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

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Case 8<br />

Case history: A 50 year old female with round firm beast mass.<br />

Submitted diagnosis: Medullary <strong>Carcinoma</strong> (poorly differentiated invasive<br />

breast carcinoma, overall mBR grade 3; nuclear grade 3, tubular grade 3,<br />

mitotic grade 3).<br />

MEDULLARY CARCINOMA<br />

Background: Medullary carcinoma is defined in the WHO classification <strong>of</strong> breast<br />

tumors (1) as a “well circumscribed carcinoma composed <strong>of</strong> poorly differentiated cells<br />

with scant stroma, no glangular component and prominent lymphoid infiltration." The<br />

diagnosis <strong>of</strong> “true” medullary carcinomas requires strict application <strong>of</strong> diagnostic<br />

criteria; and, when done properly, some report that it constitutes ~5% <strong>of</strong> breast<br />

carcinomas (2-5). Like others, I believe those tumors with atypical features are best<br />

considered within the group <strong>of</strong> InvDC,NST (until additional studies indicate<br />

otherwise)(6,7) or invasvie ductal carcinoma with medullary features (42).<br />

Successful treatment by lumpectomy and primary radiotherapy has been reported (8),<br />

and many (but not all) studies indicate an improved prognosis over InvDC,NST, when<br />

compared stage for stage with InvDC,NST (2-4,9-12). For example, Moore and Foote<br />

(9) reported that 11.5% <strong>of</strong> their patients with medullary carcinoma died <strong>of</strong> tumor<br />

within five years, despite the fact that 42% <strong>of</strong> patients had axillary node metastases.<br />

Richardson (4) reported that the 5-year disease-free survival <strong>of</strong> their 99 patients was<br />

78%, with death due to disease in only 10%. There was 95% 20-year disease free<br />

survival for stage I patients in this series and 61% for stage II patients (2). Patients<br />

with medullary carcinoma tend to have a lower frequency <strong>of</strong> axillary node metastases<br />

than patients with atypical medullary or InvDC,NST (3,11,10); and, when present,<br />

nodal metastases are usually found in three or fewer nodes (3,10). Stage II medullary<br />

carcinoma patients have a more favorable prognosis than comparable patients with<br />

non-medullary carcinoma. But, patients with tumors larger than 3 cm or with four or<br />

more positive nodes have high recurrence rates that are not appreciably different from<br />

the recurrence rates <strong>of</strong> patients with infiltrating duct carcinoma.<br />

Ellis et al. (13) and Periera et al. (14) do not report a significantly different survival<br />

for all patients with medullary carcinoma, compared to patients with InvDC,NST (i.e.,<br />

51% vs. 46%, respectively, at 10 years follow up). They emphasize that this holds<br />

even when strict criteria are applied for the diagnosis. However, review <strong>of</strong> their data<br />

show that the 10-year survival for mBR grade 3 InvDC,NST is 39%, whereas it is<br />

51% for medullary carcinoma. All medullary carcinomas were mBR grade 3 in their<br />

series. Possibly, this group will accept some improved survival for patients with<br />

108

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