About 30% of patients experience pain and less than 10% develop facial paralysis. Pathology and genetics of head and neck tumours, 1st edition, 2005 p216. This type of neoplasm is commonly found in the parotid gland and has a diffuse infiltrative growth pattern, with small acinar or glandular structures composed of a monotonous proliferation of cells with. Acc is often found in parotid 8090% and occasionally identified in. Acinic cell carcinoma of the posterior buccal mucosa.
This clinical trial combined pembrolizumab and vorinostat in recurrentmetastatic squamous cell carcinomas of the head and neck hn, and salivary gland cancer sgc. A gradual mass progression of the terminal end of the parotid gland usually affected. In these data, acinic cell carcinoma comprised 17% of primary malignant salivary gland tumors or about 6% of all salivary gland neoplasms. At the time of diagnosis, signs and symptoms have usually been present for less than a year. Its particular histopathological and immunohistochemical characteristics are reminiscent of breast secretory carcinoma. A 27yearold woman presented with an enlarging painless right preauricular mass at 28 weeks pregnant. Metastasis of acinic cell carcinoma of the parotid gland to. We aimed to analyze the diagnosis and treatment experience for this malignancy in our hospital. Dedifferentiated acinic cell carcinoma of the parotid. The latter seems to arise from striated ducts and is composed of peculiar clear cells. Clinical analysis of acinic cell carcinoma in parotid gland. The reported 10year survival rate is approximately 70%45.
The signals correlate with the histology, suggesting vascularity, hemosiderin deposition. Salivary gland neoplasm, papillary cystic acinic cell carcinoma. Acinic cell carcinoma an overview sciencedirect topics. Salivary duct carcinoma is in many studies the fifth most common malignant tumour after mucoepidermoid carcinoma, adenoid cystic carcinoma, acinic cell carcinoma and carcinoma ex pleomorphic adenoma. Aspirates from these tumors can be diagnostically challenging. Acinic cell carcinoma of the parotid salivary gland presenting as a. It is also known as acinic cell adenocarcinoma it should not to be confused with pancreatic acinar cell carcinoma. Pdf acinic cell carcinoma of the parotid gland with four. Acinic cell carcinoma is the second most common salivary gland malignancy, representing approximately 4% to 6% of all salivary gland neoplasms and up to 17% of malignancies. The parotid gland is the predominant site of origin.
Acinic cell carcinoma reported to be the second most common malignant tumor of the accessory parotid gland 12. Immunohistochemistry also enables differentiating sc from acinic cell carcinoma. I present with this, a case report where incidental results detected by histopathology examination with acinic cell carcinoma of the parotid gland which is a. Acinic cell carcinoma acc is an infrequent malignant salivary gland tumor, and most cases arise in the major glands, particularly the parotid. This tumor is usually a lowgrade, highly differentiated carcinoma. The mass had been stable for more than 10 years, but showed rapid growth during pregnancy. Mar 30, 2012 strong staining can be used to support the diagnosis of acinic cell carcinoma. Ultrastructure of the acinic cell carcinoma and clear cell. Unusual occurrences of this neoplasm are reported in hard palate, maxillary sinuses, lip, etc. Acinic cell carcinoma acc is a lowgrade malignant salivary neoplasm. It is important to differentiate rcc from acinic cell carcinoma of the parotid gland, as the latter can also have clear cells 3.
Keywords acinic cell carcinoma 4 pathology 4 salivary glands 4 therapy 4 prognosis 4 mammary analog secretory carcinoma. Management of metastatic acc is challenging due to limited data in the literature. Acinic cell carcinoma of the parotid gland radiology. However, rcc metastasising to the parotid glands is an extremely rare event. Pdf acinic cell carcinoma of palate a rare presentation. On the core biopsy, a diagnosis of acinic cell carcinoma. Characteristically slow growing with clinical behavior reflective of a lowgrade tumor, although 25 50% of patients with acinic cell carcinoma can recur if not treated adequately. Apr 01, 2019 of patients with malignant parotid tumors, 720% present with facial nerve weakness or paralysis, which almost never accompanies benign lesions and indicates a poor prognosis. Pdf acinic cell carcinoma acc is a lowgrade malignant salivary neoplasm that.
