Adenoid Cystic Carcinoma of the Breast Rosemary Leeming, MD; Mark Jenkins, MD; Geoffrey Mendelsohn, \s=b\ Adenoid



cystic carcinoma of the breast is a rare neoonly 140 cases having been reported to date.

Data on 123 of these cases are reviewed herein and another case is presented in detail. Several features distinguish this

type of breast cancer from more typical histologic types and suggest that it may have a unique tumor behavior. The

prognosis appears to be favorable and the incidence of axillary lymph node involvement is lower. Distant metastases are uncommon, but they tend to occur without prior lymph node involvement. This lack of prognostic significance for negative axillary lymph nodes underscores the need for other prognostic markers in this disease and suggests that axillary dissection can be eliminated in most cases. Similarities to typical breast cancer include the incidence of local recurrence and the lack of effect of surgical treatment on distant metastases and overall survival. These data suggest that breast-conserving treatment may be applicable to adenoid cystic carcinoma.

(Arch Surg. 1992;127:233-235)

carcinoma of the breast is a rare neo¬ for fewer than 1% of all breast carcinomas. Approximately 140 cases of adenoid cystic carcinoma of the breast have been reported in the litera¬ ture to date.1"21 Since the prognosis for this type of tumor is favorable and the clinical behavior unusual, the impor¬ tance of correctly diagnosing adenoid cystic carcinoma of the breast is clinically relevant. A review of our medical records revealed three cases of adenoid cystic carcinoma of the breast. This report de¬ scribes our most recent case in detail. The literature is re¬ viewed and recommendations are made regarding prog¬ nosis and treatment.

cystic Adenoid plasm, accounting

REPORT OF A CASE A 47-year-old premenopausal woman was referred to her sur¬ geon for evaluation of abnormal mammography findings of the right breast. Her medical history included morbid obesity, insulin-dependent diabetes mellitus, and hypertension. She be¬ gan menarche at age 12 years and had never been pregnant. The mammogram showed a poorly delineated, irregularly margin-

Accepted From the



for publication September 7, 1991. Departments of Surgery (Drs Leeming and Jenkins) and

(Dr Mendelsohn), The Mount Sinai Medical Center, Ohio.

Reprint requests to the Department of Surgery, The Mount Sinai

Medical Center, One Mount Sinai Dr, Cleveland, OH 44106 (Dr



ated, nodular density located interiorly along the midline of the

right breast.

finding was new, having been absent on a performed 17 months earlier. Physical examina¬ tion revealed no masses or changes in skin or nipple, and there was no abnormal adenopathy. The patient underwent a lumpectomy of the right breast after This


needle localization. The segment of breast tissue removed mea¬ sured 5x3x2 cm and contained a firm, gritty, irregular, graytan mass measuring 1.5 cm in greatest dimension. Intraoperative cytologie and frozen-section analysis suggested the possibility of adenoid cystic carcinoma; this diagnosis was confirmed by per¬ manent histologie section. One week later, a right axillary dissection was performed, and 13 lymph nodes were negative for tumor. The patient did well after surgery except for a minor wound infection. She received adjuvant radiation therapy to the right breast in daily fractions of 2 Gy over 5 weeks for a total of 50 Gy. She received no other adjuvant therapy and was well with no evidence of disease 12 months later. The tumor consisted of variably sized ovoid, stellate, and trabecular nests of cells in a rather fibrous stroma (Fig 1). The tu¬ mor cells were compact and polyhedral to slightly fusiform. A focally prominent cribriform or cylindromatous pattern was present and many of the cribriform spaces contained an amor¬ phous, eosinophilic, periodic acid-Schiff-positive material typ¬ ical of adenoid cystic carcinoma (Fig 2). Immunocytochemical studies were positive for S100 protein and weakly positive for cytokeratin. Carcinoembryonic antigen and estrogen receptor stains



COMMENT Adenoid cystic carcinoma of the breast is a rare neoplasm. The most extensive review was reported by Qizilbash et al14 in 1977. They found 95 cases in the literature and described an additional two patients. Peters and Wolff13 reported an additional 11 cases and Ro et al15 reviewed the tumor regis¬ try at the University of Texas M. D. Anderson Cancer Cen¬ ter and found another 12 cases. Several other reports of sin¬ gle cases and smaller series bring the total number of cases of adenoid cystic carcinoma of the breast reported in the lit¬ erature to 140.7'91618'21 Data on 123 of these patients were available for analysis, and together with the patient de¬ scribed above form the basis of this review. The most fre¬ quent presenting symptom of adenoid cystic carcinoma of the breast is a dominant mass, which is occasionally tender to palpation.1'2-510 It is uncommon for the tumor to be fixed to the overlying skin or nipple or to the pectoral muscles. The tumors are usually grossly circumscribed but typically ex¬ hibit microscopic invasion into the surrounding tissue.16 The histologie appearance of adenoid cystic carcinoma in the breast is similar to that occurring in other tissues, such as salivary glands, upper respiratory tract, and

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Fig 1. Histologie appearance of adenoid cystic carcinoma of the breast. Note the ovoid, trabecular, and irregularly shaped nests of tumor cells with a focally cribriform pattern.

