April 2001: a 70 year old woman with recurrent meningioma.

The April Case of the Month (COM). The contributors report a case of a 70 year-old woman with recurrent meningiomas, one of which showed rhabdoid and lipomatous differentiation. Histopathological study of the first and second previous resections showed only typical meningothelial meningioma. On the third craniotomy, a new tumor specimen showed an admixture of classic meningothelial meningioma with lipomatous and rhabdoid foci. Immunohistochemical studies showed diffuse reactivity for epithelial membrane antigen and vimentin, as well as focal positivity for desmin and smooth muscle actin in the areas with rhabdoid features and S100 protein in the lipomatous foci. The presence of these three different and concomitant histological patterns only in the third surgical resection might support a metaplastic origin and, also, corroborates the concept that rhabdoid features are suggestive of an aggressive behavior.

Pleomorphic xanthoastrocytoma: report of a case diagnosed by intraoperative cytopathological examination.

The authors present an unusual case of a 13-yr-old boy with a 3-mo history of seizures. A CT scan showed a contrast-enhancing mass located in the left temporal lobe. The patient underwent a stereotatic-guided craniotomy; intraoperative cytological diagnosis was performed by the smear technique, showing a pleomorphic xanthoastrocytoma. The tumor was totally resected. Definitive diagnosis was established by examination of paraffin-embedded material. Six months after the surgical intervention, the patient is doing well, with no radiological evidence of recurrence. The cytological differential diagnosis of giant cell lesions of the central nervous system is emphasized.

Mammary epithelioid myofibroblastoma arising in bilateral gynecomastia: case report with immunohistochemical profile.

Myofibroblastoma of the breast is a rare benign neoplasm, which has rarely been reported in association with gynecomastia. We report a case of a 25-year-old male patient with an epithelioid myofibroblastoma arising in a context of bilateral gynecomastia. The lesion was composed of nests and cords of epithelioid cells, with round to oval nuclei, granular chromatin, and distinct nucleoli dispersed in a myxoid to collagenous stroma with marked vascular proliferation. Immunohistochemical profile showed diffuse positivity for vimentin and focal immunoreactivity for desmin, whereas cytokeratins (CAM5.2 and AE1/AE3), EMA, alpha smooth muscle actin, actin HHF35, CEA, S100, factor VIII, neuron-specific enolase, CD31, and CD34 were all negative. We emphasize that this diagnosis is difficult to establish, owing to the rarity of this variant and clinical presentation.

Cavernous sinus thrombosis caused by zygomycosis after unrelated bone marrow transplantation.

Invasive zygomycosis is a devastating fungal infection occurring as an opportunistic infection after bone marrow transplantation (BMT). Sinusitis can lead to fungal infection in immunosuppressed patients, and cavernous sinus thrombosis, an uncommon condition in immunocompetent patients, typically follows an infection involving the medial third of the face, nose, or paranasal sinuses. Patients undergoing unrelated-donor BMT (UD-BMT) are prone to develop life-threatening infections because of poor recovery of cellular immunity. Despite adequate clinical evaluation and treatment, the prognosis of patients with invasive fungal infections is dismal, especially when intracerebral structures are affected. We describe a case of a patient who underwent an UD-BMT and developed cavernous sinus thrombosis after sinusitis due to zygomycosis. Moreover, he also had disseminated fungal (Zygomycetes and Aspergillus) and viral (cytomegalovirus and adenovirus) infections.

[Neurocysticercosis: a clinical and pathological study of 27 necropsied cases]. / Neurocisticercose: estudo clínico e patológico de 27 casos de necrópsia.

Neurocysticercosis is the most frequent and widespread neuroparasitosis of the human being. The development of brain and leptomeningeal lesions, with subsequent symptoms, are mainly related with the immune status of the host, and to the number and evolutional phase of the parasites. We present the pathological findings in 27 necropsies of patients with neurocysticercosis, which accounted for 3.1% of the necropsies. 77% of the patients were male and the age ranged from 18 to 85 years. In 26% there was previous history of alcoholism. Clinicopathological study showed that 50% of the cases were classified as asymptomatic form, 11% epileptic form, 11% intraventricular form and 11% combined form. 33% of the patients presented seizures as a factor of aggravation of the clinical picture. There was a single cysticercus in 60% of the cases, the cellulosae form present in 82% and the racemous form in 7% of the cases; the remaining 11% had both forms present. In 30% of the patients the cause of death was directly related with the presence of the cysticercus in the central nervous system. Our findings confirm the high morbidity of this disease.

