Sclerosing polycystic adenosis of parotid gland: A unique report of two cases occurring in two sisters

Sclerosing polycystic adenosis (SPA) of salivary glands is a tumorous lesion of salivary glands, with clinical presentation of a slow-growing mass characterized by a combination of histological features, some of which are reminiscent of mammary fibrocystic disease. SPA is mostly unifocal, but rarely may be multifocal and/or bilateral. Recurrences have been reported in up to 19% of cases. Although originally considered pseudoneoplastic, the occurrence of “dysplasia” and carcinoma in situ of ductal epithelium, and recent evidence of clonality suggest a possible neoplastic nature. Herein we describe, for the first time, two cases of SPA in two sisters (7 and 33 years old). The younger patient experienced multiple recurrences. This is the first report of familial occurrence of SPA, suggesting a possible genetic background.     

Juvenile sclerosing polycystic adenosis cytologically mimicking Warthin tumor

Sclerosing polycystic adenosis (SPA) is a rare salivary gland disease. Histologically it resembles a low‐grade ductal carcinoma in situ or sclerosing adenosis of the breast, characterized by lobular proliferation of ducts with apocrine cellular features surrounded by fibrosclerotic stroma. Although SPA is typically benign, recurrence is not uncommon, and cases with a malignant component have been documented. Thus, complete excision is desirable but preoperative diagnosis is challenging. A 12‐year‐old boy presented with a painless mass in the right neck. We identified a well‐demarcated mass in the right parotid region measuring approximately 2 cm using cervical echography and magnetic resonance (MR) imaging. Fine‐needle aspiration (FNA) revealed two cell types. There were loosely cohesive clusters of polymorphic epithelioid cells with irregular nuclei and abundant vacuolated cytoplasm containing zymogen granules. Some of these cells were binuclear. The other cell types represented normal ductal cells. The original cytological diagnosis was Warthin tumor. Right parotidectomy was performed. Histologically, we observed proliferation of ducts with granular, vacuolated, zymogen granules, and apocrine‐like features in the cytoplasm with hyalinizing sclerotic stroma and some binuclear cells. Four years after parotidectomy, there has been no recurrence or malignant transformation.Cytological diagnosis of SPA is challenging on FNA specimens since SPA is a very rare entity of the salivary gland that can mimic other salivary gland neoplasms. A mixture of apocrine‐like cells and sebaceous‐like cells, nuclear pleomorphism, and zymogen granules can help to diagnose this rare lesion during the initial cytological diagnosis.     

Sclerosing polycystic adenosis: A rare tumor misdiagnosed as retention cyst on fine needle aspiration cytology

Sclerosing polycystic adenosis (SPA) is a rare benign neoplasm of the salivary gland which resembles the fibrocystic disease of the breast clinically as well as morphologically. This entity has varied morphological presentation on fine needle aspiration. Only a few case reports and occasional case series are found in the literature describing its cytology. Here we are presenting a case of SPA in the parotid gland in a 13‐year‐old male patient who presented with a slow growing infra‐auricular mass since one year without any other symptoms. Radiological investigation suggested a possibility of matted level II cervical lymph nodes. Fine needle aspiration of the same showed only crystalloids in a dirty background. Hence, a possibility of retention cyst was given and excision was advised. On histopathological examination, the mass was diagnosed as Sclerosing polycystic adenosis of the parotid gland. Since SPA is a rare entity with diverse morphological features, it is more likely to be misdiagnosed on cytology. Recognition of this benign entity is important since the differential diagnosis includes other more common benign and malignant salivary gland neoplasm particularly mucoepidermoid carcinoma and other tumors with cystic and oncocytic features. Repeat aspiration from multiple sites should be considered in cystic lesions of the salivary gland so that its varied cytological components can be yielded to help with the diagnosis. Diagn. Cytopathol. 2017;45:640–644. © 2017 Wiley Periodicals, Inc.     

The pathogenic role of Epstein–Barr virus (EBV) in sclerosing polycystic adenosis

Sclerosing polycystic adenosis (SPA) is a pathology of the salivary gland which occurs infrequently and has a controversial etiology. In this study, we investigated the possible roles of HPV and EBV in the pathogenesis of SPA.     

