Sclerosing polycystic adenoma - A review.

This review summarizes the current state of knowledge on sclerosing polycystic adenoma, including epidemiological/clinical, histopathological/cytopathological, ultrastructural, immunohistochemical and etiopathogenetic/molecular genetic aspects. Differential diagnostic issues are briefly discussed.

[Sclerosing polycystic adenosis of the parotid gland : surgical treatment of an extensive lesion of deep tissue without superficial damage]. / Adénose sclérosante polykystique de la parotide : traitement chirurgical d'une lésion extensive du lobe profond sans atteinte superficielle.

Sclerosing polycystic adenosis (SPA) is a rare benign epithelial lesion of the salivary glands, of unknown etiology, mainly affecting the parotid gland. We report the first clinical case of SPA involving the deep parotid gland with extension in the parapharyngeal space and the masticatory region. It has been resected by an external parotidectomy approach exclusively, despite the median extension of the lesion. The objective of this article is to complete the small series of cases described in the literature, and to update the knowledge of this rare disease.

L'adénose sclérosante polykystique (SPA) est une lésion épithéliale bénigne rare des glandes salivaires, d'étiologie inconnue, atteignant principalement la glande parotide. Nous rapportons le premier cas clinique de SPA dont l'origine est le lobe profond de la parotide et qui envahit la région masticatrice et l'espace parapharyngé. Elle a été réséquée par une voie d'abord externe de parotidectomie, exclusivement, malgré l'extension médiane de la lésion. L'objectif de cet article est de compléter la petite série de cas décrits dans la littérature, et d'actualiser les connaissances de cette pathologie rare.

Clinicopathological profile of sclerosing polycystic adenoma/adenosis: A systematic review.

In this systematic review, we aimed to evaluate the clinicopathological profile of sclerosing polycystic adenoma (SPA). PubMed, Scopus, EMBASE, Lilacs, Web of Science, and gray literature were searched to access cases of SPA in salivary glands. One hundred and thirty cases of SPA were found across 61 selected articles. SPA affected mainly the parotid gland of adults with a mean age of 44.6 years old, with a slight preference for females. The lesion was usually presented as a painless firm mass with a long period of evolution. Histologically, they are well-delimitated lesions composed of acinar and ductal elements with a variety of cytomorphologic features surrounded by a densely collagenized stroma. PI3K was the most common gene mutation related to SPA. SPA is a benign condition that mainly affects the parotid gland of female patients and it is usually treated by surgical resection with a good prognosis.

Excision of a sclerosing polycystic adenosis of the deep parotid gland.

A 15-year-old male presented to the hospital with a 2-month history of a right parotid mass. Magnetic resonance imaging revealed an encapsulated 3.7 cm mass. Fine-needle aspiration suggested a monomorphic adenoma (prior to utilization of the Milan Classification system). Total parotidectomy was performed with dissection and preservation of the facial nerve. Surgical pathology identified the lesion as sclerosing polycystic adenosis (SPA) after examining the histopathological and immunohistochemistry findings. SPA is a rare lesion which leads to inflammatory changes in the salivary gland. The most common site impacted is the parotid gland but also can affect other major or minor salivary glands. The fibrocystic changes are morphologically like the histological developments of sclerosing adenosis of breast tissue. Those changes consist of fibrosis, apocrine metaplasia, and different degrees of proliferation of ducts, acini, and myoepithelial cells. The pathogenesis of SPA is unknown but recent studies suggest that it could be a neoplasm. Treatment with surgical excision is effective and its' recurrence is rare. This case report details the cytomorphology, histology, and immunohistochemical profile of SPA.

A Case of Sclerosing Polycystic Adenoma of the Parotid Gland.

Sclerosing polycystic adenoma (SPA) is a rare salivary gland tumor with about 100 cases reported in the literature. We describe a case of SPA in the parotid gland and review the diagnostic tools used for identifying SPA. A 24-year-old male with a two-year history of right-sided face mass, initially thought to be pleomorphic adenoma of the parotid gland after fine needle aspiration (FNA). Following superficial parotidectomy, histologic features were consistent with SPA. This case illustrates the challenge of pre-operative assessment for SPA. Recent study has suggested SPA is a neoplasm and definitive treatment is surgical excision.

Sclerosing Polycystic Adenoma: Conclusive Clinical and Molecular Evidence of Its Neoplastic Nature.

