Inflammatory pseudotumor (IPT)-surgical cure of an inflammatory syndrome.

UNLABELLED: We report on four female adolescents, who presented with inflammatory symptoms. Extensive diagnostic workup revealed tumors on different locations. After surgical removal, clinical and laboratory signs of inflammation disappeared rapidly. On histology, the tumors showed a mixture of inflammatory cells characteristic of inflammatory pseudotumors in three of the patients. CONCLUSION: In patients with unclear inflammatory symptoms, inflammatory pseudotumor should be added to the differential diagnosis. WHAT IS KNOWN: • The inflammatory pseudotumor (IPT) is a mostly benign myofibroblastic tumor of the soft tissue and causes inflammatory symptoms. What is new: • IPTs have may wider than hitherto defined histologic features. Removal of IPT is curative.

Gastric outlet obstruction after long-term prostaglandin administration mimicking hypertrophic pyloric stenosis.

Prostaglandin E1 (PGE1) is widely used in neonates with cyanotic congenital heart disease who depend on the patency of the ductus arteriosus for oxygenation. Side effects of prostaglandin therapy are common and include respiratory depression, generalized flushing, and cardiovascular and neurological effects. Little is known about the complex effects on the gastrointestinal tract. We report on an infant with gastric outlet obstruction after long-term prostaglandin administration. At the age of 1 month, feeding problems developed with projectile vomiting. Ultrasonography showed progressive elongation of the antropyloric channel without wall thickening, which was causing gastric outlet obstruction. Three days after cardiac surgery and cessation of prostaglandin therapy, the infant fed normally and rapidly gained weight. The clinical signs in such patients can mimic hypertrophic pyloric stenosis. Therefore, the sonographic findings should not be confused with pyloric wall thickening to avoid a false diagnosis and unnecessary surgery. The symptoms diminish with cessation of the prostaglandin therapy after a corrective cardiac operation.

Posaconazole for treatment of refractory invasive fungal disease.

Invasive fungal infections are usually associated with immunocompromised states About 40-60% of these patients are refractory to standard antifungal therapy We describe the effect of posaconazole in the treatment of a 12 years-old girl with uncontrolled diabetes mellitus with life-threatening cerebral mucor mycosis and a 4 year old girl boy with chronic granulomatous disease presenting with invasive Aspergillus nidulans infection.

Paclitaxel: an effective antineoplastic agent in the treatment of xenotransplanted hepatoblastoma.

BACKGROUND: Hepatoblastoma is an uncommon liver tumor of infancy and early childhood. Though most patients with nonmetastatic hepatoblastomas can be cured by defining surgical strategies and chemotherapy regimes, new drugs are needed for children with advanced hepatoblastomas. The activity of paclitaxel as a new antineoplastic agent with limited experience in pediatric oncology was studied in a xenograft model. PROCEDURE: Hepatoblastoma cell suspensions from three children were transplanted subcutaneously into nude mice NMRI (nu/nu). One of the primary tumors was an embryonal multifocal hepatoblastoma, whereas the other tumors were embryonal/fetal hepatoblastomas localized on a liver lobe. After 4 weeks, xenografted tumor sizes reached 50-100 mm3. The xenografted tumors resembled their originals histologically and produced high levels of alpha-fetoprotein. The efficiency of paclitaxel at equitoxic doses was analyzed. RESULTS: Paclitaxel produced an effect in all three hepatoblastomas. There was a significant reduction of tumor volume (P < 0.001) and alpha-fetoprotein levels after chemotherapy (P < 0.0001). The proliferation activity of the tumor cells corresponded with these results. Histologically, after treatment with paclitaxel the tumor regression was 35%-49%. The mechanism of paclitaxel action could be demonstrated by light microscopy immunohistochemistry and electron microscopy. CONCLUSIONS: The preliminary results in phase I trials of solid tumors in children and the results of this study suggest that paclitaxel in phase II studies can now be entertained for patients with hepatoblastoma.