Lipomatous medulloblastoma in adults. A distinct clinicopathological entity.

We report on three patients who presented with a cerebellar medulloblastoma at age 48, 53, and 59 years. Histopathology showed typical features of medulloblastoma, in one case with marked neuronal differentiation. In addition, all neoplasms contained focal accumulations of mature fat cells. Immunoreactivity of adipocytes for S-100 protein, neuron-specific enolase, synaptophysin, microtubule-associated protein-2, and glial fibrillary acidic protein and the lack of immunoreactivity to type IV collagen suggest lipomatous differentiation of neoplastic primitive neuroectodermal cells rather than an admixture of mesenchymal elements. Mitotic activity was low and the growth faction, as determined by the MIB-1 labeling index, was less than 5%. All patients are alive with a recurrence-free interval ranging from 3.5 to 12 years. These three patients and five similar previously reported cases all fit into the concept of the lipomatous medulloblastoma as a new clinicopathological entity characterized by (a) typical features of a cerebellar medulloblastoma with advanced neuronal differentiation, (b) areas of lipomatous differentiation, (c) low proliferative potential, (d) manifestation in adults (mean age, 50 years), and (e) apparent favorable clinical prognosis.

Chondrosarcoma of the temporal bone in an infant: case report and review of the literature.

Cranial chondrosarcomas are rare. The majority of cases arise from the base of the skull and are most common in adult life. In this report, an infant with a chondrosarcoma of the temporal bone is discussed. We are not aware of a previously reported case of chondrosarcoma of the temporal bone in this age group.

Intramedullary epidermoid cyst of the cervical spinal cord associated with an extraspinal neuroenteric cyst: case report.

A 4-year-old girl presented with an intramedullary epidermoid cyst of the cervical spinal cord. The clinical, radiological, and surgical features and a brief critical review of the literature are included in this report. The cyst contents were removed totally in two operations. The child had a coexisting neuroenteric cyst in the posterior mediastinum. To our knowledge, this coexistence has not been previously reported. Contemporary imaging modes and prospects of the surgical treatment are discussed.

Vertebral hemangioma symptomatic during pregnancy--report of a case and review of the literature.

A spinal tumor complicating pregnancy is a rare condition. A 25-year-old woman who became paraplegic during the 35th week of her second pregnancy presented during the postpartum period. She underwent two surgical interventions, and the cord compression caused by a T5 vertebral body hemangioma with laminar involvement and extradural extension was relieved. The occurrence of vertebral hemangiomas during pregnancy is discussed; the radiological features with special reference to magnetic resonance imaging are outlined; and cases from the literature are reviewed.

Optic glioma of childhood: clinical, histopathological, and histochemical observations.

This report documents the long-term clinical and histopathological behaviour of eight intraorbital and 16 intracranial optic nerve gliomas and relates the therapeutic data to the prognosis for both visual acuity and survival. The mean age at onset of symptoms was 8.6 years and at the diagnosis 10.9 years. It is generally held that proptosis is mild in intraorbital glioma, but we encountered marked proptosis ranging from 7 to 12 mm in six of the eight intraorbital gliomas, which contained abundant Alcian-blue-positive mucoid material. Of the patients with intracranial optic nerve gliomas 37.5% survived for a mean of eight years after treatment with radiotherapy or surgery combined with radiotherapy. At follow-up ranging from five months to 11 years only one of the six patients with intracranial gliomas had full visual acuity. Our observations emphasise that, although optic nerve gliomas are benign hamartomas, the prognosis for visual acuity and survival is unfavourable in cases which are diagnosed and treated late. Histopathological and histochemical observations suggest that increase in the amount of mucoid material may contribute to rapid enlargement of intraorbital and intracranial optic gliomas.

Intracranial solitary chondroma. Case report.

A patient is described who had a solitary left frontal intracranial chondroma originating from the falx cerebri. The tumor was totally removed. The diagnostic value of computerized tomography and the surgical findings in this rare pathological condition are discussed.

Balo's concentric sclerosis. Report of two patients with magnetic resonance imaging follow-up.

Balo's concentric sclerosis was diagnosed antemortem in 2 patients, by magnetic resonance (MR) imaging showing striking concentric alternating rings in 1 patient and by characteristic histopathological features in the other. The course of the lesions and the concentric pattern were followed by MR imaging for 3 years and 18 months, respectively. One patient demonstrated spontaneous remission that has not been reported in Balo's disease. Balo's disease may not have a fulminant course as described in the past and the MR appearance of the chronic lesion may resemble that of a chronic multiple sclerosis plaque.

Acoustic neurinoma with intratumoral bleeding.

The Authors present a case of acoustic neurinoma which has caused intratumoral bleeding. Although acoustic neurinomas are about 8-10% of all intracranial tumors there are only few cases about acoustic neurinomas causing hemorrhage. Pathologic changes of intratumoral vessels in tumors larger than 2 cm were considered to be a cause of the hemorrhage. This rare pathology must be remembered as one of the causes of the intracranial bleeding.

Case report and review of the literature: arachnoid cyst of the fourth ventricle presenting as a syndrome of normal pressure hydrocephalus.

The Authors report a case of 4th ventricular arachnoid cyst that presented clinically with the criteria of normal pressure hydrocephalus (NPH). Only a few cases of intraventricular arachnoid cyst have been recorded in the literature; the present case is the second reported case diagnosed as a syndrome of NPH.

Adult medulloblastoma presenting as a large neck tumor. Case report.

Extension of neuroepithelial tumors outside the neuroaxis is uncommon and usually follows surgical intervention. We report a patient with medulloblastoma of the right cerebellar hemisphere with extracranial extension presenting as a large neck tumor and discuss the clinical and pathological features of this case.

