. Hypertension was the most common etiological factor, accounting for 70% of the cases. The clinical presentation appeared to fall into two basic groups. The first group (60% of the cases) ran a progressive course with early brain-stem compression. The second group had a benign course with findings of focal cerebellar dysfunction. The CT findings reflected the two clinical categories. The scans of Group 1 patients revealed a lorge hematoma (greater to or equal to 3cm) and substantial ventricular dilatation. In contrast, scans of Group 2 patients demonstrated a small hematoma (less than 3 cm) without ventricular dilatation. Use of CT scanning allowed the accurate diffferentiation of cerebellar hemorrhage from primary brain-stem and intraventricular hemorrhage. The findings of the CT investigations proved very helpful in defining appropriate therapy.     

Cerebellar swelling after surgery for medulloblastoma with leptomeningeal dissemination in children. A case based-update

ObjectDespite the improvement in the overall management of medulloblastomas in recent years, certain phenomena and in particular postoperative cerebellar swelling remain an enigma. This rare complication, little described in the literature, is nonetheless life threatening for the patients.Case reportsWe report our experience about two children who developed severe cerebellar swelling with hydrocephalus and upward herniation soon after a gross total resection of a fourth ventricle medulloblastoma by a telo-velar approach. Despite rapid management of ventricular dilation and optimal medical intensive treatment of intracranial hypertension, both children died quickly after the surgery. Pathological examination analyses were in favour of anaplastic/large cell medulloblastoma.DiscussionDiffuse cerebellar swelling with upward herniation may occur postoperatively in young children with anaplastic/large cell medulloblastoma with leptomeningeal spread. In the literature, only 4 cases have been so far described with delayed onset of symptoms. Two children survived with an aggressive management (decompressive surgery and early radio-chemotherapy).ConclusionCerebellar swelling is an unrecognised and sudden complication of posterior fossa surgery for metastatic anaplastic medulloblastoma with leptomeningeal dissemination in young children. An initial less invasive surgical approach could be considered in such cases, in order to prevent this complication with potentially tragic issue, and which cannot be managed with a CSF shunt alone.     

Cerebellar pilocytic astrocytoma in adults: a management paradigm for a rare tumour

Background

Pilocytic astrocytoma is one of the commonest subtypes of glioma to affect children. However, they are rarely diagnosed in patients over the age of 18 years. In adults, these tumours appear more frequently supra-tentorially than in the cerebellum and some reports suggest a different clinical course in adults. We reviewed ten patients aged 18 or over who had been operated on for cerebellar pilocytic astrocytoma to assess the impact of tumour biology and extent of resection on outcome in adults.

Method

Patients were identified from a neuropathology database and a retrospective chart review of ten patients was performed. Recorded data included patient demographics, tumour location, presenting features, radiological appearance, extent of surgical resection, tumour recurrence and Ki-67 proliferation index.

Results

Nine patients were men and one patient was a woman. Median follow up is 41.5 months (range 15–334 months). Complete surgical resection was achieved in nine of the patients operated in our institution. One patient had prior subtotal resection elsewhere. Tumour recurrence was seen only in the two patients with subtotal resection, at 7 and 25 years. Ki-67 ranged from <1 to 10 % and appears to have no correlation to recurrence. No patients in this series had adjuvant treatment.

Conclusions

Cerebellar pilocytic astrocytomas in adults should be treated with macroscopic complete surgical resection whenever possible. If this is achieved, long-term survival rates are excellent, whereas subtotal resection carries a high risk of tumour recurrence. Ki-67 is less important prognostically than the extent of initial resection.     

Cerebellar mutism in adults after posterior fossa surgery: a report of 2 cases

BACKGROUND: Mutism has been associated with injury to midline cerebellar structures secondary to degenerative disease, tumors, hemorrhage, or surgery. Typically, cerebellar mutism syndrome (CMS) has been seen in children and only rarely described in adults after surgery of the posterior fossa. This syndrome typically arises 48 hours after the initiating event and resolves approximately 7 to 8 weeks later. Characteristics of CMS include complete absence of speech without impaired consciousness, other cranial nerve deficits, or long tract signs. CASE DESCRIPTION: The authors report on 2 patients each of whom developed cerebellar mutism after tumor resection using a posterior fossa approach. The first patient underwent gross total resection of a pineal region tumor via a supracerebellar approach. The second patient underwent posterior fossa decompression for a left cerebellar hemispheric renal cell carcinoma metastasis with adjacent hemorrhage. One patient displayed a variant of cerebellar mutism with severe ataxic dysarthria without complete absence of speech, whereas the other demonstrated frank mutism. After neuroimaging studies confirmed the absence of a surgically treatable postoperative cause for the patients' symptoms, they were managed in a supportive fashion (eg, speech therapy) and improved within 3.5 months and 1 year, respectively. CONCLUSION: It is paramount that neurosurgeons be aware of cerebellar mutism with regard to its very rare occurrence in adults, its time of onset, and typical self-limiting course.     

