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[Primary central nervous system malignant lymphoma originating from the cerebellum and extending along the lower cranial nerves].

We report a case of primary central nervous system (CNS) malignant lymphoma of the central nervous system originating from the cerebellum and growing along the lower cranial nerves. A 67-year-old woman presented with hoarseness, vertigo, nausea, and vomiting. Gd-DTPA enhanced MRI showed a homogeneous enhanced mass lesion extending from the cerebellum to the medulla oblongata around the jugular foramen on the right side. Although pre- and intra-operative diagnosis had been schwannoma, histopathological examination revealed a B-cell, diffuse malignant lymphoma. The growth pattern of malignant lymphoma in the present case, which extended extra-axially, is considered to be rare. We discuss here the growth patterns and difficulties of diagnosis of primary CNS malignant lymphoma in this area.

Aged↗

Supratentorial hemangioblastoma in a patient with breast cancer. A case report.

Patients with breast cancer may develop cerebral metastasis. Radio- and chemotherapy are advocated as an alternative to surgery in such patients. A woman operated on for breast cancer 2 years earlier developed a cerebral lesion. A definite preoperative diagnosis of the lesion was not possible on the basis of CT and MRI findings. The lesion proved to be a supratentorial hemangioblastoma. Neurosurgical treatment is recommended for patients with breast cancer who present a cerebral lesion, since a correct diagnosis may only be possible in the operating theater.

Aged↗

Significant differences in the matrix metalloproteinase expression profiles of spontaneous medulloblastomas/primitive neuroectodermal tumors as compared with their xenografted, established tumor cell line derived counterparts.

Matrix metalloproteinases (MMP) are a family of zinc-dependent enzymes which degrade various components of the extracellular matrix (ECM) and play an important role in facilitating neoplastic cell invasion and metastasis. Structural changes in the extracellular matrix are necessary for cell migration during tissue remodeling and tumor invasion. Expression of MMP-2, -3, -9, -10, and -13 was investigated in both spontaneous and xenografted (cells derived from an established cell-line [DAOY#3]) childhood medulloblastomas (MEDs)/primitive neuroectodermal tumors (PNETs) employing an indirect alkaline phosphatase conjugated immunocytochemical technique. Evaluation of the results was based on (a) the percent of neoplastically transformed tissue that reacted positively and (b) a measure of staining intensity [graded from A (highest) to D]. The two forms of stromelysin (SL), types 1 (MMP-3) and 2 (MMP-10), share 82% sequence homology, but exhibit differences in cellular synthesis and inducibility by cytokines and growth factors in vitro. Strong overall expression of MMP-3 and -10 was found only in the spontaneous MEDs/PNETs, especially in the ECM adjacent to blood vessels. Positive immunoreactivity could be seen for these two MMPs in the ECM surrounding over 90% of the neoplastically transformed cells in the spontaneous cases, and the staining intensity was also the strongest possible (A,B). Focal (surrounding less than 10% of the neoplastically transformed cells) but strong (A,B) immunoreactivity for collagenase-3 (MMP-13) was also only detected in spontaneous MEDs/PNETs, an endopeptidase characterized by a potent degrading activity against a wide spectrum of substrates. Weak (surrounding anywhere between 10% and 90% of the neoplastically transformed cells, and of B and B,C intensity) expression of MMP-2 (gelatinase A) and MMP-9 (gelatinase B), two cytokine-induced MMPs, was also observed in the spontaneous cases. Staining for MMP-2 was negative in the xenografted MEDs/PNETs. The only positive immunoreactivity in the xenografted MEDs/PNETs was observed in the case of MMP-9, with expression of strong intensity in the ECM surrounding over 90% of the neoplastically transformed xenografted MED/PNET cells (++++; A,B). It is clear that the activation of MMPs and their inhibitors occurs in a very well orchestrated manner. The data presented here suggest that there are significant differences in the pathophysiology of spontaneous and xenografted human neoplasms, which further establishes the already detected limitations of such models in preclinical cancer research.

Adolescent↗

Spontaneous neoplastic regression: the significance of apoptosis.

