33 Special Types of Invasive Breast Carcinoma: Diagnostic Criteria ...
33 Special Types of Invasive Breast Carcinoma: Diagnostic Criteria ... 33 Special Types of Invasive Breast Carcinoma: Diagnostic Criteria ...
at least some lesions developing from these ducts should show both myoepithelial and duct luminal epithelial-cell differentiation. The most commonly encountered benign myoepithelial lesion of the breast is sclerosing adenosis, and its variants, but myoepithelial features are also expected in sclerosing papillary lesions, which have protean histologic presentations and monikers (e.g., radial scar, complex sclerosing lesion, indurative mastopathy, etc.). True neoplasms expected to show myoepithelial differentiation are many and the list continues to grow. Included in this latter category are adenomyoepithelioma and variants, salivary gland-like tumors primary in the breast, metaplastic breast carcinoma (a.k.a., “myoepithelial carcinoma”), adenoid cystic carcinoma, low-grade adenosquamous carcinoma, and breast carcinoma, “basalcell” variant, the latter of which is thought to show, at least in some cases, myoepithelial-like differentiation. Clinicopahological features: Of the malignant breast tumor showing myoepithelial differentiation adenoid cystic carcinoma deserves extensive discussion. Adenoid cystic carcinoma of the breast closely resembles adenoid cystic carcinoma of salivary gland origin, but it is much rarer in the breast, accounting for only ~ 0.1% of all breast carcinomas (1-6). Electron microscopic studies have revealed the same diverse cell types in mammary adenoid cystic carcinoma that are encountered in adenoid cystic carcinoma arising in the salivary glands. This likely reflects the common ectodermal “sweat gland” origin of both breast and salivary gland; and, it seems that there should be even more overlap in the patterns of tumors arising in both locations, but this is not usually the case. Other salivary gland-like tumors arising within the breast are very uncommon. Indeed, breast glands and salivary glands are tubulo-acinar exocrine glands that can manifest as tumours with similar morphological features, but that differ in incidence and clinical behavior depending on whether they are primary in breast or salivary glands. Salivary gland-like tumours of the breast are of two types: tumours with myoepithelial differentiation and those devoid of myoepithelial differentiation. The first and more numerous group comprises a spectrum of lesions ranging from "bona fide" benign, such as benign adenomyoepithelioma and pleomorphic adenoma, to low grade malignant, such as adenoid cystic carcinoma, low grade adenosquamous carcinoma, and adenomyoepithelioma, to high grade malignant lesions such as metaplastic breast carcinoma (a.k.a., “malignant myoepithelioma”). A second group comprises lesions that have only recently been recognized, such as acinic-cell carcinoma, oncocytic carcinoma of the breast, and the rare mucoepidermoid carcinoma (7). 69
Adenoid cystic carcinoma (AdCC) is clearly a tumour with adenomyoepithelial differentiation and characterized by the presence of a dual population of basaloid and luminal cells arranged in specific growth patterns. These adenomyoepithelial features are unscored by Van Dorpe and coworkers who reported a case of adenoid cystic carcinoma arising in a tubular adenomyoepithelioma (8). AdCCs, regardless of the anatomical site, are characterized by expression of the protooncogene and therapeutic target c-KIT, and seem to harbor a specific chromosomal translocation t(6;9) leading to the fusion gene MYB-NFIB and overexpression of the oncogene MYB. However, as already noted the clinical behavior of salivary gland and breast AdCC differs; while salivary gland lesions have a relatively high proclivity to metastasize, patients with breast AdCCs have an excellent outcome (9). Mastectomy has been curative in the vast majority of cases (1, 3-6, 10-14); but, chest wall recurrence has been reported after simple mastectomy (14). Moreover, there can be isolated systemic metastases, which occur in ~10% of cases (2, 10, 15-18). This contrasts with a ~43% distant metastasis rate for salivary gland adenoid cystic carcinoma (18). In a review of ~100 cases of adenoid cystic carcinoma of the breast, there were only 12 with distant metastases. Pulmonary metastases are by far the most common site, and metastases may be detected 6 to 12 years (10, 15, 16, 19) after finding the primary breast tumor. Other metastatic sites include bone, liver, kidney, brain, thigh, pleura, mediastinal lymph node, supraclavicular lymph node, and inferior