Protein Losing Enteropathy – An Unusual Presenting Symptom of Wilms Tumor

Abstract

We report a case of a 2 month old male presenting with edema and hypoalbuminemia who was found to have a Wilms tumor. This is the first reported case of Wilms tumor presenting as protein losing enteropathy. History: The patient presented to his primary care physician with fever and irritability. On exam he was noted to have pitting edema of the bilateral lower extremities and periorbital swelling. This was long standing, as parents did not consider this a change in his baseline. Labs on admission were remarkable for a total protein of 3.8g/dL and albumin of 2.3g/dL. CBC with differential was unremarkable and ALC was slightly low at 2.97k/uL. Urine was negative for protein. US of the abdominal vasculature was negative for thrombosed vessel and no mass was appreciated at that time. He was treated with albumin infusions and IV furosemide. His feeds were switched to hydrolysate and then elemental formula with no significant improvement in his clinical or lab status. Cardiac ECHO was normal. The patient had an EGD and flexible sigmoidoscopy with biopsy. These were visually normal, and pathology was negative for any inflammatory changes or evidence of dilated lacteals. MR of the lower extremities and pelvis were negative for evidence of lymphangiectasia. Stool for α-1-antitrypsin was markedly elevated. A tagged albumin scan was done hoping to localize the source of protein loss. The study was non-diagnostic, however a lack of perfusion was described in the upper pole of the right kidney. Subsequent imaging confirmed the mass. He underwent right radical nephrectomy with lymph node sampling. Intraoperatively dilated lymphatics of the mesentary and a small amount of chylous ascities were noted. Pathology showed Wilms tumor with invasion of the renal capsule and no lymph node involvement. Post operatively the patient has successfully maintained albumin levels above 3.5g/dL with no infusions and his ALC has been increasing. Clinically he is less edematous and his growth has been good. Discussion: Protein losing enteropathy is a clinical entity multiple potential etiologies. Typically it is caused by mucosal inflammation or lymphatic obstruction. Severe infections or inflammitory states may also increase intestinal permeability leading to protein loss. One potential mechanism for our patient's presentation is mass effect with impingement of the efferent lymphatics. Also possible is a paraneoplastic syndrome or humoral effects of substances secreted by the tumor.