In mammalian cells, neoplastic transformation has a direct relationship with the expression of oncogenes, the production of certain growth factors and with the mutation, loss or simple inactivation of the function of tumor suppressor genes. Genes for suppression of the development of the malignant immunophenotype, as well as inhibitory growth factors have regulatory functions within the normal processes of cell division and differentiation. Telomerase (a ribonucleoprotein polymerase) activation is frequently observed in various types of neoplastic cell transformation. Telomerase activation is regarded as essential for cell immortalization and its inhibition may result in spontaneous regression (SR) of neoplasms. SR of neoplasms occurs when the malignant tumor mass partially or completely disappears without any treatment or as a result of a therapy considered inadequate to influence systemic neoplastic disease. This definition makes it clear that the term SR applies to neoplasms in which the malignant disease is not necessarily cured, and to cases where the regression may not be complete or permanent. A number of possible mechanisms of SR are reviewed, with the understanding that no single mechanism can completely account for this phenomenon. The application of the newest immunological, molecular biological and genetic insights for more individualized anticancer immunotherapy (biotherapy) is also discussed. In conclusion, of all the possible mechanisms of SR of neoplasms, programmed cell death (PCD) or apoptosis is involved in each. The immunological mechanism is probably the main effector mechanism of SR in human neoplasms with its trigger being apoptosis. The treatments of the tumor, such as with various anti-neoplastic drugs or radiation or immunotherapy, all include the basic mechanism of programmed cell death or apoptosis. Without apoptosis, there is practically no tumor regression, none of any kind.

Adult↗

Investigation and comparison of the effects of rehabilitation on balance and coordination problems in patients with posterior fossa and cerebellopontine angle tumours.

BACKGROUND: This study was planned to investigate and compare the effects of acute rehabilitation programme on balance and coordination problems in patients with posterior fossa and cerebellopontine angle tumours. METHODS: This study was carried out at Hacettepe University, School of Physical Therapy and Rehabilitation, neurosurgical rehabilitation unit on 40 patients whose ages varied between 18-60 years. Subjects were subdivided into two groups. Group I consisted of 20 patients with posterior fossa tumour, while the group II was composed of 20 patients with cerebellopontine angle tumour. Patients were evaluated according to the localisation of the tumour, the number of the surgical operations, the level of the surgical excision of the tumour, percent of having shunt, radiotherapy and chemotherapy. Balance problems were assessed according to Mokken's functional independence assessment scale in 5 selected positions and standing balance was also evaluated according to the method of Bohannon. The rehabilitation of the patients included balance and coordination training. The patients were re-evaluated before their discharge and the results of the two evaluations were compared. RESULTS: A significant improvement was found in the second group when pre- and post-treatment evaluations were compared. There were more coordination problems in group 1 in the pretreatment period. Both groups showed significant improvement of coordination problems after the treatment. However, this improvement was more obvious in the second group (p<0.05). There was a negative significant correlation between the decrease of balance problems and the amount of the tumour excided (I Group r=-0.51, II Group r=-0.57). CONCLUSIONS: It can be concluded that rehabilitation approaches are effective in balance and coordination problems observed in both tumour groups, but the responses to treatment are more obvious in the cerebellopontine angle tumours, which have better prognosis.

Adolescent↗

Diagnosis and results of treatment with radiation therapy in gliomatosis cerebri patient: case report.

Gliomatosis cerebri (GC) is a rare disease loosely defined as a diffusely infiltrating glioma involving extensive areas of the brain. The prognosis is poor and no definite treatment has proven effective for GC. Little information exists regarding the role of radiation therapy (RT) for GC, but some researchers have suggested that it is a good choice of treatment from their limited experience. In this report, we present a case with imaging and histological diagnosis of GC and demonstrate the treatment results of RT. The patient was a 39-year-old woman with progressive symptoms of dizziness, unsteady gait, headache, vomiting, and consciousness disturbance for 6 months. She received a series of radiographic examinations and surgical interventions for diagnosis. The definite diagnosis of GC was made by a combination of magnetic resonance imaging (MRI) findings and histological examinations. Forty Gray (Gy) of whole brain irradiation followed by 14 Gy reduced-field boosts were given to her. The MRI, following treatment, showed regressive changes, and clinical symptoms were slightly improved. The patient survived 19 months after the diagnosis, which is longer than the average survival time of patients without treatment.

Adult↗

Abnormal brain scans: Contribution of blood radioactivity to image.

Images obtained with 99m-Tc-labeled red blood cells were compared with 99m-Tc-pertechnetate scans in 26 patients with primary and secondary brain tumors, intracerebral infcts, and hemorrhage. The results indicated that the contribution of blood pool radioactivity to a positive brain scan was minor.

Astrocytoma↗

[Chemo-radiotherapy for malignant brain tumors].

