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33 Special Types of Invasive Breast Carcinoma: Diagnostic Criteria ...

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invasive micropapillary carcinoma may be highly aggressive (8). In any event, it is<br />

important that, whether cystic and/or solid, papillary carcinoma without invasion can<br />

be considered a form <strong>of</strong> “intraductal” carcinoma (1,9,10), and patients with<br />

noninvasive papillary carcinoma are essentially cured by mastectomy (1). But,<br />

recurrences can occur with pre-invasive examples, and they can be either in situ<br />

and/or invasive diseases.<br />

Clinicopathologic features: Papillary carcinomas occur in an older age group (mean<br />

ages from 63 to 67 years) than InvDC,NST (11). They tend to occur centrally, and<br />

nipple discharge and/or bleeding develops in ~<strong>33</strong>% <strong>of</strong> patients (1,4). <strong>Invasive</strong><br />

papillary carcinomas can be large tumors, due to the bulky, cystic component<br />

frequently present. The average size is 2 to 3 cm, and, as their <strong>of</strong>ten low histologic<br />

grade would predict, papillary carcinomas are hormone receptor-positive in the<br />

majority <strong>of</strong> cases (12,13). Papillary carcinomas are usually circumscribed, as are<br />

fibroadenomas, benign cystic lesions, and medullary or mucinous carcinomas (14,15).<br />

Invasion is suggested by an irregular contour (14). Cut surfaces are brown or<br />

hemorrhagic, but usually they are tan or grey.<br />

Although variable, the arborescent fronds <strong>of</strong> papillary carcinoma are usually<br />

composed <strong>of</strong> very delicate fibrovascular cores; indeed, the fibrous component may be<br />

inconspicuous. The lining epithelial cells are <strong>of</strong> a single type, showing high nuclear to<br />

cytoplasmic ratios, increased mitotic activity, and usually uniform, hyperchromatic<br />

nuclei. Furthermore, the tumor cells may show stratification and resemble<br />

adenomatous polyps <strong>of</strong> the colon. Mitotic figures are variably present and more<br />

numerous in lesions that exhibit the most severe cytologic atypia. Myoepithelial cells,<br />

distributed relatively uniformly and proportionately within the epithelium in benign<br />

papillary lesions, are largely overgrown in papillary carcinomas; but, they may not be<br />

entirely absent from a papillary carcinoma. The finding <strong>of</strong> myoepithelial cells in some<br />

parts <strong>of</strong> a papillary lesion is not inconsistent with a diagnosis <strong>of</strong> carcinoma (16-18).<br />

“Intracystic papillary carcinomas” (IPC) is an uncommon breast tumor usually<br />

developing in elderly patients (median age ~70, range 27 to 99); and, they present as<br />

discrete, circumscribed solitary masses (usually subareolar), which are present within<br />

ducts <strong>of</strong> at least 1 cm in diameter by definition (1,9). 3.5% occur in men (19). They<br />

<strong>of</strong>ten contain dark brown, partly clotted blood and detached degenerated papillary<br />

fragments <strong>of</strong> the tumor. Mural nodules <strong>of</strong> residual tumor can usually be found on the<br />

inner or luminal surface or in the cyst wall. Presenting as a discrete cystic mass,<br />

“intracystic papillary carcinoma” has been considered a special form <strong>of</strong> preinvasive or<br />

32

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