Undifferentiated sarcoma of the liver: a rare pediatric tumor.

Undifferentiated embryonal sarcoma of the liver (UESL) is an uncommon hepatic tumor of mesenchymal origin recognized as a unique clinicopathologic entity since 1978. UESL has historically been considered an aggressive neoplasm with an unfavorable prognosis. Survival has improved using recent multimodal approaches, designed for patients with soft tissue sarcomas at other sites. Several small series have reported survival of up to 70% of children. We report a case of a 12-year-old boy from the United Arab Emirates who relapsed after complete surgical resection and was then successfully treated with re-resection followed by chemotherapy and radiotherapy. With a follow-up of 5 years, he is well and asymptomatic, and is leading a healthy life. This case emphasizes the fact that these poorly prognostic tumors may benefit from post-surgery chemotherapy. This case illustrates the improved survival of UESL patients following the multimodality therapy with a relatively long follow-up. This is the first case of UESL reported in this region of the world.

Congenital epulis.

Epulis is a rare tumour, with female preponderance that is only seen in the newborns. It arises from the mucosa of the gingiva and protrudes out of the infant's mouth. It can potentially obstruct the airways and may require an EXIT (ex-utero intrapartum treatment) procedure which involves establishing an airway before the feto-maternal circulation is interrupted. We present a female newborn with such a mass, which was diagnosed antenatally. A multidisciplinary team including the neonatologist, anaesthesiologist and ENT specialist should be present in the delivery room to establish the airways, which may require an EXIT procedure. Recommended treatment is early surgical resection. Recurrences of the tumour and damage to future dentition have not been reported, suggesting that radical excision is not warranted.

Reversal of iron-induced dilated cardiomyopathy during therapy with deferasirox in beta-thalassemia.

A 15-year-old male with beta-thalassemia major developed dilated cardiomyopathy secondary to iron-overload (Z-scores of left ventricle (LV) dimensions >3, ejection fraction (EF) 33%). Treatment with deferoxamine was unsuccessful, presumably due to poor compliance. After 15 months of using deferasirox (DFX), LV end-diastolic dimension normalized (Z-scores <2), and EF improved to 58%. We conclude that treatment with DFX resulted in a reversal of iron-induced cardiomyopathy.

Vincristine-induced urinary bladder paralysis.

The authors describe the development of bladder paralysis in a child with acute lymphoblastic leukemia undergoing maintenance chemotherapy. Immediately before the adverse clinical event, the child had received vincristine intravenously and triple therapy with hydrocortisone, cytosine arabinoside, and methotrexate intrathecally and had begun a 5-day pulse of prednisolone. The authors conclude that the ensuing reversible bladder paralysis was related to the vincristine. The clinical event resolved, and vincristine was deleted from the child's subsequent therapy until full recovery was achieved. The authors advise recognition of this problem and discontinuation of the vincristine if transient bladder paralysis develops until symptoms completely disappear.

Prenatal presentation supports the in utero development of congenital leukemia: a case report.

Congenital leukemia is a rare disease developing within the first 4 to 6 weeks of life. We report a female infant born with facial mass and multiple subcutaneous nodules. The facial mass was discovered by ultrasound during a routine prenatal examination at the 36th week of gestation. Biopsies were consistent with the diagnosis of acute monoblastic leukemia (AML, FAB M5b). Cytogenetic studies showed 46 XX, t(11;19)(q23;p13.1), which is only found in acute monoblastic leukemia and involves the gene. The infant died at 12 days of age and autopsy revealed a large leukemic tumor burden in several body organs. The discovery of the facial mass prenatally and massive extramedullary leukemic burden support the notion of the in utero development of congenital leukemia.