Sclerosing polycystic adenosis of the parotid gland: Report of one case diagnosed by fine‐needle cytology with in situ malignant transformation

Sclerosing polycystic adenosis (SPA) is a rare pathological condition affecting the salivary glands, first described by Smith etal. in 1996. Even though this lesion is being increasingly diagnosed, less than 50 cases have been published in the world literature to date. In line with numerous other pathological analogies between breast and salivary gland lesions, SPA shares with fibrocystic disease of the breast many histopathological features, i.e., fibrosis, oncocytic (apocrine) changes, hyperplasia of ductal and acinar epithelium, cystic dilation of ducts, and, often, atypical epithelial changes. Most of the described cases have followed a benign clinical course, despite the frequent possibility of atypical hyperplasia in more than 50% of the cases and of the more than occasional in situ malignant transformation. In this article, we introduce a new case occurring in the parotid gland of a 57‐year‐old male showing atypical epithelial hyperplasia and low‐grade in situ mucoepidermoid carcinoma. Fine‐needle cytology (FNC) was performed on the lesion and, when a diagnosis of SPA was prospected, the variegated cytological features of the obtained sample posed several differential diagnostic problems. The spectrum of pathological lesions entering differential diagnosis comprised sebaceous adenoma, Warthin's tumors with presence of sebaceous remnants, and low‐grade mucoepidermoid carcinoma. Histopathological examination disclosed SCA with intraductal neoplastic transformation resembling noninvasive low‐grade mucoepidermoid carcinoma. The cytological diagnosis of SPA should be entertained whenever a polymorphous picture is found on FNC samples comprising oncocytic/apocrine changes, sebaceous cells, cystic background, and epithelial hyperplasia with low‐grade cytological atypias. Diagn. Cytopathol. 2010. © 2009 Wiley‐Liss, Inc.     

Atypical sclerosing adenosis of the prostate: a rare mimic of adenocarcinoma

Cheng L & Bostwick D G
(2010) Histopathology 56 , 627–631 Atypical sclerosing adenosis of the prostate: a rare mimic of adenocarcinoma Aims: Sclerosing adenosis of the prostate is a benign, small, acinar proliferation in dense spindle cell stroma, with a distinct immunohistochemical profiles. It is incidentally found in about 2% of transurethral resection specimens. The aim was to describe cases with significant cytological atypia mimicking cancer, which have not been previously reported. Methods and results: We describe five cases of sclerosing adenosis with significant cytological atypia, referred to as atypical sclerosing adenosis (ASA), which were initially considered suspicious or diagnostic of adenocarcinoma. Seven other cases of typical sclerosing adenosis were used as controls. All cases of typical and atypical sclerosing adenosis displayed an intact basal cell layer, which was immunoreactive for high‐molecular‐weight keratin, S100 protein, smooth muscle actin, and prostate‐specific antigen, with no differences between ASA and the control group. Alpha‐methylacyl‐coenzyme A racemase was negative. Three of four cases of ASA had aneuploid DNA content by digital image analysis. All cases of typical sclerosing adenosis were diploid. During a mean follow‐up of 33 months (range 5–73 months), none developed recurrence or prostatic cancer. Conclusions: ASA is an unusual small, acinar proliferation of the prostate that may be mistaken for adenocarcinoma, and should be distinguished from other mimics, including atypical adenomatous hyperplasia, mesonephric remnant hyperplasia, and post‐atrophic hyperplasia. ASA is a benign lesion and aggressive treatment is unwarranted.     

Intraductal carcinoma of the breast arising in sclerosing adenosis

An unusual case of intraductal carcinoma of the breast arising in sclerosing adenosis is reported. A 49 year old Japanese woman noticed a lump in her right breast 3 years before she sought medical advice. Histologic examination of the lumpectomy specimen showed, adjacent to intraductal papilloma, sclerosing adenosis involved in a neoplastic cellular proliferation with cribriform pattern and comedo necrosis. lmmunohistochemical study with antl-actin antibodies discriminated intraductal carcinoma from adjacent sclerosing adenosis by highlighting myoepithelial components in the latter. Extensive sampling revealed no carcinoma outside the sclerosing adenosis, implying that the intraductal carcinoma did originate in the tubules of sclerosing adenosis. A review of the literature indicated that the ductal to lobular ratio among carcinoma in situ concurring with sclerosing adenosis is about 1:2. The average age of patients with ductal and lobular carcinoma in situ in sclerosing adenosis is 39 and 43.7, respectively. It is suggested that carcinoma in situ arising in sclerosing adenosis and fibroadenoma have a similar biological basis.     