Sclerosing polycystic adenosis, initially considered a non-neoplastic salivary gland lesion and classified as such in the 2017 WHO Classification of Head and Neck Tumors, has been the subject of controversy regarding its possible neoplastic nature. The reporting of recurrent PI3K pathway alteration represents evidence to support these lesions as being neoplastic and more appropriately referred to as sclerosing polycystic adenoma (SPA). Herein, we provide additional evidence that supports the classification of SPA as a true neoplasm. Eight cases of SPA were identified in our database and consultation files. All cases were subjected to PTEN immunohistochemistry (IHC) and next-generation sequencing (NGS). In addition, one patient underwent genetic counseling and germline testing. The cases included 5 men and 3 women with a mean age of 41 years (range 11-78) and all tumors arose in the parotid gland. One patient had multiple recurrences over a period of 2 years. Morphologically the tumors were circumscribed and characterized by an admixture of acini, ducts and cysts embedded in a fibrotic/sclerotic stroma. The cells lining the ducts and cysts showed variable granular, vacuolated, foamy and apocrine cytoplasmic features, as well as acinar cells contained intracytoplasmic brightly eosinophilic granules. The apocrine intraductal proliferations showed mild to moderate atypia in 6 cases. One case showed overt malignant morphology that ranged from intraductal carcinoma to invasive salivary duct carcinoma. Seven cases tested for PTEN IHC showed loss of nuclear expression in the acinar and ductal cells with retained PTEN expression in the myoepithelial cell and stroma. NGS detected PIK3CA or PIK3R1 genetic alterations in 7 cases, including a novel TFG-PIK3CA fusion. Coexisting PTEN mutations were seen in 4 cases, including in a patient with clinical stigmata of Cowden syndrome and confirmed by germline genetic testing. Our findings herein documented including recurrence of tumor, malignant transformation, high prevalence of PI3K pathway oncogenic alterations and the possible heretofore undescribed association with Cowden syndrome add support to classifying SPA as true neoplasms justifying their designation as adenoma, rather than adenosis.

Sclerosing Polycystic Adenosis Arising in the Parotid Gland Without PI3K Pathway Mutations.

A 15-year-old old Japanese male with a 2-month history of swelling of his left subauricular area was admitted to our department. A thumb-sized, hard mass with mild tenderness was palpated on the left parotid gland. Ultrasonography revealed a well-circumscribed, hypoechoic mass exhibiting heterogeneity in the left parotid gland measuring 1.7 × 1.5 × 1.3 cm. Computed tomography scan revealed a well-circumscribed, solid mass exhibiting slight peripheral enhancement in the left parotid gland. Magnetic resonance imaging revealed a hypointense mass in the left parotid gland on both T1- and T2-weighted images. Clinicoradiologic findings suggested a benign or low-grade malignant parotid tumor. The patient underwent left superficial parotidectomy with adequate safety margins. The facial nerve was identified and preserved. Neither facial paralysis nor tumor recurrence was observed as of 1 year postoperatively. Histologically, the nodule consisted of a vaguely nodular arrangement of variably sized ducts and acini in a hyalinized fibrous background with focal myxoid changes. The ductal/acinar component exhibited a bilayered arrangement of cuboidal luminal and flattened abluminal cells exhibiting a variety of epithelial proliferative patterns, including micropapillary and cribriform. Areas of oncocyte-like changes with intracellular coarse eosinophilic granules, apocrine-like feature, foamy/vacuolated changes, and clear cells were noted in the proliferating epithelium. Immunohistologically, the luminal cells were positive for gross cystic disease fluid protein-15. The Ki-67 labeling index was 2-3%. The histologic features and immunohistologic profile were consistent with sclerosing polycystic adenosis. Targeted next-generation sequencing of 160 cancer-related genes using the surgical specimen revealed no mutations, including known significant mutations in PTEN, PIK3CA, or PIK3R1.

Sclerosing polycystic adenosis of the submandibular gland: Two case reports.

BACKGROUND: Sclerosing polycystic adenosis (SPA) is a rare disease of salivary glands, similar to fibrocystic disease of the breast. It occurs over a wide age range and exhibits a slight female preference. Most SPA cases have occurred in the parotid gland. The exact nature of SPA is unclear, but its tumor nature has recently been proposed. Although SPA has a good prognosis after adequate surgery, atypical lesions might occur, ranging from mild dysplasia to carcinoma in situ in some cases. To the best of our knowledge, only five cases of SPA in the submandibular gland have been reported to date. Here, we present two new cases of SPA involving the submandibular gland. CASE SUMMARY: A 50-year-old woman and a 52-year-old woman were referred to Tongji Hospital in Wuhan, China, with complaints of moderate pain, recurrent swelling, and a mass in the submandibular area. After admission, the two cases of the submandibular mass were examined physically. The boundary of the submandibular tumor was clear, and the range of motion was good. After preoperative examinations, surgery was performed on a selective basis. Postoperative histopathological examination revealed a well-defined mass with acinar structures, ducts, or cystic dilated glands of various sizes scattered in a large number of proliferative sclerosing stroma. There were flat and cuboidal cells, and eosinophils in the duct epithelium. There was also a eosinophilic substance in the lumen of dilated cysts. No atypical epithelial hyperplasia, invasive growth, or carcinoma in situ was found. Based on the above findings, the mass was diagnosed as SPA. Both patients have remained asymptomatic and no recurrence or distant metastasis had occurred by the 7-mo and 5-year follow-up, respectively. CONCLUSION: SPA is a rare disease of the salivary gland. Even though it has a good prognosis after adequate surgery, atypical lesions may occur from mild dysplasia to carcinoma in situ. However, no recurrence, distant metastasis, or mortality has been reported for submandibular gland SPA. Clinicians and pathologists should be familiar with the characteristics of SPA in the submandibular gland to avoid misdiagnosis and overtreatment.

