Breast – Malignant, Males, Children

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Breast-malignant, males, children

Morphologic variants of DCIS

Neuroendocrine DCIS

Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.

Reviewer: Daniel Visscher, M.D., University of Michigan Hospitals, February 2009 (see Reviewers page)

Revised: 15 August 2009

Last major update: August 2009

Definition

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● DCIS in which 50% or more cells express neuroendocrine markers (Histopathology 2008;53:288)

Terminology

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● First described in 1985 (Histopathology 1985;9:21)

● Also called endocrine DCIS, intraductal or solid papillary carcinoma (AJSP 1995;19:1237)

Epidemiology

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● 7% of DCIS cases

● Mean age 70 years, usually > age 60 years (Zhonghua Bing Li Xue Za Zhi 2006;35:594)

Clinical

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● May have associated invasive component, either mucinous (colloid) or with neuroendocrine features (AJSP 1996;20:921)

● Presents as breast mass or nipple discharge (AJSP 1996;20:921)

● Neuroendocrine DCIS is associated with intraductal papilloma and pagetoid involvement by tumor cells

● Neuroendocrine markers are expressed in 67% of solid intraductal papillary carcinomas (Virchows Arch 2007;450:539)

● Almost all DCIS with spindle cells have neuroendocrine differentiation (Histopathology 2004;45:343)

Case reports

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● 68 year old woman with 1.5 cm breast nodule (Am Surg 2000;66:1163)

● 72 year old woman with breast mass (Case of the Week #45)

Microscopic description

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● Solid lobular growth, neuroendocrine-like festoons and rosettes and prominent fibrovascular septa

● Cells are polygonal, oval or spindled with abundant granular eosinophilic cytoplasm and bland ovoid nuclei

● Accumulation of basophilic intracellular mucin

● Often pagetoid spread and well developed vascular network

● Variable stromal sclerosis or signet ring cells

● Usually no necrosis

Micro images

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Case of the Week #45

Synaptophysin #1 #2

Solid proliferation Cribriform pattern

Tumor cells in cords and festoons with Chromogranin

well defined fibrovascular septa and vascular cores

Small cells with dense, deeply stained Focal Grimelius+ cells

amphophilic cytoplasm and uniform round nuclei

Cytology description

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● Plasmacytoid tumor cells and arborizing papillary fronds (Cancer 2000;90:286)

Cytology images

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Highly cellular with cell clusters Multiple single detached cells with plasmacytoid

with papillary architecture appearance (eccentric nuclei and granular cytoplasm)

Single plasmacytoid tumor cells Papillary fronds in moderately cellular background

with cytoplasmic granularity

Virtual slides

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Two cases

Positive stains

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● Chromogranin, synaptophysin, neuron-specific enolase

● ER, PR (Histopathology 2004;45:343)

● Low Ki-67

Negative stains

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● p53, HER2

Electron microscopy

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● Dense core neurosecretory granules, larger mucigen granules

Differential diagnosis

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● Florid epithelial hyperplasia and papilloma - lack the monomorphic cell population associated with DCIS, negative for neuroendocrine markers

End of Breast – Malignant, Males, Children > Neuroendocrine DCIS

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