A Case of an Adult Wilms Tumour in a Patient With Velocardiofacial Syndrome.

Adult Wilms tumors (nephroblastomas) are exceedingly rare with less than 500 cases reported in the literature. To our knowledge, ours is the first reported case of a patient with velocardiofacial syndrome (Shprintzen syndrome) acquiring an adult Wilms tumor. The case highlights the possible role of chromosome 22q aberrations toward the pathogenesis of a subset of Wilms tumors.

Paranasal sinus teratocarcinosarcoma with intradural extension.

Teratocarcinosarcoma, although a rare neoplastic entity, should be considered as a differential diagnosis in any middle-aged adult presenting with a history of intermittent unilateral epistaxis and nasal obstruction. Tissue biopsy may fail to reveal a full spectrum of histologic heterogeneity in these tumours, and definitive diagnosis is usually made with tumour resection. Aggressive treatment including surgery followed by adjuvant radiation therapy is advocated and confers a better rate of survival than radiotherapy alone.