Guillain-Barre syndrome in patient with Burkitt's lymphoma and type 2 diabetes mellitus

Although peripheral neuropathies are commonly observed in patients with non-Hodgkin's malignant lymphomas (NHML), Guillain-Barre syndrome (GBS) belongs to the occasional complications of lymphoproliferative disorders. It appears in less than 0.3 per cent of NHML. It is worthy of note that in the reported case there occurred three independent risk factors of peripheral neuropathy: Burkitt's lymphoma, chemotherapy and type 2 diabetes mellitus. Based on clinical course, EMG finding and neuropathological examination, in spite of normal cerebrospinal fluid protein content, GBS as a paraneoplastic disorder was diagnosed. It was assumed that chemotherapy and diabetes mellitus conduced to severe neuropathy.     

Experience in surgical management of tumours involving the cavernous sinus.

Potential injury to the neurovascular structures within the cavernous sinus often prohibits aggressive removal of tumours involving it, however, fully understanding the anatomy and selecting an appropriate surgical approach can often resolve this problem with acceptable morbidity. Moreover, a tumour may originate from different anatomical structures of the cavernous sinus which will influence the difficulty and outcome of the surgery. In general, tumours in this region can be classified as intradural, intracavernous and invasive types. The strategy of surgical treatment varies among these different anatomical types. Therefore, preoperative evaluation of tumours in the cavernous sinus is critical for the selection of an appropriate microsurgical approach. During the past 5 years, 12 tumours involving the cavernous sinus have been operated upon which included four neuromas, three meningiomas, three cavernous haemangiomas, one plasmacytoma and one chondroma. Nine of these twelve tumours were totally resected after one or two operations. There was no surgical mortality and the most common morbidity was transient cranial nerve palsy. At 2 months after surgery there was no additional postoperative cranial nerve deficit in all the patients; however, one patient developed a postoperative middle cerebral artery infarct due to accidental injury to the internal carotid artery during surgery. The respectability of the tumour mostly depends on its consistency and the involvement of the adjacent anatomy. The pathoanatomical features of the tumours and the clinicoradiological findings, as well as the selection of the microsurgical approach, are discussed.     

Review: Molecular characteristics of long‐term epilepsy‐associated tumours (LEATs) and mechanisms for tumour‐related epilepsy (TRE)

Brain tumours are the second most common cause of seizures identified in epilepsy surgical series. While any tumour involving the brain has the potential to cause seizures, specific subtypes are more frequently associated with epilepsy. Tumour‐related epilepsy (TRE) has a profound impact on patients with brain tumours and these seizures are often refractory to anti‐epileptic treatments, resulting in long‐term disability and patient morbidity. Despite the drastic impact of epilepsy‐associated tumours on patients, they have not traditionally enjoyed as much attention as more malignant neoplasms. However, recently a number of developments have been achieved towards further understanding of the molecular and developmental backgrounds of specific epilepsy‐associated tumours. In addition, the past decade has seen an expansion in the literature on the pathophysiology of TRE. In this review, we aim to summarize the mechanisms by which tumours may cause seizures and detail recent data regarding the pathogenesis of specific developmental epilepsy‐associated tumours.     

Role of stereotactic biopsy in multifocal brain lesions: considerations on 100 consecutive cases.

One hundred patients affected by multifocal brain lesions were investigated by serial stereotactic biopsy. Systemic diseases and primary neoplasms elsewhere were previously ruled out. The histological diagnosis obtained in this series comprises malignant gliomas in 37% of patients; primary non-Hodgkin's brain lymphoma in 15%; metastatic brain tumours in 15% (no evidence of the primary tumour at the time of stereotactic surgery); low grade gliomas in 12%; infective diseases in 10% (including brain abscesses and multifocal viral encephalitis); and ischaemic lesions in 6%. In addition, two patients with germinomas, two with primitive neuroepithelial tumours, two with multiple telangiectases, and one with a teratoma were also included in this series. Histological findings obtained by stereotactic procedures guided the choice of treatment, avoiding the risks of blind treatments. Indications and future perspectives for stereotactic surgery in multifocal brain lesions are discussed with emphasis on advances in diagnostic and therapeutic tools.     

