Polymorphous low-grade adenocarcinoma (PLGA) is a neoplasm that occurs frequently in the mucosa of the soft and hard palates, in the buccal mucosa and in the upper lip and is very rare within the nasopharynx. We present a case of PLGA, which presented as a nasal polyp

Gulati, A

Kaul, R

Kaushik, R

Raina, S

English

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Polymorphous low grade adenocarcinoma-an unusual presentation

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Case Report:
Polymorphous low grade adenocarcinoma-an unusual presentation
Rashmi Kaul, Registrar, Department of Pathology
Anchana Gulati, Assistant Professor, Department of Pathology
Rajni Kaushik, Associate Professor, Department of Pathology, 
Sujeet Raina,Assistant Professor, Dept. of Medicine
Indira Gandhi Medical College, Shimla – 171001, Himachal Pradesh
Address For Correspondence:
Dr Rashmi Kaul,
Fire Officers Building,
Stokes Place, Shimla - 171002
Himachal Pradesh, India.
E-mail: shivanshraina@yahoo.co.in
Citation: Kaul R, Gulati A, Kaushik R, Raina S. Polymorphous low grade adenocarcinoma-an unusual presentation. Online J Health  
Allied Scs. 2010;9(2):18
URL: http://www.ojhas.org/issue34/2010-2-18.htm
Open Access Archives: http://cogprints.org/view/subjects/OJHAS.html and http://openmed.nic.in/view/subjects/ojhas.html
Submitted: May 23, 2010; Accepted: Jul 13, 2010; Published: Jul 30, 2010
Abstract:
Polymorphous low-grade adenocarcinoma  (PLGA) is a neo-
plasm that occurs frequently in the mucosa of the soft and hard 
palates, in the buccal mucosa and in the upper lip and is very 
rare  within  the  nasopharynx.  We  present  a  case  of  PLGA, 
which presented as a nasal polyp.
Key  Words: Polymorphous  low-grade  adenocarcinoma, 
minor salivary gland neoplasm, nasal polyp
Introduction:
Polymorphous low grade adenocarcinomas (PLGA) are minor 
salivary gland neoplasms with a predilection for intraoral sites. 
Extraoral location of the tumor is rare and the nasal tumors are 
less than 1%.[1] It generally involves the palate, but it has also 
been reported in the base of the tongue, upper lip, buccal mu-
cosa, tonsil,  and retromolar  pad.[2] So far,  only 3 cases have 
been reported, where PLGA  presented as a sinonasal mass.
[3,4] Histologically,  PLGA  resembles  pleomorphic  adenoma  , 
benign mixed tumor and to an extent, to adenoid cystic  car-
cinoma.We present a case of PLGA, which presented as a nas-
al polyp.
Case Report:
A male patient presented to the Dept. of ENT with a history of 
nasal obstruction and a single episode of epistaxis. There was 
no history of an upper respiratory tract infection or  trauma. 
Nasopharyngoscopic examination revealed a polypoidal struc-
ture in left nasopharynx. No cervical lymph node enlargement 
was found. The FNAC of the polypoidal structure showed cel-
lular smears. The cells had high N/C ratio, scanty cytoplasm, 
nuclei of variable chromatin density and inconspicuous to con-
spicuous nucleoli.  Beaded fragments of hyaline stroma were 
present between cell clusters.[Fig. 1] A diagnosis of adenoid-
cystic carcinoma was made. Polypectomy was done and sent 
for histopathological examination. HPE revealed mildly pleo-
morphic plump columnar cells arranged in tubular, cribriform, 
solid and fasicular patterns. No area revealed papillary config-
uration. Mitotic activity was low. Basement membrane materi-
al was found associated with the tumor cells. Peripherally the 
tumor cells revealed infiltrative pattern with foci of neurotrop-
ism[Fig  2],  suggesting  a  diaganosis  of  Polymorphous  low 
grade adenocarcinoma. 
Figure-1: Microphotograph showing pleomorphic tumor 
cells with interspersed beaded fragments of hyaline stroma 
present between cell clusters.(x400,Giemsa)
Figure-2: Microphotograph showing mildly pleomorphic 
plump columnar cells arranged in tubular, cribriform, 
solid and fasicular patterns along with basement 
membrane material.(x400,HPE)
1
This work is licensed under a
Creative Commons Attribution-
No Derivative Works 2.5 India License
Online Journal of Health and Allied Sciences
Peer Reviewed, Open Access, Free Online Journal
