Undifferentiated carcinoma of nasopharyngeal type arising in the larynx is unusual. This type of carcinoma-which occurs almost exclusively in nasopharynx-is very infrequent in the larynx (0.2%). Till date only 17 cases are reported in the medical literature. We present the clinical and histopathological findings along with the management of one additional case of undifferentiated carcinoma of nasopharyngeal type in the larynx which was managed successfully with radiotherapy

Bansal, S

Gupta, AK

Shankar, A

English

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Undifferentiated Carcinoma of Larynx of Nasopharyngeal Type

Sandeep Bansal

Amit Shankar

Ashok K Gupta

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Case Report:
Undifferentiated Carcinoma of Larynx of Nasopharyngeal Type
Sandeep Bansal, Amit Shankar, Ashok K Gupta,
Department of Otolaryngology, Head and Neck Surgery, Post Graduate Institute of Medical Education and Research, 
Chandigarh, INDIA.
Address for Correspondence:
Dr. Sandeep Bansal,
Assistant Professor,
Department of Otolaryngology,
PGIMER, Sector 12,
Chandigarh – 160012, India.
E-mail: drsandeepb@rediffmail.com
Citation: Bansal S, Shankar A, Gupta AK. Undifferentiated Carcinoma of Larynx of Nasopharyngeal Type. Online J Health Allied 
Scs. 2011;10(1):24
URL: http://www.ojhas.org/issue37/2011-1-24.htm
Open Access Archives: http://cogprints.org/view/subjects/OJHAS.html and http://openmed.nic.in/view/subjects/ojhas.html
Submitted: Jan 31, 2011; Accepted: March 29, 2011; Published: April 15, 2011
Abstract:
Undifferentiated carcinoma of  nasopharyngeal  type  arising in 
the larynx is unusual. This type of carcinoma-which occurs al-
most  exclusively  in  nasopharynx-is  very  infrequent  in  the 
larynx (0.2%). Till date only 17 cases are reported in the medic-
al literature. We present the clinical and histopathological find-
ings along with the management of one additional case of un-
differentiated carcinoma of nasopharyngeal type in the larynx 
which was managed successfully with radiotherapy.
Key Words: Nasophryngeal type of carcinoma larynx; Chemo-
therapy; Rare tumour
Introduction:
In 1978 the World Health Organization (WHO) classified car-
cinomas  of  the  nasopharynx  into  three  histologic  subtypes1: 
squamous cell carcinoma (Type A), non-keratinizing carcinoma 
(Type  B),  and  undifferentiated  carcinoma  of  nasopharyngeal 
type (Type C). The WHO Type C corresponds to the lympho-
epithelial carcinoma (lymphoepithelioma)  Schmincke-Regaud, 
characterized  by  various  degrees  of  lymphocytic  infiltration. 
This  tumor  is  essentially  encountered  in  the  nasopharynx, 
where surface epithelium contains indigenous lymphoid tissue. 
Undifferentiated carcinoma of the nasopharyngeal type is very 
rare in the hypopharynx and larynx. To our knowledge only a 
few have been reported in the English literature.2 We present 
the clinical and histopathologic findings of a case of undifferen-
tiated carcinoma of nasopharyngeal type in the larynx.
Case Report:
A 62 years old man was referred with a six months history of 
progressive  hoarseness and occasional  dysphagia  and odyno-
phagia. He complained of a progressive cough but denied dys-
pnea, hemoptysis and weight loss. He had a 60 pack/year his-
tory of tobacco use and a significant history of alcohol abuse. 
Indirect  laryngoscopy  showed  mucosa  covered  growth  in-
volving left aryepiglottic fold, left arytenoid, left false cord, left 
true cord, and medial wall of left pyriform fossa with restricted 
left vocal cord mobility.  There was left level II 2x3 cm hard 
mobile non tender lymph node. Fine needle aspiration cytology 
from the neck node revealed squamous cell  carcinoma meta-
static. The results from metastatic work up, including a CT scan 
of the chest and abdomen were negative.  A computed tomo-
graphy of  neck confirmed the location  of  the  mass and also 
showed involvement of the preepiglottic space and thyroid car-
tilage  invasion  (Fig 1).  Endoscopy under general  anaesthesia 
was then performed and findings were confirmed. Biopsy was 
taken from the representative sites which revealed undifferenti-
