This study is an analysis of the prevalence of polymorphous low grade adenocarcinoma plga in. Comparison of immunohistochemical markers between adenoid. Recently, two cases of plga with microscopically confirmed distant metastases have been reported. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for polymorphous lowgrade adenocarcinoma. Patients twentyfour patients with plga treated from january 1. A 54yearold male presenting with a mass at the base of the tongue performed transoral laser microsurgery. Polymorphous lowgrade adenocarcinoma of the parotid. In all but one there was a history of a painless intraoral mass of.
Adenoidcystic carcinoma adcc and polymorphous lowgrade adenocarcinoma plga are two malignant salivary gland tumours that show many histopathologic and some clinicopathologic features in many respect. Polymorphous low grade adenocarcinoma of the salivary gland, plga. Seldom does the plga present an aggressive behaviour and demonstrate distant metastasis, as in the present case where a 73yearold female patient with a swelling in the maxillary. Polymorphous lowgrade adenocarcinoma is a malignant epithelial tumour with low metastatic rate and infiltrative growth pattern. It is important to differentiate plga from other salivary gland tumors with myoepithelial differentiation such as pleomorphic adenoma and adenoid cystic carcinoma.
Polymorphous lowgrade adenocarcinoma plga is a rare tumor of the salivary glands that is limited, to a great extent, to the minor salivary glands and commonly, but. Oral surg oral med oral pathol oral radiol endod 91 2. Plgas are histologically heterogeneous and thus are difficult to distinguish from more aggressive salivary gland cancer subtypes. Central rii cellece i e ccess journal of ear, nose and throat disorders cite this article.
Jun 12, 2015 cancer of the appendix is very rare and is typically found incidentally during appendectomies, in about 1% of the cases. Shorter time presented due to lack of consistent records. Polymorphous low grade adenocardnoma plga is a rare, slow growing malignant neoplasm with a propensity to arise predominantly from minor salivary glands. Polymorphous lowgrade adenocarcinoma plga is a slowgrowing, distinct neoplasm of minor salivary glands. Polymorphous lowgrade adenocarcinoma plga is a lowgrade malignant neoplasm usually arising in the minor salivary glands. Polymorphous lowgrade adenocarcinoma located in the. A contemporary approach to pathologic diagnosis of. Polymorphous low grade adenocarcinoma plga is a low grade malignant epithelial tumor that is the second most common malignant neoplasm of minor salivary glands. We report a case of plga arising from the parotid gland. Pdf polymorphous low grade adenocarcinoma of the palate a. A contemporary approach to pathologic diagnosis of endometrial cancer joseph t rabban md mph associate professor, department of pathology university of california san francisco joseph. Introduction polymorphous low grade adenocarcinoma plga was first described as a distinct clinicopathologic entity in 1984 by evans and batsakis. The 57 surveys for which it was possible to calculate the percentage of plgas among all malignant minor salivary gland. Polymorphous lowgrade adenocarcinoma of the sublingual gland.
The growth patterns were characteristic, being tubulopapillary with back. Dec 17, 2018 what are the signs and symptoms of polymorphous low grade adenocarcinoma of salivary gland. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for polymorphous low grade adenocarcinoma. Plga occurs primarily in the oral cavity, especially in the palate, followed by the oral mucosa and upper lip 1,2. Plga is predominantly found in females in their sixth to seventh decade, and it most commonly arises in the minor salivary glands, with more than half of cases involving the palate. Polymorphous low grade adenocarcinoma plga is a rare, asymptomatic, slowgrowing malignant salivary gland tumor. Polymorphous lowgrade adenocarcinoma symptoms, diagnosis. Polymorphous lowgrade adenocarcinoma is a minor salivary gland neoplasm which occurs frequently in the mucosa of the soft and hard palates, in the buccal mucosa and in the upper lip. Metastases to locoregional lymph nodes occur in only 610% of cases. Polymorphous lowgrade adenocarcinoma plga is almost exclusively a minor salivary gland tumour and has been recognised since the early 1980s. Polymorphous low grade adenocarcinoma plga is a relatively indolent malignant salivary tumor that metastasizes to the lymph nodes in approximately 29% of patients but rarely metastasizes to distant sites. Polymorphous lowgrade adenocarcinoma is a lowgrade and indolent neoplasm, with 1020% risk of local recurrence, and low risk for distant metastasis and death due to plga. We report a case of a 55 year old male who presented with an asymptomatic swelling on the hard palate.