vena cava (2, 20). Many patients with systemic metastases will have negative axillary lymph nodes, but axillary metastases may occur (1, 17, 21). In fact, only three cases of axillary lymph nodal metastases had occurred in ~100 cases reviewed (2). Those with axillary metastases usually develop pulmonary metastases, and two such cases were considered to have died of metastatic mammary adenoid cystic carcinoma, but the diagnosis was not well established in one of the cases (17). This metastatic pattern clearly suggests that hematogenous spread is most common and that the clinical course is very slow with symptoms developing years after primary diagnosis. Moreover, surgical resection of these metachronous metastases has been successful in maintaining disease control (2, 18). Adenoid cystic carcinoma occurs in adult women of the same age group as for mammary carcinoma (i.e., mean ages 50 to 63 years; range 25 to 80 years of age) (2, 3, 5, 6, 10-12, 22). Adenoid cystic carcinoma usually presents as discrete, firm masses. Uncommonly, they are detected by mammography (12). They can present “acutely” but some have been present for 10 years or more (11). Most are hormone receptor negative (12, 22, 23). Sizes vary from 0.2 to 12 cm with most between 1 and 70
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- Page 255: Am J Clin Pathol 1979;72:383-389. 1
at least some lesions developing from these ducts should show both myoepithelial and<br />
duct luminal epithelial-cell differentiation. The most commonly encountered benign<br />
myoepithelial lesion <strong>of</strong> the breast is sclerosing adenosis, and its variants, but<br />
myoepithelial features are also expected in sclerosing papillary lesions, which have<br />
protean histologic presentations and monikers (e.g., radial scar, complex sclerosing<br />
lesion, indurative mastopathy, etc.). True neoplasms expected to show myoepithelial<br />
differentiation are many and the list continues to grow. Included in this latter category<br />
are adenomyoepithelioma and variants, salivary gland-like tumors primary in the<br />
breast, metaplastic breast carcinoma (a.k.a., “myoepithelial carcinoma”), adenoid<br />
cystic carcinoma, low-grade adenosquamous carcinoma, and breast carcinoma, “basalcell”<br />
variant, the latter <strong>of</strong> which is thought to show, at least in some cases,<br />
myoepithelial-like differentiation.<br />
Clinicopahological features: Of the malignant breast tumor showing myoepithelial<br />
differentiation adenoid cystic carcinoma deserves extensive discussion. Adenoid<br />
cystic carcinoma <strong>of</strong> the breast closely resembles adenoid cystic carcinoma <strong>of</strong> salivary<br />
gland origin, but it is much rarer in the breast, accounting for only ~ 0.1% <strong>of</strong> all breast<br />
carcinomas (1-6). Electron microscopic studies have revealed the same diverse cell<br />
types in mammary adenoid cystic carcinoma that are encountered in adenoid cystic<br />
carcinoma arising in the salivary glands. This likely reflects the common ectodermal<br />
“sweat gland” origin <strong>of</strong> both breast and salivary gland; and, it seems that there should<br />
be even more overlap in the patterns <strong>of</strong> tumors arising in both locations, but this is not<br />
usually the case. Other salivary gland-like tumors arising within the breast are very<br />
uncommon.<br />
Indeed, breast glands and salivary glands are tubulo-acinar exocrine glands that can<br />
manifest as tumours with similar morphological features, but that differ in incidence<br />
and clinical behavior depending on whether they are primary in breast or salivary<br />
glands. Salivary gland-like tumours <strong>of</strong> the breast are <strong>of</strong> two types: tumours with<br />
myoepithelial differentiation and those devoid <strong>of</strong> myoepithelial differentiation. The<br />
first and more numerous group comprises a spectrum <strong>of</strong> lesions ranging from "bona<br />
fide" benign, such as benign adenomyoepithelioma and pleomorphic adenoma, to low<br />
grade malignant, such as adenoid cystic carcinoma, low grade adenosquamous<br />
carcinoma, and adenomyoepithelioma, to high grade malignant lesions such as<br />
metaplastic breast carcinoma (a.k.a., “malignant myoepithelioma”). A second group<br />
comprises lesions that have only recently been recognized, such as acinic-cell<br />
carcinoma, oncocytic carcinoma <strong>of</strong> the breast, and the rare mucoepidermoid<br />
carcinoma (7).<br />
69
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