Biomedical subjects
Publications and source records attributed to L Rigobello.
Cytogenetic studies on a supratentorial ependymoma from a 1-year-old boy showed a t(11;17)(q13;q21). This is the second ependymoma reported with a rearrangement at 11q13; to our knowledge the 11q13 is the first recurring breakpoint reported in ependymoma.
We present four cases of infantile cerebellar neoplasms composed of cells with large vesicular nuclei with prominent nucleoli. All four cases were strongly immunoreactive for synaptophysin, and one case showed immunoreactivity for neurofilaments. Filter hybridization for N-myc and c-myc oncogenes showed a 27-fold c-myc amplification in one case. The cytogenetic analysis in this case showed Double-Minutes and isochromosome 17q. An intracerebral xenograft in nude mice obtained from one such tumor showed a similar morphology to that of the original tumor as well as strong immunoreactivity for synaptophysin and neurofilaments. All the neoplasms were characterized by highly aggressive behavior leading to early cerebrospinal fluid dissemination despite radiotherapy and chemotherapy. We conclude that large-cell medulloblastoma represents a distinct and more aggressive variant of medulloblastoma that requires more aggressive therapy.
N-myc and c-myc amplification was investigated in 27 medulloblastomas. DNA was extracted from 19 formalin fixed and paraffin embedded tumors and from fresh frozen tumor tissue in 8 other cases. The results showed no evidence of amplification of N-myc oncogene and only 1 case had a 27 fold amplification of c-myc. Cytogenetically, this neoplasm presented numerous double minute chromosomes (DMs). Moreover, it had an unusual rapidly aggressive course with massive cerebrospinal fluid dissemination unresponsive to intrathecal chemotherapy. Our results indicate a low incidence of N-myc and c-myc gene amplification in medulloblastomas, suggesting that the oncogenic mechanism in these neoplasms is not closely related to DNA gene amplification. C-myc amplification, although not frequently observed, may however provide a growth advantage for medulloblastoma cells in vivo, favoring their rapid dissemination. Medulloblastomas with c-myc activation may represent a subgroup of tumors with a more aggressive behavior.
The case of a 68-year-old woman who had relatively acute, unilateral ophthalmoplegia is reported. Radiological studies indicated a mass lesion involving the pituitary gland and left cavernous sinus. Pathological tissue obtained by the transsphenoidal approach revealed the presence of a Coccidioides granuloma. This pathological entity should be considered when evaluating patients with a pituitary mass and ophthalmoplegia.
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The authors discuss the criteria of differential diagnosis for a correct use of the term "multiple meningiomas". Reviewing a series of 934 meningiomas, of which 834 were intracranial and 100 were spinal, they found 14 cases of multiple meningiomas, i.e. an incidence of 1.5%. The study shows that multiple meningiomas are quite similar to solitary meningiomas in all their biological characteristics. Furthermore, the possible presence of more than one meningioma must always be kept in mind in the clinical and radiological evaluation of a patient. The aetiology of multiple separate meningiomas is discussed: the hypothesis which suggests their origin from multicentric neoplastic foci activated by a supposed "tumour-producing factor" appears to be the most reliable.
Two cases of pituitary abscess are reported. Bacteriological and histological findings of both cases are discussed, together with data available from the literature.
A rare case of intracranial hypertension associated with subarachnoid bleeding from a spinal tumour is reported. Clinical features, review of the literature and pathogenetic factors are discussed.
5 patients with gliomas of the anterior optic pathways, histologically assessed or with strong diagnostic evidence, who developed precocious or pseudoprecocious puberty (2 cases) before the operation or after radiation treatment, are presented. Only 2 patients presented familial and/or personal evidence of neurofibromatosis. The production mechanism of these sexual changes is discussed.
Tapia's syndrome is characterized by unilateral paralysis of the tongue and vocal cord, and is caused by a lesion of the 10th and 12th cranial nerves below the nodose ganglion, without involvement of the pharyngeal branches of the 10th nerve. The authors report the case of a 25-year-old man who presented with a 4-year history of progressive glossolaryngeal paralysis. Operation through the right laterocervical region allowed complete removal of a neurofibroma involving the 10th and 12th nerves at their crossing below the nodose ganglion.