[Haemangioblastomas: clinical, epidemiological and pathological findings in 14 cases]. / Hemangioblastomas: achados clínicos, epidemiológicos e anatomopatológicos em 14 casos.

We report the clinical, epidemiological and pathological findings of 14 patients with haemangioblastoma. Sixty-four percent occurred in males, with ages ranging from 16 to 60 years, with an average of 34, 4 years. Most of the tumours were confined to the cerebellum (n=9). The most frequent symptoms were headache (n=7) and dizziness (n=7), with a mean duration of 70 days. Von Hippel-Lindau syndrome (vHL) was diagnosed in 3 patients. Eleven patients were submitted to total surgical removal and in 3 the tumour was partially resected. A relapse rate of 28% in 3 years of follow-up was found. The patients with vHL showed recurrence in 66% of the cases. These findings corroborate those in current medical literature, showing an increased morbidity of this tumor when associated with vHL.

[Medulloblastomas: clinical, epidemiological and pathological findings in 28 cases]. / Meduloblastomas: achados clínicos, epidemiológicos e anátomo-patológicos de 28 casos.

We report the clinical, epidemiological and pathological findings of 28 patients with medulloblastoma: 22 were male; age ranged from 1 to 50 years, with a mean of 15 years. The most frequent symptoms and signs were headache (64%) and vomiting (64%). Only one patient showed a desmoplastic medulloblastoma variant, the others showed classical medulloblastomas. Regarding treatment, most patients were submitted to total resection (n=10) or partial tumorectomy (n=7). A low rate of tumoral recurrence was observed, occurring in 21% of the patients (n=6) after 4 years of follow-up. Chemotherapy seemed to contribute to a lower recurrence rate amongst our patients. Our findings are similar to those reported in literature, thus helping to understand the biological behavior of this type of tumor.

Evaluation of cell proliferation, epidermal growth factor receptor, and bcl-2 immunoexpression as prognostic factors for patients with World Health Organization grade 2 oligodendroglioma.

BACKGROUND: Prognostic factors in oligodendrogliomas are an area of controversy in neuropathology. Although grading and the study of some morphologic variables may be of value as prognostic parameters, the variability of postoperative disease free survival in patients with World Health Organization Grade 2 oligodendroglioma indicates that the biologic behavior of this entity remains unknown. The objective of the current study was to evaluate immunoexpression of the proliferation index (PI), epidermal growth factor receptor (EGFR), and bcl-2 as prognostic factors in patients with Grade 2 oligodendroglioma. METHODS: In a series of 19 cases of pure Grade 2 oligodendroglioma, we assessed the mitotic count, labeling index for MIB-1 and PCNA, and immunoreactivity for EGFR and bcl-2 with semiquantitative parameters and compared these with postoperative disease free survival. Statistical analyses using the Cox-Mantel nonparametric test and Spearman correlation coefficient were used to evaluate the data. RESULTS: Disease free survival was significantly shorter when the MIB-1 PI was > 5% (P = 0.0096) and the PCNA PI was > 9% (P = 0.00011) and when mitoses were observed (P = 0.00004). The paired variables also were found to correlate: MIB-1 versus PCNA (P = 0.04), MIB-1 versus mitotic count (P = 0.0184), and PCNA versus mitotic count (P = 0.0079). In all cases, there was immunoreactivity for EGFR; conversely, all cases were negative for bcl-2 in the cells with an oligodendrocyte phenotype. CONCLUSIONS: The PI was found to correlate with the postoperative disease free survival in patients with Grade 2 oligodendroglioma; therefore, the authors suggest a possible subdivision of Grade 2 oligodendrogliomas into two groups based on the mitotic count and PI.

