Case Reports in Pathology

Case Reports in Pathology / 2014 / Article

Case Report | Open Access

Volume 2014 |Article ID 982432 |

Sepideh Mokhtari, Saede Atarbashi Moghadam, Abbas Mirafsharieh, "Sclerosing Polycystic Adenosis of the Retromolar Pad Area: A Case Report", Case Reports in Pathology, vol. 2014, Article ID 982432, 4 pages, 2014.

Sclerosing Polycystic Adenosis of the Retromolar Pad Area: A Case Report

Academic Editor: E. Miele
Received20 Sep 2013
Accepted11 Oct 2013
Published03 Mar 2014


Sclerosing polycystic adenosis is a rare pathological lesion that affects salivary glands. The majority of cases involve the parotid and its occurrence in minor glands is exceedingly rare. Here, we report the first case of this lesion in the retromolar pad area and discuss its histological features and immunohistochemical reactivity with αSMA and Ki67 markers. A review of the literature on its immunohistochemical profile is also provided. Sclerosing polycystic adenosis has a diverse histomorphology and should be differentiated from other more important pathologic lesions.

1. Introduction

To the best of our knowledge, 54 cases of sclerosing polycystic adenosis (SPA) of salivary glands have been reported. SPA characteristically arises in the major glands, and the majority of cases involve the parotid [1]. Some cases have also been reported in minor salivary glands of mucobuccal fold, hard palate, floor of mouth, and buccal mucosa [2, 3]. SPA has been reported in a wide age range from childhood to the eighth decade of life [1]. Here, we report the first case of SPA in the retromolar pad area.

2. Case Report

A 60-year-old male presented with swelling in his retromolar pad area with two months’ duration. There was no tenderness or ulceration. Excisional biopsy of the lesion was performed and a well-circumscribed soft tissue lesion was excised. Histopathologic examination showed lobules of hyalinized connective tissue with epithelial components of ductal and acinar differentiation. Ductal structures formed variably sized cysts or they were packed as small ducts similar to the sclerosing adenosis of the breast. Ducts were lined by flattened to cuboidal epithelial cells and some cells had apocrine metaplasia. Mucous cells were frequently seen (Figures 1, 2, and 3). Periductal fibrosis with lamellar architecture was a common feature. Occasional hyaline globules were also present. Epithelial hyperplasia of ductal structures, formed solid nests, cribriform structures and intraductal anastomosing bridges. Few chronic inflammatory cells were infiltrated throughout the lesion.

Immunohistochemical staining for αSMA and Ki-67 was performed. Myoepithelial cells, surrounding ductal elements, demonstrated immunoreactivity for αSMA (Figure 4). Immunohistochemical examination with Ki-67 revealed less than 1% positivity in lesional cells (Figure 5). The proliferative cells were present within ductal elements of cribriform structures, which explained transluminal duct hyperplasia.

3. Discussion

There is a controversy whether SPA is a neoplasm or reactive lesion. Clonal nature of cells has been demonstrated in some cases [4]. Some viruses such as human papilloma virus (HPV) and Epstein-Barr virus (EBV) may also have a role in pathogenesis of salivary gland diseases. One recent study has demonstrated EBV expression in tumor cells supporting the neoplastic nature of SPA and a possible association with Epstein-Barr virus. Interestingly, no immunoreactivity has been observed for HPV [5].

Reports of cytological atypia or dysplasia within some SPA have added to controversies about the nature of this lesion. Atypia may be found within the ductal epithelial cells ranging from mild to severe dysplasia and carcinoma in situ. However, the lobular architecture is always maintained and invasive carcinoma has not been identified in SPA cases [6].

SPA has diverse histological features. This lesion has a strong resemblance to the fibrocystic disease of breast [2]. Sclerosis and marked adenosis of ductal elements were evident in this case, but adenosis of acini was lacking. Sebaceous, squamous, foamy, and vacuolated cells as well as acinar cells with cytoplasmic zymogen granules have been described in this lesion [2]. However, our case was devoid of these features. Gnepp et al. have also reported two cases with lipomatous stroma [7].

Some authors have investigated immunohistochemical staining profile of cells in SPA. A review of previous studies is presented in Table 1. However, more investigations are required to establish the immunohistochemical profile of this lesion.

InvestigatorsMarkersImmunohistochemical reactivity

Fulciniti et al. (2010) [9]Collagen IV Enhanced lobular architecture
Cytokeratin 14Enhanced the ratio of apocrine cells present in the epithelial lining of lobular structures
Gross cystic disease fluid protein (GCFDP)Sebaceous cells

Gurgel et al. (2010) [8]Ki-67 Positive in less than 1% of cells
CKAE1/AE3, EMA, GCDFP-15Tubuloacinar elements
Estrogen, progesterone, and CK 34βE12 Negative
SMA, S100 Myoepithelial layer

Swelam (2010) [5]S100 Lesional ductal and spindle-shaped cells
Bcl-2Strong, diffuse cytoplasmic immunoreactivity in basal cells of neoplastic cells
Ki-67Sporadic positivity in Basal cells of neoplastic ductal epithelium
EBV Expression in neoplastic S100 positive cells

Meer and Altini (2008) [2]P63Peripheral layer of cells surrounding acini, ducts, and cystic spaces outlining these structures
AE1/AE3 In ductal lining cells of tubuloacinar elements
S100Ductal cells and spindled myoepithelial cells
AE1/AE3, CAM5.2, EMA, antimicrobial antibody, BRST-2, S100Luminal cells

Bharadwaj et al. (2007) [10]Cytokeratin In ductal and acinar elements
SMA, S100Myoepithelial layer

Skálová et al. (2006) [4]
CKAE1/AE3Positive in ductal and acinar cells
EMA, S100, antimitochondrial antibodyVariably positive
CEA, p53, and HER-2/neuNegative
GCDFP-15Acinar cells with coarse eosinophilic cytoplasm
Progesterone receptorsPositive in 15% to 20% of epithelial cells
Estrogen receptorsAt least focally in 5% of ductal cells in dysplastic and hyperplastic foci
SMA, P63, and calponinMyoepithelial layer

Gnepp et al. (2006) [7]Calponin, SMA, muscle specific actin, S100Myoepithelial layer

SPA is treated with conservative surgical excision with tumor-free margins and recurrence is rarely encountered [8].

Conflict of Interests

The authors declare that there is no conflict of interests regarding the publication of this paper.


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Copyright © 2014 Sepideh Mokhtari et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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