Acinic cell carcinoma, abbreviated accc, is a rare type of salivary gland cancer. A phase ii trial of pembrolizumab and vorinostat in. We report a rare case of acinic cell carcinoma acc arising from heterotopic salivary gland tissue within intra parotid lymph node. Pleomorphic adenoma was the commonest salivary gland tumor observed in both sexes. Adenoid cystic carcinoma is especially virulent, with significant neurotropism and a propensity to metastasize to the lung. Late recurrence of acinic cell carcinoma of the parotid gland. At scanning power, basophillia and prominent lymphoid infiltrate should raise suspicion of acinic cell carcinoma tumor shows multidirectional differentiation towards acinar, ductal as well as myoepithelial elements some tumor cells must demonstrate differentiation towards acinar cells. Distant metastasis dm is a common treatment failure pattern in acinic cell carcinoma acicc of the major salivary glands.
In afip data of salivary gland neoplasms, acinic cell carcinoma is the third most common salivary gland epithelial neoplasm after mucoepidermoid carcinoma and adenocarcinoma, nos. Acinic cell carcinoma of the parotid gland with four morphological. Acinic cell carcinoma salivary glands radiology reference. Jan 21, 2019 acinic cell carcinoma acicc is a rare salivary gland carcinoma that is poorly understood. It was initially thought to be benign disease entity and foote and frazell 1,2 first coined it as a carcinoma in the early 1950s. Skull base involvement by acinic cell carcinoma of the. Acinic cell carcinoma is the fourth most common malignant parotid gland tumour after mucoepidermoid carcinoma mec, adenoid cystic carcinoma adcc and carcinoma ex pleomorphic adenoma1. Mar 10, 2016 i present with this, a case report where incidental results detected by histopathology examination with acinic cell carcinoma of the parotid gland which is a rare entity of parotid tumour. Immunostaining of all salivary tumors is summarized in table 1. Because acicc is rare and was recently recognized as the entity of malignancy, acicc has been difficult to study. Acinic cell carcinoma is the malignancy of salivary glands that most often occurs bilaterally. Acinic cell carcinoma primarily arising in ectopic. Cell proliferation correlates with prognosis in acinic cell carcinomas of salivary gland origin. Dog1 may also be a marker of a transformed myoepithelial phenotype in a subset of biphasic salivary gland.
Dec 15, 2011 acinic cell carcinoma acicc is a rarely encountered malignancy in parotid gland. Before its description, sc was frequently classified as acinic cell carcinoma acc or adenocarcinoma, not otherwise specified. The disease presents as a slow growing mass, associated with pain or tenderness in 50% of the cases. Pathology pathology may superficially resemble normal serous acinar cells of the salivary glands. Acinic cell carcinoma acc is a malignant epithelial neoplasm of the salivary glands in which at least some of the neoplastic cells demonstrate serous acinar cell differentiation characterized by cytoplasmic zymogen secretory granules. Trk expression is not entirely sensitive or specific for secretory carcinoma, nuclear staining distinguishes secretory carcinoma from mimics. On the core biopsy, a diagnosis of acinic cell carcinoma was made, a rare diagnosis. Arch pathol lab medvol 9, january 2015 selected salivary gland and head and neck tumorszhu et al 59. Acinic cell carcinoma of minor salivary glands acinic cell carcinoma of minor salivary glands ferlito, a. Acinic cell carcinoma of the parotid gland with four. Acinic cell carcinoma occurs in the parotid gland more often than primary planoepithelial carcino ma.
The three most common tumors of the parotid gland in order are mucoepidermoid carcinoma, adenoid cystic carcinoma, and acinic cell carcinoma. Approximately 80% of patients with facial nerve paralysis have nodal metastasis at the time of diagnosis. Immunohistochemical study of 30 cases using the mib 1 antibody in formalinfixed paraffin. Acinic cell carcinoma of minor salivary gland origin volume 105 issue 9 peter zbaeren, willy lehmann, sven widgren skip to main content accessibility help we use cookies to distinguish you from other users and to provide you with a better experience on our websites. Acinic cell carcinoma cytologic features and diagnostic pi. Review and updates of immunohistochemistry in selected. Here the authors perform genomic, transcriptomic and epigenomic profiling of acicc and find highly. This is the only instance of primary pulmonary acinic cell carcinoma with lymph node metastasis among 15 cases in the literature. Acinic cell carcinoma arising in salivary glands is a rare tumor, accounting for.
