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WHO Classification of Tumours
Mixed adenoneuroendocrine carcinoma
Appendix


Definition

Mixed adenoneuroendocrine carcinomas (MANEC) have a phenotype that is morphologically recognizable as both gland-forming epithelial and neuroendocrine, and are defined as carcinomas since both components are malignant and should be graded. A component of squamous cell carcinoma is rare. Arbitrarily, at least 30% of either component should be identified to qualify for this definition. The identification in adenocarcinoma of scattered neuroendocrine cells by immunohistochemistry does not qualify for this definition
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Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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In the appendix, this term has been proposed to designate carcinomas that arise by progression from a pre-existing goblet cell carcinoid

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Tang LH, Shia J, Soslow RA, Dhall D, Wong WD, O'Reilly E, Qin J, Paty P, Weiser MR, Guillem J, Temple L, Sobin LH, Klimstra DS (2008)
Pathologic classification and clinical behavior of the spectrum of goblet cell carcinoid tumors of the appendix.
Am J Surg Pathol 32: 1429-43



. Either a signet-ring cell type or poorly differentiated adenocarcinoma type may be encountered
Click to access Pubmed
Tang LH, Shia J, Soslow RA, Dhall D, Wong WD, O'Reilly E, Qin J, Paty P, Weiser MR, Guillem J, Temple L, Sobin LH, Klimstra DS (2008)
Pathologic classification and clinical behavior of the spectrum of goblet cell carcinoid tumors of the appendix.
Am J Surg Pathol 32: 1429-43




Click to access Pubmed
Wang HL, Dhall D (2009)
Goblet or signet ring cells: that is the question.
Adv Anat Pathol 16: 247-54



. The latter type typically exhibits immunohistochemical expression of p53 and MUC1, together with loss of MUC2
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Tang LH, Shia J, Soslow RA, Dhall D, Wong WD, O'Reilly E, Qin J, Paty P, Weiser MR, Guillem J, Temple L, Sobin LH, Klimstra DS (2008)
Pathologic classification and clinical behavior of the spectrum of goblet cell carcinoid tumors of the appendix.
Am J Surg Pathol 32: 1429-43



. These carcinomas usually occur in the apparent absence of neoplastic change in the mucosal epithelium
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Burke AP, Sobin LH, Federspiel BH, Shekitka KM, Helwig EB (1990)
Goblet cell carcinoids and related tumors of the vermiform appendix.
Am J Clin Pathol 94: 27-35



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Table: Types and characteristics of neuroendocrine neoplasms of the appendix.

Commentary: Neuroendocrine tumours of the digestive system