Six cases of polymorphous lowgrade adenocarcinoma terminal duct adenocarcinoma of the minor salivary glands are presented. Polymorphous lowgrade adenocarcinoma genetic and rare. According to a report published by the national cancer institute, using the surveillance, epidemiology, and end results seer database, appendix cancer account for about 0. Patients twentyfour patients with plga treated from january 1, 1973, through december 31, 2005. A case report in a male 48 he had history of foul smelling discharge from the mouth, dysphagia, odynophagia, bleeding, weight loss. Polymorphous low grade adenocarcinomas plgas are thought to be indolent tumours that are localised preferentially to the palate and affect the minor salivary glands almost exclusively. Polymorphous lowgrade adenocarcinoma of the nasopharynx. Polymorphous low grade adenocarcinoma of the hard palate. Polymorphous low grade adenocarcinoma, salivary gland tumors. As the name implies, polymorphous lowgrade adenocarcinoma plga is a lowgrade neoplasm consisting of a uniform population of tumor cells that are arranged in a variety of architectural patterns polymorphous.
Histologically, two main types of cells have been observed. The tumor mass may grow at a slow rate in the mouth and may present no pain. Nonencapsulated uniform plump columnar cells with bland nuclei with diverse growth patterns tubular, cribriform, papillary, solid, fascicular, microcystic, single file, pseudoadenoid cystic without true lumens, strandlike, mixed. These nonpapillary, low grade adenocarcinomas are part of the spectrum of polymorphous low grade adenocarcinoma. More detailed information about the symptoms, causes, and treatments of polymorphous low grade adenocarcinoma is available below. Polymorphous lowgrade adenocarcinoma is characterised histologically by a bland cytological uniformity combined with a remarkable histological diversity tubular, papillary, cribriform, solid and fascicular growth patterns and clinically as an indolent tumour, invasive and locally persistent but slow to. Plga generally follows a benign clinical course with frequent perineural invasion but low. A 31 yr old male who cameto the department of periodontics for a routine deaning of teeth was incidentally detected with a painless swelling on. Polymorphous lowgrade adenocarcinoma plga is a malignant epithelial tumor characterized by cytological uniformity, morphological diversity, an infiltrative growth pattern and low metastatic potential. Paranasal sinuses are uncommon venues for polymorphous lowgrade adenocarcinoma. A carcinoma commonly misclassified as polymorphous low grade adenocarcinoma hematoxylineosin staining used in both parts of figure. Polymorphous low grade adenocarcinoma is a rare, malignant salivary gland tumor, which is found almost exclusively in minor salivary glands. Polymorphous lowgrade adenocarcinoma of salivary gland signs and symptoms may include the following. Polymorphous low grade adenocarcinomas are minor salivary gland neoplasms with a predilection for intraoral sites.
Polymorphous lowgrade adenocarcinoma of the upper lip. Polymorphous low grade adenocarcinoma has only rarely been reported in britain, but we believe it deserves wider recognition as a distinct clinicopathological entity and, in particular, separation. Polymorphous low grade adenocarcinoma of salivary gland signs and symptoms may include the following. Wang tc, yang yt, tsai mt 2016 polymorphous lowgrade adenocarcinoma. Polymorphous low grade adenocarcinoma versus pleomorphic adenoma of minor salivary glands.
Salivary gland adenocarcinomas have been known as aggressive however progress. Epidemiology represents 2 nd or 3 rd most common minor salivary gland malignancy 1726% 1st. What are the signs and symptoms of polymorphous lowgrade adenocarcinoma of salivary gland. The polymorphous lowgrade adenocarcinoma of the nasopharynx is a rare disease. The clinicopathologic features of five terminal duct carcinomas arising in minor salivary glands are presented. Methods one hundred sixtyfour cases of other studies have reported a relative frequency of acinic cell carcinoma from 0% to 19% of malignant salivary gland neoplasms. Polymorphous low grade adenocarcinoma plga is a rare salivary gland neoplasm with an. Polymorphous lowgrade adenocarcinoma of the parotid gland. Adenocarcinoma with mucinous component adenocarcinoma is no longer considered definitionally high grade msih carcinomas are low grade. These tumors tend to start in the minor salivary glands. Polymorphous low grade adenocarcinoma is a malignant epithelial tumour with low metastatic rate and infiltrative growth pattern.