经典型与促纤维增生型髓母细胞瘤MRI表现

目的比较经典型和促纤维增生型髓母细胞瘤MRI表现及ADC值的差异。方法回顾性分析49例经手术病理证实为髓母细胞瘤患儿的影像学资料,观察其MRI表现,测量ADC值;根据2007年WHO标准对髓母细胞瘤进行病理分型,比较经典型和促纤维增生型髓母细胞瘤MRI表现和ADC值的差异。结果 49例中,经典型髓母细胞瘤41例,促纤维增生型髓母细胞瘤8例,常规MRI征象如肿瘤位置、T2信号、囊变、瘤周水肿及强化等差异无统计学意义(P均0.05);促纤维增生型髓母细胞瘤ADC值[(0.78±0.12)×10-3 mm2/s]低于经典型髓母细胞瘤[(0.88±0.10)×10-3 mm2/s;P0.05)。结论经典型和促纤维增生型髓母细胞瘤常规MRI表现无明显差异;促纤维增生型髓母细胞瘤的ADC值低于经典型。     

Optimal therapy in medulloblastoma

Summary Twenty-eight children with medulloblastoma (19 boys and 9 girls) were treated at the Departments of Neurosurgery and Radiotherapy in Essen between 1969 and 1977, and were followed prospectively after surgical treatment and postoperative irradiation. Because different radiation techniques had been used, these patients were divided into two groups, one given a limited irradiation volume and the other irradiation of the entire central nervous system. The results obtained in the former group were inferior to those in the latter. All living patients in the second group are in good condition. Special attention is paid to the technique of irradiation and the factors which may influence prognosis. Even after the relatively short follow up period of one to five years, it may be concluded that high-dose irradiation of the entire central nervous system considerably improved the prognosis of medulloblastoma.     

New research directions in medulloblastoma

IntroductionMedulloblastoma is the most common type of pediatric malignant brain tumor where the most important amount of clinical and radiological data has been accumulated in recent years. This has led to its sophistication in the management of these patients with a clear benefit for the patients. Long-term outcome and sequelae have been described and their causes well understood such as preventive measures which can now be implemented.Material and methodsThis review paper does not attempt to make a systematic review of the literature in the field of research regarding medulloblastoma. It rather reflects more the opinion of a pediatric oncological team involved for a long time in this type of research. Therefore, a relevant literature review was carried out and selected by the senior author.ResultsMedulloblastoma is no longer a single entity but a group of at least 4 different diseases with a specific oncogenesis. In addition, biomarkers for prognosis have emerged to complement the known clinico-radiological risk factors. If this biological classification has allowed to modulate the therapeutic strategies, it has not yet brought many new drugs (except for the Sonic Hedgehog inhibitors) in the armamentarium against medulloblastomas. Consequently, some high-risk tumors remain difficult to cure. Combining data on oncogenesis and prognostic biomarkers will allow to define risk groups more specifically. New targeted therapies that are more effective and less toxic are desperately needed. Alternatively, it is also justified to study preventive measures to decrease the sequelae of the tumor and its treatments. From the therapeutic point of view, we scarcely know the biological determinants of chemosensitivity and radiosensitivity, as well as those associated with metastases which are indeed invaluable for tailored therapeutic strategies.ConclusionIf some genetic causes of medulloblastoma are known, the occurrence of the disease is largely unexplained for the others, justifying more research in this area. If genomics (and to a lesser extent epigenomics) of these neoplasms has been well studied, little is known on their proteomics and on the regulatory networks involved in the biological behavior of these tumor cells. New models are developed to test these aspects.     

Atypical adult medulloblastoma

Medulloblastomas are malignant primitive neuro-ectodermal neoplasms of childhood. In adults, clinical manifestations, imaging and prognosis can be different from that observed in children. Three adult patients with confirmed diagnoses of medulloblastoma are discussed in this report. They presented with unusually prolonged clinical courses and with imaging more suggestive of L’hermitte-Duclos disease. Medulloblastoma should be considered in all adults with posterior fossa masses despite having clinical and radiological features suggestive of a low-grade tumour. Definitive diagnosis requires histological confirmation in all cases.     

Congenital cerebellar medulloblastoma

Congenital cerebellar medulloblastoma is extremely rare. Reported here is a female infant who presented her first abnormal clinical manifestations on the sixth day of life and who was subsequently found to have medulloblastoma at operation. Electron microscopy revealed glial filaments and short cytoplasmic projections in some neoplastic cells and the presence of junctional complexes between neoplastic cells with and without glial filaments, which suggests the astrocytic differentiation of the tumor. Including our own case, there have been only 21 reported cases of congenital cerebellar medulloblastoma. A strong female preponderance and some familial occurrence are noted in congenital cerebellar medulloblastoma.