Carcinoma in situ involving sclerosing adenosis: a mimic of invasive breast carcinoma

The distinction between invasive and in situ carcinoma of the breast is important with regard to the treatment and prognosis of the patient. When carcinoma in situ involves breast tissue in which the normal architecture is altered by pre-existing sclerosing adenosis, the resulting histological picture may closely mimic an invasive carcinoma. We record the histopathological features in 13 cases where there was difficulty in identifying the presence or extent of invasive carcinoma. The most useful clue was attention to the low power appearances of distorted lobular units in the areas of malignancy and comparison with surrounding breast tissue which usually showed recognizable sclerosing adenosis. The use of immunohistochemical stains for myoepithelium (α-actin and S-100 protein) and for basement membrane (collagen type IV and laminin) proved to be of considerable value in identifying the preservation of these features around glandular structures in areas of sclerosing adenosis containing in situ carcinoma.     

A case of matrix-producing carcinoma of the breast with micoglandular adenosis and review of literature

Matrix-producing carcinoma (MPC) of the breast is an extremely rare variant of metaplastic breast carcinoma that contains a mixture of epithelial and mesenchymal elements. As overt carcinoma with direct transition to a cartilaginous and/or osseous stromal matrix cells, MPC is of no spindle cells between those two elements. This is the case of a 43 year-old female patient with MPC which coexisted with microglandular adenosis (MGA), atypical MGA (AMGA) and carcinoma in situ arising in MGA (MGACA in situ). MGA is a rare, infiltrative, benign lesion of the breast with an indolent clinical course. Histological evidence of carcinoma arising from MGA has previously been documented. MPC arising in MGA is an extremely rare subtype of breast carcinoma and has been seldom detailed described in the previous studies. This report highlights one such case with cytomorphological and histopathological correlation, along with a review of pertinent literature and differential diagnosis.     

Invasive carcinoma arising from sclerosing polycystic adenosis of the salivary gland

Sclerosing polycystic adenosis is a rare salivary gland lesion. Recently, this entity has been regarded as a neoplastic lesion of low-grade malignant potential but, to date, no invasive carcinoma, metastases, or associated mortality have been reported. We report the first case of an invasive carcinoma component in a recurrent sclerosing polycystic adenosis lesion.     

Adenomyoepithelioma of the breast with a distinctive type of apocrine adenosis

A newly recognized type of dimorphic carcinoma of breast, distinct from adenoid cystic carcinoma, is described. It is characterized by a predominantly solid, clear cell myoepithelial proliferation, with centrally situated glandular lumina lined by apocrine cells. All cases arose in association with a distinctive type of atypical apocrine adenosis which has to be distinguished from microglandular adenosis and from tubular carcinoma. The biological behaviour of the tumour remains to be ascertained on the basis of longer follow-up, but it appears to have only limited malignant potential.     

A case of IgG4-related sclerosing mesenteritis

IgG4-related disease has been recognized as a systemic syndrome characterized by mass-forming lesions with lymphoplasmacytic infiltration and sclerosis. This disease has been identified in various sites, including the pancreas, retroperitoneum, lung, head, and neck. Herein we report a case of IgG4-related sclerosing mesenteritis. An 82-year-old woman was admitted to our hospital due to persistent abdominal pain. Abdominal computed tomography demonstrated a solitary mass with a maximal diameter of 11.7 cm in mesentrium of the small intestine. On her laboratory examination, only C-reactive protein level was elevated. Although the pre-operative diagnosis was indefinite, she underwent ileocecectomy. Grossly, an elastic soft mass with foci of hemorrhage was seen in the mesentrium. Microscopically, the lesion was composed of fibroblastic or myofibroblastic spindle cells with abundant stromal fibrosis and inflammatory infiltrate, such as lymphocytes and plasma cells accompanied by lymphoid follicles with a germinal center. Obstructive phlebitis was observed. Immunohistochemically, numerous IgG4-positive plasma cells were observed, and the IgG4/IgG ratio was 75.9%. The serum level of IgG4 examined at post-operation was high. These findings suggested that this lesion was consistent with IgG4-related sclerosing mesenteritis.     