Recent studies on polymorphous low grade adenocarcinoma pdf. Polymorphous lowgrade adenocarcinoma of salivary gland. Paranasal sinuses are uncommon venues for polymorphous low grade adenocarcinoma. Polymorphous low grade adenocarcinoma plga is a rare malignancy which could usually be seen in minor salivary glands. Polymorphous low grade adenocarcinoma plga of the paranasal sinus is an extremely rare disease. Adenocarcinoma of the appendix genetic and rare diseases. Plga is a minor salivary gland neoplasm that occurs mostly in the junction of the soft and hard palate, followed by the buccal mucosa and upper lip. Polymorphous lowgrade adenocarcinoma nord national. Polymorphous lowgrade adenocarcinoma definition of. Here, we report a polymorphous lowgrade adenocarcinoma case of the maxillary sinus, a very rare. It is more frequent in the age range from 30 to 70, with a clear female predilection in a 2. This is a low grade malignancy characterised by bland cytology and various architectural patterns.
It can also occur in minor salivary glands in the rest of the upper aero. In such cases, it is critical to make a definite diagnosis as each of these tumors has an entirely different treatment protocol. Polymorphous low grade adenocarcinoma of the palate a case. Mar 16, 2020 as the name implies, polymorphous low grade adenocarcinoma plga is a low grade neoplasm consisting of a uniform population of tumor cells that are arranged in a variety of architectural patterns polymorphous. Polymorphous low grade adenocarcinoma of the parotid in a. Polymorphous low grade adenocarcinoma of tongue case. Polymorphous low grade adenocarcinoma of the parotid in a teenager divya khosla1, shalini verma1, nitin gupta2, rajpals punia3, gurbir kaur1, awadheshk pandey1, kislay dimri1 abstract introduction.
Polymorphous lowgrade adenocarcinoma of the salivary glands with transformation to high grade carcinoma. The treatment of choice is wide surgical resection, and the efficacy of radiotherapy has not been confirmed. A rare type of tumor of the salivary gland that occurs mainly on the roof of the mouth. Polymorphous low grade adenocarcinoma plga is a rare malignant neoplasm arising almost exclusively in the minor salivary glands. All three tumors were histologically of a low grade, and they comprised a uniform population of cells that lacked nuclear pleomorphism. The incidence of pgla in all minor salivary gland tumor are 10% and 25% in all malignant minor salivary gland tumor. Jun 09, 2017 pathologists are often overdiagnosing cases of low grade appendiceal mucinous neoplasm as invasive appendiceal adenocarcinoma, according to recent study data. The palate or roof of the mouth, is the most common site for plga.
Lobular carcinoma of the breast metastatic to the oral. This study analyzes the demographic, clinicopathologic, incidence, and survival characteristics of head and neck plga hn. Low magnification micrograph of a polymorphous lowgrade adenocarcinoma, showing the typical variation of architectural arrangement. B fibrous connective tissue concentration about a polymorphous low grade adenocarcinoma with infiltrating tubular glands extending into the adjacent minor salivary gland parenchyma. Polymorphous low grade adenocarcinoma of the head and neck. It has previously been known as terminal duct carcinoma, lobular carcinoma, and lowgrade papillary adenocarcinoma. We report a case of plga that arose at the base of the tongue. Polymorphous low grade adenocarcinoma plga is a recently described, relatively uncommon tumor that arises preferentially in minor salivary glands, favoring the palace. Polymorphous lowgrade adenocarcinoma of the tongue base. To date this entity has been identified within the oral cavity and only one case within the nasopharynx and some cases in the. Karyotypic variability and evolutionary characteristics of a polymorphous low grade adenocarcinoma in the parotid gland. Sep 12, 2008 low grade papillary adenocarcinoma lgpa represents a relatively rare histological variant of polymorphous low grade adenocarcinoma plga. Only a few cases have been reported in the sinonasal area.
It is nonaggressive when compared to other oral cavity. Original article polymorphous lowgrade adenocarcinoma. Jan 15, 20 this study is an analysis of the prevalence of polymorphous low grade adenocarcinoma plga in epidemiological surveys of salivary tumors published in the english language from 1992 to 2012. Pdf background polymorphous low grade adenocarcinomas plga are minor salivary gland neoplasms with a predilection for intraoral sites. Here, we report a polymorphous low grade adenocarcinoma case of the maxillary sinus, a very rare. Immunohistochemical pattern of pleomorphic adenoma. It is exclusively observed in the soft and hard palate regions, exhibiting an indolent biology. This tumor was recognized as a distinct entity in 1983 by freedman and lumerman and batsakis et al. It was first described in 1983 as lobular carcinoma by freedman and lumerman due to its similarity to lobular carcinoma of the breast freedman and lumerman 1983, and also by batsakis et al. Polymorphous lowgrade adenocarcinoma in the upper lip.
