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Breast-malignant, males, children
Neuroendocrine carcinoma
Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.
Reviewer: Daniel Visscher, M.D., University of Michigan Hospitals, February 2009 (see Reviewers page)
Revised: 23 September 2009
Last major update: September 2009
Copyright: (c) 2002-2009, PathologyOutlines.com, Inc.
Definition
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● Carcinoma with neuroendocrine features in at least 50% of cells
● Usually restricted to low grade tumors (high grade tumors are usually considered small cell carcinoma)
● See also small cell carcinoma
Terminology
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● Often (although not consistently) refers to cases lacking another specific histologic type, such as solid papillary, mucinous/colloid or micropapillary
● Also called endocrine carcinoma or solid cohesive neuroendocrine carcinoma
● Some cases were formerly called carcinoid tumor (Eur J Surg Oncol 1995;21:609)
Clinical
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● Up to 5% of all breast carcinomas, depending on how defined
● Frequency increases with age
● Similar clinical presentation as ductal NOS; i.e. no carcinoid syndrome is present
● Similar prognosis as ductal NOS
● Apocrine phenotype (androgen receptor positive in 50% of cells) is present in elderly women (Mod Path 2001;14:768)
Case reports
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● Presenting as perianal mass (Clin Breast Cancer 2007;7:892)
● Presenting as kidney and adrenal metastases (Pathol Res Pract 2008;204:851)
● Metastases to skin (Am J Clin Dermatol 2007;8:379)
Treatment and prognosis
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● Treatment similar to ductal carcinoma NOS, but possibly add somatostatin for nuclear scanning and treatment of metastatic disease (G Chir 2008;29:203, Breast 2008;17:111)
Clinical images
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10 cm mass
Gross description
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● No distinctive gross features
Microscopic description
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● Small, low grade tumor cells in nests separated by fibrous tissue
● Rarely ribbons, rosettes or mitotic figures
● Usually no mucin, no DCIS
● No specific histologic patterns, such as solid papillary, small cell or mucinous/colloid
Micro images
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Fig 2: H&E, chromogranin Distinct nests of cells
& synaptophysin
Various markers (Fig G, H, I) (Fig A-D, H-I)
Ribbon type growth pattern with Tumor cells often have argyrophilic Grimelius+
some spindled cells (AFIP) granules, tumor is immunoreactive for chromogranin (AFIP)
Other images: well differentiated tumor with alveolar growth pattern #1; #2; #3 with carcinomatous lymphangitis; #4-synaptophysin+
Cytology description
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● Markedly cellular with mostly dispersed tumor cells, also some loose clusters, acinus-like formations, small sheets, rosette like formations, ribbons
● Cells are small and regular with moderate cytoplasm, fairly uniform and round/oval nuclei, often plasmacytoid with eccentric nuclei (Acta Cytol 1994;38:73, Indian J Pathol Microbiol 2007;50:65)
Positive stains
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● Chromogranin, synaptophysin, neuron-specific enolase, ER
● GCDFP-15 (50%), TTF1 (20%)
Electron microscopy
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● Dense core secretory granules
Electron microscopy images
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Dense core granules throughout the Dense core granules that measure
cytoplasm, but increased at cell membrane 270-430 nm cluster near cell membrane
Differential diagnosis
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● Lobular carcinoma
● Metastatic carcinoid tumor - Am J Surg 2006;191:799, Diagn Cytopathol 2007;35:306
● Other breast tumors with neuroendocrine features - small cell carcinoma, colloid carcinoma, invasive ductal carcinoma NOS with <50% neuroendocrine tumor cells
● Neuroendocrine DCIS - not invasive
Additional references
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End of Breast – Malignant, Males, Children > Neuroendocrine carcinoma
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