An end should be considered in patients with acinic cell carcinoma of the parotid gland due to i a notable rate of occult neck metastases in lowgrade tumors and ii the worse dss of patients. Acinic cell carcinoma with highgrade transformation of the salivary gland is an unusual variant with less than fifty cases being reported in the literature. Highgrade transformation dedifferentiation of acinic cell. Renal cell carcinoma metastasis to the parotid gland. Pathology clinic salivary gland acinic cell carcinoma. Mar 23, 2018 an acinic cell carcinoma acc of salivary gland is a type of malignant epithelial tumor affecting either the major or minor salivary glands. Acinic cell carcinoma of minor salivary gland origin the. Acinic cell carcinoma is an uncommon malignant neoplasm, mostly arising in the parotid gland. Acinic cell carcinoma is an uncommon lowgrade malignant tumor of the salivary glands, in which some cells resemble normal acinic cells. The latter is usually negative for protein s100 and mammaglobin and positive for dog1. In the present patient, the parotid gland metastasis was thought to be solitary and thus was surgically excised. It should not to be confused with pancreatic acinar cell carcinoma. The former appears as a differentiated tumor, made of secretory serous acinar cells. Dedifferentiated acinic cell carcinoma of the salivary gland is an uncommon variant of acinic cell carcinoma, characterized by the coexistence of both an usual lowgrade acinic cell carcinoma and a highgrade dedifferentiated component, as well as by an accelerated clinical course.
Acinic cell carcinoma is a slowgrowing malignant tumor, normally developing in the parotid glands and the major and minor salivary glands, upper lip, buccal mucosa, and the palate. Metastatic acinic cell carcinoma of the parotid gland to. According to the world health organization, acinic cell carcinoma acc is a malignant epithelial neoplasm of the salivary glands in which at least some of the neoplastic cells demonstrate serous acinar cell differentiation characterized by cytoplasmic zymogen secretory granules. Most acc occur in the parotid gland, representing between 2 and3% of all epithelial parotid neoplasms. Monotonous proliferation of cells with abundant finely granular, weakly eosinophilic or clear vacuolated cytoplasm, central round nuclei and prominent nucleoli, resembling acinic cells of salivary gland j clin pathol 2003. Acinic cell carcinoma causes, treatment, prognosis. Update in salivary gland pathology arup laboratories. Pdf nodal metastases in acinic cell carcinoma of the. Acinic cell carcinoma of the parotid gland radiology case. The acinic cell carcinoma and the clear cell carcinoma of the parotid are morphologically different neoplasms under electron microscopic examination. Acinic cell carcinoma, parotid gland, prognosis, parotidectomy introduction acinar cell carcinoma acc is a relatively rare malignancy of the salivary gland and accouts for 68% of salivary gland tumors and 17% of parotid malignancies 1. The patient underwent superficial parotidectomy for acinar cell rich lesion. On histopathology it was difficult to determine whether it was a primary tumor from intra parotid lymph node or metastatic from the parotid.
Discovered on gastrointestinal stromal tumor protein 1 dog1 expression in acinic cell carcinoma original magnification 3400. We explore histopathologic, histochemical, electron microscopic and immunohistochemical aspects and discuss histologic grading, histogenesis, animal models, and genetic events. Acinic cell carcinoma acc is a rare, slowgrowing, lowgrade salivary gland cancer. As parotid cancer research has evolved, so have the treatment recommendations for parotid cancers, including acc. Acinic or acinar cell carcinoma of the parotid gland is a rare tumor that represents approximately 10% of salivary gland tumors. It is observed in a wide age category of both children and adults.
Acinic cell carcinoma is the fourth most common malignant parotid gland tumour after mucoepidermoid carcinoma mec, adenoid cystic carcinoma adcc and carcinoma ex pleomorphic adenoma. Enhancer hijacking activates oncogenic transcription factor. Acinic cell carcinoma is a malignant tumor representing 2% of all salivary tumors. Acc has a tendency to recur and metastasize, rarely enough to soft tissue as well as other sites. Almost 17 percent of the primary salivary gland carcinoma is related to the acinic cell carcinoma. Original article clinical characteristics, therapy and. Histopathologic grading of salivary gland neoplasms. Dr yousef khelfa md of the edward comprehensive care center, at marshal university in huntington, west virginia presented a not so benign case. Prof vinod kumar arora, department of pathology, university college of medical sciences and gtb hospital, delhi. Acinic cell carcinoma of the lung with metastasis to lymph. Salivary gland cancer treatment options include surgery with or without postoperative radiation therapy, radiation therapy, and chemotherapy. Acinic cell carcinoma of the parotid gland in pregnancy. T1 skull base involvement by acinic cell carcinoma of the parotid gland. About 81%98% of the cases occur in the parotid gland, 11% in the submandibular gland, salivary glands.