This parotid epithelialmyoepithelial carcinoma shows a solid growth pattern with scattered tubules and mostly shows elongated nuclei with open chromatin and delicate nuclear membranes reminiscent of polymorphous. Adenocarcinoma of the lung tends to stain mucin positive as it is derived from the mucusproducing glands of the lungs. Triad of infiltrative growth, multiple architectural growth patterns, cellular uniformity nonencapsulated but often well circumscribed tumor with diverse polymorphous growth patterns cribriform, fascicular, microcystic, mixed, papillary focal, pseudoadenoid cystic without true lumens, single file, solid, strandlike, tubular am j surg pathol 1984. Polymorphous lowgrade adenocarcinoma plga is a rare, asymptomatic, slowgrowing malignant salivary gland tumor.
Patients are usually middleaged to elderly, more often women. Oct 12, 2014 polymorphous low grade adenocarcinoma plga is one such tumor, misunderstood, underdiagnosed, controversial in nomenclature and unpredictable in behavior. Polymorphous low grade adenocarcinoma plga is an indolent malignant tumor affecting the minor salivary gland almost exclusively. Polymorphous lowgrade adenocarcinoma basicmedical key. Lowgrade appendix tumors often overdiagnosed as invasive cancer. Prkd1 mutations characterize salivary gland polymorphous. Polymorphous low grade adenocarcinoma is a rare malignancy arising from the minor salivary glands in the aerodigestive system, most frequently the hard palate. It is the second most common intraoral malignant minor salivary gland tumor accounting for 17. Nodal recurrence of salivary polymorphous lowgrade.
Low grade nonintestinal sinonasal adenocarcinoma shashi gopalan 1,mohd razif mohamad yunus, suriahayati md pauzi 2, noraidah masir 2, bee see goh 1 1 department of orlhns, and 2 department of pathology, universiti kebangsaan. Also called terminal duct carcinoma, lobular carcinoma, low grade papillary adenocarcinoma 10% of minor salivary gland tumors but only 1% of all malignant salivary gland tumors. There has been a debate as to whether lgpa is associated with greater aggressive potential compared to plga. Polymorphous lowgrade adenocarcinoma plga is a slowgrowing salivary gland malignancy with a nonaggressive behaviour. Polymorphous lowgrade adenocarcinoma libre pathology. Use tnm staging see colorectal adenocarcinoma staging for problematic staging issues. Ductal growth pattern of polymorphous low grade adenocarcinoma hex625. Lobular carcinoma of the breast metastatic to the oral cavity mimicking polymorphous low grade adenocarcinoma of the minor salivary glands kathleen k.
It has previously been known as terminal duct carcinoma, lobular carcinoma, and low grade papillary adenocarcinoma. It has a varied microscopic architectural appearance, i. Polymorphous low grade adenocarcinoma of the salivary glands with transformation to high grade carcinoma. Sinonasal low grade adenocarcinoma 239 and we investigated the expressions of ck 7, ck 20, cdx2 and p53, and the ki67 labeling index. It shares many similar histological patterns with adenoid cystic carcinoma, but the biological behaviour is significantly different. Polymorphous lowgrade adenocarcinoma is a rare, malignant salivary gland tumor, which is found almost exclusively in minor salivary glands. Polymorphous low grade adenocarcinoma plga is a low grade malignant neoplasm that occurs almost exclusively in the minor salivary glands, primarily those in the palate. Polymorphous low grade adenocarcinoma plga is an epithelial salivary gland malignancy almost exclusively seen in the minor salivary glands. Polymorphous low grade adenocarcinoma with distant. Diagnostic dilemma of polymorphous low grade adenocarcinoma. Polymorphous low grade adenocarcinoma plga originating mostly in the minor salivary glands of the posterior hard and soft palate is characterised by its indolent growth and a slower rate of metastasis. Polymorphous lowgrade adenocarcinoma december 5 th, 2008.
The tumor is characterized by a proliferation of uniform, cytologically bland cells and exhibits a broad range of growth patterns including tubular, solid, cribriform, and. Polymorphous lowgrade adenocarcinoma plga is a rare tumor of the salivary glands that is limited, to a great extent, to the minor salivary glands and commonly, but not exclusively, localized in the palate of the mouth. Iskandar, mbbch, phd c the oral cavity is a rare site of metastatic lesions. Objective to reappraise the clinical and histologic variables associated with a more aggressive outcome in polymorphous low grade adenocarcinoma plga. Pdf polymorphous low grade adenocarcinoma james castle. Although adenoid cystic carcinoma adcc is a high grade malignant tumor, in most cases, it does not show cell atypia and mitotic features, except in the solid type 3. Printable colorectal mucinous adenocarcinoma surgical. It is more frequent in the age range from 30 to 70.
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