I) Malignant gliomas: Randomized clinical trials conducted in the USA showed that radiotherapy plus chemotherapy with nitrosoureas offered a long-term survival advantage to patients younger than 60 years old with malignant gliomas. Combination chemotherapy, such as procarbazine/CCNU/vincristine (PCV) must be tested further, and intra-arterial chemotherapy with nitrosoureas offered no survival advantage. Combination chemotherapy with PCV showed efficacy for patients with anaplastic oligodendroglioma and anaplastic oligoastrocytoma. II) Medulloblastoma: The addition of chemotherapy to radiotherapy improved the survival of patients with poor risk medulloblastoma, and may reduce the required craniospinal radiation dose in patients with good risk medulloblastoma. III) Primary CNS lymphoma (PCNSL): Combination of chemotherapy with high-dose MTX and radiotherapy improved survival of patients with PCNSL; however, the neurotoxicity produced by this treatment modality is a serious problem in older patients. IV) Intracranial germ cell tumors: The addition of chemotherapy to radiotherapy may produce long term survival with good quality of life in patients with germinoma. Neoadjuvant therapy consisting of chemotherapy and radiotherapy followed by complete surgical excision improved survival of patients with intracranial nongerminomatous germ cell tumors.

Antineoplastic Combined Chemotherapy Protocols↗

Contribution to the study of 150 cases of cerebral metastases. II. Neuropathological study.

This study deals with the morphology of 150 cases of cerebral metastases. They are, for the most part, associated to other cases of metastases elsewhere in the body (96.7%). A histological study shows that the nervous parenchyma is more frequently invaded by expansion than infiltration; the majority of metastases settle in the grey matter or where the white matter meets the cortex. There is no predominance for one of the two hemispheres. The frequency of metastases in the various lobes is proportionate to their respective volumes. In 30% of the cases, there is only one single metastasis; these are then, very often, tumours from the digestive tract and from the breast. Usually they are large, and especially if they originate from the digestive tract. Single metastases are three times more frequent in the brain than in the cerebellum. In 10% of the cases, only the cerebellum is affected by metastases. Breast tumours are by far the most frequent kind of tumor to invade the dura. The widely accepted principle that the brain is invaded by means of the arteries is confirmed by our series, where no document shows an illustration in support of the venous theory. In most cases, the metastases are of the same histological type as the primary tumour. More or less deep necroses are found quite often. These disorders of vascular permeability explain fairly clearly the varying responses of the environing nervous tissue to the tumours invasion, particularly in the presence of hemorrhages. When they are voluminous, they may clinically have appeared in the form of an ictus. These hemorrhages occur preferably in the white matter. Necroses are sometimes cavitary, their centre being taken up by a necrosis of liquefaction, surrounded by a more or less continuous neoplastic border. The most frequent inflammatory reaction is lymphoplasmocytarian, particularly accentuated on the periphery of the metastasis and in the neighbouring meninges. Oedema predominates in the white matter. They nearly always lead to demyelinization, with its aftermath of granulofatty bodies or astrocytarian reactions. Fibrosis is also frequent, it is intra-metastatic and in direct relationship with the tumour's histological type; it is more rarely perimetastatic, developing from the vascular mesenchyma. The vascular changes constitute one of the most constant peri-metastatic parenchymatous alterations. They may appear in the form of vascular ectasia or neoformations of vessels, the most characteristic aspect of which is the formation of vascular glomerules. These undergo a very quick evolution and are indubitably linked to the degree of malignancy.

Adult↗

Epigenetic changes in pilocytic astrocytomas and medulloblastomas.

Aberrant methylation of CpG islands located in promoter regions represents one of the major mechanisms for silencing of cancer-related genes in tumour cells. We determined the frequency of aberrant CpG island methylation of several tumour-associated genes: MGMT, GSTP1, DAPK, p14ARF, THBS1, TIMP-3, p73, p16INK4A, RB1 and TP53 in 24 neurogenic tumours consisting of pilocytic astrocytomas (n=13) and medulloblastomas (n=11). The methylation index (number methylated genes/total genes analysed) displayed slight differences (0.18 and 0.25, respectively), and the profile of methylated genes in the two neoplasms was distinct, as predicted. The main differences involved the methylation rate of GSTP1 (0% in pilocytic astrocytomas vs. 18% medulloblastomas) and p14ARF (0% in pilocytic astrocytomas vs. 45% in medulloblastomas) genes. Pilocytic astrocytomas also demonstrated some differences when compared to methylation data from other astrocytic tumours, primarily regarding the MGMT methylation rate. Despite the fact that these differences do not show specific tumour-associated gene methylation patterns, our findings should help us understand the pathogenic mechanisms of both neurogenic neoplasm types.

Adolescent↗