Topographical, morphological and immunohistochemical characteristics of carcinoma in situ of the breast involving sclerosing adenosis. Two distinct topographical patterns and histological types of carcinoma in situ

Moritani S, Ichihara S, Hasegawa M, Endo T, Oiwa M, Shiraiwa M, Nishida C, Morita T, Sato Y, Hayashi T, Kato A, Aoyama H & Yoshikawa K
(2011) Histopathology  58 , 835–846
Topographical, morphological and immunohistochemical characteristics of carcinoma in situ of the breast involving sclerosing adenosis. Two distinct topographical patterns and histological types of carcinoma in situ Aim: To examine the histopathological features of 24 surgically resected carcinoma in situ (CIS) involving sclerosing adenosis (SA), with special reference to the topographical relationship between CIS and SA. Methods and results: In 13 (54%) lesions, CIS was entirely surrounded by SA (type A) and in 11 (46%), CIS involved SA at least focally but was not confined to the SA area (type B). The mean size of CIS in type B (30.45 mm) was significantly larger than in type A (18.00 mm). The mean size of SA in type A (39.46 mm) was significantly larger than in type B (19.54 mm). Most type A CIS were non‐high‐grade, and the oestrogen receptor (ER)(+)/progesterone receptor (PgR)(+)/HER2(?) immunophenotype predominated. Most type B CIS were high‐grade and six (54%) were ER(?)/PgR(?). Most type A were bcl‐2(+)/p53(?) in both SA and CIS areas, but two (18%) apocrine ductal CIS of type B were bcl‐2(?)/p53(+) in both SA and CIS areas. Expression of ER and cyclin D1 in SA was not different from that of SA unassociated with cancer. Conclusions: Most CIS involving SA arises within SA and high‐grade DCIS tends to grow beyond SA. Occasional CIS may arise outside SA and secondarily involve SA.     

Salivary duct carcinoma: report of a case and review of the literature

Summary Salivary duct carcinoma is a rare primary tumour of the salivary glands arising most frequently in the parotid gland. It has a male preponderance and occurs most often in patients over the age of 50 years. Its distinctive histological features include dilated ducts containing cells arranged in cribriform, papillary or solid patterns often with central necrosis and reminiscent of intraduct carcinoma of the breast. These features are associated with an obvious invasive component. It is an aggressive neoplasm and may metastasize widely, causing death in a high proportion of cases.     

A case report of sclerosing thymoma of the anterior mediastinum: an exceedingly rare morphology

The morphology of thymoma is diverse, although 5 basic subtypes are recognized in the World Health Organization classification system. Sclerosing thymoma was first documented in 1994 and to date only 13 cases have been reported. Sclerosis itself is considered to be an ancient change and can occur in various histological subtypes. Herein, we present a case of a 62-year-old woman incidentally found to have an anterior mediastinal mass, 31 × 24 × 17 mm in size, without an associated autoimmune disease such as myasthenia gravis. The mass was finally diagnosed as sclerosing thymoma derived from type A thymoma. Intraoperative pathological examination using a limited amount of sample did not allow a definitive diagnosis of thymoma in this case. When dealing with fibrous lesions observed in limited samples such as biopsy and intraoperative frozen specimens, recognizing sclerosing thymoma is important since there are several disease entities accompanying fibrosis in the anterior mediastinum.     

Malignant progression of adenomyoepithelial adenosis of the breast

A case of breast tumor is described, which consisted of dense and uniform proliferation of ducts and lobules composed of both epithelial and myoepithelial cells and in which multiple foci of adenocarcinoma were observed. The tumor surrounding the carcinoma foci was identified as 'adenomyoepithelial adenosis'. Adenomyoepithelial adenosis was not monoclonal by clonal analysis, but revealed a relatively high labeling index for proliferating cell nuclear antigen by immunohistochemistry. Although it was still undetermined whether adenomyoepithelial adenosis is a non-clonal nonneoplastic lesion or a biclonal neoplastic one, the lesion was shown to reveal high proliferative activity in both glandular epithelial and myoepithelial cells and was considered to be prone to progress to obvious carcinoma.     

Apocrine adenosis of the breast: clonal evidence of neoplasia

AIMS: We report here a case of apocrine adenosis of the breast in a 66-year-old woman. To clarify the nature of this lesion, we examined it by clonal analysis. METHODS AND RESULTS: The lesion, 43 mm at its greatest dimension, was ill-circumscribed, lacking a fibrous capsule, and was composed of compact glands that showed typical histological features of apocrine adenosis. Clonality analysis, using the polymerase chain reaction (PCR)-based method for females heterozygous for a BstXI polymorphism of the X-linked phosphoglycerokinase (PGK) gene, revealed the monoclonal nature of the lesion. CONCLUSIONS: This result strongly suggests that some populations of apocrine adenosis are already neoplastic, and this could contribute to the premalignant potential of this lesion.