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

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medullary carcinoma, when compared to matched patients with grade 3 InvDC,NST.<br />

Clinicopathologic features: The mean age at presentation in several series has ranged<br />

from 46 to 54 years (10,16,16). Medullary carcinomas have circumscribed margins, a<br />

s<strong>of</strong>t to moderately firm consistency, and are <strong>of</strong>ten mistaken for fibroadenomas,<br />

especially in young women. Bilateral carcinomas and multicentricity may occur<br />

(3,11,16,17).<br />

(“Multicentricity” is defined here as microscopic foci <strong>of</strong> carcinoma outside the<br />

primary quadrant, and it implies the presence <strong>of</strong> multiple clonally distinct tumors.<br />

Synchronous multicentric tumors are uncommon in my experience. “Multifocal”<br />

tumors are much more common, and “multifocal” refers to separate, satellite foci <strong>of</strong><br />

one invasive tumor clone. This feature is <strong>of</strong>ten observed in invasive lobular<br />

carcinoma. It likely results from “skip” invasive tumor, resulting from lymphaticvascular<br />

spread and/or relatively synchronous invasion from multiple separate points<br />

along the breast duct system by one carcinoma clone that has previously spread<br />

through the breast along the duct system.)<br />

In patients with medullary carcinoma, ipsilateral axillary lymph nodes are <strong>of</strong>ten<br />

enlarged, even when there are no nodal metastases, due to reactive lymphoid and<br />

histiocytic hyperplasia. The median size <strong>of</strong> the primary tumors is 2 to 3 cm. Peripheral<br />

fibrosis may suggest encapsulation, and some small tumors appear less well<br />

circumscribed due to an intense lymphoplasmacytic reaction that extends into adjacent<br />

breast tissue (10). InvDC,NST may also be well-circumscribed. Cut surfaces reveal a<br />

round or lobulated, pale brown to grey tumor, which is s<strong>of</strong>ter than the usual breast<br />

carcinoma. Hemorrhage, necrosis, and cystic changes may be present, especially in<br />

larger lesions.<br />

Those who believe medullary carcinoma is a tumor with a favorable prognosis agree<br />

that it is necessary to adhere to strict morphologic criteria for the diagnosis<br />

(3,11,10,18). As described by Foote and Stewart (19), the definitive features must<br />

include 1) a prominent lymphoplasmacytic reaction, 2) circumscription, 3) a syncytial<br />

growth pattern (i.e., broad anastomosing sheets <strong>of</strong> tumor cells with indistinct cell<br />

borders), 4) poorly differentiated nuclear grade, and 5) high mitotic rate. The<br />

lymphoplasmacytic reaction must be intense enough to be "graded" at least as<br />

“intermediate” or “moderate” in amount (i.e., the mononuclear infiltrate involves at<br />

least 75% <strong>of</strong> the periphery and is present diffusely in the substance <strong>of</strong> the tumor).<br />

According to Rosen et al. (15) the lymphoplasmacytic reaction commonly encompasses<br />

ducts and lobules in the surrounding breast occupied by carcinoma in situ<br />

as well as nearby benign ducts and lobules not containing carcinoma. He believes<br />

expansile growth <strong>of</strong> in situ carcinoma in ducts and lobules leads to the formation <strong>of</strong><br />

109

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