Wilms' tumor, pancreatic islet cell carcinoma, and pheochromocytoma in a child

Pancreatic Tumors

“…Pancreatic neuroendocrine tumors (pNETs) are rare in children and may present sporadically or in conjunction with congenital syndromes such as multiple endocrine neoplasia (MEN) or von Hippel-Lindau syndromes (Sola et al 2008;Ellison and Edil 2012). Specifically, insulinomas, gastrinomas, and VIPomas have been reported in children (Wolfe and Jensen 1987;Grosfeld et al 1990;Park et al 1996;Nazir 2011;Peranteau et al 2013).…”

“…For obvious reasons, nonfunctioning lesions are more difficult to diagnose; these tumors are important, however, as they comprise approximately 85% of all pNETs (Ellison and Edil 2012). In these cases, as with other malignancies with poor prognoses, patients present only after complications of mass effect and/or malignant spread (Sola et al 2008).…”

“…As with the other histologic types, surgical intervention is the cornerstone of curative treatment for insulinomas (Grosfeld et al 1990;Peranteau et al 2013). Due to its encapsulated morphology, insulinomas may be amenable to enucleation alone once the pancreatic duct is identified on intraoperative ultrasound (Grosfeld et al 1990;Sola et al 2008). These lesions may occur anywhere along the pancreas, however, without a specific preference for head, body, or tail (Peranteau et al 2013).…”

“…Vasoactive intestinal peptide (VIP), substance P, and possibly secretin cases of diffuse islet cell carcinoma, total pancreatectomy may be required (Sola et al 2008). Due to an overall earlier presentation, however, these malignancies have an improved prognosis compared to other tumors of the pancreas.…”