Polymorphous lowgrade adenocarcinoma plga is a rare, asymptomatic, slowgrowing malignant salivary gland tumor. A mucinous adencarcinoma is a type of cancer that produces what is called mucin, which is the main component of mucus. 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 lowgrade adenocarcinoma plga is a malignant epithelial tumor characterized by cytological uniformity, morphological diversity, an infiltrative growth pattern and low metastatic potential. Douglas frank, md2 1albert einstein college of medicine. We outline a case of this rare entity displaying a not hitherto described immunophenotype. Low grade polymorphous adenocarcinoma is a malignant neoplasm of salivary glands with uncommon occurrence in the head and neck region, almost exclusively affecting minor salivary glands. Complete examination and timely surgery are the key components of a successful treatment strategy for such rare disorders. Polymorphous lowgrade adenocarcinoma definition of. Although adenoid cystic carcinoma adcc is a highgrade malignant tumor, in most cases, it does not show cell atypia and mitotic features, except in the solid type 3. This is a lowgrade malignancy characterised by bland cytology and various architectural patterns.
The tumor mass may grow at a slow rate in the mouth and may present no pain. Over recent years, little doubt remains that this tumour represents a separate entity based on morphology, ultrastructural features and behaviour. Evans and batsakis, in 1984, eventually coined the term, polymorphous low grade adenocarcinoma plga. Plga was first described in 1983 by batsakis et al. Karyotypic variability and evolutionary characteristics of a polymorphous low grade adenocarcinoma in the parotid gland. It has previously been known as terminal duct carcinoma, lobular carcinoma, and low grade papillary adenocarcinoma. Polymorphous lowgrade adenocarcinoma nord national. Noncomedo dcis is low grade or intermediate grade, which means that it is slow growing and less likely to become an invasive breast cancer. It is more frequent in the thirdseventh decade of life, with a clear female predilection in a 2.
Polymorphous low grade adenocarcinoma plga shows histologic diversity. Polymorphous lowgrade adenocarcinoma is a low grade and indolent neoplasm, with 1020% risk of local recurrence, and low risk for distant metastasis and death due to plga. Polymorphous lowgrade adenocarcinomas are minor salivary gland neoplasms with a predilection for intraoral sites. It is important to differentiate plga from other salivary gland tumors with myoepithelial differentiation such as pleomorphic adenoma and adenoid cystic carcinoma. It is more frequent in the age range from 30 to 70, with a clear female predilection in a 2. What are the signs and symptoms of polymorphous lowgrade adenocarcinoma of salivary gland. Polymorphous lowgrade adenocarcinoma plga is an uncommon type of lowgrade minor salivary cancer. Lowgrade nasopharyngeal papillary adenocarcinoma is an extremely rare tumor, with only a limited number of cases reported in the literature. Polymorphous lowgrade adenocarcinoma of the palate.
Plga and cribriform adenocarcinoma of salivary gland. 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. It is more frequent in the age range from 30 to 70. Polymorphous lowgrade adenocarcinoma plga is a rare, asymptomatic, slow growing malignant salivary gland tumor.
Polymorphous lowgrade adenocarcinoma semantic scholar. Polymorphous lowgrade adenocarcinoma located in the. Nodal recurrence of salivary polymorphous lowgrade. The following histological factors predicted disease free survival on univariate. Polymorphous lowgrade adenocarcinoma plga has been known as a. Synonyms for polymorphous lowgrade adenocarcinoma in free thesaurus. Common tumor of palate with infiltrative growth, multiple architectural growth patterns and cellular uniformity, first described in 1983 oral surg oral med oral pathol 1983. Breast cancer grade and other tests doctors use information from yourbreast biopsy to learn a lot of important things about. Biphasic lowgrade nasopharyngeal papillary adenocarcinoma. Lowgrade nasopharyngeal papillary adenocarcinoma lgnppa is distinctly rare. 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. Nodal recurrence of salivary polymorphous lowgrade adenocarcinoma caitlin p. A carcinoma commonly misclassified as polymorphous lowgrade adenocarcinoma hematoxylineosin staining used in both parts of figure. It is twice more common in females, with a mean age of presentation at 59 years.
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. Pdf polymorphous low grade adenocarcinoma of the palate. 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 lowgrade adenocarcinoma. Paranasal sinuses are uncommon venues for polymorphous lowgrade adenocarcinoma. Polymorphous lowgrade adenocarcinoma plga is a malignancy arising. My breast cancer coach is a questionnaire designed to help you better understand your type of breast cancer so you can focus on the information thats most relevant to you. Polymorphous lowgrade adenocarcinoma is a rare malignancy arising from the minor salivary glands in the aerodigestive system, most frequently the hard palate. This is the rare case of chronic epistaxis caused by plga. Polymorphous low grade adenocarcinoma plga is an indolent malignant tumor affecting the minor salivary gland almost exclusively.
Polymorphous lowgrade 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 lowgrade adenocarcinoma of salivary gland. The palate or roof of the mouth, is the most common site for plga. Polymorphous lowgrade adenocarcinoma of the salivary glands with transformation to highgrade carcinoma. Polymorphous low grade adenocarcinomas plga are distinctive salivary gland neoplasms with an almost exclusive propensity to. Immunohistochemical pattern of pleomorphic adenoma.
