I like reading the words of Dr Mark Lewis, an Oncologist and a Neuroendocrine Tumour (NET) patient himself (with MEN1).  He always delivers with “enthusiastic vigour”, a term he reduces to “brio” (which I had to google!)

His article as usual sets a scene and he has form for looking back in the history of NETs. I’m sure he does this as it can often illustrate just how much clinical progress has been made since way back then. And that is the purpose of the recent article entitled “Continuing the Odyssey in the Land of Small Tumors“.  He quotes from a 1987 article written by Dr Charles Moertel entitled “An Odyssey in the Land of Small Tumors” and I suspect he selected this article from Dr Moertel as he too writes with “brio”.

“Patients present in an almost surrealistic manner – a mix of tumor-related symptoms and signs, with bizarre and sometimes grotesque endocrine syndromes”

The article from 1987, when somatostatin analogues were starting to be used, must have been a hugely exciting time, perhaps more so than when peptide receptor radiotherapy (PRRT) became a thing in the 21st Century. After setting the scene using Dr Moeortel’s work, Dr Lewis then introduces a recent paper by well-known NET Specialist Dr Pamela Kunz and Dr Janie Zhang, a Hematology/Oncology Fellow at Yale-New Haven Hospital.  Dr Lewis introduces their work with “After more than three decades of interim research, the article in this issue by Zhang and Kunz provides a contemporary framework for approaching the titular small tumors, with updates on nomenclature, diagnostic workup, and management“.  Please note this review will focus on tumor control in NETs (i.e. not in Neuroendocrine Carcinomas).

In his own paper, Dr Lewis quite rightly points out the continued confusion whereby the classification of neuroendocrine tumors (NETs) has long been obfuscated by the etymology of the German word karzinoide, a misnomer if it is translated to mean that this cancer-like disease is uniformly benign. Clearly there’s still a lot of work to do to get rid of that obfuscation, the thoracic guys are still behind the curve.

As a patient himself, Dr Lewis also covers the issue of patient quality of life.  Clearly there’s a balance required making therapy decisions often tough for the physician and patient, particularly when the therapy may be different across the broad spectrum of NET types and primary locations. Another area where many unmet needs still remain.

And he approaches the tricky subject of therapy sequencing with the words “This risk-benefit calculus is crucial in caring for patients whose longevity can be measured in years, if not decades, and for whom therapeutic options have multiplied to the point that the sequence of their use must be considered“. Working out the best sequencing is difficult, particularly with so many factors involved not least the different types of NET and primary locations.  This is really a work in progress and perhaps will always be.

Both papers make for excellent reads and summaries of just how far we’ve come.  Clearly there’s still a long way to go!  I encourage you to read both recently published papers in this sequence:

1. Continuing the Odyssey in the Land of Small Tumors, Mark A. Lewis, MD, DOI: 10.1200/OP.21.00598 JCO Oncology Practice, published online October 15, 2021, PMID: 34652964 (click here)

2. Making Sense of a Complex Disease: A Practical Approach to Managing Neuroendocrine Tumors, Janie Y. Zhang MD, Pamela L. Kunz MD, DOI: 10.1200/OP.21.00240 JCO Oncology Practice, Published online October 15, 2021, PMID: 34652954 (click here)

And for those who, like Dr Mark Lewis and myself, like reading articles with “memorable brio”, you may also enjoy the 1987 work from Dr Moertel:

Moertel CG: Karnofsky memorial lecture. An odyssey in the land of small tumors. J Clin Oncol 5:1502–1522, 1987 Click this Link