Usefulness of smears in intra‐operative diagnosis of newly described entities of CNS

The recent edition of World Health Organisation (WHO) Classification of Tumours of the Central Nervous System has incorporated a substantial number of important changes. It has recognised several new entities, many of which are rare. Intra‐operative diagnosis of these tumours can be difficult with the freezing artefact that often cripples brain frozen sections. In many instances intra‐operative smears are extremely useful adjuvants in neuropathological diagnosis. In this article, we describe intra‐operative smear findings of three of the newly described tumours. Their characteristic cytologic features are illustrated along with differentiating features from the common mimics, together with a general approach to brain smears. The entities we discuss here are papillary glioneuronal tumour, papillary tumour of the pineal region and angiocentric glioma. All three tumours share at least focal pseudo‐papillary/vasculocentric architecture. Smears from papillary glioneuronal tumour demonstrated dual population of cells in a neuropil background, whereas papillary tumour of the pineal region and angiocentric glioma comprise a single population of cells. These two tumours can further be differentiated based on their cell morphology and background.     

Posterior fossa tumors in children with neurofibromatosis type 1 (NF1)

Background

Tumours of the posterior fossa associated with neurofibromatosis type 1 (NF1) are very infrequent. Series studying this association are seldom reported.

Personal experience

In a series of 600 NF1 patients studied during 39 years (1965-2004) only five (0.83%) had posterior fossa tumours. They were studied clinically, radiologically by computerized tomography (CT) or magnetic resonance (MR) and histologically. Four of them had astrocytomas but only in one case was the tumour primarily cerebellar while the tumour was primarily of the brain stem with invasion of the adjacent regions of one or both cerebellar hemispheres in three patients. The fifth tumour was a medulloblastoma that had a survival of 3 years following treatment. The patient with primary cerebellar astrocytoma is apparently cured 7 years after the removal of the tumour. The patients with the brain stem tumours extending to the cerebellum, showed a chronic slowly progressive cerebellar disease, but remain alive at age of more than 20 years (one was lost to follow-up).

Discussion and conclusion

The aim of this study was to present five children (one male and four females) less than 16 years of age when they were initially seen in our service, who had NF1 associates with posterior fossa tumours. This location is very uncommon in patients with NF1, in contrast with those located in other regions, such as pathway optic tumours and brain stem tumours. Most of these tumours are histologically benign (low grade astrocytomas). Only one patient in this series had a medulloblastoma, an exceptionally rare tumour seldom reported in patients with NF1.     

A rare case of chondromyxoid fibroma of the parietal bone

The authors present a very rare case of the chondromyxoid fibroma (CMF) of the parietal bone. This is an uncommon chondroid tumour which constitutes less than 1% of primary bone tumours and which is usually localised in the metaphysis of the long bones, often in the knee region. There are 23 cases of cranial localisation of the chondromyxoid fibroma reported in several papers, 14 cases involved the cranial base and 9 the calvaria. A different ossification process of the skull-base and calvaria is probably responsible for this distribution. In our case we found the focal, lytic lesion in the parietal bone and fibro-greasy tumour mass. The dura was spared. The tumour was removed totally. Curettage of these kind of lesions should be avoided because it may lead to tumour recurrence. Histologically this tumour may cause problems in a differential diagnosis with other chondroid tumours like chondrosarcoma or chondroblastoma.     

