A Century of Advances in Neuroendocrine Tumor Biology
and Treatment
Foreword
On September 17, 1907, a paper entitled “Carcinoid
Tumors of the Small Intestine” was presented by the German pathologist
Siegfried Oberndorfer of Munich to the German Pathological Society in
One hundred years later, Ipsen Pharma memorialized the
anniversary of Oberndorfer’s extraordinary contribution by inviting 300
leading international physicians and scientists to celebrate his discovery.
The discussions reflected the current status of neuroendocrine tumor disease
and cutting-edge developments in the understanding of amine and peptide
secretion and suppression, as well as progress in the clinical management of
the ubiquitous entity of carcinoid disease.
The meeting was chaired by Irvin Modlin (USA) and Kjell
Oberg (Sweden), and the speakers included numerous eminent figures in the
field of gastroenterology and gastrointestinal surgery. Among them was the
Nobel Prize winner Sir James Black, whose seminal work produced the first
agents to effectively suppress cell secretion and block receptor activation
(beta adrenergic and histamine-2 receptor antagonists – H2RAs). A
similar concept led to the award of the Nobel Prize to Guillemin and Schally
for the identification of somatostatin and the development of the
somatostatin receptor class of agonists so effective in the treatment of
neuroendocrine tumor disease. Their efficacy and unparalleled anti-secretory
activity has revolutionized the management of neuroendocrine tumor disease.
In the century since Oberndorfer’s discovery, the
knowledge of the physiology and pathophysiology of neuroendocrine cells and
tumors has increased dramatically. Medical therapy has become widely
accepted as a life-long adjunct to all other interventions due to its safety
and high efficacy. The advances in symptom suppression have been especially
augmented by the identification of improvement in quality of life and the
avoidance of potentially lethal complication of carcinoid crisis.
As recently as three decades ago, carcinoid was considered
to be an exotic, rare and almost unmanageable condition caused by diverse
lesions that were ill-understood, difficult to diagnose and for whom no
rational therapeutic strategy existed. However, in the past few years, a
growing body of evidence has demonstrated the molecular and genomic basis,
defined the mechanistic elements of secretion and proliferation and provided
evidence that neuroendocrine cells can be identified, regulated and even
ablated. The identification of an increasingly larger population of patients
exhibiting the disease yet diagnosed very late, has proved frustrating and
highlighted the need for a sensitive and specific surveillance test. A
further important observation has been that neuroendocrine disease is
symptomatically represented by a constellation of mundane symptoms that are
often ignored, indicating the need for increased patient and physician
education. For therapy to be regarded as effective and successful, it is
necessary to provide control not only of the symptoms and quality of life
but also the growth and spread of the neoplasm. In this respect much
progress is still required.
This book serves to provide a broad state-of-the-art
overview of the substantial developments in the field of neuroendocrine
tumors in the century since Oberndorfer’s seminal description of carcinoid
tumors. It presents a compendium of clinical and scientific information that
summarizes the advances in neuroendocrine tumor biology, pathology and
clinical practice in the last century. Both we and the distinguished
physicians present acknowledge and celebrate the life and times of
Oberndorfer, whose great accomplishment led to the elucidation of
neuroendocrine tumor disease.
Irvin M Modlin MD, PhD and Kjell Oberg MD, PhD
Return to Meeting Publications