Neuroendocrine tumor

About neuroendocrine tumors in children

Neuroendocrine tumors (NET) arise from cells which have characteristics of both endocrine cells and nerve cells. These cells are distributed throughout the body, and therefore neuroendocrine tumors can arise in different organs in the body. NETs are extremely rare in children and young adults with an incidence rate of 2.8 per million per year. Most NET in children are low-grade tumors and are clinically benign. Although majority are sporadic, NET can be associated with familial syndromes such as multiple endocrine neoplasia (MEN) type 1 and 2, neurofibromatosis (NF-1), Von Hippel Lindau (VHL) and tuberous sclerosis complex (TSC).

Evaluation and treatment

Often the symptoms of neuroendocrine tumors are nonspecific and there can be a significant delay in diagnosis. Therefore, these patients may be treated by multiple medical specialists. There are no consensus guidelines for treatment of NET in children, and most physicians follow adult guidelines. At Texas Children’s Cancer and Hematology Center, a multidisciplinary team of physician scientists are involved in the diagnosis and treatment of children with neuroendocrine tumors. Based on the evidence that the biology of NET in children is different from adults, we strive to tailor innovative treatment plans and clinical trials towards better outcomes in children.

Types of neuroendocrine tumors

• Neuroendocrine tumor of appendix (carcinoid of the appendix). This is the most common neuroendocrine tumor in children. Research conducted at Texas Children's Hospital has shown that the tumors that occur in children are different from that in adults. Hemicolectomy operation in children with neuroendocrine tumor of the appendix is rarely indicated. If you child is scheduled for a hemicolectomy operation we highly recommend contacting us for a second opinion by emailing raretumors@texaschildrens.org.
• Bronchial carcinoid
• Pancreatic neuroendocrine tumor
• Pheochromocytoma
• Paraganglioma
• High grade neuroendocrine carcinoma

Second opinions

We welcome oncologists to contact us for second opinion consultations about the care and management of individual patients.

Research

At Texas Children’s Cancer and Hematology Center, we  are further interested in understanding the  biology of neuroendocrine tumors  with the goal of offering more successful therapies for pediatric patients  We have established the North American Pediatric Neuroendocrine Tumor Registry to collect information on children with neuroendocrine tumors. Email us at raretumors@texaschildrens.org for information on how to enroll in the registry.

Publications

Venkatramani R, Wu H, Chintagumpala M. Neuroendocrine tumor of the appendix in children. Neuroendocrine Tumor Symposium 2015, Austin, Texas, October 15-17, 2015. Abstract