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Management of Large Cell Neuroendocrine Carcinoma
Virginia Corbett1, Susanne Arnold2, Lowell Anthony2
1Department of Internal Medicine, Icahn School of Medicine at Mount Sinai, New York, NY, United States.
Background:
Large cell neuroendocrine carcinoma (LCNEC) is a rare, aggressive cancer with a dismal prognosis. The majority of cases occur in the lung and the gastrointestinal tract; however, it can occur throughout the body. Recently advances in the understanding of the molecular underpinnings of this disease have paved the way for additional novel promising therapies. This review will discuss the current best evidence for management of LCNEC and new directions in the classification and treatment of this rare disease.
Methods:
We performed a PubMed search for "Large cell neuroendocrine carcinoma" and "High grade neuroendocrine carcinoma." All titles were screened for relevance to the management of LCNEC. Papers were included based on relevance to the management of LCNEC.
Results:
Papers were included reviewing both pulmonary and extra pulmonary LCNEC. We summarized the data driven best practices for the management of both early and advanced stage LCNEC. We describe emerging therapies with promising potential.
Discussion:
LCNEC are rare and aggressive neoplasms. In advanced disease, the historical regimen of platinum based therapy in combination with etoposide or irinotecan remains among the commonly used first line therapies, however for extra thoracic LCNEC regimens like FOLFOX, FOLFOIRI and CAPTEM can also be used. Further effective and safe treatment options are desperately needed. Recently, new advances including a new understanding of the genetic subcategories of LCNEC and immunotherapy agents may guide further treatments.
Insights
Large cell neuroendocrine carcinoma (LCNEC) is a rare, aggressive cancer. Current treatments include platinum-based chemotherapy, but new therapies and a better understanding of LCNEC subtypes offer hope for improved management.
Area of Science:
- Oncology
- Cancer Research
- Medical Science
Background:
- Large cell neuroendocrine carcinoma (LCNEC) is a rare and aggressive malignancy.
- While most common in the lungs and GI tract, LCNEC can arise anywhere.
- Recent molecular insights are driving novel therapeutic strategies.
Purpose of the Study:
- To review current management strategies for LCNEC.
- To discuss emerging treatments and classification systems for LCNEC.
- To provide an evidence-based overview of LCNEC care.
Main Methods:
- A PubMed search was conducted for "Large cell neuroendocrine carcinoma" and "High grade neuroendocrine carcinoma."
- Titles were screened for relevance to LCNEC management.
- Relevant papers were included for review.
Main Results:
- The review encompasses both pulmonary and extra-pulmonary LCNEC.
- Best practices for early and advanced stage LCNEC management are summarized.
- Promising emerging therapies are highlighted.
Conclusions:
- LCNEC is a rare, aggressive neoplasm requiring further treatment options.
- Platinum-based chemotherapy with etoposide or irinotecan is a common first-line therapy for advanced LCNEC.
- New genetic insights and immunotherapies show promise for future LCNEC treatment.

