Old drugs still work! Oral etoposide in a relapsed medulloblastoma.

Medulloblastoma is the most common malignant brain tumor in children. Approximately 30% of children with medulloblastoma will progress or relapse despite being treated. New therapies have been proposed in recent years, including high-dose chemotherapy, immunotherapy, and targeted therapy. However, the best treatment for these patients remains unclear, and in this situation prognosis is poor. Oral etoposide has been used as a single agent or in combination for treating relapsed brain tumors since the 1990s. We report an 8-year-old patient with recurrent metastatic medulloblastoma who had an excellent response after treatment with oral etoposide, maintaining a great quality of life. As clinicians, we must always try to include our patients in clinical trials; however, when this is not possible, we should not forget that "old drugs" such as oral etoposide may work in some patients, with a good response of the tumor, and what is most important, providing the patient with a good quality of life.

A persistent epidural mass in a child with B-lineage ALL.

Epidural spinal cord compression as the initial presentation of acute lymphoblastic leukemia (ALL) is a rare and serious complication. Extramedullary disease is rarely reported in patients with ALL. The most common sites are bone, followed by soft tissue, skin and lymph nodes. We describe a child with common B-lineage ALL who presented with a mass in the spinal epidural space. She was initially treated with intrathecal chemotherapy and intravenous dexamethasone with total resolution of her clinical symptoms but a persistent epidural mass. An open biopsy of the residual epidural mass was performed 7 months after diagnosis. The histological examination did not reveal any tumor infiltration, only fibrosis. We conclude that a persistent epidural mass in patients with ALL may not indicate resistant disease and may require over a year for resolution, even when response to therapy is adequate.

CT findings after laryngectomy.

The goal of surgical treatment of laryngeal cancer is to achieve tumor control while preserving, whenever possible, the three primary functions of the larynx: breathing, swallowing, and phonation. The surgical procedure may consist of either a partial, conservative excision (eg, cordectomy, vertical partial laryngectomy, horizontal supraglottic laryngectomy, supracricoid laryngectomy with cricohyoidopexy or cricohyoidoepiglottopexy, or near total laryngectomy) or a radical excision (total laryngectomy). The procedure depends largely on the location and extension of the tumor, the stage of disease, and the patient's needs and preferences. Familiarity with the typical imaging appearance of the larynx after each procedure is crucial for differentiating normal postsurgical changes from persistent or recurrent disease as well as for diagnosing associated second primary malignancies. Since computed tomography (CT) is often used for follow-up evaluations, an ability to interpret the characteristic CT features is particularly important.

[Natural course of septo-optic dysplasia: retrospective analysis of 20 cases]. / Evolucion natural de la displasia septooptica: analisis retrospectivo de 20 casos.

INTRODUCTION: Septo-optic dysplasia (SOD) is the variable combination of signs of dysgenesis of the midline of the brain, hypoplasia of the optic nerves and hypothalamus-pituitary dysfunction, which is sometimes associated with a varied spectrum of malformations of the cerebral cortex. AIMS: To describe the natural history and neuroimaging findings in a series of 20 diagnosed patients. PATIENTS AND METHODS: We review the epidemiological, clinical and neuroimaging characteristics of 20 consecutive patients diagnosed with SOD between January 1985 and January 2010. Data obtained from computerised tomography, magnetic resonance imaging of the head, electroencephalogram, visual evoked potentials, ophthalmological evaluation, karyotyping and endocrinological studies were analysed. In seven patients, a study of the gene Homeobox HESX1 was conducted. RESULTS: Pathological antecedents in the first three months of gestation were presented by 60% of the cases, with normal results in the foetal ultrasound scans. Clinically, the most striking features were visual manifestations (85%), endocrine disorders (50%), mental retardation (60%) and epileptic seizures (55%). Fifty-five per cent were associated to abnormal neuronal migration. In 45%, SOD was the only finding in the neuroimaging scans. Karyotyping was performed in all cases, the results being normal. Gene HESX1 was positive in two of the seven cases studied (both with isolated SOD). None of those with mutation in gene HESX1 presented familial consanguinity. No gene study was conducted with the parents. CONCLUSIONS: SOD must be classified as a heterogeneous malformation syndrome, which is associated to multiple brain, ocular, endocrine and systemic anomalies. The most severe forms are associated with abnormal neuronal migration and cortical organisation.

Long bone florid reactive periostitis ossificans: a case in the distal femur mimicking osteosarcoma.

Florid reactive periostitis ossificans is a well-known benign lesion classically described in hands and feet which histopathological features can lead to a misdiagnosis of osteosarcoma. To the best of our knowledge, there is only one previous report of this lesion in a long bone. In this study we report a case of florid reactive periostitis ossificans located in the distal metaphysis of the left femur that histologically mimicked an osteosarcoma and discuss the differential diagnosis between these two entities to warn about a diagnostic pitfall.