Sclerosing Polycystic Adenoma.

Sclerosing polycystic adenoma (SPA) is the more appropriate name for sclerosing polycystic adenosis. SPA is an uncommon salivary gland lesion with a constellation of unusual histologic findings that were originally interpreted as analogous to breast fibrocystic changes. The histologic findings in SPA include fibrosis, cystic alterations, apocrine metaplasia, and proliferations of ducts, acini, and myoepithelial cells in variable proportions. Because of its unusual mixed histology, SPA may be confused with a variety of lesions, ranging from reactive conditions to benign or even malignant neoplasms. The features of SPA are reviewed, with an emphasis on resolving its differential diagnosis.

Sclerosing polycystic adenosis of minor salivary glands: Report of a rare case with diagnostic approach and review of literature.

Sclerosing polycystic adenosis (SPA) is an uncommon entity occurring in the salivary glands, with majority of the cases reported in major salivary glands reminiscent of fibrocystic disease of the breast. SPA arising in minor salivary glands of the oral cavity constitutes an exceedingly rare phenomenon. Here, we report a case of SPA that presented as a solitary, submucosal mass on the left lower labial mucosa in a 19-year-old male. The pathology features and a clinicopathologic diagnostic approach highlighting key features are discussed here. Similar cases published in the English literature are reviewed.

Coexistence of sclerosing polycystic adenosis and dysgenetic polycystic disease of parotid, Report of a case.

Sclerosing polycystic adenosis (SPA) is a rare benign salivary gland lesion. Dysgenetic polycystic disease (DPD), which is a histologically similar lesion, may cause a lattice-like gross appearance with bilateral enlargement of the entire salivary glands. In this report, we present a case of SPA in the right parotid and coexistent DPD involving the both parotid.

Sclerosing Polycystic "Adenosis" of Salivary Glands: A Neoplasm Characterized by PI3K Pathway Alterations More Correctly Named Sclerosing Polycystic Adenoma.

Sclerosing polycystic adenosis (SPA) is a rare benign salivary gland lesion that usually arises from the parotid gland. SPA was originally interpreted to be a non-neoplastic alteration analogous to fibrocystic changes of the breast, but now there is uncertainty about whether it may represent a neoplasm. SPA often contains intraductal proliferations with an appearance similar to ductal neoplasia of the breast, and one study reported X-chromosome inactivation using polymorphisms of the human androgen receptor (Skalova et al., in AJSP 30:939-944, 2006). We investigated the genetics of SPA through targeted next generation sequencing (NGS). Four cases of SPA were retrieved from the authors' consultation files. A custom, targeted NGS panel including 1425 cancer-related genes was performed on all cases, followed by immunohistochemistry for PTEN. All four cases developed in females, ranging from 40 to 69 years (mean 52.5 years), affecting the parotid (n = 3) and submandibular glands (n = 1). All cases exhibited characteristic histologic features of SPA: well-circumscribed lesions with fibrosis and an admixture of ducts, myoepithelial cells and acinar cells, the latter containing brightly eosinophilic intracytoplasmic granules. Two cases had intraductal apocrine epithelial proliferations. By targeted NGS, loss-of-function mutations in PTEN were revealed in all 4 cases. In addition, 2 of 4 cases harbored PIK3CA mutations and 2 of 4 possessed PIK3R1 alterations; one case lacked both PIK3CA and PIK3R1 mutations. PTEN expression by immunohistochemistry was lost in the ductal and acinar elements but not the myoepithelial cells in all cases. SPA is characterized by genetic alterations in the PI3K pathway, with PTEN mutations seen most frequently. This molecular profile is similar to salivary duct carcinoma and the apocrine variant of intraductal carcinoma (i.e., salivary duct carcinoma-in situ). PI3K pathway alterations were found in cases both with and without intraductal apocrine proliferations, and PTEN immunohistochemistry suggested that the ductal and acinar cells, but not myoepithelial cells, were affected. Taken together, these findings strongly support that SPA is a neoplasm, more correctly named "sclerosing polycystic adenoma." The salivary duct carcinoma-like genetic alterations, coupled with the fact that the surrounding myoepithelial cells appear to be non-neoplastic, suggest a close relationship between SPA and apocrine intraductal carcinoma.