A retrospective appraisal of traditional neuroradiological techniques (such as direct röntgenograms, cerebral angiography RISA-cisternography, and pneumo-cisterno-encephalography, as opposed to the "new" technique of computer-assisted tomography) was carried out in a series of 82 cases of opto-chiasmatic arachnoiditis, all surgically verified. It is concluded that none of these examinations can provide a reliable diagnosis of opto-chiasmatic arachnoiditis, which preoperatively can only be a tentative diagnosis that becomes final only when confirmed by surgical findings. In the presence of certain progressive neuro-ophthalmological symptoms and signs, a negative neuro-radiological investigation should not deter the neurosurgeon from exploring the chiasmatic region. Computer-assisted tomography, of which the authors have no personal experience in these cases, may hold the future answer to the diagnostic problem.
One hundred and forty consecutive subarachnoid haemorrhages (SAH) which presented either an intracranial vascular malformation (102 cases: aneurysm 80 cases, AVH 22 cases) or remained of "unknown aetiology" (38 cases) were studied. SAH caused by other factors (neoplasms, thrombo-embolisms, systemic diseases etc.) were excluded. The 38 cases with bleeding of "unknown aetiology" have been studied by complete cerebral angiography, pneumoencephalogram or CT scan, and have been followed for two years. The most important clinical factors in the three groups have been compared by a statistical method to verify the hypothesis that SAH of "unknown aetiology" is caused by vascular micromalformations which are angiographically not evident either because of their small size or because of their "spontaneous" recovery after bleeding due to thrombosis and disappearance of the malformation. From the data collected it may be concluded that SAH of "unknown aetiology" is a benign lesion, typically occurring in middle age, but with no clear characteristics that enable identification of the anatomical substratum from it originates.
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Six patients (3 couples of brothers) with adhesive arachnoiditis of the opto-chiasmatic cistern, surgically verified, are presented. Only one couple of brothers offered a possible family history of a similar condition in one maternal uncle. The visual damage had almost constantly a sudden onset and was predominantly of the axial type. No gross pathology, other than the arachnoiditic involvement of the chiasmal cistern and alterations of the anterior optic pathways, was found at surgery. Pneumocisternoencephalographic findings did not prove constantly reliable for a preoperative diagnosis of the condition when compared with surgical pathology. The overall results of the neurosurgical operations (exploration of the chiasm and removal of the adhesions) can be considered as favorable and this type of treatment is once again recommended as the therapy of choice without undue delay when general or local medical treatment proves of no avail after a reasonable length of time.
The so called colloid cyst of the third ventricle is believed to originate from the neuroepithelium in the diencephalic roof. The diagnosis is notoriously difficult and contributes significantly to the high mortality. A case in an eleven-year-old boy is reported. Computed tomography, also because not invasive, is chiefly indicative in this lesion, reducing the overall mortality.
Cysts of Rathke's cleft are considered as benign lesions, a practically permanent cure of which can be achieved by aspiration of the fluid content and ample opening of the capsule. The case of a patient who suffered from a recurrence of the cyst, 13 years after a first operation carried out according to such surgical criteria, is reported in order to signal the actual possibility of recurrence of such lesions.
Plexus-chorioideus carcinomata or malignant plexus papillomas are very rare; only few cases have been described in the literature. A further observation is added now. In a 13-year-old girl, signs of brain pressure occured two weeks before admission to a hospital. Scintigraphy and vertebral angiography showed a process in the atrium of the right lateral ventricle which was removed by temporo-parietal trephination. The tumour had the size of an apricot and a reddish colouration, it proceeded from the plexus. After-irradiation with 5 000 rad. Six years after the operation, signs of brain pressure appeared again. Neuroradiologically the process was located in the right cerebellar hemisphere. After trephination of the cerebellum, the largely vascularised tumour, which appeared to originate from the tela choroidea of the 4th ventricle, could be removed. The further course was without pathological findings. Histologically, the typical criteria were found: Invasion of surrounding brain structures, loss of the regular papillary structure, malignisation of the cells and originating from the plexus choroideus.
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