Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2022 May;23(5):721-735.
doi: 10.1007/s11864-022-00968-y. Epub 2022 Mar 26.

An Update on the Management of Mixed Neuroendocrine-Non-neuroendocrine Neoplasms (MiNEN)

Aasems Jacob  1 , Rishi Raj  1 , Derek B Allison  2   3 , Heloisa P Soares  4 , Aman Chauhan  5
Affiliations
Review

An Update on the Management of Mixed Neuroendocrine-Non-neuroendocrine Neoplasms (MiNEN)

Aasems Jacob et al. Curr Treat Options Oncol. 2022 May.

Abstract

The classification of mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) is evolving, and no clear management guidelines are currently available. However, recent studies provide insight into factors affecting outcomes and could help develop treatment decisions for patients with these rare malignancies. The majority of MiNENs have a poorly differentiated neuroendocrine carcinoma (NEC) component which is associated with an aggressive clinical course and poor outcomes. Due to the paucity of clinical trials, strategies adopted in gastrointestinal cancers and NECs are used to manage MiNENs. It is also to be noted that the thoracic neuroendocrine neoplasm WHO 2021 classification does not recognize MiNEN terminology but suggests an equivalent terminology called "combined neuroendocrine non neuroendocrine neoplasm." Surgical management is appropriate in early-stage disease with a low threshold for addition of adjuvant chemotherapy. Multimodality treatment with chemotherapy offers a survival benefit in advanced disease or when surgical resection is not possible without significant morbidity. Chemotherapy should be directed at the more aggressive component which is often the NEC component. In addition, molecular testing should be employed to evaluate patients for enrollment in clinical trials and other targeted treatments. Being a rare disease with retrospective studies and case series providing the majority of data on treatment selection, it is essential to include more granular details of pathology (e.g., Ki-67, mitotic index, percentage of each component, staging information) and treatment modalities (e.g., type and duration, rationale, radiologic response, survival outcomes) in future studies to make systematic reviews possible and help derive meaningful conclusions.

Keywords: Hepatopancreatobiliary minen; Lower gi minen; MiNEN treatment; Mixed neuroendocrine and non-neuroendocrine tumors; Neuroendocrine tumors; Upper GI minen.

PubMed Disclaimer

References

References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
    1. Nagtegaal ID, Odze RD, Klimstra D, Paradis V, Rugge M, Schirmacher P, et al. The 2019 WHO classification of tumours of the digestive system. Histopathology. 2020;76(2):182-8. This reference is of outstanding importance since the pathologic classification and grading of MiNEN is described in this version of WHO classification.
    1. Nuñez-Valdovinos B, Carmona-Bayonas A, Jimenez-Fonseca P, Capdevila J, Castaño-Pascual Á, Benavent M, Pi Barrio JJ, Teule A, Alonso V, Custodio A, Marazuela M, Segura Á, Beguiristain A, Llanos M, Martinez del Prado MP, Diaz-Perez JA, Castellano D, Sevilla I, Lopez C, et al. Neuroendocrine Tumor Heterogeneity Adds Uncertainty to the World Health Organization 2010 Classification: Real-World Data from the Spanish Tumor Registry (R-GETNE). Oncologist. 2018;23(4):422–32. - PubMed - PMC - DOI
    1. Uccella S, La Rosa S, Metovic J, Marchiori D, Scoazec JY, Volante M, et al. Genomics of High-Grade Neuroendocrine Neoplasms: Well-Differentiated Neuroendocrine Tumor with High-Grade Features (G3 NET) and Neuroendocrine Carcinomas (NEC) of Various Anatomic Sites. Endocr Pathol. 2021;32(1):192–210. - PubMed - DOI
    1. La Rosa S, Sessa F, Uccella S. Mixed Neuroendocrine-Nonneuroendocrine Neoplasms (MiNENs): Unifying the Concept of a Heterogeneous Group of Neoplasms. Endocr Pathol. 2016;27(4):284–311. - PubMed - DOI
    1. Rindi G, Arnold R, Bosman FT, Capella C, Klimstra DS, Klöppel G, et al. Nomenclature and classification of neuroendocrine neoplasms of the digestive system. In: Bosman FT, Carneiro F, Hruban RH, Theise ND, editors. WHO Classification of Tumours of the Digestive System. Lyon: WHO Press; 2010.

LinkOut - more resources