Carcinoid Cancer Awareness Month: Exploring Diagnosis, Treatment, and Patient-Centered Care

In recognition of Carcinoid Cancer Awareness Month, this episode of Oncology Data Advisor brings together Editorial Board and Fellows Forum members Dr. Waqas Haque, Dr. Joseph Kalis, Dr. Nagashree Seetharamu, and Dr. Matthew Hadfield to discuss the latest advancements in managing carcinoid cancers.

Understanding Carcinoid Tumors and Neuroendocrine Tumors

Dr. Waqas Haque, Oncology Fellow at the University of Chicago, opened the discussion by explaining the distinction between carcinoid tumors and neuroendocrine tumors. Neuroendocrine tumors (NETs) arise from neuroendocrine cells and are often found in the gastrointestinal (GI) tract, pancreas, and lungs. Over recent years, the incidence of NETs has risen, which may be due to improved imaging technology, greater awareness, or lifestyle changes. Carcinoid tumors represent a subtype of NETs, with specific terminology shifting based on location. For instance, lung NETs are often still referred to as carcinoid tumors, whereas GI tract tumors are now more commonly labeled as NETs.

Carcinoid Syndrome: Recognizing Symptoms

One distinguishing feature of carcinoid tumors is their potential to cause carcinoid syndrome, a condition that occurs when the tumor metastasizes to the liver. Symptoms of carcinoid syndrome include flushing, diarrhea, and, in advanced cases, heart-related symptoms. However, these symptoms are only present in around 10% of patients, with the rest experiencing a more indolent disease course.

The panelists noted that, due to the slow-growing nature of many carcinoid tumors, they are often detected incidentally. Nevertheless, awareness of this condition is essential, as some populations may be at a higher risk. For example, African Americans have been suggested to have a higher risk of GI NETs, although this may be due to factors such as healthcare access and delays in diagnosis.

Treatment Approaches: Surgery and Symptom Management

Treatment for carcinoid tumors varies widely depending on the disease’s aggressiveness and stage. Surgery remains the primary curative approach for resectable tumors, while asymptomatic, slow-growing tumors may not require immediate treatment. For patients with unresectable or symptomatic disease, medical management, primarily with somatostatin analogs like lanreotide or octreotide, helps control symptoms without directly targeting the tumor.

Dr. Kalis and Dr. Hadfield discussed the use of somatostatin analogs in carcinoid management, emphasizing that while these drugs are effective in symptom management, they are generally less effective in significantly slowing tumor progression. For patients with metastatic disease, the slow-growing nature of carcinoid tumors can make achieving response rates challenging, even with chemotherapy.

Advanced Therapies and Clinical Trial Challenges

The limited treatment options for metastatic carcinoid tumors underscore the importance of exploring novel therapies. While some clinical trials have investigated immunotherapies, including checkpoint inhibitors, response rates have generally been modest compared to other solid tumors. The low prevalence of carcinoid tumors presents a challenge for conducting large-scale randomized clinical trials, and many studies rely on smaller, single-arm trials or basket trials that include various NET subtypes.

The panelists highlighted peptide receptor radionuclide therapy (PRRT), approved in 2018, as a promising option for refractory disease. PRRT can help reduce progression rates and improve symptom control, but it is often associated with side effects such as fatigue and weight loss. Access to PRRT can also be a barrier, as it is regionally dependent and frequently limited to larger academic medical centers, necessitating travel for many patients.

Another option discussed was cabozantinib, a multitargeted tyrosine kinase inhibitor, recently tested in carcinoid tumor trials. Although cabozantinib has shown potential efficacy, particularly in renal cell carcinoma, its side effects often necessitate dose adjustments. Panelists raised the issue of dose optimization in clinical practice, questioning the common approach of using the maximum tolerated dose from trials rather than identifying a potentially lower effective dose. This topic aligns with the FDA’s Project Optimus initiative, which seeks to refine dose-selection practices for targeted therapies.

Emphasizing Patient-Centered Care

The panel concluded by underscoring the importance of patient-centered and multidisciplinary care for individuals with carcinoid tumors. Carcinoid patients often face a unique set of challenges, as their symptoms can be both burdensome and unpredictable. Coordinating care among oncologists, surgeons, radiologists, and supportive care teams is crucial to optimize treatment outcomes and quality of life.

Many patients also seek guidance on alternative therapies and supplements. However, supplements can sometimes interfere with carcinoid syndrome symptoms or affect biomarker tracking, making open communication essential. For instance, turmeric and proton pump inhibitors may cause diarrhea or artificially elevate serotonin levels, leading to diagnostic confusion.

Conclusion

Carcinoid cancer management requires a nuanced approach, with treatments tailored to the tumor’s location, aggressiveness, and patient symptoms. For healthcare professionals, understanding the latest therapeutic options and being attuned to patient-centered care principles are critical in providing comprehensive support to this population. As new therapies continue to emerge, and as awareness grows through initiatives like Carcinoid Cancer Awareness Month, the field moves closer to addressing the unique needs of patients with this rare disease.

About the Speakers

Waqas Haque, MD, MPH, is a Hematology/Oncology Fellow at the University of Chicago. He recently completed his Internal Medicine Residency at New York University (NYU) in a Clinical Investigator Track. Dr. Haque has balanced his patient care work with a variety of research projects. During his fellowship training at the University of Chicago, he plans to further his work in innovative clinical trial design, value-based care delivery to cancer patients, and clinical investigation.

Joseph Kalis, PharmD, BCOP, is an Ambulatory Oncology Clinical Pharmacy Specialist at the University of Colorado Health. In this position, he educates patients about their chemotherapy and immunotherapy treatments, reviews treatment plans and dose adjustments, and assists with supportive care. Dr. Kalis’ professional interests include multiple myeloma and hematologic malignancies. He enjoys teaching learners from all walks of life. Dr. Kalis has spoken extensively for continuing education programs, along with various peer-reviewed papers on oncology and pharmacy.

Nagashree Seetharamu, MD, is a Professor in the Department of Medicine, Division of Hematology and Oncology, at the Barbara and Donald Zucker School of Medicine at Hofstra/Northwell Health. In addition to being a nationally and internationally renowned thoracic and head and neck oncologist, with a busy clinical practice, she is a very active academician. She has conducted several research projects in the fields of lung cancer prevention, early detection, predicative and prognostic biomarkers, as well as novel therapeutics in thoracic and head and neck oncology. In addition, Dr. Seetharamu is passionate about mentoring the new generation of oncologists and plays an active role in the fellowship program at Northwell Health. She also serves as a mentor for trainees in other institutions through the ASCO mentorship program.

Matthew Hadfield, DO, is a Medical Oncologist at Brown University/Legorreta Cancer Center, with a pending faculty appointment at the Warren Alpert School of Medicine of Brown University. Dr. Hadfield’s clinical and research interests are in phase 1 clinical trials and developmental therapeutics, as well as cutaneous malignancies and immunotherapy-related adverse events. He recently completed his Hematology/Oncology Fellowship at Brown University.