[Krabbe s disease - case report] / Doença de Krabbe - relato de caso.

OBJECTIVE: Report a case of Krabbés disease with necropsy. METHODS: Review of medical and necropsy records. RESULTS: An 8 months-old male patient developed tremors, swallowing difficulty and excessive salivation for 4 months prior to admission, evolving with vomiting and fever. Physical examination showed microcephaly and diffuse pigmentation of the retinae. Neurological examination showed flexion of upper limbs with spastic hyperthony, symmetrical global hyperreflexia, nystagmus and spontaneous spasms. EEG showed multifocal irritative activity. There was increase in both CSF protein and gamaglobulin. The patient evolved with transitory hyperthermia, vomiting and pneumopathy, dying on the 23rd day after admission. Post mortem studies revealed microcephaly with widening of brain sulci. Histological examination revealed several globoid cells in the deep portion of the white matter, reactive gliosis and demyelination. CONCLUSIONS: These findings were similar to those in the world literature, indicating a poor prognosis due to substantial brain damage.

[Craniopharyngioma: clinical, epidemiological and pathological findings in 25 cases]. / Craniofaringiomas. Achados clínicos, epidemiológicos e anatomopatológicos de 25 casos.

We report the clinical and pathological findings of 25 cases of craniopharyngiomas. Fourteen patients were males and 11 were females. The ages ranged between 3 and 64 years, with a mean of 30.52 years. The symptoms reflected the growth and topography of the tumours; visual disorders (72%), headache (68%), vomits (40%) and papilledema (24%) were the most frequent symptoms. Twelve cases were suprasellar; 10 tumours arose from sellar region, from which 8 presented suprasellar extension; frontal lobe (n = 2) and ponto cererebellar angle (n = 1) were also affected. Eleven patients undergone total resection of the tumor and 14 were submitted to partial resection, from which 1 received adjuvant chemotherapy. The overall recurrence rate was 48%. Eight recurrences were observed in the patients submitted to partial resection and 4 in which total resection were performed. These results are similar with the literature, corroborating to the extension of residual tumour after the surgical resection as the main prognostic factor for this neoplasm.

[True neurogenic outlet syndrome: report of 2 cases]. / Síndrome do desfiladeiro torácico tipo neurogênico verdadeiro. Relato de dois casos.

True neurogenic thoracic outlet syndrome is caused by compression of the lower trunk of the brachial plexus usually by a cervical rib, fibrous band or an elongated transverse process of C7. We describe two cases of female patients (23 and 19 years old) with pain in the right superior limb and progressive muscular weakness and atrophy of the intrinsic muscles of hand. Electrodiagnostic studies showed reduced amplitude of compound muscle action potential of median nerve and decreased amplitude of ulnar sensory nerve action potential. Motor and sensory nerve conduction velocities were normal in both patients. Needle electromyography were findings compatible with chronic denervation in the intrinsic muscles of the right hand of both patients. Radiological investigation showed cervical ribs in one case and elongated transverse process of C7 in the other. A discussion about the clinical and electrophysiological features and the treatment of the syndrome was performed.

[Neurocutaneous melanosis] / Melanose neurocutânea.

OBJECTIVE: Describe the morbidity associated with a rare disease due to an embryological defect. METHODS: Retrospective revision of medical and necropsy reports. Bibliographic research using MEDLINE, LILACS and Index Medicus databases. RESULTS: 1 year-old male patient, admitted with generalized tonic-clonic seizures, evolving to deep coma and death in a few hours. Necropsy showed diffuse leptomeningeal malignant melanoma in brain stem, cerebellum, spinal cord and temporal lobe associated with a giant melanocytic nevus and satellite lesions. CONCLUSIONS: Neurocutaneous melanosis is a rare congenital syndrome characterized by the presence of large and/or multiple melanocytic nevi and pigmented tumors of the leptomeninges. It has a poor prognosis as demonstrated by the present report. It's physiopathology is believed to be due to a migration defect of the cells arising from the primitive neural crest. In these cases, an early diagnosis may improve the survival time.