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.     

Adult cerebellar medulloblastomas: the pathological, radiographic, and clinical disease spectrum

The records of 34 patients over 16 years of age with cerebellar medulloblastoma were retrospectively reviewed. All patients were treated by surgery, and all surviving patients were given radiation therapy. The imaging characteristics of this rare entity were evaluated with regard to the tumor location in the cerebellum, and the prognostic effects of histological characteristics such as neuronal or glial differentiation and the presence of desmoplasia were investigated. Neither histological parameters nor tumor location (median, paramedian, or lateral cerebellar) affected patient survival. The desmoplastic variant was encountered in 38% of these adult medulloblastomas and occurred in all three cerebellar locations. The degree of surgical resection did not have a major effect on long-term survival; long-term survival was possible even in patients who had received only a biopsy. The extent of initial radiation therapy was positively correlated with recurrence-free survival; full neuraxis irradiation was associated with a 13% incidence of delayed spinal metastases, whereas 75% of patients treated with irradiation of only the posterior fossa and/or the whole brain developed spinal deposits. A similar local recurrence rate (12.5%) was noted in both irradiation groups. Chemotherapy resulted in palliation in some patients with metastatic disease.     

A review of the factors influencing the prognosis of medulloblastoma. The importance of cell differentiation

The clinical, therapeutic, and histological features of 54 patients with medulloblastoma were analyzed retrospectively by a multivariate approach with regard to prognosis. The overall 5-year survival rate was 60%, with 48% of patients free of recurrence at 5 years. Cell differentiation, when present, was associated with a significantly longer recurrence-free period. Seventy-two percent of patients with the histological finding of cell differentiation were recurrence-free at 5 years. A marginally significant increase in the 5-year survival rate was also seen in association with differentiation. Only 34% of the patients whose tumor exhibited necrosis were alive at 5 years. There was no statistically significant difference in 5-year survival for children under 3 years of age or for the group of children aged 5 years or under. However, a significantly larger fraction (72%) of the group aged 5 years or under had a recurrence-free period of 5 years or more. Other factors including sex, extent of surgical resection. Chang tumor stage, posterior fossa radiation dose, and adjuvant chemotherapy did not influence prognosis.     

Treatment of medulloblastoma in children: Long-term results following surgery,radiotherapy and chemotherapy

Summary Forty-seven children with histologically confirmed medulloblastoma are considered. Forty-five cases were surgically treated by direct approach to the tumour, while two cases were treated only with a shunt. A shunt was inserted preoperatively in 14 cases, postoperatively in 4 cases. Surgical resection was total or subtotal in 52% of cases, partial in 35%, and limited to a biopsy in 13%. Radiation treatment to the entire neuraxis was done in 37 cases: 10 of these cases received additional chemotherapy—mostly with CCNU—as primary treatment for medulloblastoma.Ten patients died within 30 days after surgery. Twenty-two patients died months after treatment, mainly from tumour recurrence (19 cases). One patient was lost to follow-up. Thirteen patients are survivors from 10 months to 20 years after treatment. As a whole, the one year survival rate has been 67%, 3 year survival 43%, and 5 year survival 27%. Complications affecting prognosis have been presented by tumour recurrence and metastases. CSF shunting and lack of prophylactic irradiation to the cerebral hemispheres have been considered responsible for the high incidence of supratentorial metastases in our series.Factors influencing prognosis have been the extent of tumour resection and association of primary chemotherapy with radiotherapy. Within 3 years after surgery survival has been 52% in cases with total resection against 31% in cases with partial resection of tumour. As regards chemotherapy, 3 year survival has been 60% for patients with combined treatment (chemo- and radiotherapy) against 37% in patients with radiotherapy alone. It is concluded that the best results in children with medulloblastoma are achieved by a radical resection, associated with a combined primary treatment of radiotherapy and chemotherapy.     

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

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 (greater than or equal to 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.     

Infants with medulloblastoma: a single institution review of survival

Although the prognoses of children with medulloblastoma have improved dramatically over the last several decades, the reported survival for very young children with this tumor remains poor. We undertook a retrospective review of patients less than 36 months of age at the time of the diagnosis of a medulloblastoma who were treated at our institution during a 36-year period. Of 28 such patients, 10 patients are presently surviving without disease at a median of 150 months from diagnosis. The presence of a metastatic tumor at the time of diagnosis was identified as a significant indicator of a poor prognosis, and all completely staged patients without metastasis are surviving without a recurrence of disease. This report suggests that the survival of very young children with a medulloblastoma may not be as poor as has been previously reported, particularly if a disseminated tumor is not present at the time of diagnosis.     