Fig 2. —Nests of tumor in a fibrous stroma. The tumor cells are poly¬ hedral to slightly fusiform with rather hyperchromatic nuclei. Note the presence of variably sized cribriform spaces, many of which contain amorphous eosinophilic material (arrows).

vulva. The cell of origin is obscure, with evidence of both ductal epithelial and myoepithelial cell derivation. Zaloudek et al21 have suggested an origin from a pluripotent cell capable of both lines of differentiation. Positive stain¬ ing for S100 protein, negative staining for carcinoembry¬ onic antigen, and only focal, very weak staining for cytokeratin in our case indicate predominantly myoepi¬ thelial differentiation. Other breast carcinomas can be confused histopathologically with adenoid cystic carcinoma of the breast. This is especially true of cribriform intraductal carcinoma and invasive ductal carcinoma with a cribriform pattern.15,16 In cases that have been evaluated for estrogen receptor sta¬ tus, most tumors have been negative. Only one case of estrogen receptor positivity has been reported, to our knowledge.22 Estrogen receptors were negative in the pa¬ tient described in this report. The significance of this fea¬ ture of adenoid cystic carcinoma is unknown. A review of the literature suggests an excellent prog¬ nosis for patients with adenoid cystic carcinoma of the breast. While overall survival is difficult to determine, most patients seem to do well and only 10 cases of met¬ astatic disease have been documented.4'9,n"13'15'17-18 While the reason for this favorable prognosis is unclear, it may be that certain biologic characteristics of adenoid cystic carcinoma are different from those of more common his¬ tologie types of breast cancer (ie, typical breast cancer). In addition to the better prognosis, there is a much lower incidence of axillary lymph node metastasis, with only three cases reported in the literature.1518,20 One of these cases20 was not confirmed histologically. Investigators have shown that patients with breast cancer with clini¬ cally negative axillary lymph nodes have approximately a 40% incidence of histologically proven axillary mé¬ tastases.23 This is considerably higher than the 2.4% inci¬ dence of lymph node métastases reported in patients with adenoid cystic carcinoma of the breast. As with all types of breast cancer, treatment options for patients with adenoid cystic carcinoma of the breast have varied. Since adenoid cystic carcinoma is such a rare neo¬ plasm, it will be impossible to prospectively compare


options. Furthermore, one must be wary about ex¬ trapolating data from large prospective trials made up primarily of patients with typical breast cancer to patients with adenoid cystic carcinoma, which may behave differ¬ ently. From this retrospective review, however, certain

observations can be made. First, it is clear that there is much less prognostic significance of negative axillary lymph node métastases in these patients. Eight (80%) of 10 patients found to have distant metastatic disease in this study had no axillary lymph node involvement. A third case of axillary mé¬ tastases and distant metastatic disease has been reported, but it is not considered to be a case of true adenoid cystic carcinoma.17 It seems apparent that we must find other prognostic determinants to use in this disease. It has been suggested that the histologie grade of the tumor, based on the system used for adenoid cystic carcinoma of the sal¬ ivary glands, is prognostic and should be used to guide treatment options.15 Unfortunately, this observation is based on a small number of cases and its accuracy cannot be fully evaluated. Certainly, nuclear grade is known to be an important prognostic determinant in typical breast cancer,24 and both these features should be evaluated fur¬ ther in these patients. A second observation from this analysis pertains to the incidence of tumor recurrence following excision alone. Nine (37.5% ) of 24 patients undergoing excision alone had a local recurrence and this incidence is similar to that re¬ ported by Fisher et al25 in patients with typical breast can¬ cer undergoing lumpectomy alone. Two additional pa¬ tients were described by Zaloudek et al21 as having local recurrence; on further review of the original report,3 this cannot be confirmed. Only two of these patients devel¬ oped distant métastases15 and one was found later to have axillary lymph node métastases.20 Thus, the incidence of distant métastases following local excision of adenoid cystic carcinoma was 8.3%, which is similar to that following mastectomy (8.1%). These findings suggest that local treatment has little impact on the development of metastatic disease or overall survival. Again, this is con¬ sistent with prospective data previously published by

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Fisher et al25 and supports the alternate theory of breast cancer biology rather than the halstedian theory.26 Adenoid cystic carcinoma of the breast is a rare neo¬ plasm with certain histopathologic and biologic charac¬ teristics that distinguish it from more common forms of breast cancer. WTiile prospective data do not exist to sup¬ port one treatment option over another for this disease, it is similar to typical breast cancer both in terms of local recurrence as well as in the lack of effect of local treatment on the development of distant disease. These data suggest that breast-conserving treatments may be applicable. It remains to be seen whether postoperative radiation ther¬ apy can reduce the incidence of local recurrence in this subtype of breast cancer as it does for more common his¬ tologie types; retrospective data do not suggest why it would not be as effective. Since the information gained from axillary lymph node dissection is less valuable in these patients and the inci¬ dence of axillary métastases is so low, it may be appropri¬ ate to eliminate this part of the staging procedure unless clinically suspicious lymph nodes are present. Finally, it is imperative that newer prognostic determinants be evaluated in these patients since, at present, there does not appear to be a way of predicting patients who may benefit from systemic adjuvant therapy. References 1. Anthony PP, James PD. Adenoid cystic carcinoma of the breast: prevalence, diagnostic criteria, and histogenesis. J Clin Pathol. 1975;28:647-655. 2. Cavanzo FJ, Taylor HB. Adenoid cystic carcinoma of the breast: an analysis of 21 cases. Cancer. 1969;24:740-745. 3. Cammoun H, Contesso G, Rouesse J. Les adenocarcinomes cylindromateux du sein. Ann Anat Pathol Paris. 1972; 17:

