First manifestation of lambda positive plasmacytoma in the orbital apex with acute unilateral loss of vision.

INTRODUCTION: An intracranial plasmacytoma is a rare form, which can involve the calvarium, dura or the cranial base. Only few case reports describe the manifestation of plasmacytoma of the skull base with affection of visual acuity. CASE REPORT: We describe the case of a 43-year-old woman, presenting with an acute unilateral loss of vision. The presumption diagnosis was retrobulbar neuritis as first manifestation of multiple sclerosis. MR imaging disclosed a tumour in the left orbital region and a meningioma was suspected. After complete resection with decompression of the optic nerve, the neuropathological examination revealed a lambda positive plasmacytoma. Additional work-up disclosed an involvement of multiple vertebral bodies. Due to the diagnosis of multiple myeloma, oncological therapy had been initiated. CONCLUSION: Skull base plasmacytoma is a rare disease. Solitary lesions causing neurological deficits should be treated aggressively including surgery for histological diagnosis and decompression of neural structures. Prognosis and further therapy depends on the systemic stage of disease, which has to be defined by diagnostic work-up.

T-cell lymphoma mimicking schwannoma of a cervical nerve root.

T-cell lymphoblastic lymphoma is a rare form of non-Hodgkin lymphoma, which shows preponderance for young men. Most common symptoms are painless swelling of lymph nodes, accompanied by B symptoms and large mediastinal masses. Most often, an involvement of the nervous system is due to paraneoplastic symptoms or side effects of treatment. In a literature research, we could not find a case with affection of a cervical nerve root as the first symptom for T-cell lymphoblastic lymphoma. A 39-year-old man presented with right-sided C8 radiculopathy, including pareses and paresthesia. Since the magnetic resonance image disclosed a right-sided mass lesion in the region of the neuroforamen C8, compressing the corresponding nerve root, a schwannoma was suspected. The tumor was removed using a dorsal approach. Neuropathological examination revealed the diagnosis of T-cell lymphoblastic lymphoma. The patient underwent diagnostic staging and received further treatment. He experienced a very grim course and succumbed to his disease 12 months after surgery. T-cell lymphoblastic lymphoma is a rare disease, and tropism of lymphoma cells to neural structures is seldom encountered. However, the presence of radiculopathy, together with signs, referring to B symptoms, should prompt the physician to consider this coincidence in the differential diagnosis of schwannoma.

Metastatic tumours mimicking vestibular schwannoma.

In this review of the literature we discuss the rare occurrence of metastatic tumours mimicking bilateral vestibular schwannoma and present an own case with pancreatic signet-ring cell carcinoma as primary tumour.

Measurement of intradiscal pressure after lumbar discectomy.

OBJECT: Early physiotherapeutic exercises after lumbar discectomy have been shown to be helpful for pain control and in reducing the delay until the return to work. Many strategies exclude sitting body positions during the first weeks, because raised intradiscal pressure and an increased motion range in the lower lumbar disc levels have been found in unoperated individuals when seated. To evaluate whether these results can be extrapolated to the situation of an operated patient, we examined the feasibility of an intracranial pressure sensor for measuring intradiscal pressure after lumbar discectomy. PATIENT AND METHOD: An otherwise healthy patient underwent lumbar discectomy for a right-sided lumbar disc herniation at the level L4/5. Before surgery he had agreed voluntarily to the implantation of a pressure sensor and the manufacturer had provided data on the safe use of the sensor in the disc space. Intraoperatively the sensor was positioned in the disc space from the right side. Measurements were performed by means of individual readings by three different examiners at different time points. Before removal of the sensor its correct position was demonstrated by X-ray and computed tomography. RESULTS: The patient tolerated surgery very well, recovery and wound healing were un-eventful, and symptoms improved. The pressure monitoring system showed reproducible results without any signs of technical problems. The intradiscal pressure values were about ten times lower than the values known from unoperated individuals (lying: up to 130 mmHg, sitting: up to 50 mmHg, standing: up to 450 mmHg. 100 mmHg=0.013332 MPa). CONCLUSION: We present an easy to perform and technically safe technique for the measurement of intradiscal pressure after lumbar discectomy using an intracranial sensor. To assess the impact of intradiscal pressure on recovery after lumbar microdiscectomy a study with a larger cohort of patients and with long-term follow-up will be needed.

Glioblastoma simultaneously present with meningioma--report of three cases.

BACKGROUND: Most primary intracranial tumors occur as solitary lesions; multiple locations of one tumor, the occurrence of two different tumors or even collision tumors have been described only in a few patients. From a statistical point of view, in less than 100 glioblastoma cases will a meningioma be simultaneously present in the brain. We report three cases with this coincidence and display the results of CGH and chromosome analysis in two patients, in whom the tumors arose in very close spatial correlation to each other. PATIENTS: We describe three case histories with simultaneous occurrence of meningioma and glioblastoma as shown by MRI on admission. After neurosurgical removal of mass lesions, specimens from two patients were cultivated in cell culture and the cells were examined for chromosomal aberrations by conventional karyotyping as well as comparative genomic hybridization (CGH). RESULTS: Examinations disclosed characteristic genetic aberrations for one meningioma and two glioblastomas. In one patient it was possible to compare the data for the meningioma and the glioblastoma; in this case we did not find a common genetic aberration in tumor cells with a different histology. CONCLUSION: Genetic testing of tumor cells should be performed routinely when different histological types of brain tumors are present in a close spatial relationship. We favor the hypothesis of statistical coincidence for the simultaneous occurrence of the two tumors rather than a common pathway giving rise to two tumor entities.

[L-2-hydroxyglutaric aciduria -- a rare cause of macrocephaly]. / L-2-Hydroxy-Glutarazidurie -- eine seltene Differenzialdiagnose der Makrozephalie.

We report on a 9-year-old girl who was referred to our department because of increasing macrocephaly and school problems. The neurological examination disclosed mild cerebellar dysfunction and positive pyramidal tract signs. An MRI of the brain revealed extensive signal alterations of the white matter. Biochemical investigations established the diagnosis of L-2-hydroxyglutaric aciduria which has to be kept in mind as a rare cause of macrocephaly.

Endolymphatic sac tumour. A case report.

We present the case of a patient with an endolymphatic sac tumour (ELST). This rare tumour entity has only recently been defined and despite a well characterized clinical appearance misdiagnosis as jugular paraganglioma is frequent. A 68-year-old woman was admitted to our Neurosurgical Department with a mass lesion extending from the left temporal bone to the cerebello-pontine angle (CPA). Radiological features were high vascularization, contrast enhancement and destruction of the os petrosum. After preoperative angiographic embolization the tumour was removed in two surgical interventions, first via a lateral suboccipital approach and second by petrosectomy. The lesion proved to be a typical endolymphatic sac tumour by a synopsis of histological, radiological and clinical features. ELST should be taken into consideration in patients with mass lesions in the cerebellopontine angle destroying the petrous bone and resembling paraganglioma. Since slow growth rate and lack of metastases are particular features of ELST, complete resection of the tumour results in long survival times without adjuvant chemo- or radiotherapy.