Adult medulloblastoma. Review of 22 patients

BACKGROUND AND PURPOSE: Medulloblastoma is a malignant neuro-ectodermal tumor classically considered as a pediatric tumor. Adult medulloblastoma is rare. This low incidence results in a lack of data concerning the management of treatment. We report our experience and propose a review of the literature to clarify the main therapeutic options that are nowadays suggested.METHODS: We reviewed 22 adult patients treated for cerebellar medulloblastoma between 1979 and 1999. Actuarial relapse-free and overall survival were determined by the Kaplan-Meier method. Prognosis factors were studied by Log- Rank test.RESULTS: The five years relapse free and overall survival rates were respectively 63.1% and 81.3%. These rates are superior to those reported in the literature. None of the studied factors (age, gender, histological subtype, total or partial surgery, presence of a CSF derivation device, radiotherapy, chemotherapy) were significantly associated to remission or survival. However our statistical results should be interpreted with caution in this small population.CONCLUSION: Adult medulloblastoma prognosis seems to improve since chemotherapy has been introduced in the therapeutic protocols. Prospective and multicentric studies should determine the exact pattern of treatment.     

Treatment of medulloblastoma with procarbazine, hydroxyurea, and reduced radiation doses to whole brain and spine

Forty-seven patients with medulloblastoma were treated postoperatively with procarbazine, followed by craniospinal radiation therapy in combination with hydroxyurea. The radiation dose to the posterior fossa was 55 Gy; the spinal cord received 25 Gy and the whole brain 25 to 35 Gy (mean 33 Gy). Seventeen tumors recurred. Only one initial recurrence was in the spinal subarachnoid space; 10 (59%) were in the posterior fossa, and four (24%) were supratentorial. The estimated 5-year disease-free survival probability was 55%; the 5-year overall survival rate was 66%. Myelotoxicity occurred in 38% of patients, but in only one case was it severe enough to warrant reducing the total dose of radiation. It was concluded that good-risk medulloblastoma patients may be treated with radiation dosages as low as 25 Gy to the spinal axis and to the whole brain without increasing the risk of recurrence outside the posterior fossa. Chemotherapy with procarbazine followed by radiation therapy and hydroxyurea during radiation therapy was well tolerated and may play a role in reducing radiation dosages outside the posterior fossa.     

Prognostic factors for hepatitis C recurrence in patients undergoing orthotopic liver transplantation

End-stage liver disease caused by the hepatitis C virus (HCV) is a major indication for liver transplantation. HCV re-infection after LT is constant, and it significantly impairs patient and graft survival. Factors that may influence histological recurrence in the graft remain unclear. The aim of our study is to analyse the factors that influence the histological recurrence of HCV in a series of 142 patients undergoing orthotopic liver transplantation. In this series, donors age until 1995 were mostly younger than 30 years, however, from 2000 onwards most of the cases had graft implants from elderly donors. Histological recurrence was 92% after a mean follow-up of 60 months (12-120 months), 17% of which had poor-prognostic recurrence. Half of the patients presenting with recurrence did so in the first six months and more than two thirds developing hepatitis in the graft did so in the first year. The accumulated probability of histological recurrence is 40.1%, 51.5%, 70.3%, 83.5% and 92% at 6, 12, 24, 36 and 60 months respectively. In our experience, the variables associated with the development of histological recurrence in the graft, in the multivariate analysis, are donors age > or =31 years and immunosuppressive treatment without mycophenolate mophetil (MMF). Treatment with MMF still acts as a protection factor and is associated with histological non-recurrence.     

Medulloblastoma: Survey of factors possibly influencing the prognosis

Summary In a series of 310 cases the role of the factors age, sex, location of the tumour (midline or lateral), and medulloblastoma type (classic or desmoplastic) were analysed with regard to the prognosis. The influence of therapy is not considered because of the inhomogeneity of the material and the view nowadays that treatment is insufficient. The occurrence of lateral (or hemispheric) medulloblastomas increases in the group of the 11–15 years old patients. The proportion of the desmoplastic type is greater in adults than in children, but they are distributed almost equally medially and laterally. There seems to be no definite correlation between sex and the survival time. The mean survival time increases with age. With regard to the histological subclassification into children and adults, the mean survival times are nearly identical. The only obvious factor with a decisive influence on the prognosis seems to be the time of appearance of the tumour. Additionally, the data support the conclusion that a desmoplastic medulloblastoma should be considered as a histological variant without clinical relevance.     

Single brain metastasis of non-small cell lung carcinoma. Study of survival among 54 patients

We studied 54 patients treated for non-small cell lung carcinoma with single brain metastasis presenting between 1980 and 1985. Better survival was obtained in cases of patients presenting a fair neurological condition who were treated by surgery. Histological condition and date of onet of metastasis had no significant influence on survival. Combined treatment of both primary lung tumour and brain metastasis was a favourable prognosis element, and surgical resection of both locations led to the best results in terms of duration and quality of survival.     