143-154. 4. Elsner B. Adenoid

cystic carcinoma of the breast: review of the literature and clinico-pathologic study of seven patients.

Pathol Eur (Bruxelles). 1970;5:357-364. 5. Friedman BA, Oberman HA. Adenoid cystic carcinoma of the breast. Am J Clin Pathol. 1970;54:1-14. 6. Groshong LE. Adenocystic carcinoma of the breast. Arch

Surg. 1966;92:424-427. 7. Hjorth S, Magnusson PH, Blomquist

P. Adenoid cystic carcinoma of the breast: report of a case in a male and review of the literature. Acta Chir Scand. 1977;143:155-158. 8. Koss LG, Brannan CD, Ashikari R. Histologic and ultrastructural features of adenoid cystic carcinoma of the breast. Cancer. 1970;26:1271-1279. 9. Lim SK, Kovi J, Warner OG. Adenoid cystic carcinoma of the breast with metastasis: a case report and review of the lit-

J Natl Med Assoc. 1979;71:329-330. 10. Lusted D. Structural and growth patterns of adenoid cystic carcinoma of the breast. AmJ Clin Pathol. 1970;54:419-425. 11. Nayer HR. Cylindroma of the breast with pulmonary metastases. Dis Chest. 1957;31:324-327. 12. O'Kell RT. Adenoid cystic carcinoma of the breast. Mo erature.

Med. 1964;61:855-858. 13. Peters GN, Wolff M. Adenoid cystic carcinoma of the breast: report of 11 new cases: review of the literature and discussion of biological behavior. Cancer. 1982;52:680-686. 14. Qizilbash AH, Patterson MC, Oliveira KF. Adenoid cystic carcinoma of the breast: light and electron microscopy and a brief review of the literature. Arch Pathol Lab Med.

1977;101:302-306. 15. Ro JY, Silva EG, Gallager HS. Adenoid cystic carcinoma of the breast. Hum Pathol. 1987;18:1276-1281. 16. Sumpio BE, Jennings TA, Sullivan PD, Merino MJ. Ade-

noid cystic carcinoma of the breast: data from the Connecticut Tumor Registry and a review of the literature. Ann Surg.

1987;205:295-301. 17. Verani RR, van der Bel-Kahn J. Mammary adenoid cystic carcinoma with unusual features. Am J Clin Pathol. 1973;59:653\x=req-\ 658. 18. Wells noma

CA, Nicoll S, Ferguson DJP. Adenoid cystic carciof the breast: a case with axillary lymph node metastasis.

Histopathology. 1986;10:415. 19. Wilson WB, Spell JP. Adenoid cystic carcinoma of breast: a case with recurrence and regional metastasis. Ann Surg.

1967;166:861-864. 20. Woyke S, Domagala W, Olszewski W. Fine structure of mammary adenoid cystic carcinoma. Pol Med J. 1970;9:1140\x=req-\


21. Zaloudek C, Oertel YC, Orenstein JM. Adenoid cystic carcinoma of the breast. Am J Clin Pathol. 1984;81:297-307. 22. Kern WH. Morphologic and clinical aspects of estrogen receptors in carcinoma of the breast. Surg Gynecol Obstet.

1979;148:240-242. 23. Fisher B, Redmond C, Fisher ER,

et al. Ten-year results of randomized clinical trial comparing radical mastectomy and total mastectomy with or without radiation. N Engl J Med. a

1985;312:674-681. 24. Fisher B, Redmond C, Fisher ER, et al. Relative worth of estrogen or progesterone receptor and pathologic characteristics of differentiation as indicators of prognosis in node negative breast cancer patients: findings from National Surgical Adjuvant Breast and Bowel Project Protocol B-06. J Clin Oncol.

1988;6:1076-1087. 25. Fisher B, Redmond C, Poisson R,

et al. Eight-year results of a randomized clinical trial comparing total mastectomy and lumpectomy with or without irradiation in the treatment of breast cancer. N Engl J Med. 1989;320:822-828. 26. Osbourne MP. The biologic basis for breast cancer treatment options. Am Coll Surg Bull. 1986;71:4-13.

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Adenoid cystic carcinoma of the breast.

Adenoid cystic carcinoma of the breast is a rare neoplasm, with only 140 cases having been reported to date. Data on 123 of these cases are reviewed h...
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