Undifferentiated embryonal sarcoma is the third most common malignant tumor of the liver in children, accounting for 13% of hepatic malignancies in this age group. It has been considered an aggressive neoplasm with very poor prognosis until the late 1980s, when long-term survivors were reported after multiagent chemotherapy followed by resection. We, herein, report two pediatric cases of undifferentiated embryonal sarcoma treated successfully with surgical resection after neoadjuvant chemotherapy based on therapy used in childhood soft tissue sarcomas and in childhood hepatic malignancies. The first patient also had a concurrent cerebellar tumor (pilocytic astrocytoma), for which he first underwent craniotomy and resection, delaying the liver tumor resection by 10 weeks. They are alive and tumor free at 48 months (case no. 1) and 18 months (case no. 2) following neoadjuvant chemotherapy and liver resection.
It is unclear which induction therapy yields the best outcomes in pediatric kidney transplantation. Retrospective data of 88 children receiving a renal allograft between November 1996 and October 2003 were analyzed. Patients received ATGI (n = 12), BI (n = 29), or NAI (n = 47). The mean ATG dose was 5.1 ± 2.1 mg/kg. At 12 months, graft survival rates were 91.7%, 100%, and 97.9% for ATGI, BI, and NAI groups, respectively. Acute rejection rates at 12 months were 0 (ATGI), 20.6% (BI), and 10.7% (NAI). The mean GFR for ATGI (42.4 ± 25.9 mL/min) was lower than for BI (78.3 ± 27.2 mL/min), and NAI (66 ± 28.3 mL/min) at 12 months (p < 0.05). One ATGI patient developed CMV pneumonia but none developed post‐transplant lymphoproliferative disorder. Although there was no renal allograft survival benefit with either ATGI or BI, relative to NAI, the absence of acute rejection and equivalent rates of viral infections in the higher‐risk ATGI recipient group suggests that the treatment strategy is promising. A large prospective study is needed to better define the role of ATGI in pediatric kidney transplantation.
Intrapyloric injection of botulinum toxin A (BoTx) successfully improved symptoms in idiopathic and diabetic gastroparesis (DGP) refractory to medical treatment. Therefore, we used it in three pancreas transplant patients done in our institution during the last 18 months. They had severe, persistent DGP despite successful pancreas transplantation. They received 100 units of BoTx during the first injection. The clinical effect became evident within 2 weeks after the treatment, and lasted for an average of 29 weeks (range 14-44 weeks). The patients' subjective evaluation showed improvement of their symptoms and quality of life following BoTx. Patients 2 and 3 had recurrent symptoms at 44 and 24 weeks, respectively, after the first injection; they required a second dose of 90 and 80 units, respectively. They are doing well at 3 months followup. Intrapyloric injection of BoTx is safe and efficient. It should be considered for treating residual DGP following successful pancreas transplantation.
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