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Table of Contents
Definition / general | Terminology | Clinical features | Case reports | Treatment | Clinical images | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Electron microscopy description | Electron microscopy images | Differential diagnosis | Additional referencesCite this page: Neuroendocrine carcinoma - general. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/breastmalignantneuroendocrine.html. Accessed July 14th, 2017.
Definition / general
- Carcinoma with neuroendocrine features in at least 50% of cells
- Terminology usually restricted to low grade tumors (high grade tumors are often called small cell carcinoma)
- May arise from neuroendocrine cell hyperplasia (Pathol Int 2012;62:331, J Clin Pathol 2012;65:699)
Terminology
- 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 features
- 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 Pathol 2001;14:768)
Case reports
- 60 year old woman with neuroendocrine breast carcinoma metastatic to renal cell carcinoma and ipsilateral adrenal gland (Pathol Res Pract 2008;204:851)
- 63 year old woman with breast carcinoma with neuroendocrine differentiation and myocardial metastases (Clin Breast Cancer 2007;7:892)
- 63 year old woman with solid neuroendocrine carcinoma (Ann Ital Chir 2013;84:81)
- 76 year old woman with multinodular cutaneous spread in neuroendocrine tumor of the breast (Am J Clin Dermatol 2007;8:379)
- 77 year old woman with HER-2 positive primary solid neuroendocrine carcinoma of the breast (Breast Cancer 2012 Jun 19 [Epub ahead of print])
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
Images hosted on other servers:
10 cm mass
Gross description
- No distinctive gross features
Microscopic (histologic) description
- 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
Microscopic (histologic) images
Images hosted on PathOut server:
Ribbon growth pattern with spindled cells (AFIP )
Cells often have argyrophilic
grimeliius+ granules, tumor is
immunoreactive for chromogranin (AFIP )
Images courtesy of Dr. Semir Vranic
Focal mucin production
Chromogranin expression
Synaptophysin expression
Estrogen receptor
HER2 protein
Images hosted on Flickr:
Neuroendocrine carcinoma of the breast
Images hosted on other servers:
Cells in small and large nests
No pleomorphism
Hint of lumen
Well differentiated tumor with alveolar growth pattern
Fig 2: H&E, chromogranin & synaptophysin
Chromogranin immunoreactivity
Various markers (Fig G, H, I)
(Fig A-D, H-I)
Carcinomatous lymphangitis
Synaptophysin+
Cytology description
- Markedly cellular with mostly dispersed tumor cells, also some loose clusters, acinus-like formations, small sheets, rosette like formations and 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
- ER, PR (usually, Med Oncol 2012;29:2613)
- Also chromogranin, synaptophysin, neuron-specific enolase, GCDFP-15 (50%), TTF1 (20%)
Electron microscopy description
- Dense core secretory granules
Electron microscopy images
Images hosted on PathOut server:
Dense core granules
throughout the
cytoplasm, but increased
at cell membrane
Dense core granules
measure 270 - 430 nm
cluster near cell membrane
Differential diagnosis
- Lobular carcinoma: linear and targetoid patterns, nuclear features not neuroendocrine
- Metastatic carcinoid tumor (Am J Surg 2006;191:799, Diagn Cytopathol 2007;35:306)
- Neuroendocrine DCIS: not invasive
- Other breast tumors with neuroendocrine features include small cell carcinoma, colloid carcinoma and invasive ductal carcinoma NOS with < 50% neuroendocrine tumor cells
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