Midterm results after treatment of liposarcoma in the extremities

PURPOSE: In this retrospective analysis, survival time, local recurrence and rate of metastasis were appraised in patients with primary liposarcoma of the extremities depending upon the applied primary and adjuvant therapy procedures. Furthermore, we compared the representativity of histological results of the biopsy with the final histology of the resected tumour. MATERIAL AND METHOD: Between 1990 and 1998, 27 patients were surgically treated who suffered from a primary liposarcoma of the extremities. At the mean follow-up time of 62.4 months (39-141 months) postoperative survival rate, rate of metastasis and local recurrence were determined. The results were analysed with regard to intraoperative resection distance, tumour size and localisation as well as histological classification of previous biopsy and finally resected tumour. The influence of radiotherapy on the results was also determined. RESULTS: In 5 patients (18.5%) the amputation and in 22 cases (81.5%) the limb sparing resection of the liposarcoma was performed. 6 patients underwent a marginal resection and one patient an intralesional resection. At follow-up we observed a local recurrence rate of 22.2% (n = 6) and a survival rate of 74.1%. 18.5% of the patients had metastases. After adjuvant radiotherapy three patients (50%) showed local recurrence after marginal resection. In only 8 cases (29.6%) the results of primary biopsy and final tumour classification (entity, subtype and grading) were identical. CONCLUSION: The development of metastases (often pulmonary) and local recurrences even after long tumour-free interval makes adequate follow-up investigations mandatory in liposarcoma patients. With regard to the problems in assessing liposarcoma biopsies, MRI-orientated biopsies from several tumour areas via one biopsy approach are recommended.     

Soft-tissue sarcoma of the thigh: Surgical margin influences local recurrence but not survival in 152 patients

Between 1979 and 1998, 152 patients with a soft-tissue sarcoma arising in the thigh were surgically treated in the Sarcoma Center in Aarhus, Denmark. We studied clinicopathologic factors prognostic for local recurrence and survival. 27 patients (18%) had a low-grade tumor, 26 (17%) an intermediate-grade and 99 (65%) a high-grade tumor. 27 patients (18%) were amputated and 125 (82%) had a local resection. 21 (14%) underwent a marginal resection, 82 (54%) a wide resection and 49 (32%) a compartmental resection. 32 patients were also given radiotherapy, 11 of these had a marginal resection. The 5-year local recurrence-free rate was 91%. Multivariate analysis selected marginal resection and histological high grade as unfavorable prognostic factors for local recurrence. The 5-year survival rate was 68%. High age and histological high grade were unfavorable prognostic factors for survival in a multivariate analysis. Surgical margin influenced local recurrence, but not the overall survival.     

Soft-tissue sarcoma of the thigh: surgical margin influences local recurrence but not survival in 152 patients

Between 1979 and 1998, 152 patients with a soft-tissue sarcoma arising in the thigh were surgically treated in the Sarcoma Center in Aarhus, Denmark. We studied clinicopathologic factors prognostic for local recurrence and survival. 27 patients (18%) had a low-grade tumor, 26 (17%) an intermediate-grade and 99 (65%) a high-grade tumor. 27 patients (18%) were amputated and 125 (82%) had a local resection. 21 (14%) underwent a marginal resection, 82 (54%) a wide resection and 49 (32%) a compartmental resection. 32 patients were also given radiotherapy, 11 of these had a marginal resection. The 5-year local recurrence-free rate was 91%. Multivariate analysis selected marginal resection and histological high grade as unfavorable prognostic factors for local recurrence. The 5-year survival rate was 68%. High age and histological high grade were unfavorable prognostic factors for survival in a multivariate analysis. Surgical margin influenced local recurrence, but not the overall survival.     

Brain tumors during the first twenty-four months of life

One hundred patients with brain tumors were diagnosed and treated during the first 24 months of life. They represent 16% of 608 children with brain tumors treated from 1952 through 1984. The most common histological type of brain tumors during the first 24 months are benign astrocytoma, medulloblastoma, and choroid plexus papilloma. The tumor location is distributed relatively evenly among the cerebellum and the 4th ventricle, the cerebral hemisphere (including the lateral ventricle), and the suprasellar region and 3rd ventricle. The chief presenting signs and symptoms are a full fontanelle, macrocephaly, changes of behavior, and delayed developmental milestones. Localizing signs are infrequent. Ninety-two patients underwent craniotomy with a one-month surgical mortality rate of 12%. The surgical mortality rate was 5.6% among 36 recent patients diagnosed by computed tomography. The 5-year survival rate is 41% in the patients younger than 12 months and 74% in patients diagnosed during the 2nd year of life. This study indicates the validity of Collins' rule for medulloblastomas during the first 24 months of life, but not for benign or malignant astrocytomas.     

