Primary intraosseous papillary intralymphatic angioendothelioma of the distal femoral epiphysis: a case report with literature review.

INTRODUCTION: Papillary intralymphatic angioendothelioma (PILA) is an exceptionally rare metastasizing soft tissue tumor. It tends to arise in the subcutaneous tissues of distal extremities in children. Only four intraosseous PILA cases have been reported until now in English language literature. CASE REPORT: We present a case of PILA arising in the distal femoral epiphysis of a 50-year-old female patient. It started as a relentless pain in her left knee. A plain radiography revealed a radiolucent area in the left internal femoral condyle. Computerized tomography revealed a 1-cm lytic lesion with a sclerotic rim. Magnetic resonance images showed a significant bone marrow edema signal focused on a 1-cm subchondral lesion suggestive of an intraarticular osteoid osteoma. Histologically, the tumor contained vascular channels covered by a single endothelial layer with intraluminal papillary endothelial structures lined with hobnail cells. Immunohistochemically, the cells were positive for ERG, CD31, and D2-40. The tumor underwent cryoablation and 6 months later, after local recurrence or tumor persistence, a wide tumor resection was referred. After 7 years of follow-up, the patient displayed neither local recurrence nor distant metastases. CONCLUSION: Primary intraosseous PILAs are exceedingly rare tumors that should be considered in the differential diagnosis of vascular bone tumors.

Chronic expanding hematoma.

Chronic expansive hematoma (CEH) is a rare lesion, characterized by the persistence and increase in size of an hematoma for a period greater than one month since the initial hemorrhage. The clinical importance of this pathology is due to the fact that it can simulate malignant soft tissue neoplasms, both clinically as a result of its progressive growth and radiologically for its findings in imaging studies. This article will review three cases of CEH in different scenarios, explaining the radiological findings in different imaging techniques such as ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), and PET-CT.

Osteomielitis pélvica y piomiositis del músculo obturador interno. Revisión del tratamiento a propósito de un caso / Pelvic osteomyelitis and obturator internus muscle pyomyositis. Revision of the treatment with regard to a case

La osteomielitis pélvica asociada a infección del músculo obturador interno es una infección poco difundida en la literatura científica. Presentamos el caso de un adolescente con ambas infecciones y una revisión del abordaje terapéutico de ambas patologías

Pelvic osteomyelitis associated with pyomyositis of the obturador internus muscle is rarely reported. We present the case of a teenager with both infections and a review of the therapeutic approach of both pathologies

Tumor benigno de células notocordales / Benign notochordal cell tumors

Los tumores benignos de células notocordales (TBCN) son lesiones con diferenciación notocordal que afectan al esqueleto axial. Se caracterizan por cursar asintomáticos o con sintomatología inespecífica, y por pasar desapercibidos radiológicamente por su pequeño tamaño, o bien por ser confundidos con otras lesiones óseas benignas, como hemangiomas vertebrales. Cuando son grandes o sintomáticos pueden plantear el diagnóstico diferencial con metástasis, tumores óseos primarios y cordomas. Presentamos el caso de un TBCN de difícil diagnóstico, en una mujer de 50 años de edad, con una lesión sacra vista en la resonancia magnética (RM). Se programó una biopsia guiada por tomografía computarizada (TC) para filiar la lesión, pero la tumoración no era claramente identificable en la TC, por lo que se utilizaron las referencias anatómicas de la RM para seleccionar el plano adecuado. Tanto la planificación del abordaje como la correlación radiopatológica fueron determinantes para llegar al diagnóstico definitivo

Benign notochordal cell tumors (TBCN) are lesions with notochordal differentiation which affect the axial skeleton. They are characterized by asymptomatic or non-specific symptomatology and are radiologically unnoticed because of their small size, or because they are mistaken with other benign bone lesions, such as vertebral hemangiomas. When they are large, or symptomatic, can be differential diagnosis with metastases, primary bone tumors and chordomas. We present a case of a TBCN in a 50-year-old woman, with a sacral lesion seen in MRI. A CT-guided biopsy was scheduled to analyze the lesion, finding that the tumor was not clearly recognizable on CT, so the anatomical references of MRI were used to select the appropriate plane. The planning of the approach and the radio-pathological correlation were determinant to reach the definitive diagnosis

Benign notochordal cell tumors. / Tumor benigno de células notocordales.

