Leonardo Nercolini Faoro

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

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.

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 chrornatin, and distinct nucleoli dispersed in a myxoid to collagenous stroma with marked vascular proliferation. Immunohistocrhemical profile showed diffuse positivity for vimentin and focal immunoreactivity for desmin, whereas cytokeratins (CAM5.2 and AE1/AE3), EMA, alph-a smooth muscle actin, actin HHF35, CEA, S1OO, 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.

Neurocysticercosis: a clinical and pathological study of 27 necropsied cases

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.

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

Abstract: 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.

Meduloblastomas: achados clínicos, epidemiológicos e anátomo-patológicos de 28 casos

Relatamos os achados clinicos, epidemiologicos e anatomo-patologicos de 28 casos de meduloblastomas. Dos 28 pacientes analisados, 22 eram do sexo masculino e seis do sexo feminino. As idades variaram de 1 a 50 anos, com media de 15 anos. Os sinais e sintomas de maior frequencia foram cefaleia (64%) e vomitos (64%). Em relacao ao tratamento, a maioria dos pacientes foi submetida a resseccao cirurgica total (n=10) ou a exerese parcial (n=7). Com a excecao de um paciente que apresentava meduloblastoma desmoplasico, os demais apresentavam a forma classica do meduloblastoma. Foi evidenciada uma taxa de recidiva tumoral total de 21% (n=6) em 4 anos de seguimento clinico. A quimioterapia adjuvante parece contribuir para melhor evolucao clinica dos pacientes. Estes achados aproximam-se dos encontrados na literatura, contribuindo para a compreensao do comportamento biologico deste tumor.

Unrelated bone marrow as a source of Bacillus cereus causing septic shock.

Bone marrow grafts are commonly found to host bacteria diagnosed by surveillance culture, and graft contamination can be found in 2% to 25% of samples. In a retrospective 10 year evaluation of the prevalence of marrow contamination in our service, a 15.2% contamination rate was noted. However, studies have consistently reported no clinical impact due to this fact. 3,4 Recently, we faced a situation that led us to rethink our position about graft contamination. An 18-year-old male with the diagnosis of acute biphenotypic leukemia received an unrelated bone marrow transplantation. The donor was selected through the National Marrow Donor Program and the marrow harvested in USA. A courier transported the marrow in a cooler at room temperature to our service in Curitiba, Brazil. In our Cryobiology Laboratory, the volume of marrow and media was reduced from 1047 ml to 700 ml, and samples collected before and after processing were sent for cultures. In the BM Unit, 4 h after the beginning of the infusion the patient developed fever (40 °C) and chills. Blood culture sets were collected, and imipenem-cilastatin started intravenously. Nine hours after fever and chills, hypotension and shock developed, being managed with vasoactive drugs. The sequence of events is detailed in Table 1. The clinical picture persisted for the next 24 h. Meanwhile, the Bacteriology Laboratory reported that a Grampositive bacillus was found in both samples (donor marrow and patient blood) sent for culture. Vancomycin and ampicillin were prescribed immediately in an attempt to cover the three most likely Gram-positive bacilli: Bacillus, CorynebacteriumandListeria. Three days later, the microorganism was identified as Bacillus cereus , growing in the bone marrow and blood samples. Fortunately, the patient recovered from the septic shock. Despite careful attention to sterile procedures, low-level contamination of hematopoietic stem cell components can occur. Bacteria from the skin flora (coagulase-negative Staphylococcus , Propionibacterium and Corynebacterium species) are the contaminants in |90% of cases and no infections attributable to these germs have been demonstrated following stem cell infusion. 5 However, potentially pathogenic organisms ( Bacillus, Pseudomonas and Gram-negative enteric bacteria) have also been detected as contaminants. 6

Krabbe s disease - case report

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.

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 ressected. 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.