Prognostic significance of Tks5 expression in gliomas

A pathological hallmark of gliomas is their extensive invasion into the brain parenchyma regardless of tumour grade. Clinically this is a major factor in tumour recurrence as surgery and adjuvant therapies are unable to eradicate all the infiltrating malignant cells. Tyrosine kinase substrate with five SH3 domains (Tks5, also known as SH3PXD2A) and cortactin are required for the formation of invadopodia, actin-based protrusions of tumour cells with associated proteolytic activity implicated in tumour invasion. We investigated the prognostic significance of Tks5 and cortactin expression in 57 patients with various grades of glioma. Expression of Tks5 or cortactin occurred in all grades of tumours and expression of Tks5, but not cortactin, was associated with significantly reduced patient survival among glioma patients. This association was clearest in patients with low-grade astrocytomas and oligoastrocytomas. These results suggest a prognostic relevance for the Tks5 invadopodial protein in glial-derived brain tumours.     

Surgical treatment of intramedullary tumors: preliminary results

Since 1995 in the Department of Neurosurgery, Mining Hospital in Sosnowiec a surgical technique has been used of total or gross total removal of tumour. It includes the use of CO2 laser for myelotomy and tumour vaporisation as well as ultrasonic aspirator, microsurgical techniques and intraoperative monitoring of SSEP. Ten patients 11-61 years of age were operated on. Four tumours were located in the cervical part of the spinal cord, three in the cervico-thoracic area while the remaining three in the thoracic part. The condition of four patients before the operation was assessed as I-II degree on the McCormick scale, of one patient as--III degree--and of the other five patients as IV-V degree. Only in two cases the removed tumour was ependymoma; in six cases these were benign astrocytomas and in two other--anaplastic tumours. After the treatment, the clinical condition of almost all patients improved. Pain and trouble some paraesthesias subsided. Five patients are completely self-dependent and three other walk with the aid of handrails. The treatment proved unsuccessful in one patient with an anaplastic tumour in whom paralysis of lower limbs occurred before the operation. Six months after the operation the patient died of tumour metastasis. The observation period ranged from 6 months to 3 years. On the basis of our own observations it is suggested that total safe removal of even infiltrating intramedullary tumours is possible when proper surgical techniques are used. Best results are obtained in patients in good clinical condition irrespective of tumour extent.     

Histopathological patterns of papillary tumour of the pineal region

Papillary tumour of the pineal region (PTPR) is a rare neoplasm that has been formally included in the 2007 WHO classification of central nervous system tumours. The critical diagnosis of this neoplasm is often difficult because of its similarity to other primary or secondary papillary lesions of the pineal region, including parenchymal pineal tumours, papillary ependymoma, papillary meningioma, choroid plexus papilloma and metastatic papillary carcinoma. We present the variability of the histopathological pattern in three cases of PTPR. All cases showed predominant epithelial-like morphology but with various degrees of papillary formation and intensity of cellular pleomorphism. One tumour was highly cystic and exhibited cellular sheets containing vessels covered by several layers of uniform columnar to cuboidal tumour cells. The second tumour showed distinct papillae covered by layers of polymorphous cells with atypical, often hyperchromatic nuclei. Numerous cells displayed foamy, eosinophilic or clear, sometimes vacuolated cytoplasm. The third case consisted of solid cellular areas composed of pseudostratified columnar cells, most often arranged in perivascular pseudorosette formations. The cells lining papillary structures exhibited marked polymorphism with atypical, often plump nuclei. Mitotic figures were rare and areas of necrosis were observed only in one case. Immunohistochemical staining showed diffuse immunoreactivity for neuron-specific enolase, S-100 protein, cyto-keratin and vimentin. Focal reaction for synaptophysin and chromogranin A and epithelial membrane antigen (EMA) were observed. The tumours lacked expression of GFAP. The Ki-67 labelling index was relatively low but its focal increase was noted in two cases. The final diagnosis of PTPR was based on both predominant papillary morphology and immunohistochemical results. PTPR should be considered in diagnosis of pineal tumours but their natural history, therapeutic strategy and prognosis remain controversial.     

Tumours of the cauda equina.

