Adenoid cystic carcinoma of the breast: case report and review of literature

We report the case of a patient who presented with a painful breast lump that turned out to be an adenoid cystic carcinoma of the breast. The literature is reviewed, highlighting the good prognosis associated with this rare condition and the current preferred treatment modalities.     

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 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.     

Adenoid cystic carcinoma of the male breast

We present an 82-year-old male patient who presented with complaints of gradually an increasing ulcero-proliferative lesion with persistent mucinous discharge in the left breast. Left side-modified radical mastectomy was done. This was histopathologically diagnosed as an adenoid cystic carcinoma of the left breast. Periodic acid schiff (PAS) staining confirmed the diagnosis. Three of the five axillary lymph nodes excised were positive for malignancy. Although the patient was advised to have postoperative radiotherapy, he did not comply. After 2 years, the patient again presented with local recurrence of the disease. Wide excision of the recurrent malignant nodules over the anterior chest wall was done, and the defect was covered primarily with split thickness skin grafting. Postoperative radiation was given. For the past 9 months, the patient has maintained a regular follow-up on an outpatient basis. He does not have any evidence of recurrence of the tumor--neither locally nor distant metastasis.     

Adenoid cystic carcinoma of the esophagus: Report of a case and review of the Japanese literature

We report herein the case of a 79-year-old man with adenoid cystic carcinoma (ACC) of the esophagus. The tumor had a polypoid appearance and was covered by thin esophageal mucosa. As the biopsy specimens suggested a diagnosis of poorly differentiated adenocarcinoma, the patient underwent subtotal esophagectomy with reconstruction of the gastric tube via the posterior mediastinum. Histologically, the carcinoma contained basaloid cells, cribriform foci, and a certain amount of eosinophilic hyaline substance. Some of the basaloid cells were stained immunohistochemically for keratin, muscle actin, and S-100 protein, a pattern which was identical to the pattern of immunoreactivity of the myoepithelium. We reviewed 36 other cases of ACC of the esophagus reported in Japan, with special reference to the criteria for histological diagnosis.     

Adenoid cystic carcinoma of the breast: Report of a case

Adenoid cystic carcinoma of the breast is an uncommon form of cancer, and only a few articles have described the cytological findings of this disease. We report herein the case of a 48-year-old woman who presented with a breast mass beneath the nipple, the aspirate from which consisted of globules of mucous balls surrounded by epithelial cells with scant cytoplasm and hyperchromatic nuclei. Microscopically, the tumor was formed by myoepithelial cells and glandular epithelial cells in a biphasic pattern. Immunohistochemical study revealed positivity for smooth muscle actin. A left total mastectomy with axillary lymph node dissection was performed. None of the 22 axillary lymph nodes contained metastases, and the patient remains well and free from recurrence 29 months after her operation. This case report provides some information about the cytological diagnosis and the accuracy of fine-needle aspiration, which must be considered despite the rarity of this disease. Received: June 24, 1999 / Accepted: January 7, 2000     

Adenoid cystic carcinoma of the breast in a 19-year-old girl

A case of adenoid cystic carcinoma of the breast in a 19-year-old girl is presented. As this tumor has specific characteristics and diagnostic criteria, this case illustrates the importance of an accurate histological diagnosis.     

Adenoid cystic carcinoma with high-grade transformation: a report of 11 cases and a review of the literature

High-grade transformation of adenoid cystic carcinoma (ACC) (previously referred to as dedifferentiation) is a rare phenomenon that does not fit into the traditional ACC grading schemes. The importance and minimal criteria for distinction from solid (grade III) ACC are not well established. We report 11 new cases and review the literature to further define the profile of this tumor. The median age was 61 years (range: 32 to 72 y) with a male predominance (male to female ratio of 1.75:1). The most commonly involved sites were sinonasal (4/11) and submandibular (4/11). Lymph nodes were pathologically positive in 4/7 (57.1%) cases. Distant metastases to the lung (n=2) and soft tissue of the shoulder (n=1) were observed. Five of 9 patients (55.6%) died, all within 5 years with a median overall survival of 12 months. Histologically, ACC with high-grade transformation was distinguished from conventional ACC by nuclear enlargement and irregularity, higher mitotic counts, and the loss of the biphasic ductal-myoepithelial differentiation. Useful supportive criteria were prominent comedonecrosis and fibrocellular desmoplasia. The most common morphologies for the high-grade component were poorly differentiated cribriform adenocarcinoma and solid undifferentiated carcinoma. Micropapillary and squamoid patterns were occasionally present. Ki-67 and p53 labeling indices were elevated in the high-grade components, though c-kit and cyclin-D1 were not. ACC-high-grade transformation is a highly aggressive salivary gland tumor with a variety of histologic patterns. The high propensity for lymph node metastases suggests a role for neck dissection in patients with this rare tumor.     

