Protein-Losing Enteropathy: An Unusual Presentation of Neuroblastoma

Intestinal lymphangiectasia as a first manifestation of neuroblastoma

“…In one of these patients, PLE was attributed to neurohumoral mechanisms of catecholamines secreted by neuroblastoma cells 3. In the others, intestinal lymphangiectasia due to lymphatic obstruction by the tumor was reported as in our case 1,2,4. It is necessary to consider secondary causes of intestinal lymphangiectasia, especially neuroblastoma, in young children with PLE, and sufficient investigation should be done before the diagnosis of congenital lymphangiectasia is made.…”

“…Lymphosarcoma, gastrointestinal carcinomas, mesenteric mesenchymoma, and lymphoma have been associated with protein‐losing enteropathy 1,2,6. Proposed mechanisms for the occurrence of PLE in malignancy include protein loss through the inflamed or ulcerated mucosa, such as in gastrointestinal carcinomas, direct tumor infiltration such as lymphoma, lymphatic obstruction with heart failure, and chronically elevated right‐sided venous pressure from carcinoid syndrome, neurohumoral effects of substances secreted by tumor such as VIP or catecholamines, and lymphatic obstruction by tumor and development of secondary intestinal lymphangiectasia 1–4. Neurohumoral factors such as VIP can be responsible for the development of PLE in neuroblastoma.…”

“…It has been reported that neuroblastoma can cause chronic diarrhea by neurohumoral effects on the gastrointestinal tract of various substances secreted by the tumor. However, chronic diarrhea due to protein losing enteropathy (PLE) is an uncommon presentation in children with neuroblastoma 1–4. In this report, we present an unusual case of intestinal lymphangiectasia, which cause a protein‐losing enteropathy as an initial manifestation of neuroblastoma.…”

The Update of Treatment for Primary Intestinal Lymphangiectasia

“…This investigation is usually performed simultaneously with endoscopy, which is used to validate intestinal lymphangiectasia. Echocardiography and abdominal imaging is generally required since the secondary origins are usually cardiac and associated with tumor conditions such as lymphoma, sarcoma and neuroblastoma [ 11 12 13 ]. Central venous pressure is often elevated above the thoracic duct pressure in patients with right-sided heart failure or single-ventricle circulation [ 14 ].…”

Emergencies Caused by Pheochromocytoma, Neuroblastoma, or Ganglioneuroma

“…Patients may present with leg palsy or weakness of the lower extremities if the tumor involves the spinal cord or results in spinal cord compression [162,163]. Other tumor mass-related presentations include blindness owing to metastatic neuroblastoma [164] and protein-losing enteropathy as the result of lymphatic obstruction [165] or elevated catecholamines [166], resulting in periorbital edema and severe hypoproteinemia.…”