A retrospective study of 70 consecutive patients with a cauda equina tumour who were admitted to Neurosurgical Department at the Radcliffe Infirmary, Oxford is presented. The diagnosis of these tumours is often difficult and delayed. The quality of life largely depends upon the neurological disability at presentation. The diagnostic features and investigations are discussed together with the treatment and prognosis.     

The relationship between neuropeptide Y expression and headache in pituitary tumours

Patients with pituitary tumours often present with disabling headache but there is no clear relationship between tumour size and headache. Neuropeptide Y (NPY) has been identified in pituitary tumours and may serve as a biochemical marker of the propensity for headache. Using immunohistochemical techniques we examined 27 consecutive pituitary adenoma specimens for NPY (including one normal postmortem control anterior pituitary specimen). A separate observer divided the patients into two groups: headache and non-headache. The association between the presence of NPY and headache was tested. NPY positive immunoreactivity was seen in 13 tumour specimens (50%, 13 of 26 pituitary tumour specimens), characterized by cytoplasmic and nuclear staining patterns. There was no significant association between the presence of NPY and headache (chi(2) = 0.9, P = 0.34). We did not observe NPY in the normal anterior pituitary control specimen. NPY was present in four of five (80%) growth hormone-secreting tumours and two of two (100%) prolactinomas, compared with four of 11 (36%) non-functioning adenomas. The mechanism of many pituitary tumour-associated headaches remains undetermined. The significance of NPY positivity in pituitary tumours is unknown, although the results of this study may implicate this peptide in the control of somatotroph and lactotroph activity. Our data do not support a clear role for NPY pituitary tumour-associated headache.     

Familial occurrence of hemangioblastoma

Haemangioblastoma is a benign vascular neoplasm accounting for approximately 1-2% of all intracranial tumours. Patients with haemangioblastoma are aged usually about 40 years, the tumour is situated mostly in the cerebellum, more frequently in males. The tumour is a component of the Hippel-Lindau syndrome with familial-hereditary dominant aetiology, often associated with retinal angiomatosis, cysts of the pancreas and kidneys, renal carcinoma and phaeochromocytoma. A familial haemangioblastoma is reported in father and son. The father had the tumour in cerebellar vermis but the son had multiple tumours in cerebellum and medulla.     

Hippel-Lindau disease]

Hippel-Lindau disease is one of inherited tumour susceptibility syndromes. The most common lesions are located in central nervous system, retina and visceral organs. In Poland the disease was rarely diagnosed although the prevalence is much higher than it was supposed and is estimated as 1: 30-50,000. It is inherited in an autosomal dominant manner with age related penetrance reaching almost 98% penetrance at the age of 60 and variable expression. The VHL gene is located near the tip of the short arm of chromosome 3 (3p25-26). Classical lesions in VHL patients are: haemangioblastomas of CNS, retina, cysts and clear cell carcinoma of kidney, cysts and tumours of pancreas, phaeochromocytoma and paraganglioma, papillary cystadenoma of epididymis and endolymphatic sac tumours. Multifocal, often bilateral lesions in form of benign cysts, vascular tumours or carcinomas occur. Management of the lesions often differs from that in sporadic cases of the tumours. Non-symptomatic lesions of CNS need no treatment, neither do non-symptomatic tumours of epididymis and some of phaeochromocytomas. Kidney carcinoma is treated when it reaches a certain size preferably by nephron-sparing surgery. Special care should be provided to pregnant VHL patients. Available DNA testing enables to identify VHL carriers. Although the mean age of death in VHL patients is 41 at the moment a proper prophylactic, diagnostic and treatment management can probably prolong survival of the patients and limit complications of the disease. The coordination between genetic consultants and clinicians is crucial in the management of the patients. The authors coordinate work of Polish VHL Registry and Polish VHL Association.     

Scintigraphic assessment of vascularity and blood-tissue barrier of human brain tumours.