Published Quarterly :  Mangalore, South India : ISSN 0972-5997
Volume 9, Issue 2; Apr-Jun 2010
Discussion:
Since the original description of these tumors, PLGA has been 
recognized as a distinct salivary gland tumor that has a pre-
dilection to occur in the minor salivary glands and is associ-
ated with  slow growth  and  indolent  biology.  PLGA occurs 
over a wide age range but does not seem to occur in the first or 
second decades of life. There is a nearly 2:1 female to male ra-
tio  for  the  patients.  Clinically,wide  age  range  has been de-
scribed(23 to 94 years). PLGA in women tends to occur at a 
slightly younger age (56.6 years) compared with male patients 
(59.7 years), but there is no statistically significant difference. 
The typical clinical presentation of PLGA is that of an asymp-
tomatic mass located within the oral cavity. Rare intraosseous 
cases have been described. Nasal cavity and nasopharyngeal 
involvement  in PLGA is rare(1% and 0.5%). To  date only  
few cases have been reported in nasal cavity. Clinical sympto-
matology ranges in duration from a few days to 40 years, with 
an average length of symptoms of 27 months.  Patients who 
present with pain, bleeding, or ulceration do not have more ag-
gressive  disease  nor  are  they more  prone  to  develop  recur-
rences. Grossly, the tumor is usually unencapsulated, well cir-
cumscribed, lobular and firm. Microscopically, different archi-
tectural patterns can be seen in various areas. The most com-
mon patterns of growth included tubular, trabecular, solid, and 
cribriform patterns,  with  a  focal  papillary pattern identified 
less frequently. Myxoid change in the background may also be 
seen. In contrast to the architectural polymorphism, the nuclei 
are uniform and bland with absent or negligible mitoses.[5]
The differential  diagnoses of  PLGA are adenoid  cystic  car-
cinoma and pleomorphic adenoma.  Both adenoid cystic  car-
cinoma and pleomorphic adenoma can have similar architec-
tural patterns; however, the cells in ACC tend to be smaller 
with  hyperchromatic  nuclei  and  coarser  chromatin.  Mitoses 
are numerous. The infiltrative growth pattern and neurotrop-
ism are an important diagnostic clue favouring PLGA, when 
the differential includes pleomorphic adenoma. Although, the 
tumor has variable architectural patterns, the cells have min-
imal  cellular  pleomorphism.  Local  recurrences  can  occur. 
Rarely  regional  lymph  node  metastasis  and  distant  (lung) 
metastasis have been documented. PLGAs chiefly involve the 
minor  salivary  glands.  Only  few cases  involving  the  major 
salivary glands have been reported so far..
A definitive  diagnosis  of  PLGA is  established  by detecting 
this  characteristic  histological  feature of  the resected tumor. 
The patient has been followed-up without evidence of recur-
rence and did not undergo radiation therapy. We suggest that 
radiation therapy may be reserved for patients with recurrence 
and regional metastasis.
References:
1. González-Lagunas J, Alasà-Caparrós C, Vendrell-
Escofet G, Huguet-Redecilla P, Raspall-Martin G. 
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2. Charous DD, Cunnane MF, Rosen MR,Keane WK. 
Recurrent  polymorphous  low-grade  adenocar-
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3. Wenig BM, Harpaz N, DelBridge C. Polymorph-
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2


Polymorphous low-grade adenocarcinoma of seromucous glands of the nasopharynx. A report of a case and a discussion of the morphologic and immunohistochemical features.

Polymorphous low-grade adenocarcinoma of the nasal fossa. Med Oral Patol Oral Cir Bucal

Polymorphous low-grade adenocarcinoma of the nasopharynx. Case report and review of the literature. Strahlenther Onkol2000;176:40–2.

Recurrent polymorphous low-grade adenocarcinoma manifesting as a sinonasal mass: a case report. Ear Nose and Throat Journal.

Rashmi Kaul

Anchana Gulati

Rajni Kaushik

Sujeet Raina

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Polymorphous low grade adenocarcinoma-an unusual presentation

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