Benign notochordal cell tumors (TBCN) are lesions with notochordal differentiation which affect the axial skeleton. They are characterized by asymptomatic or non-specific symptomatology and are radiologically unnoticed because of their small size, or because they are mistaken with other benign bone lesions, such as vertebral hemangiomas. When they are large, or symptomatic, can be differential diagnosis with metastases, primary bone tumors and chordomas. We present a case of a TBCN in a 50-year-old woman, with a sacral lesion seen in MRI. A CT-guided biopsy was scheduled to analyze the lesion, finding that the tumor was not clearly recognizable on CT, so the anatomical references of MRI were used to select the appropriate plane. The planning of the approach and the radio-pathological correlation were determinant to reach the definitive diagnosis.

Pulmonary hypertension and its management in patients undergoing non-cardiac surgery.

Pulmonary hypertension is a complex disorder of the pulmonary vasculature that leads to increased peri-operative morbidity and mortality. Non-cardiac surgery constitutes a significant risk in patients with pulmonary hypertension. The management of right ventricular failure is inherently challenging and fraught with life-threatening consequences. A thorough understanding of the pathophysiology, the severity of the disease and its treatment modalities is required to deliver optimal peri-operative care. This review provides an evidence-based overview of the definition, classification, pathophysiology, diagnosis and treatment of pulmonary hypertension and focuses on the peri-operative management and treatment of pulmonary hypertensive crises in a non-cardiac setting.

High frequency of childhood ADHD history in women with fibromyalgia.

Fibromyalgia and ADHD share some clinical features, and a reduced dopamine function has been proposed for both disorders. Here we found, in a large sample of fibromyalgia female patients, a higher frequency of childhood ADHD antecedent when compared with healthy women. Our data suggest that Fibromyalgia and ADHD have some common etiopathological mechanism.

MRI of a family with focal abnormalities of gyration.

Focal abnormalities of gyration (FAG) are developmental disorders that may occur in isolated patients or, as in the case being reported, as part of a familial disorder. Analysis of individuals in a family spanning three generations was carried out using MRI. Abnormalities, present in all members of generations II and III, included focal cortical dysplasia (three patients), focal cortical infolding (two patients) and schizencephaly (one patient); associated minor anomalies, such as white matter abnormalities, were seen in the remaining three members of generations II and III. MRI recognition of FAG in the family being reported proved useful in defining their phenotypical expression and providing proper counselling for individual family members.

Vaccination of humans against cutaneous leishmaniasis: cellular and humoral immune responses.

Brazilian army conscripts were vaccinated against American cutaneous leishmaniasis by using nonliving polyvalent promastigote Leish vaccine 5 or Leish vaccine 6 (vaccines with five or six Leishmania stocks) with or without Corynebacterium parvum. No statistically significant differences in lymphocyte stimulation indices were found between vaccinated groups with or without C. parvum, but lymphocyte stimulation indices of all vaccinees were significantly higher (P less than 0.001) than those of the placebo group. A correlation of 90% was found between positive skin test results and positive lymphocyte stimulation indices. Eight major antigens with estimated molecular masses of 13.5, 25, 40, 63, 73, 85, 97, and 160 kilodaltons were recognized by Leish vaccine 5 sera. Our finding also demonstrated the predominance of immunoglobulin M antibody in sera of vaccinated subjects and that a component of Leish vaccine 5, gp63, was immunogenic in humans both at the T-cell level and at the antibody level.

Spontaneous hematomas caused by microangiomatosis of the basal ganglia. Presentation of two cases in the newborn period.

