Polymorphous Low-Grade Adenocarcinoma

Polymorphous Low-Grade Adenocarcinoma

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Polymorphous Low-Grade Adenocarcinoma is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • PLGA
  • Lobular Carcinoma of the Minor Salivary Glands
  • Low-Grade Papillary Carcinoma of the Palate
  • Pleomorphic Adenoma
  • Terminal Duct Carcinoma

Disorder Subdivisions

  • None

General Discussion

Polymorphous low-grade 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. The major salivary glands are the parotid glands (at the side of the face, below the ears), the sublingual glands (below the tongue), and the submandibular glands (below the lower jaw). As the name suggests, each of the major salivary glands is of substantial size and visible to the naked eye. There are about 600 to 1,000 minor salivary glands that are microscopic in size. These minor salivary glands are found in the lining (mucosa) of the lips, tongue, and hard and soft palate, as well as inside the nose, cheeks, and sinuses.



Less than one (1%) per cent of all cancers reported in the USA are salivary cancers and, of these, 80% begin in the parotid glands, and about 15% begin in the submandibular glands, leaving only 5% that begin in the sublingual and minor salivary glands. Most of the tumors that start in the major salivary glands turn out to be benign, while most, but not all, of the cancers that start in the minor salivary glands turn out to be malignant.

Symptoms

A lump or mass in the palate or near any of the salivary glands should be seen by a physician as soon as possible. A lingering pain in the area of the salivary glands is a signal to see a physician. A change in the size and/or shape of one of the salivary glands (asymmetry) may be a sufficient reason to see a doctor. Unexplained bleeding in the mouth is also a symptom warranting attention, and any persistent numbness on any part of the face or a weakening of the muscles on one side of the face should be brought to the attention of a physician. Early diagnosis and treatment are important.

Causes

Why a normal cell becomes malignant is not yet fully understood.

Affected Populations

In most instances PLGA affects people in their middle age. The median age at which patients are diagnosed is 57 years, with a range of from 22 to 71 years. For reasons that are unknown, this disease affects slightly more women than men.

Standard Therapies

Diagnosis

The diagnosis of PLGA may be suspected on history and clinical examination, but can be confirmed only by biopsy of the affected tissue.



Treatment

The primary mode of treatment for low-grade stage I tumors of the salivary gland is surgery (resection). Radiation therapy may be prescribed as a supplement in some cases. Palate rehabilitation may be needed depending on the size of the tumor.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

Robins KT, Fu YS. Uncommon Epithelial Tumors of the Oral Cavity. In: Raghaven D, Brecher ML, Johnson DH. Eds. Textbook of Uncommon Cancer. 2nd ed. John Wiley & Sons. New York, NY; 1999:119-20.



JOURNAL ARTICLES

Edwards PC, Bhuiya T, Kelsch RD. C-kit expression in the salivary gland neoplasms adenoid cystic carcinoma, polymorphous low-grade adenocarcinoma, and monomorphic adenoma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2003;95:586-93.



Young S, Leon M, Talerman A, et al. Polymorphous low-grade adenocarcinoma of the vulva and vagina: a tumor resembling adenoid cystic carcinoma. Int J Surg Pathol. 2003;11:43-49.



Sahai K, Kapila K, Dahiya S, et al. Fine needle aspiration cytology of minor salivary gland tumours of the palate. Cytopathology. 2002;13:309-16.



Simpson RH, Pereira EM, Ribeiro AC, et al. Polymorphous low-grade adenocarcinoma of the salivary glands with transformation to high-grade carcinoma. Histopathology. 2002;41:250-59.



Darling MR, Schneider JW, Phillips VM. Polymorphous low-grade adenocarcinoma and adenoid cystic carcinoma: a review and comparison of immunohistochemical markers. Oral Oncol. 2002;38:641-45.