Assessment of vascularity and blood-tissue barrier was performed by sequential scintigraphy in 43 patients with brain tumours. The blood-tumour barrier was evaluated by use of 99mTc-pertechnetate, and vascularity using 99mTc-labelled red blood cells. Three groups of tumours were found: tumours with low vascularity and permeable barrier, tumours with high vascularity and permeable barrier, and tumours with low vascularity and relatively impermeable barrier. The first group indicates that when vessels are permeable, there may be a rapid penetration of large amounts of pertechnetate into the tumour even when vascularity is not increased. In the other two groups penetration of pertechnetate into the tumour is affected by vascularity, as it determines the total area where passage of the radiopharmaceutical takes place. It is suggested that the permeability of the blood-tumour barrier and the amount of vascularity may have an effect on the success of chemotherapy in brain tumours.     

Interstitial radiobrachytherapy of malignant cerebral neoplasms: rationale, methodology, prospects

The local use of radionuclides in the management of neoplastic processes was initially considered over 80 yr ago and has enjoyed increasing enthusiasm in the treatment of somatic and central nervous system tumours during the past 30 yr. The marriage of complex neuroimaging techniques and modern stereotactic devices has markedly enhanced the technical precision of interstitial radiobrachytherapy of malignant cerebral neoplasms. In applying these techniques, it is imperative to achieve an optimal placement of radionuclide sources in order to develop a geometrically homogenous, controlled distribution of radiation. Critical considerations include determination of tumour volume and contour, and development of a homogenous dose rate (dependent upon multiple sources at varying intensity) that will not only effect tumour cell kill but do this without excessive production of radionecrosis which necessitates craniotomy because of mass. Using the Brown-Roberts-Wells (BRW) stereotactic guidance system and an image-defined, volumetrically determined target, implants of multiple iridium 192(192Ir) sources were used to establish appropriate isodose envelopes. A methodology for achieving the described objectives is detailed as it applies to a variety of malignant intracerebral neoplasms (glioblastoma multiforme, malignant astrocytoma, malignant mixed glioma, primary cerebral lymphoma, metastatic carcinoma and malignant pineal region tumours). Technical realization of precision implantation relying upon imaging data may be acheived with this method with satisfactory responses that are dependent upon histological tumour type and the morphology of the tumour distribution as related to the image. Early and late complications related to the surgical technique and radionuclide applications were less than 5%. Although encouraging, these techniques require further definition and greater data accrual before uniform application outside major medical centres can be justified. It is anticipated that improvement in results with intrinsic gliomas and other invasive neoplasms will be realized with further definition of tumour boundaries by tract biopsy techniques and concurrent utilization of hyperthermia and brain protective methods.     

Effect of brain tumour laterality on patients' perceived quality of life

OBJECTIVES: There is little reliable quantitative information on preoperative quality of life of patients with brain tumours. The aim of this study was to clarify the effect of the volume, location, and histological grade of brain tumours on the preoperative quality of life of patients. METHODS: The study population consisted of 101 successive patients with brain tumour at Oulu Clinic for Neurosurgery studied with CT or MRI for preoperative determination of tumour location and size. The Nottingham health profile (NHP) and Sintonen's 15D scale were used at that time to measure quality of life. RESULTS: Tumour size did not correlate linearly with impairment of quality of life. Large tumours (>25 ml) were associated with poorer quality of life than small tumours (< or =25 ml). The patients with a tumour located on the right side or in the anterior region reported a poorer quality of life than those with a tumour on the left side or posteriorly. Quality of life assessments made by doctors using the Karnofsky performance scale showed no differences between the two hemispheres. Patients with the most malignant gliomas (grades III-IV) displayed the poorest quality of life. CONCLUSIONS: Large tumours apparently damage several parts of the brain and/or raise intracranial pressure to a level that exceeds the brain's compensatory capacity. Contrary to earlier understanding, tumours in the right hemisphere seemed to be related to poorer quality of life. This effect was especially clear in the patients' subjective evaluation of their quality of life. As the location of the brain tumour thus affects perceived quality of life, any measurements of the quality of life of patients with brain tumours should take into account the location and laterality of the tumour.     

