Polymorphous lowgrade adenocarcinoma of the upper lip. Patients are usually middleaged to elderly, more often women. 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 low grade adenocarcinoma of the upper lip. Plga accounts for 7 11% of all benign and malignant neoplasms and about 19 26% of all malignancies in minor salivary glands.
The list of treatments mentioned in various sources for polymorphous lowgrade adenocarcinoma includes the following list. The concept of dedifferentiation had previously been used in salivary gland carcinomas. These surveys included studies from different researchers, countries and continents. 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. It has a varied microscopic architectural appearance, i. Women are affected twice as frequently as men, and generally present in the fifth to sixth decade of life with a painless ihaoral mass. This case lent further support to the suggestion that these lesions are a relatively nonspecific hepatic.
Polymorphous low grade adenocarcinoma with distant metastases. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. The diagnosis is mainly based on an incisional biopsy. A carcinoma commonly misclassified as polymorphous low grade adenocarcinoma hematoxylineosin staining used in both parts of figure. Previously, pac was referred to as polymorphous low grade. Polymorphous lowgrade adenocarcinoma of submandibular gland. These tumors tend to start in the minor salivary glands. Always seek professional medical advice about any treatment or change in treatment plans. 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 low grade adenocarcinoma original magnification.
Polymorphous low grade adenocarcinoma plga is a slowgrowing salivary gland malignancy with a nonaggressive behaviour. What are the signs and symptoms of polymorphous lowgrade adenocarcinoma of salivary gland. It has previously been known as terminal duct carcinoma, lobular carcinoma, and lowgrade papillary adenocarcinoma. Polymorphous lowgrade adenocarcinoma synonyms, polymorphous. Pdf polymorphous lowgrade adenocarcinoma plga, is a rare, salivary gland intraoral tumor with complexities in. 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. In such cases, it is critical to make a definite diagnosis as each of these tumors has an entirely different treatment protocol. 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. A 67yearold man was initially seen with painless right upper eyelidswelling and conjunctival hyperemia across a 2day period. This study analyzes the demographic, clinicopathologic, incidence, and survival characteristics of head and neck plga hn. Polymorphous lowgrade adenocarcinoma plga is a slowgrowing salivary gland malignancy with a nonaggressive behaviour. Polymorphous low grade adenocarcinoma of the hard palate.
Polymorphous lowgrade adenocarcinoma of the salivary glands with transformation to highgrade carcinoma. Like many of the salivary glandlike tumors of the breast, polymorphous adenocarcinoma is a triple negative, basallike tumor appl immunohistochem mol morphol 20. Pdf polymorphous low grade adenocarcinoma james castle. Polymorphous low grade adenocarcinomas are minor salivary gland neoplasms with a predilection for intraoral sites. It is more frequent in the thirdseventh decade of life, with a clear female predilection in a 2. Polymorphous lowgrade adenocarcinoma libre pathology. It has previously been known as terminal duct carcinoma, lobular carcinoma, and low grade papillary adenocarcinoma. Synonyms for polymorphous low grade adenocarcinoma in free thesaurus. This tumor was recognized as a distinct entity in 1983 by freedman and lumerman and batsakis et al. Polymorphous lowgrade adenocarcinoma lester dr thompson.
Polymorphous adenocarcinoma of the salivary glands. The palate or roof of the mouth, is the most common site for plga. First described in 1983, this entity has been recognized to have an indolent course with rare metastases or deaths. We describe our experience with 17 patients treated at our institution for plga from 1984 to 2012. Pdf polymorphous low grade adenocarcinomas plga are minor salivary gland neoplasms with a predilection for. Abstractobjective to study the immunohistochemical pattern of cd 117, glial fibrillary. Polymorphous lowgrade adenocarcinoma plga is a rare tumor of the salivary. Among salivary gland malignancies, polymorphous lowgrade adenocarcinoma plga clearly illustrates such diagnostic dif. For language access assistance, contact the ncats public information officer. Salivary glands primary salivary gland neoplasms malignant polymorphous adenocarcinoma. Polymorphous lowgrade adenocarcinoma of the lacrimal gland.
Polymorphous low grade adenocarcinoma plga is a rare malignancy most commonly seen in the minor salivary glands. This tumor consisted of cellular lobules with distinct cribriform, papillary, and cystic patterns. Polymorphous lowgrade adenocarcinoma definition of. I describing the occurrence of hepatic fibrinring granulomas in a patient with systemic lupus erythematosus. Polymorphous low grade adenocarcinoma plga is an indolent malignant tumor affecting the minor salivary gland almost exclusively. Polymorphous lowgrade adenocarcinomas are minor salivary gland neoplasms with a predilection for intraoral sites. It can also occur in minor salivary glands in the rest of the upper aero. Polymorphous lowgrade adenocarcinoma of seromucous.