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.

Non-cystic Sclerosing Polycystic Adenosis: Diagnosis of a Hitherto Undescribed Pattern.

A case of sclerosing polycystic adenosis without cysts is described. The lesion occurred as a painless slow growing superficial right parotid lump in a 47 years old woman. The tumor measured 14 × 10 mm and displayed extreme well circumscription, sclerotic stroma with scattered hyaline nodules, sprinkling of mononuclear inflammatory cells and islands of mature fat. The epithelial component was predominantly composed of tubules lined by low columnar to cuboidal epithelium with bland nuclear features. Only focal incomplete acinar differentiation with few cells containing small eosinophilic granules was present. The stroma contained a significant fibromyxoid component with increased cellularity. There was no recurrence on follow-up at 14 months.

Sclerosing polycystic adenosis of lower lip: A new and rare salivary gland entity.

Sclerosing polycystic adenosis (SPA) was first described in 1996 by Smith et al. and was characterized by resemblance to epithelial proliferative lesions of the breast such as fibrocystic disease and sclerosing adenosis. Etiopathogenetically, it is generally believed to represent a nonneoplastic sclerosing and inflammatory process. The age range is broad (typically fourth decade), with a slight female predilection. The vast majority are parotid lesions, with very few in minor salivary glands. As of 2017, not more than 60 cases have been reported worldwide. Microscopically, it is characterized by a well-circumscribed to partially circumscribed tubulocystic proliferation of a gland within a sclerotic-fibrous stroma. Ductal epithelium showing variations such as foamy, mucous and apocrine are seen. We report a case of SPA of lower lip in a 70-year-old male.

Sclerosing polycystic adenosis of the oral cavity.

Minor salivary glands are ubiquitous in the oral cavity, and related diseases are often indolent and asymptomatic. We describe the unusual features of sclerosing polycystic adenosis, and its similarities to more sinister conditions of the minor salivary glands. Its importance is currently uncertain and research points to a pathogenesis that is neoplastic, which can create ambiguity. Sclerosing polycystic adenosis is a newly-discovered condition that merits further discussion and research to evaluate its full impact.

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.

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.

A unique case of sclerosing polycystic adenosis of the sinonasal tract.

Sclerosing polycystic adenosis is an extremely uncommon, recently described, sclerosing lesion of the salivary glands that appears histologically similar to fibrocystic changes of the breast. The key histopathologic features of sclerosing polycystic adenosis include lobular proliferation of ductal and acinar elements, cystically dilated ducts exhibiting frequent apocrine and sebaceous metaplasia, eosinophilic intracytoplasmic granules within some acinar-type cells, intraductal epithelial hyperplasia, and dense fibrosis. Most described cases have occurred in the major salivary glands, particularly the parotid gland. Although most authorities consider sclerosing polycystic adenosis to be a pseudoneoplastic process, the occurrence of dysplasia and carcinoma in situ of ductal epithelium reported recurrence rates of up to 30%, and recent evidence of clonality suggests a possible neoplastic etiology. However, there have been no cases of metastasis. Herein, we report the first case of sclerosing polycystic adenosis of the sinonasal tract in a 79-year-old woman presenting with a sinonasal mass.

Sclerosing polycystic adenosis of the buccal mucosa.

Sclerosing polycystic adenosis (SPA) is a rare lesion of salivary glands with a striking resemblance to fibrocystic disease of the breast. Most of the 47 reported cases have occurred within the parotid gland, with only a single case being described within the buccal mucosa. We report an additional case of SPA of the buccal mucosa. The exact nature of this entity is unknown, but has up until recently believed to be a pseudoneoplastic reactive and inflammatory sclerosing process. Even though SPA has satisfied the criteria for monoclonality, the debate as to whether SPA represents a true neoplasm or a pseudoneoplastic inflammatory sclerosing process, with low-grade neoplastic potential continues. Awareness of the occurrence of this lesion in both major and minor salivary glands is important to promote its differentiation from other more sinister salivary gland pathology. Cure is effected by localized surgical excision and all reported cases of SPA show an excellent prognosis with no true recurrence or metastasis.