Esophageal carcinoma in children and adolescents.

BACKGROUND: Esophageal cancer is rare in children and is limited to isolated case reports. We describe 2 cases of esophageal carcinoma (1 case each of squamous cell carcinoma and adenocarcinoma) and present literature review of esophageal carcinoma in childhood. OBSERVATIONS: Both of our patients had common symptoms of progressive dysphagia and significant weight loss at presentation. We were unable to identify any specific predisposing factors for either adenocarcinoma (caustic ingestion, reflux disease, Barrett esophagus) or squamous cell carcinoma (caustic ingestion, inherited bone marrow failure syndromes). Both patients responded poorly to chemotherapy and died of progressive disease. CONCLUSIONS: On account of the rarity of esophageal carcinoma in this age group, there are no management guidelines for the pediatric oncologist. There is a strong need for collaborative efforts between adult and pediatric oncologists to establish cooperative diagnostic and therapeutic protocols for successful management of rare pediatric tumors like esophageal carcinoma.

Iatrogenic spinal epidermoid cyst after lumbar puncture using needles with stylet.

Iatrogenic spinal epidermoid tumors are extremely rare and may be caused by implanted skin fragments in the spinal canal, most commonly due to lumbar punctures using hollow needles without a stylet. The authors report for the first time an iatrogenic spinal epidermoid tumor that developed 26 months after several lumbar punctures, performed using disposable spinal needles with stylet, in an 8-year-old boy with persistent lower back pain. The authors hypothesize that an ill-fitting stylet compounded by multiple therapeutic lumbar punctures and young age as possible risk factors for the development of the tumor in the present case. A total excision of the tumor with laminectomy yielded good symptomatic recovery.

Sickle cell disease and plumbism in children.

Childhood lead poisoning is common, affects multiple organs and is preventable. Co-existence of plumbism and sickle cell disease (SCD) is uncommon, with major overlap in signs and symptoms. We describe a patient with SCD and plumbism. Dysfunctional eating patterns may cause ingestion and increased absorption of lead in these patients. Routine screening for pica and other dysfunctional eating habits with appropriate behavioral interventions is emphasized. Annual screening for lead in patients with SCD of all ages (1-21years) and supplementation of micronutrients like zinc and iron in deficient cases may help prevent plumbism in these patients.

Characterization of novel biomarkers in selecting for subtype specific medulloblastoma phenotypes.

Major research efforts have focused on defining cell surface marker profiles for characterization and selection of brain tumor stem/progenitor cells. Medulloblastoma is the most common primary malignant pediatric brain cancer and consists of 4 molecular subgroups: WNT, SHH, Group 3 and Group 4. Given the heterogeneity within and between medulloblastoma variants, surface marker profiles may be subtype-specific. Here, we employed a high throughput flow cytometry screen to identify differentially expressed cell surface markers in self-renewing vs. non-self-renewing SHH medulloblastoma cells. The top 25 markers were reduced to 4, CD271/p75NTR/NGFR, CD106/VCAM1, EGFR and CD171/NCAM-L1, by evaluating transcript levels in SHH tumors relative to samples representing the other variants. However, only CD271/p75NTR/NGFR and CD171/NCAM-L1 maintain differential expression between variants at the protein level. Functional characterization of CD271, a low affinity neurotrophin receptor, in cell lines and primary cultures suggested that CD271 selects for lower self-renewing progenitors or stem cells. Moreover, CD271 levels were negatively correlated with expression of SHH pathway genes. Our study reveals a novel role for CD271 in SHH medulloblastoma and suggests that targeting CD271 pathways could lead to the design of more selective therapies that lessen the broad impact of current treatments on developing nervous systems.

Semapimod sensitizes glioblastoma tumors to ionizing radiation by targeting microglia.

Glioblastoma is the most malignant and lethal form of astrocytoma, with patients having a median survival time of approximately 15 months with current therapeutic modalities. It is therefore important to identify novel therapeutics. There is mounting evidence that microglia (specialized brain-resident macrophages) play a significant role in the development and progression of glioblastoma tumors. In this paper we show that microglia, in addition to stimulating glioblastoma cell invasion, also promote glioblastoma cell proliferation and resistance to ionizing radiation in vitro. We found that semapimod, a drug that selectively interferes with the function of macrophages and microglia, potently inhibits microglia-stimulated GL261 invasion, without affecting serum-stimulated glioblastoma cell invasion. Semapimod also inhibits microglia-stimulated resistance of glioblastoma cells to radiation, but has no significant effect on microglia-stimulated glioblastoma cell proliferation. We also found that intracranially administered semapimod strongly increases the survival of GL261 tumor-bearing animals in combination with radiation, but has no significant benefit in the absence of radiation. In conclusion, our observations indicate that semapimod sensitizes glioblastoma tumors to ionizing radiation by targeting microglia and/or infiltrating macrophages.

Brown-Séquard-plus syndrome because of penetrating trauma in children.

Brown-Séquard syndrome is an uncommon condition involving incomplete spinal cord injury, with ipsilateral motor and proprioception loss, contralateral pain, and decreased temperature. Brown-Séquard-plus syndrome is associated with additional neurologic findings involving the eyes, bowel, or bladder. We describe an adolescent with Brown-Séquard-plus syndrome attributable to a stab injury. Our patient's clinical features of spinal and neurogenic shock overlapped at presentation. He was managed with high-dose steroids, along with intense physiotherapy and rehabilitation, resulting in good neurologic recovery. Appropriate medical (and surgical, when indicated) management usually results in good to complete recovery of neurologic function, depending on the level and grade of injury. With the increasing incidence of gunshot wounds and stab injuries in children, pediatricians, including pediatric neurologists and emergency physicians, are more likely to encounter these types of spinal cord injuries in children.

Spontaneous hemopneumothorax in children: case report and review of literature.

Spontaneous hemopneumothorax is rare, occurs in young adolescents, and can be life threatening secondary to massive bleeding. An adolescent with spontaneous hemopneumothorax and shock managed by tube thorascostomy is described here. We compared our case with published data of spontaneous hemopneumothorax in the pediatric age group. Spontaneous hemopneumothorax involves the accumulation of air and blood in the pleural space in the absence of trauma or other obvious causes. Spontaneous hemopneumothorax is usually seen in adolescents, more common in males than females. The common clinical features of spontaneous hemopneumothorax include dyspnoea and chest pain, and 30% present with hypovolemic shock. The bleeding can result from a torn adhesion between the parietal and visceral pleurae, from a rupture of vascularized bullae, or from torn congenital aberrant vessels. Over the last 6 decades, the treatment has progressed from the thoracotomy to minimally invasive techniques such as video assisted thoracoscopic surgery, with great reduction in mortality and recurrence rates. Although a rare entity, diagnosis of spontaneous hemopneumothorax must be considered in young adolescents presenting with spontaneous onset of chest pain and dyspnoea with radiograph findings of hydropneumothorax and/or signs of shock.