Functional rehabilitation and brain tumour patients. A review of outcome

Abstract The increasing prevalence of brain tumours and longer duration of survival achieved by recent advances in treatment prompt a critical analysis of the impact of functional rehabilitation on patients with brain tumours. In this review brain tumours and outcome of brain tumour patients are discussed from a rehabilitation perspective, taking into account not only life expectancy but also the direct and indirect causes of functional impairment. Results of functional rehabilitation and factors involved in its effectiveness are presented and analysed to serve as a basis to neurologists involved in the management of patients with brain tumours.     

Difficulties in stereotactic biopsies of brain tumors

MATERIAL: 28 stereotactic biopsies of organic brain processes (brain tumours) were performed in the years 1997-2000 in the Department of Neurosurgery, Medical University of Warsaw. In this series the lesions were located in corpus callosum in 5 patients, in basal nuclei in 9, and deeply in the white matter of cerebral hemispheres in 14. METHOD: The Baklund biopsy kit and Leksell's stereotactic frame were used, target localisation was based on the CT scan. Histological verification was based on hematoxillin--eosin staining, completed with histochemical evaluation if necessary. In 9 patients intraoperative smear evaluation was performed. RESULTS: Sensitivity of stereotactic biopsies was 86% (24/28), although the rate of conclusive biopsies was lower, being 60% (17/28). False negative results were observed in 14% of the patients (4/28). Analysis of the results revealed, that the sensitivity was not dependent on the size, neither on the location of the tumour, but was related to its morphology. The false negative results were obtained in the tumours with significant necrosis (as seen on CT scans). There were no surgical complications in this series. CONCLUSIONS: 1. Difficulties in stereotactic biopsies of brain tumours are associated mainly with tumour morphology. In tumours with marked necrosis, other degenerative changes or cystic ones, higher risk of non-conclusive biopsy may be expected. 2. Size of the tumour and its location do not affect the diagnosis based on the stereotactic biopsies. 3. In the polymorphic tumours, the policy to take biopsy material from different tumour sites, should be a rule, as different parts of the lesion may represent different stages of malignancy and histological evaluation of separate parts of the tumour may lead to inadequate oncological treatment.     

Diffusion and perfusion MR imaging in cerebral lymphomas

Because of the increasing incidence of cerebral lymphoma, it is critical for patient management to recognize the MR features of this disease. We present the characteristic morphological and functional MRI features of this tumor. The findings on MRI studies, including morphological, diffusion and perfusion imaging, performed in 9 biopsy-proven cases of cerebral lymphoma with 13 lesions are presented and analyzed, and are discussed in comparison with published literature data. All patients underwent diffusion-weighted imaging with a single shot echo-planar pulse sequence. Dynamic susceptibility-contrast MRI was performed using a T2*-weighted gradient-echo echo-planar sequence after intravenous injection of chelates of gadolinium at the rate of 6 ml/s and a temporal resolution of 1 second. All cases of cerebral lymphoma appeared hypointense or isointense on T1-weighted images and in 75% of cases iso- or hypointense on T2-weighted images. All lesions enhanced except one in a patient receiving steroid therapy. On diffusion-weighted images, tumours were hyperintense with normal or decreased ADC values (0.717+/-0.152.10-3 mm2/sec, range: 0.550-1.014) and an ADC ratio tumour/normal white matter of 0.974+/-0.190 (range: 0.768-1.410). On perfusion, the signal intensity-time curve of each tumour showed a characteristic type of curve with a significant increase of the signal intensity above the baseline and a low maximum relative cerebral blood volume ratio (rCVBmax) of 1.43+/-0.64 (0.55-2.62). Due to their higher cellularity, the lack of neoangiogenesis, and the increased permeability of the blood-brain barrier related to the infiltration of blood vessels wall by lymphomatous cells, cerebral lymphoma presents characteristic diffusion and perfusion MRI features that should be useful for diagnosis and patient follow-up.