Serum biological markers and paraneoplastic syndromes in Wilms tumor

Abstract

Various tumors secrete tumor-specific substances capable of producing signs and symptoms in host organs not caused by direct tumor invasion or organ destruction. These symptoms are collectively referred to as "remote effects" or "paraneoplastic syndromes" of malignancy. Paraneoplastic syndromes are uncommon in childhood cancer. In Wilms tumor several distinct paraneoplastic syndromes have been reported: hypertension, erythrocytosis, hypercalcemia, Cushing syndrome, and acquired Von Willebrand disease. In addition some tumor-specific substances are known to be elevated in patients with a malignancy without causing specific symptoms. These so called "tumor markers" can be used to detect early recurrence in previously treated patients, or in the evaluation of patients undergoing adjuvant therapy. Five of particular interest are erythropoietin, neuron-specific enolase (NSE), hyaluronic acid (HA), hyaluronic acid-stimulating activity (HSA), and hyaluronidase.

Classic Wilms tumor with raised alpha-fetoprotein levels: A case report and literature review

“…Tumor markers have predictive value in diagnosis and follow-up of children with solid malignancies [6]. Elevated levels of some tumor markers such as neuron specific enolase, hyaluronic acid, carcinoembryonic antigen, erythropoietin and renin have reported in Wilms' tumor [7]. Additionally, an elevated level of serum AFP is mainly found in cases such as hepatoblastoma, hepatocelluler carcinoma, malignant germ cell tumors, and some immature teratomas containing yolk salk elements [8].…”

Wilms’ Tumor in Children: An Overview

“…Some hormones, such as erythropoietin and ACTH, can be secreted in Wilms’ tumor. In addition, hypercalcemia and hemorrhagic conditions caused by reduced von Willebrand factor are associated with Wilms’ tumor [33]. Physicians also should be alert for other associated findings, such as hemihypertrophy, aniridia, and genitourinary malformations.…”

“…The tumor is often detected by a caregiver while bathing the child. 30% of patients have hematuria, and 25% have hypertension [32, 33]. In addition, patients can suffer from malaise, fever, weight loss, anorexia, or a combination of those symptoms.…”

Hypertension in a cohort of African children with renal tumours

“…The reported incidence in cohorts of patients with Wilms' tumour varies widely from 25 to 100% [1,[3][4][5]. The incidence in the developing world is unknown.…”

“…Hypertension is a frequent problem in children with renal tumours and has been ascribed to increased renin production either as a tumour metabolite or secondary to alteration of intra-renal blood flow and stimulation of the juxta-glomerular apparatus [1][2][3]. The reported incidence in cohorts of patients with Wilms' tumour varies widely from 25 to 100% [1,[3][4][5].…”