A 32 year old man presented recurrent epistaxis was evaluated with. 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. Histologically, two main types of cells have been observed. Polymorphous lowgrade adenocarcinoma of salivary gland signs and symptoms may include the following. Here, we report a polymorphous lowgrade adenocarcinoma case of the maxillary sinus, a very rare. The patient is well and free of disease 34 months after the surgery. All three tumors were histologically of a lowgrade, and they comprised a uniform population of cells that lacked nuclear pleomorphism. It is most commonly found in the palate the name of the tumor derives from the fact that. If you have problems viewing pdf files, download the latest version of adobe reader. Polymorphous lowgrade adenocarcinoma plga is a rare tumor of the salivary glands. A 54yearold male presenting with a mass at the base of the tongue performed transoral laser microsurgery.
We report a case of a 55 year old male who presented with an asymptomatic swelling on the hard palate. Invasive ductal carcinoma is also called infiltrating ductal carcinoma or ductal adenocarcinoma. Polymorphous lowgrade adenocarcinoma plga is a minor salivary gland tumor with a low malignant potential. Polymorphous low grade adenocarcinoma is a rare, malignant salivary gland tumor, which is found almost exclusively in minor salivary glands. Polymorphous low grade adenocarcinoma lester dr thompson.
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. 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. Here, we present a case of lowgrade, nonintestinal snac in a 17yearold african american man and a discussion of the classification and pathologic features of primary snacs. Polymorphous lowgrade adenocarcinoma of the salivary gland, plga. Epidemiology represents 2 nd or 3 rd most common minor salivary gland malignancy 1726% 1st mucoepidermoid carcinoma rare in reported asian series female preponderance 2 4. Pdf polymorphous low grade adenocarcinoma polymorphous. What are synonyms for polymorphous lowgrade adenocarcinoma. Polymorphous lowgrade adenocarcinoma plga is one such tumor, misunderstood, underdiagnosed, controversial in nomenclature and unpredictable in behavior. A contemporary approach to pathologic diagnosis of. Low grade appendix mucinous neoplasm lamn is a well differentiated mucinous adenocarcinoma that arises from the appendix. Polymorphous lowgrade adenocarcinoma, salivary glands of.
Adenocarcinoma accounts for 10 to 20% of all primary malignant tumors of the nasal cavity and paranasal sinuses. 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. It can also occur in minor salivary glands in the rest of the upper aero. Polymorphous lowgrade adenocarcinoma of the parotid. Polymorphous lowgrade adenocarcinoma of the tongue base. Thyroidlike from lowgrade papillary nasopharyngeal. The growth patterns were characteristic, being tubulopapillary with back. Salivary glands primary salivary gland neoplasms malignant polymorphous adenocarcinoma. Patients are usually middleaged to elderly, more often women. Sinonasal lowgrade adenocarcinoma 239 and we investigated the expressions of ck 7, ck 20, cdx2 and p53, and the ki67 labeling index.
Polymorphous lowgrade adenocarcinoma is not listed in the existing who classification, but recent reports indicate that it may be the second most common malignant intraoral salivary gland tumour. It is usually located in the hard or soft palate, although it may be found in the rest of the oral. The book comprises chapters authored by various researchers active in the urologic oncology area. This tumor was recognized as a distinct entity in 1983 by freedman and lumerman and batsakis et al. Women are affected twice as frequently as men, and generally present in the fifth to sixth decade of life with a painless ihaoral mass. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 toll free. Polymorphous lowgrade adenocarcinoma plga is an epithelial salivary gland malignancy almost exclusively seen in the minor salivary glands. For language access assistance, contact the ncats public information officer. Your personalized treatment guide my breast cancer coach. Polymorphous lowgrade adenocarcinoma is a rare, malignant salivary gland tumor, which is found almost exclusively in minor salivary glands.
Polymorphous lowgrade adenocarcinoma genetic and rare. Well differentiated means when the cells of the tumor and the organization of the tumors tissue are almost normal. It has previously been known as terminal duct carcinoma, lobular carcinoma, and lowgrade papillary adenocarcinoma. Polymorphous lowgrade adenocarcinoma is a malignant epithelial tumour with low metastatic rate and infiltrative growth pattern. The former is usually a lowgrade tumor with papillary con. Some published studies have paid more attention to the clinicopathological features of nasopharyngeal adenocarcinoma, while little effort has been made to study the optimal therapeutic strategies. Polymorphous lowgrade adenocarcinoma has only rarely been reported. Author links open overlay panel yuantung chu a chungtai yue a b. Polymorphous lowgrade adenocarcinomamanagement and. Patients should talk with their doctor for more information about tumor grade and how it relates to their treatment and prognosis.
Adenocarcinoma of the appendix genetic and rare diseases. Lowgrade papillary adenocarcinomas of the sinonasal tract are rare neoplasms. The importance of tumor grade in planning treatment and determining a patients prognosis is greater for certain types of cancer, such as soft tissue sarcoma, primary brain tumors, and breast and prostate cancer. The treatment of choice is wide surgical resection, and the efficacy of radiotherapy has not been confirmed. 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 lowgrade adenocarcinoma plga is a slowgrowing salivary gland malignancy with a nonaggressive behaviour. It has a varied microscopic architectural appearance, i. By answering a few simple questions about your tumor type, well be able to present you with a report of treatment options that may be appropriate for patients like you.
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