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 submandibular gland origin yutaka yamazaki a, atsuko hirai a, makoto ishikawa a, shingo moriya b, tadashi iizuka c, yoshimasa kitagawa a a oral diagnosis and medicine, department of oral pathobiological science, graduate school of dental medicine, hokkaido university, kita nishi7, kitaku, sapporo. Examination revealed him to be afebrile with a visual acuity of 612 od and69 os, 6 mm of proptosis, 3mm inferior globe displacement, temporal. Polymorphous lowgrade 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 plga is the second most frequent type of malignant tumor of the minor salivary glands. Ckit expression distinguishes salivary gland adenoid. Polymorphous lowgrade adenocarcinoma nord national. Polymorphous low grade adenocarcinoma of salivary gland signs and symptoms may include the following. We report the first case of a polymorphous lowgrade adenocarcinoma plga occurring in the vulva and vagina of a 32yearold woman. Polymorphous lowgrade adenocarcinoma is a rare malignancy arising from the minor salivary glands in the aerodigestive system, most frequently the hard palate. The 57 surveys for which it was possible to calculate the percentage of plgas among all malignant minor salivary gland. Metastases to locoregional lymph nodes occur in only 610% of cases.
To date, this entity has been identified either within the confines of the oral cavity or, rarely, in the nasal cavity. Polymorphous low grade adenocarcinoma is a rare, malignant salivary gland tumor, which is found almost exclusively in minor salivary glands. Polymorphous lowgrade adenocarcinoma of maxillary alveolus. Polymorphous low grade adenocarcinoma with distant. Polymorphous low grade adenocarcinoma plga is a rare tumor that mostly affects minor salivary glands. Oral surg oral med oral pathol oral radiol endod 91 2. Polymorphous low grade adenocarcinoma of the parotid in a. 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 alveolus was. Although relatively rare, polymorphous adenocarcinoma pac is likely the second most common malignancy of the minor salivary glands misg. 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 plga is a rare malignancy most commonly seen in the minor salivary glands. In contrast, polymorphous low grade adenocarcinoma plga has a much lower. 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. Previously, pac was referred to as polymorphous lowgrade.
A 54yearold male presenting with a mass at the base of the tongue performed transoral laser microsurgery. Oct 12, 2014 polymorphous low grade adenocarcinoma plga is one such tumor, misunderstood, underdiagnosed, controversial in nomenclature and unpredictable in behavior. Dec 17, 2018 what are the signs and symptoms of polymorphous low grade adenocarcinoma of salivary gland. Immunohistochemical pattern of pleomorphic adenoma. Polymorphous lowgrade adenocarcinoma plga is a minor salivary gland neoplasm that is characterized by morphologic variability, cytologic uniformity, and an infiltrating growth pattern. We report a case of a 55 year old male who presented with an asymptomatic swelling on the hard palate. Symptoms of polymorphous lowgrade adenocarcinoma including 5 medical symptoms and signs of polymorphous lowgrade adenocarcinoma, alternative diagnoses, misdiagnosis, and correct diagnosis for polymorphous lowgrade adenocarcinoma signs or polymorphous low.
Recently, two cases of plga with microscopically confirmed distant metastases have been reported. 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. We report the first case of a polymorphous low grade adenocarcinoma plga occurring in the vulva and vagina of a 32yearold woman. Polymorphous lowgrade adenocarcinoma of salivary gland. Case report polymorphous lowgrade adenocarcinoma of submandibular gland origin yutaka yamazaki a, atsuko hirai a, makoto ishikawa a, shingo moriya b, tadashi iizuka c, yoshimasa kitagawa a a oral diagnosis and medicine, department of oral pathobiological science, graduate school of dental medicine, hokkaido university, kita nishi7, kitaku, sapporo 0608586, japan. Treatment list for polymorphous lowgrade adenocarcinoma. 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. Symptoms of polymorphous low grade adenocarcinoma including 5 medical symptoms and signs of polymorphous low grade adenocarcinoma, alternative diagnoses, misdiagnosis, and correct diagnosis for polymorphous low grade adenocarcinoma signs or polymorphous low grade adenocarcinoma symptoms. 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. 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 the lacrimal. Polymorphous lowgrade adenocarcinoma plga is a rare, asymptomatic, slowgrowing malignant salivary gland tumor. The term polymorphous lowgrade adenocarcinoma was later adopted in the 2nd who classification of 1991 and defined as a malignant epithelial tumour characterized by cytological uniformity, morphological diversity and a low metastatic potential. Before its original recognition as an independent entity in 1983, most cases of plga were included in the spectrum of adenoid cystic carcinoma adcc, and.
The purpose of this study is to report six new cases followedup during a long period. Introduction polymorphous lowgrade adenocarcinoma plga is a rare malignant tumor which mostly develops in minor salivary glands. 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. Synonyms for polymorphous lowgrade adenocarcinoma in free thesaurus. Polymorphous lowgrade adenocarcinoma apamed central.
