Hemihypertrophy, renal dysplasia and benign nephromegaly

Abstract

Hemihypertrophy is associated with malignant visceral abdominal neoplasms in childhood. Benign nephromegaly and nephroblastomas are both known to occur with hemihypertrophy; however, association with renal dysplasia has not previously reported. We describe an infant presenting with recurrent haematuria who had segmental hemihypertrophy, ipsilateral renal dysplasia and contralateral benign nephromegaly. Although debated, renal dysplasia may predispose to and predate malignant change. Rational management and optimal surveillance of renal dysplasia and benign nephromegaly in hemihypertrophy is discussed.

Focal Nodular Hyperplasia of the Liver and Elevated α Fetoprotein Level in an Infant With Isolated Hemihyperplasia

“…In using the term for all asymmetrical growths, Hoyme et al 5 reported 10 tumors (5.9%; 6 Wilms tumors, 2 adrenocortical tumors, 1 hepatoblastoma, and 1 leiomyosarcoma) in 9 patients after a prospective follow-up of 168 patients with IHH. Diverse lesions such as bilateral adrenal cystic lesions, 4,15 pancreatoblastoma, 16 renal dysplasia, and benign nephromegaly 17 have also been reported. Adrenocortical cytomegaly and adrenal hemorrhage were also present in our case together with FNH.…”