Beckwith-Wiedemann syndrome and virilizing cortical adrenal tumor in a child

Factors affecting outcomes of prenatally‐diagnosed tumours

“…Ultrasound has been used for prenatal diagnosis of fetal tumours for the past 20 years (Meizner, 2000). Improvements in antenatal diagnosis have been made by the addition of MRI (Sbragia-Neto et al, 2000;Alfonso et al, 2000), chromosomal studies of the fetal cells from amniocentesis (Al-Nemri et al, 2000;Chitty, 1998;Krapp et al, 1999;King et al, 2000) and attempts to identify tumour-associated antigens in maternal and fetal blood (Hohlfeld et al, 1994). Bolande (1971) pointed out the benign nature of most neonatal tumours because of possible cancer repression in early life.…”

Adrenocortical tumor with precocious puberty in a 2‐month‐old girl

“…In the pediatric literature on adrenocortical tumor, female to male ratio is 2:1, with three children presenting with Cushing's syndrome 2,4 and one with an associated BeckwithWiedemann syndrome. 7 The most common clinical presentation (84.2%) includes signs of virilization, but excessive linear growth is often noted. 8 Chen et al reviewed 34 patients aged <15 years with an adrenocortical carcinoma or cortical adenoma.…”

“…11 Moreover, constitutional mutations of p53 are frequently reported in young children with a diagnosis of adrenocortical tumor. Familial genetic predisposition to cancer (p53 mutations) and selected genetic syndromes (Beckwith-Wiedemann syndrome 7 ) have been associated with childhood adrenocortical cancer in general. 3 In case of positive TP53 mutation, mainly in Li-Fraumeni syndrome (a genetic disorder that greatly increases the incidence of several types of cancer, especially in the bones, soft tissue, brain, blood, and adrenal glands), the recurrence rate is significant.…”

Pancreatoblastoma associated with incomplete Beckwith-Wiedemann syndrome: case report and review of the literature

“…The Beckwith-Wiedemann syndrome apart from a constellation of congenital abnormalities with variable expressivity is known for its high risk for developing malignant and benign tumor from multiple cell lines [17][18][19][22][23][24][25][26]28,29]. Risk of tumorigenesis increases significantly when hemihypertrophy is present [17,18], and patients with incomplete forms may share the same oncogenic potential as those with classic manifestations of the syndrome [17,[30][31][32].…”