We present 2 cases of tumors of the gray basal nuclei with intraventricular hemorrhage and secondary hydrocephalus. Because of the patients' ages, the absence of traumatic antecedents and the angiographic characteristics, the findings were diagnosed as gliomas. In both cases, the existence of hematomas provoked by microangiomatosis was proved after the operation. The differential diagnostic between these two entities is discussed.

Successful surgical treatment of an odontogenic ossified craniopharyngioma. Case report.

A large, highly ossified craniopharyngioma was diagnosed and surgically treated in a 2-year-old girl. About 20 teeth were macroscopically identified in the operative specimen, some of them totally developed, complete with crown and root. Pathological study established the diagnosis of craniopharyngioma with formation of teeth. Only five cases have been reported previously, and only one patient survived the operation. The patient is alive 3 years after surgical treatment. Radiotherapy was not used.

Clivus ependymoma.

A 8-month-old girl had an ependymoma in the clivus, 2 X 6 cm in size, connected with the fourth ventricle by a cord of tissue 0.5 cm thick. There were no indications to make us suspect the origin of the tumour in the fourth ventricle, or that it was a case of ependymoma.

Imunologia da paracoccidiodomicose. / Immunology of paracoccidioidomycosis

Estudos imunologicos foram realizados em 16 pacientes adultos, de ambos os sexos, acometidos de paracoccidioidomicose. Dentre os achados mais importantes figuram os teores elevados de IgG, a baixa do 3o. componente do complemento, a presenca de anticorpos precipitantes e hemaglutinantes contra a paracoccidioidina, a presenca de complexos imunes circulantes, baixos teores de linfocitos T circulantes, teores normais de linfocitos T supressores e hiporreatividade dos linfocitos T a fitohemaglutinina e a paracoccidioidina.A histologia das reacoes cutaneas a paracoccidioidina acusou um aspecto semelhante ao da reacao de Arthus, com o maximo de intensidade do eritema em 24 horas, a presenca de vasculite e a infiltracao predominante por polimorfonucleares

Radiological Diagnosis of Periventricular and Subcortical Leukomalacia.

Nine newborn infants with histories of perinatal asphyxia are presented. The pneumoencephalographic findings which led to the diagnosis are typical and constant. They include marked subcortical atrophy with rounded, dilated, and undisplaced lateral ventricles. Cystography with 3 cc of air demonstrated multiple subcortical and pareventricular cavities, without communication with the ventricular system, but with the typical honeycomb appearance of paraventricular and subcortical leukomalacia described in postmortem findings. The CT findings are typical, and provide the location of the cavities as well as their density.

Congenital medulloblastoma. Report of two cases.

Congenital medulloblastoma caused severe hydrocephalus in two patients, one aged one day and the other 5 months.

Spontaneous ventriculostomy and ventricular diverticulum.

Four cases of ventricular diverticulum are presented, 2 with spontaneous ventriculostomy. In 2 patients, the cause was monoventricular hydrocephalus through congenital stenosis of the foramen of Monro, with paraventricular diverticula and spontaneous ventriculostomy. Both patients had a cystic space in the quadrigeminal and supracerebellar cistern. In one patient during pneumoencephalography, the ventricular diverticulum filled via the ventriculostomy. The other 2 patients had congenital stenosis of the Sylvian aqueduct with the formation of a large supratentoria cyst connecting with the posterior part of the third ventricule.

Occlusion of the cerebral arteries in Recklinghausen's disease.

Two children, aged 18 months and 6 years, who had Recklinghausen's disease, had occlusion of cerebral arteries. One child had no motor deficit but the other had right hemiparesis and partial occlusion of the left posterior cerebral artery, a fact not found in the literature.

Congenital atresia of the foramen of Monro.

Two cases of congenital atresia of the foramen of Monro are described, one 8 days old and the other 2 months old. Both are associated with a parieto-occipital paraventricular diverticulum and spontaneous ventriculostomy with the passing of the cerebrospinal fluid to the posterior fossa and the formation of a cyst in the supracerebella region which compressed and displaced the structures of the posterior fossa.