CASE 17850 Published on 17.08.2022
0

Rare case of breast adenoid cystic carcinoma: radio-pathological correlation

Section

Breast imaging

Case Type

Clinical Cases

Authors

Chua Sook Yin1, Choong Kah Lai1, Lee Sen Lin2

1. Radiology Department, Shah Alam Hospital, Malaysia

2. Pathology Department, Tengku Ampuan Rahimah Hospital, Malaysia

Connected authors
Sook Yin Chua Malaysia
Patient

61 years, female

Categories
Area of Interest Breast ; Imaging Technique Mammography, Ultrasound
Download as PDF Print Show related cases Notify admin
Clinical History

A 61-year-old lady presented with right breast lump for two years, associated with worsening pain for two months. The lump was not increasing in size. No constitutional symptoms. Family history (cousin) of breast malignancy noted. On palpation, tender swelling at right breast 9 o’clock region noted, no skin changes.

Imaging Findings

The first mammogram showed a well-defined nodular density at right breast periareolar region. Latest mammogram done this year shows no interval change in size and appearance. No suspicious grouped calcification or architectural distortion. No axillary lymphadenopathy.

Breast ultrasound showed well-defined, hypoechoic, lobulated, non-calcified solid lesion at right breast 9 o’clock peri-areolar region measuring 0.6 x 0.6 x 1.1cm. Overall no suspicious imaging feature and the lesion was initially classified as BIRADS 3. Subsequent repeat mammogram and ultrasound 2 years later showed no significant interval change in its size and appearance.

However, because of persistent pain, the lesion was subsequently classified as BIRADS 4a and an ultrasound-guided biopsy was performed. Histopathological examination (HPE) showed epithelial cells mainly of luminal ductal epithelial cells and myoepithelial cells, suggestive of salivary gland tumour favouring adenoid cystic carcinoma (ACC).

Subsequent staging CT of the neck, thorax, abdomen and pelvis confirmed no distant metastasis. Wide local excision(WLE) and axillary clearance was performed. The HPE of the WLE specimen confirmed adenoid cystic carcinoma, no axillary lymph node involvement. Patient completed radiotherapy and is currently well.

Discussion

Background

ACC is a rare tumour of the breast, accounting for less than 1 % of all breast carcinoma [1,2,3]. In contrast to ACC of salivary gland, it has indolent course with lymph node involvement and distant metastases seldom reported [4]. Despite their triple negative status (negative for estrogen (ER), progesterone (PR) receptors and HER-2 neu (c-erb2) , majority of the ACC rarely involve regional lymph nodes and is associated with high survival rate [2, 5].

Clinical perspective

Majority of the patients present at 50 to 64 years old [6] with palpable breast mass, few cases are asymptomatic with the lesion detected on routine screening mammogram[7]. The tumour size varies widely [5]. Distant metastases are uncommon and tend to occur without lymph node involvement [2, 3, 7].  The site of metastases frequently described are lung, followed by liver, kidney and bone [2, 3, 7] .

Definitive diagnosis is usually via ultrasound-guided core needle biopsy or excisional biopsy. Currently there is no consensus on the optimum treatment for ACC of breast. Surgery remains the mainstay of treatment, ranging from local excision to radical mastectomy [2, 3, 7]. Adjunctive radiotherapy is found to improve overall survival [3, 7].

Imaging perspective

The mammographic presentation varies. In one study it was reported as circumscribed lobulated nodule usually in upper quadrant or periareolar regions [6], similar to our case. In another study the tumour presented with lobulated to irregular mass, subtle architectural distortion or asymmetric densities [4]. Only few cases reported to have calcifications.[1,3]

The sonographic appearance is reported as hypoechoic or heterogeneous mass with irregular contours, some with minimal peripheral colour flow [4]. In our patient the lesion presented as well-circumscribed hypoechoic nodule on ultrasound with no interval growth over two years.

Histopathologically three different architectural patterns are described: tubular, cribriform, and solid[6]. In our patient the histopathological examination (HPE) showed epithelial cells arranged in cribriforming, tubular to trabecular patterns. Two populations of cells noted: luminal ductal epithelial cells (positive for CD117, negative for ER, PR and HER2) and myoepithelial cells (positive for CK5/6, p63, SMA).

Outcome

10-year survival rate ranging between 86-95%[2].

Take-home message

Adenoid cystic carcinoma is a rare breast carcinoma with indolent nature and good prognosis. The mammographic and sonographic appearance of adenoid cystic carcinoma of breast is non-specific thus biopsy is recommended for definitive diagnosis.

 

Written informed consent obtained from patient for publication.

Differential Diagnosis List
Adenoid cystic carcinoma of breast
Invasive carcinoma of breast
Fibroadenoma of breast
Final Diagnosis
Adenoid cystic carcinoma of breast
Case information
URL: https://www.eurorad.org/case/17850
DOI: 10.35100/eurorad/case.17850
ISSN: 1563-4086
License

This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

Figure 1

Mammogram images done 2 years ago: Right MLO view (figure 1a) and CC view (figure 1b) demonstrate a well-defined high density
Mammogram images done 2 years ago: Right MLO view (figure 1a) and CC view (figure 1b) demonstrate a well-defined high density nodule at periareolar region.
Mammogram images done 2 years ago: Right MLO view (figure 1a) and CC view (figure 1b) demonstrate a well-defined high density
Mammogram images done 2 years ago: Right MLO view (figure 1a) and CC view (figure 1b) demonstrate a well-defined high density nodule at periareolar region.
Figure 2

Current mammogram images: Right MLO view (figure 2a) and CC view (figure 2b) demonstrate similar size and appearance of high
Current mammogram images: Right MLO view (figure 2a) and CC view (figure 2b) demonstrate similar size and appearance of high density nodule at periareolar region.
Current mammogram images: Right MLO view (figure 2a) and CC view (figure 2b) demonstrate similar size and appearance of high
Current mammogram images: Right MLO view (figure 2a) and CC view (figure 2b) demonstrate similar size and appearance of high density nodule at periareolar region.
Figure 3

Ultrasound right breast (Figure 3a & 3b): Well-circumscribed hypoechoic solid lesion at right breast 9 o’clock periareolar
Ultrasound right breast (Figure 3a & 3b): Well-circumscribed hypoechoic solid lesion at right breast 9 o’clock periareolar region, no significant colour doppler flow within.
Ultrasound right breast (Figure 3a & 3b): Well-circumscribed hypoechoic solid lesion at right breast 9 o’clock periareolar
Ultrasound right breast (Figure 3a & 3b): Well-circumscribed hypoechoic solid lesion at right breast 9 o’clock periareolar region, no significant colour doppler flow within.
Figure 4

HPE of the biopsy specimen H&E x 100 (Figure 3): proliferations of epithelial cells arranged in cribriforming and tubular pat
HPE of the biopsy specimen H&E x 100 (Figure 3): proliferations of epithelial cells arranged in cribriforming and tubular pattern, displaying round spaces within. Subsequent stain results are suggestive of salivary gland type tumour, favouring adenoid cystic carcinoma.