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Medicina Oral, Patología Oral y Cirugía Bucal (Ed. impresa)

versión impresa ISSN 1698-4447

Med. oral patol. oral cir. bucal (Ed.impr.) vol.10 no.4  ago./oct. 2005


Polymorphous low-grade adenocarcinoma of the nasal fossa
Adenocarcinoma Polimorfo de Bajo Grado de fosa nasal


Javier González Lagunas (1), Cristian Alasà Caparrós (1), Gerard Vendrell Escofet (1)
Pere Huguet Redecilla (2), Guillermo Raspall Martin (1)

(1) Departamento de Cirugía Oral y Maxilofacial
(2) Departamento de Anatomía Patológica
Hospital Universitari Vall d´Hebrón
Paseo Vall d´Hebrón 129, 08035 Barcelona

Dr. Javier González Lagunas
C/ Casanova 101, Principal.
08011 Barcelona.
Teléfono : 948%537965
Fax : 934514480

Received: 23-05-2004 Accepted: 30-07-2004

González-Lagunas J, Alasà-Caparrós C, Vendrell-Escofet G, Huguet-Redecilla P, Raspall-Martin G. Polymorphous low-grade adenocarcinoma of the nasal fossa. Med Oral Patol Oral Cir Bucal 2005;10:367-70.
© Medicina Oral S. L. C.I.F. B 96689336 - ISSN 1698-4447


An unusual case of a T4N2CMx polymorphous low grade adenocarcinoma located in the nasal fossae and extending to the pterygoid area is presented. The primary tumor was excised through a Lefort I maxillotomy and the neck was managed with a supraomohyoid neck dissection. Adjuntive postoperative radiotherapy was also administered to the patient.

Key words: Minor salivary glands, salivary glands neoplasms, polymorphous low-grade adenocarcinoma, nasal fossa neoplasm.



Se presenta un inusual caso de Adenocarcinoma Polimorfo de Bajo Grado en estadio IV (T4N2CMx) localizado en la fosa nasal con extensión hacia región pterigoidea. El tumor primario fue tratado mediante una resección del mismo a través una osteotomía de maxilar superior tipo Lefort I y a nivel cervical se realizó un vaciamiento ganglionar cervical supraomohioideo homolateral. Posteriormente recibió tratamiento coadyuvante con radioterapia postoperatoria.

Palabras clave: Glándulas salivares menores, neoplasias glándulas salivares, adenocarcinoma polimorfo de bajo grado, neoplasia fosa nasal.



The term Polymorphous low-grade adenocarcinoma (PLGA) was introduced in 1984 by Evans and Batsakis (1) to describe a group of tumors of the minor salivary glands that shared a varied histomorphology and a malignant and indolent behaviour. Other terms include Lobular carcinoma and Terminal Duct Carcinoma. Since its introduction in1984, a number of series of this tumor have been presented in the literature (2-4).

Extraoral location of the tumor is rare and the nasal tumors are less than 1%. We present an infrequent case of PLGA located in the nasal fossa and that was approached through a Lefort I maxillotomy.


A 67 cases old female attended our department with a swelling in the posterior aspect of her right hemipalate. A biopsy had been performed in another center with a diagnosis of PLGA. A right submandibular adenopathy was detected Her previous clinical history revealed external radiotherapy in the head and neck area during childhood for an unknown reason.

An orthopantomogram was normal. A CT scan was performed and a T4N2cMx lesion located in the right nasal fossa was diagnosed A fine needle aspiration citology of the neck node was performed and a diagnosis of malignancy, probably PLGA was obtained. An MRI showed a well defined nasal fossa lesion arising in the floor of the nasal fosssa and extending to the right pterigoid plate (Fig.1).

Surgery under general anesthesia was scheduled. The tumor was approached through a Lefort I osteotomy. The patient was a long-standing edentulous patient so an extreme atrophy of the upper jaw was observed (Fig 2). In the neck a bilateral supraomohyoid dissection was performed. Resection of the tumor was performed and reposition of the upper jaw with precontoured 1,5mm miniplates was done.

Biopsy confirmed the diagnosis of PLGA (Fig.3), but remnants of the tumor were found in the right pterigomaxillary area. In the neck, regional metastasis (2/16) were found in the right neck.

Conventional radiotherapy was done in the pterigoid area and a control MR showed a marked reduction of the tumoral remnants. Those remanants were, anyhow, reapproached surgically and the tuberosity and pretigomaxillary defect were reconstructed with a Bichat fat flap.

Two years after treatment, the patient is free of disease.


Polymorphous low-grade adenocarcinoma (PLGA) is a salivary gland malignant neoplasm arising almost exclusively in the minor glands (3,6). Most of the cases have been described in the oral cavity, specially in the palate (hard and soft) (57%), lips(17%) and buccal mucosa (16%) being the extraoral location a rarity. The age range is 21-94, but most cases appear in the sisth and seventh decade (3-7)

PLGA requires differentiation from ACC. PLGA is a slow growing tumor with prolonged survival. Although tumor can recur, distant metastasis have not been reported. Eighty percent of patients are alive with no evidence of disease, 24% have tumor recurrrence and 6% have metastasis to the lymph nodes(3-7). Only one case of death for disease has been described (2)

Nasal cavity and nasopharynx involvement in PLGA is rare (1 and 0,5%)(7) To date few cases have been detected in the nasal area (8,9,10). In one instance, the tumor has been associated to un indifferentiated carcinoma (9). One of the cases showed an intracranial extension (8).

The general incidence ot tumors in the nasal area according to AFIP is as follows: the most frequent tumor are papillomas (545/1635), soft tissue tumors (332/1635), and glandular tumors (311) (10). In the nasal fossae and the paranasal sinuses a number of seromucosal glands arise, being more abundant in the lateral nasal cavity walls. Those glands markly ressemble minor salivary glands. Even though their secretion is not saliva, from an histologic point of view , there is only an academic difference (11).

In its series of glandular tumors the AFIP (Armed Forces Institute of Pathology)(10) founds the following distribution: high grade adenocarcinomas (29,9%), followed by mixed tumors (23,4%), low grade adenocarcinomas (including acinic cells (21,5%), adenoid cystic carcinoma (17,3%), and mucoepidermoid carcinomas (5,4%) . Li in a series of 16 cases found a 93,75% incidence of malignancies, versus a general incidence of 51,1% of benignity when considering all locations (11). No case of PLGA has been described in those series.

Regarding treatment planning , being the tumor a slow growing malignant lesion, a wide local surgical excision should be performed. Due to the location of this case, the Lefort I access osteotomy proved to be an excellent instrument for management of th lesion. The need of neck dissection treatment should be limited to those patients with positive nodes, as was the case presented. The use of chemo and radiotherapy is by the moment controversial. In one case (8) the only treatment was radiotherapy that showed a 51 months survival.


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9. Lloreta J, Serrano S, Corominas JM, Ferres-Padro E. Polymorphous low-grade adenocarcinoma arising in the nasal cavities with an associated undifferentiated carcinoma. Ultrastuct Pathol 1995;19:365-70        [ Links ]

10. Wenig BM, Harpaz N, Del Bridge C. Polymorphous low-grade adenocarcinoma of seromucous glands of the nasopharynx: a report of a case and discussion of the morphologic and inmunohistochemical features. Am J Clin Pathol 1989;92:104-9.        [ Links ]

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