Patients with progressing incurable hn and sgc, eastern cooperative oncology group ecog. Predictors of distant metastasis in parotid acinic cell. Interestingly, acicc was originally described as an adenoma, but from the 1950s the term acinic cell adenocarcinoma was coined when its ability to metastasize and recur locally had been recognized 3, 4. Highgrade transformation dedifferentiation of acinic. Oct 27, 2019 acinic cell carcinoma acc is a special type of breast carcinoma showing serous differentiation, with zymogentype cytoplasmic granules, similar to acinic cell carcinoma arising in the parotid gland eusebi et al. Metastasis of acinic cell carcinoma of the parotid gland. Third most common malignancy of major salivary gland 15%.
Acinic cell carcinoma acicc is a rare salivary gland carcinoma that is poorly understood. Acinic or acinar cell carcinoma acc is a rare parotid gland tumor, accounting for approximately 10% of salivary gland tumors. Apr 22, 2017 salivary gland acinic cell carcinoma acicc is usually considered a relatively benign disease. Enhancer hijacking activates oncogenic transcription. Acinic cell carcinoma accc of the breast is a rare histological type of malignant epithelial neoplasm exhibiting acinic cell differentiation. Pathology and genetics of head and neck tumours, 1st edition. When these tumors are welldifferentiated, the neoplasia has innocuous aspect, due to the similarity to normal parotid tissue. Acinic cell carcinoma accc is a malignant epithelial salivary gland neoplasm that demonstrates serous acinar cell differentiation with cytoplasmic zymogen. Acinic cell carcinoma is a lowgrade neoplasm occurs on the epithelial cell lining of the salivary gland. Pdf histopathological pattern of salivary gland tumors. Acinic cell carcinomas accs are uncommon salivary gland tumors that most commonly occur during the. Original article clinical characteristics, therapy and prognosis of parotid acinar cell carcinoma xilin nie 1, jialei gu, wendong wang 1, kejing wang, jinbiao shang1,2 1department of head and neck surgery, zhejiang cancer hospital, hangzhou, china. Here the authors perform genomic, transcriptomic and epigenomic profiling of.
Uncommon malignant epithelial neoplasm with serous acinar cell differentiation cytoplasmic zymogen granules and salivary ductal cell component j oral maxillofac surg 2010. Acc can be misinterpreted when it has low grade histology, due to its benign. Relapsed acinic cell carcinoma of the parotid gland with. The authors concluded that the occurrence of acinic cell carcinoma outside the parotid is rare and that, in these cases, the sc diagnosis should be considered first. Women are affected with salivary gland tumors more than men. Trk, though membranous expression of trk is common in other salivary gland.
Dec 12, 2014 acinic cell carcinoma arising in salivary glands is a rare tumor, accounting for 2% to 5% of the primary neoplasms of the parotid gland. Get detailed information about the treatment for newly diagnosed and recurrent salivary gland cancer in this summary for clinicians. Acinic cell carcinoma arising in salivary glands is a rare tumor, accounting for 2% to 5% of the primary neoplasms of the parotid gland. Acinic cell carcinomas of the salivary glands are rare malignant neoplasms that account for % of all salivary gland tumors. Salivary gland tumor being malignant is inversely proportional to the size of the gland. Acinic cell carcinoma acc is a rare epithelial malignant neoplasm of salivary glands. When these tumors are welldifferentiated, the neoplasia has innocuous aspect, due to the similarity to normal.
It is characterized by a low and highgrade component juxtaposed with one another and tends to take on a more aggressive clinical course than its lowgrade counterpart, suggesting a poor clinical outcome. Fineneedle biopsy, acinar cell carcinoma, parotid gland, parotid neoplasms address for correspondence. Secretory carcinoma sc is a recently described entity occurring in the salivary glands. Acinic cell carcinoma acicc is a relatively uncommon malignancy accounting for 1 to 6% of all salivary gland tumors and 15% of all malignant tumors in parotid gland 1, 2.
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