Jugular foramen meningioma. Report of a case and review of the literature.

The occurrence of a meningioma within the jugular foramen is very rare. We present the case of a 33-year-old man who presented with five-year history of hoarseness and was found to have paralysis of lower four cranial nerves on the left. High resolution computed tomography showed a dumbbell shaped tumor of the left jugular foramen extending intracranially over the jugular tubercle and extracranially into the parapharyngeal space. The patient underwent staged neuro-otological surgery and the mass was removed subtotally with no recurrence in six years. Histopathologic diagnosis was transitional meningioma. Our review of the literature revealed 33 previously reported cases. Four of these cases had neurofibromatosis. All but one underwent some form of otolaryngologic surgery. Although twenty had an intracranial component to their tumor, only 11 had a craniotomy. The majority of the tumors were meningothelial meningiomas (60%). Only two cases were malignant meningiomas. We reviewed the clinical and radiological characteristics and summarized the benefits and pitfalls of existing surgical options.

Solitary skull metastasis from occult thyroid carcinoma.

Metastatic tumors to the skull, though relatively rare, are most often from lung, breast or prostate. In this report, we describe a case of skull metastasis from occult thyroid carcinoma and discuss the differential diagnosis of these lesions.

Nonsuture closure of arterial defect by vein graft using isobutyl-2-cyanoacrylate as a tissue adhesive.

Nonsuture repair of intracranial blood vessel is still a challenging issue in neurosurgery. In this experimental study isobutyl-2-cyanoacrylate was used for nonsuture repairing of carotid artery to observe its histotoxicity. Isobutyl-2-cyanoacrylate was dropped over the edge of defect on carotid artery of rat under the surgical microscope and jugular vein was placed to cover the defect. Rats were divided in 6 group according to postoperative survival period (2, 5, 7, 14, 21, 28 days respectively). The carotid arteries were patent of all rats according to angiography and autopsy specimen. Histopathological results showed that: the changes are mainly inflammatory respons +, there was no necrotic tissue at any time the healing progress and no significant differences in the vein graft. Therefore isobutyl-2-cyanoacrylate is considered helpful and safe adhesive material for the nonsuture repair of blood vessel.

Cerebral cladosporiosis.

A 49-year-old woman was admitted to Hacettepe Medical Faculty Hospital with the complaints of headache, nausea, vomiting, lethargy, and weakness on her right side. She revealed a history of pulmonary Cryptococcus infection 5 years before and she had been treated with amphotericin B. After clinical and laboratory investigation she was thought to have an intracranial mass, but her deteriorating situation did not allow any surgical intervention; she died within 7 days. On necropsy, hard, gray-white nodular pulmonary lesions, ranging 0.1-4 cm in diameter, basal meningitis, infarcts, and a nodular lesion 1.5 cm in diameter similar to those of the lung were present in the white matter of the right hemisphere of the brain. Microscopic examination revealed granulomatous inflammation caused by Cladosporium, which had brown pigment and septate hyphae.

Ossified somatotropinoma.

Calcification of pituitary adenomas as a histopathologic finding is reported in up to 25% of all degenerated adenomas. On the other hand, ossification of these adenomas is an extremely rare finding, with only one reported case, a prolactinoma. Here, we report a case of somatotropinoma in a 42-year-old acromegalic female. The adenoma, about 2 cm in diameter, was removed by the transsphenoidal route. Pathologic examination revealed ossification areas in the adenoma and the patient showed dramatic progress with relief of acromegaly signs and normalization of serum growth hormone levels. This case seems to be a unique report of osteoid metaplasia of a somatotropinoma.

Familial glioma in two siblings.

We report two siblings who had intracranial gliomas, one with glioblastoma multiforme and the other with mixed oligo-astrocytoma. The genetic influences in the development of glioma are discussed.

Autosomal recessive alobar holoprosencephaly with cyclops in three female sibs: prenatal ultrasonographic diagnosis at 18th week.

A couple who were first cousins were referred for genetic counselling because of a previous spontaneous abortion and two female stillbirths with cyclopia. In the 18th week of the mother's fifth pregnancy a holoprosencephalic fetus was diagnosed by ultrasonography. The pregnancy was terminated. Postmortem computed tomography and pathological examination revealed that the female fetus had alobar holoprosencephaly with cyclopia. Chromosomal analysis of the fetus was normal.

Bilateral subpleural ectopic brain tissue in a 23-week-old fetus.

Bilateral lesions were seen in the subpleural region in a 23-week-old aborted male fetus. This fetus was not macerated and showed no central nervous system abnormality on physical examination and vertebral magnetic resonance imaging (MRI). Postmortem examination revealed bilateral, paravertebral, subpleural, circumscribed, yellowish-white, fluent lesions 2.5 x 1 x 1 cm in size. These lesions were localized on the upper part of both lungs and there was no other internal malformation. Histological examination of lesions showed adult neurones and well-differentiated neural tissue with white and gray matter, choroid plexus, ependymal structures and, rarely, some peripheral neural cells in addition to immature neuroectodermal cells. These cells were more mature than those in the brain tissue.

Meckel Gruber syndrome: a case diagnosed in utero.

A case of Meckel Gruber syndrome is presented, diagnosed prenatally from the medical history of the mother which revealed a previous malformed stillborn with anencephaly, meningomyelocele, polydactyly and ambiguous genitalia. This was the first prenatally diagnosed case ever reported in Turkey. The clinical, computed tomography and postmortem findings and the related literature are reviewed.