Medulloblastoma in childhood

Summary Fifty-one patients with medulloblastoma have been treated at the Children's Memorial Hospital during the past 11 years. The ratio between males and females was 21. The ages ranged from 4 months to 12 years; 7 patients were less than 12 months old at the time of diagnosis. After 1969, prior to definitive surgery, all patients were given a ventriculo-peritoneal shunt to decompress the hydrocephalus which was invariably present. There were no cases of systemic metastasis related to these shunts. Subsequently, posterior fossa craniotomy was performed, with total or radical resection of tumour in 13 cases, subtotal resection in 20 cases, and partial resection or biopsy in 14 cases. After completion of surgical treatment and radiotherapy, the three-year survival rate was 45.6 per cent and the five-year survival 34.5 per cent. The survival of children given total resection of the tumour was significantly longer, and females survived longer than males. Local recurrence and spinal cord or systemic metastases are discussed. Reexploration of the posterior fossa for recurrences failed to improve the prognosis. Recurrent or metastatic medulloblastoma should be treated by radiotherapy or chemotherapy, or both.     

Soft-tissue sarcoma of the thigh: Surgical margin influences local recurrence but not survival in 152 patients

Between 1979 and 1998, 152 patients with a soft-tissue sarcoma arising in the thigh were surgically treated in the Sarcoma Center in Aarhus, Denmark. We studied clinicopathologic factors prognostic for local recurrence and survival. 27 patients (18%) had a low-grade tumor, 26 (17%) an intermediate-grade and 99 (65%) a high-grade tumor.

27 patients (18%) were amputated and 125 (82%) had a local resection. 21 (14%) underwent a marginal resection, 82 (54%) a wide resection and 49 (32%) a compartmental resection. 32 patients were also given radiotherapy, 11 of these had a marginal resection.

The 5-year local recurrence-free rate was 91%. Multivariate analysis selected marginal resection and histological high grade as unfavorable prognostic factors for local recurrence. The 5-year survival rate was 68%. High age and histological high grade were unfavorable prognostic factors for survival in a multivariate analysis. Surgical margin influenced local recurrence, but not the overall survival.     

Supratentorial Leptomeningeal Metastasis of a Medulloblastoma Without Cerebellar Tumor Recurrence

Summary.  The authors report on a 15-year-old girl presenting with headache and nausea. Cranial magnetic resonance imaging (MRI) showed a diffuse leptomeningeal contrast enhancement 5 years after resection of a posterior fossa medulloblastoma followed by radio- and chemotherapy. A left frontal biopsy revealed tumor spread. Histopathological findings of the tumor were identical to those obtained from the original medulloblastoma. To the authors' knowledge this is the first report of late supratentorial leptomeningeal metastasis from a medulloblastoma while no local recurrence or spinal leptomeningeal dissemination had occurred.     

结肠癌根治术后复发的多因素分析

目的探讨结肠癌根治术后复发的因素。方法对2000年1月～2005年1月收治的135例结肠癌患者的临床资料进行回顾性分析。选择对结肠癌术后复发可能产生影响的临床因素,通过Cox比例风险模型进行多因素分析。结果全组患者复发率为16.3％。单因素分析显示,淋巴结转移、肿瘤大小、Dukes’分期和肿瘤细胞分化程度与结肠癌根治术后肿瘤复发有关。多因素分析显示,淋巴结转移和肿瘤细胞分化程度与结肠癌根治术后肿瘤复发有关。结论淋巴结转移和肿瘤细胞分化程度是影响结肠癌术后复发的独立危险因素。     

Liposarcoma of the lower extremity

Twenty patients with primary liposarcoma of the lower extremity were treated over a 25-year period. The adequacy of the initial surgical procedure and histological grade of malignancy both influenced survival rates. In addition local recurrence was noted only in patients who had undergone "inadequate" initial excision. Inguinal lymph nodes were uninvolved by tumor in all cases. In general, patients with liposarcomas of myxoid and/or round cell type survived for long periods of time. However, even patients with myxoid lesions occasionally exhibited evidence of early blood-borne metastases. This study suggests an important relationship between an inadequate primary resection, local recurrence, and eventual retroperitoneal spread. In the majority of cases, this may have resulted from failure to control the primary distal extremity tumor, with subsequent contiguous spread of metastases into the ipsilateral retroperitoneal space. If feasible, radical soft part resection should be performed as the primary surgical therapy of these neoplasms. Tumors contiguous to the knee or ankle joint should be treated by primary amputation. Failure to control local disease may result in blood-borne dissemination or local spread along musculoaponeurotic planes to involve proximal groin or retroperitoneal space.