Recent studies on polymorphous low grade adenocarcinoma pdf. Karyotypic variability and evolutionary characteristics of a polymorphous low grade adenocarcinoma in the parotid gland. Polymorphous lowgrade adenocarcinoma of the tongue base. Polymorphous lowgrade adenocarcinoma of seromucous glands of. Sep 21, 2014 polymorphous low grade adenocarcinoma plga is the second most frequent type of malignant tumor of the minor salivary glands. In contrast, polymorphous lowgrade adenocarcinoma plga has a. 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 adenocarcinoma plga is a malignant epithelial tumor characterized by cytological uniformity. What are synonyms for polymorphous lowgrade adenocarcinoma. It occurs primarily in the minor salivary glands but.
Polymorphous low grade adenocarcinoma versus pleomorphic adenoma of minor salivary glands. It is important to differentiate plga from other salivary gland tumors with myoepithelial differentiation such as pleomorphic adenoma and adenoid cystic carcinoma. Antonyms for polymorphous low grade adenocarcinoma. Polymorphous lowgrade adenocarcinoma of the parotid. Polymorphous low grade adenocarcinoma of the salivary glands with transformation to high grade carcinoma. It is most commonly found in the palate the name of the tumor derives from the fact that. Recently, the term highgrade transformation was introduced for adenoid cystic carcinoma, acinic cell carcinoma, epithelialmyoepithelial carcinoma, and polymorphous lowgrade adenocarcinoma and may better reflect this phenomenon, although transformation into moderately differentiated adenocarcinoma. The treatment of choice is wide surgical resection, and the efficacy of radiotherapy has not been confirmed. This is a lowgrade malignancy characterised by bland cytology and various architectural patterns. Unlike the poor prognosis seen in many triple negative breast tumors, salivary glandlike tumors, including polymorphous adenocarcinoma, may have a more favorable prognosis. A case report in a male 48 he had history of foul smelling discharge from the mouth, dysphagia, odynophagia, bleeding, weight loss.
The tumor mass may grow at a slow rate in the mouth and may present no pain. Polymorphous lowgrade adenocarcinoma of the salivary gland, plga. Polymorphous lowgrade adenocarcinoma plga has been known as a comparatively less aggressive malignant tumor that predominantly occurs in the minor. Treatment list for polymorphous low grade adenocarcinoma the list of treatments mentioned in various sources for polymorphous low grade adenocarcinoma includes the following list. 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.
Polymorphous low grade adenocarcinoma, salivary gland tumors. Treatments for polymorphous lowgrade adenocarcinoma. Polymorphous lowgrade adenocarcinoma plga is a rare tumor that mostly affects minor salivary glands. Polymorphous low grade adenocarcinoma plga is a minor salivary gland neoplasm that is characterized by morphologic variability, cytologic uniformity, and an infiltrating growth pattern. Examination revealed him to be afebrile with a visual acuity of 612 od and69 os, 6 mm of proptosis, 3mm inferior globe displacement, temporal conjunctivalhyperemia, and a.
Polymorphous lowgrade adenocarcinomamanagement and. We were interested to read the recent report by murphy et a1. The optimal treatment comprises wide surgical excision, often with adjuvant radiotherapy. Hotspot activating prkd1 somatic mutations in polymorphous.
This paper describes the radiographic extent of the lesion and the findings of ct imaging. Polymorphous lowgrade adenocarcinoma of submandibular. Polymorphous lowgrade adenocarcinoma of the vulva and vagina. Polymorphous lowgrade adenocarcinoma plga is one such tumor, misunderstood, underdiagnosed, controversial in nomenclature and unpredictable in behavior. Polymorphous lowgrade adenocarcinoma of salivary gland signs and symptoms may include the following. Polymorphous lowgrade adenocarcinoma of the parotid gland. The term polymorphous lowgrade adenocarcinoma plga was introduced by evans and batsakis 1 in 1984 to describe a cytologically bland but architecturally diverse malignant neoplasm of minor salivary gland origin that had been previously recognized as lobular carcinoma, 2 terminal duct carcinoma, 3 and lowgrade papillary adenocarcinoma. Ckit expression distinguishes salivary gland adenoid cystic. The term polymorphous lowgrade adenocarcinoma plga was introduced by evans and batsakis in 1984 to describe a cytologically bland. If you have problems viewing pdf files, download the latest version of adobe reader. Polymorphous lowgrade adenocarcinoma of 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.
This article is from journal of dentistry tehran, iran, volume 11. The major salivary glands are the parotid glands at the side of the face. To date, this entity has been identified either within the confines. Adenoid cystic carcinoma acc is characterized by persistent, relentless growth and a high rate of eventual metastasis. 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 genetic and rare. Polymorphous low grade adenocarcinoma plga is a rare, asymptomatic, slowgrowing malignant salivary gland tumor. Polymorphous low grade adenocarcinoma plga occurs mostly in minor salivary glands, and there are only a few reports of major salivary gland origin. Polymorphous low grade adenocarcinoma plga is a rare salivary gland neoplasm with an indolent course.