Primitive neuroectodermal tumor of the central nervous system with glial differentiation: a FISH study of an adult case.

Primitive neuroectodermal tumors (PNETs) of the central nervous system (CNS), a rare occurrence in adults, may show glial differentiation and can be misinterpreted as pure astrocytic neoplasms. Few fluorescence in situ hybridization (FISH) studies have been carried out on these tumors; isochromosome 17q was found to be the major chromosomal abnormality. We present the case of an adult in which we performed a FISH study of both the glial and neuronal components. A complex array of FISH changes, not including an isochromosome 17q were identified.

April 2003: 55-year-old male with necrotic pontine lesion.

The April Case of the Month (COM). A 55-year-old male with history of diabetes mellitus presented with progressive loss of sensitivity on the left side of the body and horizontal diplopia. Symptoms appeared after a right basal pneumonia one month before admission. A CT scan showed an large inoperable lesion in the pons. The patient was treated with corticoids. One week later, the patient showed general deterioration. The fifth and sixth right cranial nerves were affected. Ataxia and disorders in swallowing were also present. A second CT scan showed that the pontine mass had become larger. The patient died 7 days after his admission and the autopsy was limited to CNS. The right middle cerebellar peduncle showed a 2-cm well-defined white brownish necrotic lesion that extended to the pons and periventricular gray matter. Microscopic examination revealed toxoplasmosis, which was confirmed by immunohistochemical studies. The brain tissue was negative for HIV by PCR. Toxoplasmosis is a well-known complication of AIDS, and has been reported in post-transplant patients as well, but only a few reports of toxoplasmosis in diabetics have been reported.

Tumor fibroso solitario meníngeo / No disponible

El tumor fibroso solitario es una entidad conocida en pleura y otras localizaciones, pero rara en las meninges. Presentamos el caso de un tumor fibroso solitario en cola de caballo y revisamos los casos publicados en la Literatura (AU)

Solitary fibrous tumor is an entity arising in the pleura and at other locations but is rarely found in the meninges. We present one case of this tumor, located in the spinal canal (cauda equina), and review the literature (AU)

Clear cell meningioma of the lumbo-sacral spine with chordoid features.

Clear cell meningioma (CCM) is a peculiar variant that differs from conventional meningioma in affecting younger patients, arising more often in spinal or cerebellopontine locations, and showing a higher recurrence rate. Classical meningothelial areas are scarce in these tumors and the differential diagnosis with other neoplasms, particularly metastatic carcinoma, is often difficult. We report a case of clear cell meningioma from the lumbosacral spine in which location, radiologic presentation, light microscopic appearance in initial sampling, and some of the ultrastructural findings were reminiscent of chordoma. The tumor cells were diffusely positive for vimentin and very focally positive for epithelial membrane antigen. Ultrastructural demonstration of interdigitating cell processes joined by numerous desmosomes confirmed the diagnosis of CCM.

Meningeal melanocytoma: a case report and literature review.

The clinicopathologic, immunocytochemical, and electron microscopic features of a case of meningeal melanocytoma arising in the phylum terminale are reported. Meningeal melanocytoma is an uncommon tumor that must be distinguished from metastatic or primary malignant melanoma, meningeal melanocytic nevi, pigmented meningioma, pigmented schwannoma or neurofibroma, and pigmented primitive neuroectodermal tumor. This is a difficult differential diagnosis that can be best archived by complementing histological examination with a selected panel of antibodies and, most important, electron microscopic study. The distinctive ultrastructural appearance of most of these lesions point to this technique as the gold standard in pigmented proliferations of the nervous system.

[Congenital diaphragmatic hernia: evaluation of two ways of management]. / Hernia diafragmática congénita: análisis de dos formas de tratamiento.

OBJECTIVE: The aim of this study was to analyze the treatment and evolution of congenital diaphragmatic hernia in the last 16 years, distinguishing two ways of management, and to look for parameters that can predict the evolution. MATERIALS AND METHODS: Between 1978 and 1994, 29 cases of congenital diaphragmatic hernia were treated in our NICU. During the first period (1978-1988) 17 cases (group 1) were treated after birth as a surgical emergency. In the second period (1989-1994), preoperative stabilization was performed before surgery (12 cases, group 2). Two cases of group 2 were excluded because of the association of other malformations that were the cause of death. RESULTS: Both groups were similar in gestational age, birth weight and Apgar score at 5 minutes. Overall mortality was 48.1% (47.0% in group 1 and 50.0% in group 2). No infants with PaCO2 greater than 40 mmHg and OI greater than 40 or VEI over 1,000 survived. CONCLUSION: Despite preoperative stabilization, there is no difference in the mortality rate of the two groups. With congenital diaphragmatic hernia, as with other entities of low incidence, collaborative studies are needed to obtain enough cases to analyze the results more precisely.

Contralateral trigeminal neuralgia as a false localizing sign in intracranial tumors.

Three cases of contralateral trigeminal neuralgia as a false localizing sign in intracranial tumors are reported. In each patient (meningioma, 2 cases; cholesteatoma, 1 case) the tumor was asymptomatic, so that intracranial tumor had not been suspected preoperatively. In all cases, tumors were large and firm. The tumor was supratentorial in two cases. In one case, a cortically mediated mechanism may have caused the neuralgia, whereas in the remaining two cases distortion and displacement of the brain stem and compression of the contralateral Meckel's cave would explain the trigeminal nerve signs.

Symptomatic eosinophilic granuloma of the spine: report of two cases and review of the literature.

We report two cases of eosinophilic granuloma of the spine in which the first manifestation of the disease was an associated neural deficit. In addition, we review the literature for cases of histiocytosis X in which the initial diagnosis was based only upon the discovery of spinal lesions. Thirty-nine reported cases of what we designated as "symptomatic eosinophilic granuloma of the spine" were found. A review of all 41 cases revealed that most patients present with pain, muscular stiffness and/or neurologic symptoms, the disorder most often affects the cervical vertebrae, and a high incidence of osteolytic lesions (as opposed to vertebral collapse) are found. These findings vary from those of radiologic and clinical evaluations of classical, multifocal, histiocytosis X that involves the spine.

[Intracranial neurinoma of the spinal nerve (author's transl)]. / Neurinome intracranien du nerf spinal.

A patient aged 56 years has developed a syndrome affecting the last cranial nerves--IX, X, XI and XII--on the left side progressively over the last two years, and, more recently, deafness. Apart from a simple radiological examination all other radiological tests were negative. The diagnosis was made during the surgical operation which revealed a neurinoma of nerve XI (spinal) in its intracranial path. The diagnosis was confirmed histologically. After reviewing the published literature, the authors conclude that this is an exceptional case, which justifies publication and enables differential diagnosis to be made between this tumor and the Jugular Glomus tumor.