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Atypical Carcinoid

  • Living reference work entry
  • First Online:
Encyclopedia of Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

  • 28 Accesses

Synonyms

Grade 2 neuroendocrine carcinoma; Kulchitsky cell carcinoma-grade 2; Malignant carcinoid; Moderately differentiated neuroendocrine carcinoma

Currently, their use is discouraged for the sake of clarity and scientific sound.

Definition

Atypical carcinoid is a neuroendocrine-differentiated neoplasm patterning full-blown neuroendocrine morphology at the level of both neuroendocrine cell features and neuroendocrine architecture, two to ten mitoses per 2 mm2 and/or punctate foci of nonischemic necrosis. Atypical carcinoid shares the same range of histological features as typical carcinoid and most of clinical traits, so diagnostic recognition rests on the close histologic examination of resection specimens only.

Clinical Features

  • Incidence

    Atypical carcinoids are the rarest type of neuroendocrine neoplasms arising in the lung, accounting for about 0.2% of all lung cancer (one-tenth in comparison with typical carcinoids). However, the increasing incidence of this disease and the...

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References and Further Reading

  • Alcala, N., Leblay, N., Gabriel, A. A. G., Mangiante, L., Hervas, D., Giffon, T., Sertier, A. S., Ferrari, A., Derks, J., Ghantous, A., Delhomme, T. M., Chabrier, A., Cuenin, C., Abedi-Ardekani, B., Boland, A., Olaso, R., Meyer, V., Altmuller, J., Le Calvez-Kelm, F., Durand, G., Voegele, C., Boyault, S., Moonen, L., Lemaitre, N., Lorimier, P., Toffart, A. C., Soltermann, A., Clement, J. H., Saenger, J., Field, J. K., Brevet, M., Blanc-Fournier, C., Galateau-Salle, F., Le Stang, N., Russell, P. A., Wright, G., Sozzi, G., Pastorino, U., Lacomme, S., Vignaud, J. M., Hofman, V., Hofman, P., Brustugun, O. T., Lund-Iversen, M., Thomas de Montpreville, V., Muscarella, L. A., Graziano, P., Popper, H., Stojsic, J., Deleuze, J. F., Herceg, Z., Viari, A., Nuernberg, P., Pelosi, G., Dingemans, A. M. C., Milione, M., Roz, L., Brcic, L., Volante, M., Papotti, M. G., Caux, C., Sandoval, J., Hernandez-Vargas, H., Brambilla, E., Speel, E. J. M., Girard, N., Lantuejoul, S., McKay, J. D., Foll, M., & Fernandez-Cuesta, L. (2019). Integrative and comparative genomic analyses identify clinically relevant pulmonary carcinoid groups and unveil the supra-carcinoids. Nature Communications, 10, 3407. https://doi.org/10.1038/s41467-019-11276-9.

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  • Pelosi, G., Rodriguez, J., Viale, G., & Rosai, J. (2005). Typical and atypical pulmonary carcinoid tumor overdiagnosed as small-cell carcinoma on biopsy specimens: A major pitfall in the management of lung cancer patients. The American Journal of Surgical Pathology, 29, 179–187.

    Article  Google Scholar 

  • Pelosi, G., Bianchi, F., Dama, E., Simbolo, M., Mafficini, A., Sonzogni, A., Pilotto, S., Harari, S., Papotti, M., Volante, M., Fontanini, G., Mastracci, L., Albini, A., Bria, E., Calabrese, F., & Scarpa, A. (2018). Most high-grade neuroendocrine tumours of the lung are likely to secondarily develop from pre-existing carcinoids: Innovative findings skipping the current pathogenesis paradigm. Virchows Archiv, 472, 567–577.

    Article  Google Scholar 

  • Pelosi, G., Bianchi, F., Hofman, P., Pattini, L., Strobel, P., Calabrese, F., Naheed, S., Holden, C., Cave, J., Bohnenberger, H., Dinter, H., Harari, S., Albini, A., Sonzogni, A., Papotti, M., Volante, M., & Ottensmeier, C. H. (2019). Recent advances in the molecular landscape of lung neuroendocrine tumors. Expert Review of Molecular Diagnostics, 19, 281–297.

    Article  CAS  Google Scholar 

  • Travis, W., Brambilla, E., Burke, A., Marx, A., & Nicholson, A. (2015). WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press.

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Author information

Authors and Affiliations

  1. Department of Oncology and Hemato-Oncology, University of Milan, Milan, Italy

    Giuseppe Pelosi

Authors
  1. Giuseppe Pelosi
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Corresponding author

Correspondence to Giuseppe Pelosi .

Editor information

Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

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Pelosi, G. (2020). Atypical Carcinoid. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5253-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_5253-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

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