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Uncommon Presentations, Uncommon Cancers

December 30, 2021

In this column, Beth Faiman, CNP, PhD, reviews the 5th edition of the Textbook of Uncommon Cancer.

Have you read any hematology-related books recently? Let us know what we should read and review by emailing us at [email protected].


A 52-year-old marathon runner without any significant medical or family history presents with progressive shortness of breath, which has worsened over the course of 3 months. He is seen by his primary-care provider, who orders a complete blood count, comprehensive metabolic panel, pulmonary function tests, a chest x-ray, a computed tomography scan, and an electrocardiogram (ECG). The test results are all essentially normal and fail to explain his progressive shortness of breath. He is referred to a cardiologist, and an ECG shows that he has a thickened myocardium, diastolic dysfunction, a low left ventricular ejection fraction (50%), and an elevated N-terminal pro b-type natriuretic peptide (4,350 ng/mL).

An astute practitioner orders a monoclonal protein analysis, which is positive for monotypic lambda, and a serum free light chain assay shows an elevated lambda-free serum (4,880 mg/L). An endomyocardial biopsy stained with thioflavin S is positive for lambda monotypic light chains. The patient is diagnosed with immunoglobulin light chain cardiac amyloidosis.

Each of us encounter exceptional cases such as this in our daily practice, which often begin with a relatively common symptom. The 5th edition of the Textbook of Uncommon Cancer provides practitioners with valuable, evidence-based information for diagnosing and managing numerous hematologic and solid cancers. For each condition, the text lists signs and symptoms, describes practical approaches to treatment, and highlights necessary patient and family resources.

Practitioners will appreciate the information provided in this updated edition, which includes diagnostic and treatment considerations in a multitude of areas, written by international experts in the fields of hematology and oncology. With just one glance at the book's table of contents, I was particularly intrigued to see the diverse range of blood and solid tumor cancers addressed within its pages.

I was particularly intrigued to see the diverse range of blood and solid tumor cancers addressed within its pages.

Each of us encounter exceptional cases such as this in our daily practice, which often begin with a relatively common symptom. The 5th edition of the Textbook of Uncommon Cancer provides practitioners with valuable, evidence-based information for diagnosing and managing numerous hematologic and solid cancers. For each condition, the text lists signs and symptoms, describes practical approaches to treatment, and highlights necessary patient and family resources.

Practitioners will appreciate the information provided in this updated edition, which includes diagnostic and treatment considerations in a multitude of areas, written by international experts in the fields of hematology and oncology. With just one glance at the book's table of contents, I was particularly intrigued to see the diverse range of blood and solid tumor cancers addressed within its pages.

My interest was piqued further when perusing the sections on plasma cell dyscrasia and leukemia. While these cancers are already less common than, say, breast or prostate cancer, these chapters manage to cover uncommon manifestations of uncommon cancers, such as immunoglobulin D and non-secretory myeloma and chronic neutrophilic leukemia. Reading through these two chapters, I was captivated by the information that I did not even know existed. The editors and authors also provide clinical pearls and insight into recognizing and promptly treating these disorders, which, given their infrequency, are particularly useful.

That this book covers less common presentations is unique, as most textbooks tend to cover "classic" case scenarios. Such textbooks are typically more concerned with reviewing commonalities so that readers won't miss a correct diagnosis, but fail to address the other side of the coin – unusual presentations.

Accurate diagnostic considerations and prompt interventions are essential to improve outcomes and minimize morbidities, no matter what the ailment. The science is moving fast in terms of diagnosis and management of cancer. Rare and infrequently encountered malignancies are just as important to be aware of as common manifestations, particularly for those who already subspecialize within hematology, where the rare has become common, but the ultra-rare might require a refresher.

This text provides insight on exceptional scenarios, along with updated prognostic classification systems, strategies for immunophenotypic analyses, and updated genomic considerations.

This is a great text to keep on your shelf and page through as time permits.

Whether you practice in a community-based or a large, hospital-based setting, the Textbook of Uncommon Cancer is a must-have resource for all practitioners.

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