The risk of a contralateral breast cancer among women diagnosed with ductal and lobular breast carcinoma in situ: data from the Connecticut Tumor Registry

BACKGROUND: Women diagnosed with breast carcinoma in situ are at increased risk for developing a contralateral breast cancer. The magnitude of this risk and the relationship between this risk and age, time since diagnosis, histologic subtype, and treatment for the first breast cancer is continuing to be defined. METHODS: The risk of developing a contralateral breast cancer is examined among 4198 women diagnosed with breast carcinoma in situ and reported to the Connecticut Tumor Registry (CTR) between January 1, 1975 and March 14, 1998 using Kaplan-Meier estimation. A Cox proportional hazards model is used to assess the effect of surgical treatment, radiation therapy, age at diagnosis, race, histology, marital status, anatomic location within the breast, and time since diagnosis upon this risk. RESULTS: The cumulative 5- and 10-year probabilities of being diagnosed with a contralateral breast cancer among women initially diagnosed with a ductal breast carcinoma in situ (DCIS) were 4.3% (95% confidence interval, 3.6-5.0%) and 6.8% (95% confidence interval, 5.5-8.2%), respectively. These risks are 3.35 times greater than those for women without a history of breast cancer but are similar to those for women diagnosed with non-metastatic invasive ductal carcinomas of the breast. The cumulative 5- and 10-year probabilities of being diagnosed with a contralateral breast cancer among women initially diagnosed with a lobular breast carcinoma in situ (LCIS) were 11.9% (95% confidence interval, 9.5-14.3%) and 13.9% (95% confidence interval, 11.0-16.8%), respectively. CONCLUSIONS: Women diagnosed with LCIS were 2.6 (95% confidence interval, 2.0-3.4%) times more likely than women with DCIS to be diagnosed with a contralateral breast cancer within the first six months of the first breast primary. The risk of developing a contralateral breast cancer more than 6 months after the initial breast cancer was independent of surgical or radiation therapy, time since diagnosis, age at diagnosis, histology, race, marital status, or anatomic location of the cancer within the breast.     

Adenoid cystic carcinoma of the oesophagus.

A case of adenoid cystic carcinoma of the oesophagus occurring in a patient who had previously been treated for craniopharyngioma is presented. This is the thirteenth reported case of adenoid cystic carcinoma of the oesophagus and the first in association with craniopharyngioma. The previously described cases of oesophageal adenoid cystic carcinoma are critically reviewed, and it is suggested that this tumour is in general highly malignant and may not be so rare as is generally thought.     

Adenoid cystic carcinoma of the head and neck.

Adenoid cystic carcinoma (ACC) is a rare malignant tumour believed to arise from the epithelial cells of mucous-secreting glands. It is a slow-growing but aggressive tumour with a propensity for perineural invasion. A 10 year review of 45 patients (19 males and 26 females with a median age of 56 years) treated for ACC at a single institution between 1989 and 1999 was performed to analyse factors involved in treatment failure, local control, treatment-related morbidity and mortality. The data collected were treated for survival curves according to the Kaplan-Meier method and the log-rank test was used to assess the statistical significance of the various groups. The overall survivals at 3 years and 5 years were 71% and 65%, respectively, while the disease-free survivals at 3 years and 5 years were 73% and 63% respectively. Out of the 45 patients, only six had local recurrences, yielding an 87% locoregional freedom from relapse; 16 patients (35.6%) developed distant metastases, with the lung (8/16) being the commonest site. Patients treated for tumours of the nose and paranasal sinuses experienced more morbidity than those with tumours at other sites. Positive margins, perineural invasion and solid histology of ACC were associated with increased morbidity and treatment failure. Patients treated with combined therapy did better than those who underwent a single treatment modality.     

Adenoid cystic carcinoma of Cowper's gland.

We report an additional case of primary adenoid cystic carcinoma of Cowper's gland in an otherwise healthy asymptomatic 66-year-old man. Based on a review of similar lesions presenting in the head and neck, our treatment plan entailed pelvic exenteration followed by radiation therapy. Whether adenoid cystic carcinoma of Cowper's gland behaves as aggressively as it does in the head and neck remains unclear. However, in view of the extensive local invasion in our patient and a previously successful outcome, a combined surgical and radiation approach appears to be the most appropriate therapy.     

Adenoid cystic carcinoma of female breast: A case report

INTRODUCTIONAdenoid cystic carcinoma (ACC) of the breast is a rare neoplasm accounting for 0.1% of breast carcinomas.PRESENTATION OF CASEA 30 year old female patient presented with multiple lumps in the right breast. Excision biopsy of a lump in the lower outer quadrant revealed adenoid cystic carcinoma. A right sided modified radical mastectomy (MRM) was performed, with axillary clearance. Histopathology revealed a fibroadenoma accounting for one mass and adenoid cystic carcinoma in another, confirmed on Periodic acid Schiff (PAS) staining. Three out of the eight excised axillary lymph nodes contained malignancy. There was no evidence of recurrence or distant spread at follow-up 6 months postoperatively.DISCUSSIONACC of the breast is a rare neoplasm accounting for 0.1% of breast carcinomas and is of special interest because of its favourable prognosis and distinctive histological appearance. This tumor occurs predominantly in women in their sixth decade and usually presents as a tender breast mass, often in the subareolar area though in our case it was not tender and presented as multiple lumps in the right breast. The diagnosis can be made on fine needle aspiration cytology (FNAC), but in our case FNAC was inconclusive. We performed MRM with axillary clearance, and three out eight lymph nodes proved positive for malignancy.CONCLUSIONACC is a rare malignant neoplasm. Surgical treatment is the mainstay with little role for radiotherapy and chemotherapy according to the published literature.