Melhem Solh, First-Year Resident, Department of General Surgery; Mubashir Sabir, Chief Resident, Department of General Surgery; Linda Dubay, Associate Professor, Department of General Surgery; Farooq Minhas, Clinical Pathologist, Department of Pathology, Providence Hospital and Clinics, Southfield, MI
Melhem Solh, MD
Department of General Surgery
Mubashir Sabir, MD
Department of General Surgery
Linda Dubay, MD
Department of General Surgery
Farooq Minhas, MD
Department of Pathology
Providence Hospital and Clinics
Adenocystic carcinoma accounts for fewer than 1% of all primary carcinomas of the breast.1 Most of the knowledge regarding these tumors is derived from case reports or retrospective case series. Adenoid cystic carcinoma of the breast is mainly diagnosed based on morphologic features.2 It is considered a benign disease, and its treatment is still a topic of debate. We report a case of adenocystic carcinoma of the breast in a patient who was referred to a community-based hospital and review the literature regarding this rare entity.
A 65-year-old woman (gravida 5, para 3) presented with a painful lump in her left breast, which she had discovered by self-examination a few weeks earlier. She had observed no change in the size of the recently palpated lump. The patient had no history of taking hormone replacement therapy or of breast disease, breast discoloration, or breast discharge. She had a medical history of hypertension, which was controlled with amlodipine. Her surgical history included cervical fusion and total abdominal hysterectomy with bilateral oophorectomy. Her only significant family history was throat cancer, which afflicted her father at age 58. The patient was a nonsmoker and only drank alcohol socially. Physical examination revealed a hard, mobile, 3-cm lump located in the left breast at the 5 to 6 o'clock position. The rest of the breast examination was normal. No axillary or supraclavicular lymph nodes were palpable on either side.
At this time, ultrasonography of the breast showed a 2.5-cm mass, solid and noncystic, located at the 6 o'clock position on the left breast, which was consistent with our physical assessment. Op?tions were discussed with the patient, and the decision to do an excisional bi?opsy of the left breast mass was made. The patient underwent this procedure a month later. Frozen section at the time of the operation was read as suspicious of malignancy pending the permanent path?o?logy report. Pathologic slides were sent to New York Presbyterian Hospital for consultation. The result was an adenocystic carcinoma, predominantly low-grade, with a high-grade component secondary to the foci of near solid architecture in the tumor (Figure). The specimen ob?tained was 6.7 x 6.5 x 3.0 cm, with the hard mass measuring 2.7 x 2.5 x 1.4 cm. Adeno?cystic carcinoma was present within 0.1 cm of the posterior resection margin. The tumor staining for HER2/ was classified as 1 (faint/barely perceptible membrane staining in >10% of tumor cells). The tumor was focally positive for estrogen and progesterone receptors. After an extensive literature review, and because of the close resection margins, the decision was made to do a lumpectomy without lymph node dissection. The patient underwent breast-conserving surgery (lumpectomy), with wide surgical margins reaching a small portion of the pectoralis major muscle in order to achieve a good posterior margin around the cancer. The permanent pathology report confirmed the results of the fine-needle aspiration and described the tumor as 5.5 cm with fibrocystic changes, patchy moderate ductal epithelial hyperplasia, fibro?adenoma, and adenosis, with no residual tumor focus.
Adenocystic carcinoma is a rare form of invasive breast carcinoma and ac?counts for fewer than 1% of all primary carcinomas of the breast.1 A report by Arpino and colleagues identified 28 cases of adenocystic breast cancer among 50,000 patients with breast mali?g?nancies.3 A report by Sumpio and colleagues identified 37 cases of adenocystic cancer out of 40,350 invasive breast tumors.4 Ad?eno?cystic carcinoma of the breast usually presents in postmeno?pausal women during the sixth and seventh decades of life.2-5 There are also re?ports of adenocystic carcinoma of the breast in men.2,6
The most frequent clinical presentation of this neoplasm is a palpable mass in the breast, usually detected by self-examination.2,5 The mean tumor size in Arpino and colleagues' review was 1.9 cm (range, 0.5 cm?7.0 cm),3 which is com?parable with the 20-mm size reported by McClenathan and de la Roza in patients from the Northern California Cancer Registry.5 A subareolar location was re?ported in 50% of cases (10 of 20) evaluated at the University of Michigan Medical Center.2 Adenocystic carcinoma has the same incidence in both breasts.2,3
Adenocystic carcinoma of the breast is mainly diagnosed by its morphologic features. The overall histologic pattern is analogous to that of adenocystic carci-nomas in other locations.2 Adenocystic carcinoma is characterized by the presence of cribriform structures that are formed by multiple pseudocysts filled with amorphous substance. Clusters of small, monomorphic, cubic cells with round or oval nuclei form abundant islets with a characteristic sieve-pattern. Myo?epithelial cells are frequently detected in the tumor.7-10 Diagnosis of the tumor should be based on strict criteria, because it carries a very good prognosis; moreover, adenocystic cancer requires special treatment. Estrogen receptor positivity has been assessed in several reports and was found to range between 24% and 46%.2,3 Progesterone receptors were de?tected in 35% of cases in one series of 28 patients.3 The grading of adenocystic carcinoma of the breast is similar to that of adenocystic carcinomas in other locations: grade I is completely glandular, grade II has fewer than 30% solid areas, and grade III has a more than 30% solid pattern. Shin and Rosen reported a correlation between histologic grade and prognosis11; however, an earlier report from the University of Michigan showed no such correlation.2 In Shin and Rosen's study, the solid variant with basaloid features was more capable of axillary metastasis than the conventional adenoid cystic carcinoma of the breast.11
Adenoid cystic carcinoma of the breast is considered a benign disease, and its treatment is still an area of debate. Treatment includes lumpectomy, modified radical mastectomy, or total mastectomy with or without axillary lymph node dissection.1-3,11,12 Several reports show that the incidence of axillary lymph node metastasis is almost nil (0%?5%),2,3 and the value of axillary lymph node sampling is not as great as it is for invasive ductal carcinoma of the breast. Despite the minimal incidence of axillary lymph node metastasis, adenoid cystic carcinoma of the breast can result in distant metastasis.13
The outcome of treatment for this tumor is excellent, with a 5-year overall survival rate of 85% to 90% and a 5-year disease-free rate of almost 100% for survivors.2,3 The prognosis was independent of histologic grade, nuclear grade, or proliferative activity. The outcome was independent of the type of procedure performed, whether lumpectomy, modified radical mastectomy, or total mastectomy with or without axillary lymph node dissection.2,3,12 The role of radiotherapy after breast conserving surgery needs further evaluation, with long-term follow-up.
Adenoid cystic carcinoma of the breast is a rare finding. This tumor has a favorable prognosis, and treatment should consist of lumpectomy with wide margins, avoiding the need for axillary node dissection.
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