Araujo VC, Loducca SV, Sousa SO, et al. The cribriform features of adenoid cystic carcinoma and polymorphous low-grade adenocarcinoma: cytokeratin and integrin expression. Ann Diagn Pathol. 2001;5:330-34.



Martins C, Fonseca I, roque L, et al. Cytogenic similarities between two types of salivary gland carcinomas: adenoid cystic carcinoma and polymorphous low-grade adenocarcinoma. Cancer Genet Cytogenet. 2001;128:130-36.



Evans HL, Luna MA. Polymorphous low-grade adenocarcinoma: a study of 40 cases with long-term follow up and an evaluation of the importance of the papillary area. Am J Surg Pathol. 2000;24:1319-28.



Asdornato MC, Penna K, Vinoski M. Polymorphous low-grade adenocarcinoma of the oral cavity. N Y State Dent J. 2000;66:28-32.



Nicol KK, Iskander SS. Lobular carcinoma of the breast metastatic to the oral cavity mimicking polymorphous low-grade adenocarcinoma of the minor salivary glands. Arch Pathol Lab Med. 2000;124:157-59.



FROM THE INTERNET

Salivary Gland Cancer (PDQ®). National Cancer Institute. [Patient Version]. Last Modified: 11/19/2003.

www.cancer.gov/cancerinfo/pdq/treatment/salivarygland/patient



Salivary Gland Cancer (PDQ®). National Cancer Institute. [Health Professional Version]. Last Modified: 12/22/2003.

www.cancer.gov/cancerinfo/pdq/treatment/salivarygland/HealthProfessional



Detailed Guide: Salivary Gland Cancer. American Cancer Society. ©2004. Various.

www.cancer.org



Atlas of Head and Neck Pathology: Polymorphous Low-Grade Adenocarcinoma. nd 4pp.

www.med.ohio-state.edu/oto/atlas/polymorphous2.pdf



Polymorphous Low Grade AdenoCA. The Doctor's Doctor. nd. 11pp.

www.dentalcare.com/soap/ce110pc/pg31.htm



What is Salivary Gland Cancer? ©2004 Yahoo! Inc. various.

http://health.yahoo.com/health/cancer_center/acs_crc/salivary_gland/

Resources

Let's Face It

University of Michigan, School of Dentistry / Dentistry Library

1011 N. University

Ann Arbor, MI 48109-1078

USA

Tel: (360)676-7325

Email: faceit@umich.edu

Internet: http://www.dent.umich.edu/faceit



American Cancer Society, Inc.

250 Williams NW St

Ste 6000

Atlanta, GA 30303

USA

Tel: (404)320-3333

Tel: (800)227-2345

TDD: (866)228-4327

Internet: http://www.cancer.org



National Cancer Institute

6116 Executive Blvd Suite 300

Bethesda, MD 20892-8322

USA

Tel: (301)435-3848

Tel: (800)422-6237

TDD: (800)332-8615

Email: cancergovstaff@mail.nih.gov

Internet: http://www.cancer.gov



Support for People with Oral and Head and Neck Cancer, Inc.

P.O. Box 53

Locust Valley, NY 11560-0053

USA

Tel: (516)759-5333

Fax: (516)671-8794

Tel: (800)377-0928

Email: info@spohnc.org

Internet: http://www.spohnc.org



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



Friends of Cancer Research

1800 M Street NW

Suite 1050 South

Washington, DC 22202

Tel: (202)944-6700

Email: info@focr.org

Internet: http://www.focr.org



Cancer Support Community

1050 17th St NW Suite 500

Washington, DC 20036

Tel: (202)659-9709

Fax: (202)974-7999

Tel: (888)793-9355

Internet: http://www.cancersupportcommunity.org/



Lance Armstrong Foundation

2201 E. Sixth Street

Austin, TX 78702

Tel: (512)236-8820

Fax: (512)236-8482

Tel: (877)236-8820

Email: media@livestrong.org

Internet: http://www.livestrong.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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