Consensus statement for the treatment of infantile haemangiomas with propranolol.

Although most infantile haemangiomas do not require treatment due to a natural history of spontaneous involution, some require early intervention. The Australasian Vascular Anomalies Network and the Australasian Paediatric Dermatology Network have developed a consensus statement for the treatment of infantile haemangiomas with oral propranolol. Infants with haemangiomas that are life threatening, at risk of ulceration, or at risk of causing a significant functional impairment, psychological impact or physical deformity should be treated early with oral propranolol.

Bilateral Wilms tumor and early presentation in pediatric patients is associated with the truncation of the Wilms tumor 1 protein.

Objectives: To investigate the frequency of constitutional Wilms tumor 1 gene (WT1) abnormalities in children with bilateral Wilms tumor (WT) and the age of tumor onset in patients with a mutation.

Study Design: Eight patients with bilateral WT were studied. High-resolution melting and direct sequencing were used to screen for the WT1 gene.

Successful treatment of kaposiform hemangioendothelioma and tufted angioma with vincristine.

Background: Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) are rare, locally aggressive vascular tumors. Although currently classified as separate entities, they are becoming increasingly recognized as a spectrum of the same pathology. There is a well-recognized association with Kasabach-Merritt phenomenon KHE and TA are considered neoplasms of intermediate malignancy because of infiltrative growth, local aggressiveness, and variable prognosis.

Successful treatment of infantile haemangiomas of the orbit with propranolol.

Background: Propranolol is a novel therapeutic agent in the treatment of cutaneous infantile haemangiomas. We assessed the effect of propranolol therapy in infantile haemangiomas of the orbit.

Methods: A case series of four patients with orbital infantile haemangiomas were referred for management in our tertiary referral hospitals.

Lung tumours in children.

Primary lung tumours in children are rare and metastatic lung disease is uncommon. The majority of children who present with a primary or secondary pulmonary malignancy will present co-incidentally while seeking attention for another medical problem, or with non-specific abnormalities such as cough with collapse or consolidation on the chest radiograph. With improved techniques of medical imaging for diagnostic and therapeutic purposes and improved outcomes of childhood malignancies, the role of the respiratory paediatrician in the ongoing care of oncology patients is likely to increase.

The impact of Asian descent on the incidence of acquired severe aplastic anaemia in children.

Previous studies have suggested an increased incidence of acquired severe aplastic anaemia in Asian populations. We evaluated the incidence of aplastic anaemia in people of Asian descent, using a well-defined paediatric (0-14 years) population in British Columbia, Canada to minimize environmental factors. The incidence in children of East/South-east Asian descent (6.

Non-resectable congenital tumors with the ETV6-NTRK3 gene fusion are highly responsive to chemotherapy.

Background: Recently, the ETV6-NTRK3 gene fusion has been identified in both infantile fibrosarcoma and cellular mesoblastic nephroma. For both these tumors standard curative treatment has been primarily surgical with wide local excision. This has frequently involved radical and even mutilating surgery.