ated carcinoma of nasopharyngeal type (anaplastic variant) (Fig 
2). The primary tumor received a total dose of 68.4 Gy in 38 
fractions of 1.8 Gy each, which were delivered over 54 elapsed 
days. The draining regional cervical lymphatics received a dose 
of 50.4 Gy in 28 fractions of 1.8 Gy, which were delivered over  
a  similar  period  of  time.  The  patient  tolerated  therapy  with 
modest xerostomia and altered taste. Four months from comple-
tion of therapy, fiberoptic evaluation of the larynx showed near 
complete regression of the submucosal mass in the medial as-
pect of the left pyriform sinus. There is modest edema of the 
supraglottic  laryngeal structures. The left hemilarynx remains 
fixed. There is no clinical evidence of regional nodal recurrence 
or any clinical evidence of distant metastases at the time of last  
follow-up.
Fig. 1: Computed tomography showing left hemilarynx sub-
mucosal tumor mass
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 10, Issue 1; Jan-Mar 2011
Fig. 2: H& E staining (original magnification x 25) showing 
pleomorphic  tumor  cells  surrounded  by  mononuclear  in-
flammatory cells.
Discussion:
Lymphoepithelioma  is  diagnosed  most  often  in  the 
nasopharynx.  It  compromises  40% of  all  nasopharyngeal  tu-
mors and is associated with Epstein - Barr virus (EBV).3 EPV 
genomes  are  found  in over  90% of  nasopharyngeal  carcino-
mas.4 It is postulated that EBV causes lymphocytes to undergo 
blastic  transformation,  and  a  typical  cellular  arrangement  of 
anaplastic cells dispersed among a lymphocyte infiltrate is seen. 
Although uncommon, lymphoepithelioma has also been repor-
ted  in  the  nasal  fossae,  maxillary  sinus,  tongue  base, 
parapharyngeal  area, tonsil,  and thymus.3 Lymphoepithelioma 
of the larynx is extremely rare. Micheau et al, 2 noted a 0.2% 
occurrence rate from all tumors of the larynx. laryngeal lymph-
oepitheliomas is unknown; although it may arise from lymphat-
ic tissue in the laryngeal ventricle. In an attempt to evaluate the 
modes  of  invasion  of  laryngeal  and  pharyngeal  carcinomas, 
Micheau et al2 observed a single or double laryngocele in 70% 
of the 2430 laryngectomy and pharyngolaryngectomy surgical 
specimens. Microscopic evaluation of the laryngoceles showed 
cylindrical  or  squamous  epithelium with  organized lymphoid 
tissue,  typical  of  the  histology  seen in  lymph  nodes and the 
lymphoid structures of Waldeyer’s ring. This laryngeal lymph-
oepithelial tissue has been proposed to be a true tonsil  of the 
larynx and is potentially the site of origin of lymphoepithelioma 
of  the  larynx1.  Alternatively,  Toker  and  Peterson5 postulated 
that these lesions may arise from active basal epithelium in the 
larynx, which is similar to epithelium found in tonsillar crypts.
Although  smoking  is  not  considered  to  be  a  risk  factor  for  
lymphoepitheliomas of the nasopharynx, it may play a role in 
the larynx. In 1921, Regaud and Schmincke each described, the 
pathology of lymphoepithelioma separately3. Regaud described 
nests of nonkeratinizing squamous cells embedded in a lymph-
oid stroma, whereas Schmincke noted isolated transitional cells 
scattered in lymphoid  tissue which resembled  a sarcoma.3 In 
general, lymphocytes are believed to be a nonneoplastic com-
ponent of a lymphoepithelioma. When metastasis occurs, only 
the epithelial component  of the tumor  is found at the distant 
site. Diagnosis can be difficult because the tumors may arise 
from hidden, submucosal sites. Micheau et al2 described a pa-
tient who underwent two negative biopsies before a supraglottic 
laryngectomy was performed. Pathology showed a lymphoep-
ithelioma  in an intramural  diverticulum without  ulceration  of 
the mucosa.’ Laryngeal lymphomas may also present as submu-
cosal masses. In a summary of 18 cases of malignant lymph-
oma confined to the upper aerodigestive  tract,  Friedberg and 
Has6 reported four cases in which the lesions occurred submu-
cosally  in  the  hypopharynx.  Most  lymphomas  of  the  larynx 
present  as  smooth  supraglottic  masses,  usually  involving  the 
epiglottic or aryepiglottic fold.7 Lymphomas and lymphoepithe-
liomas may be difficult to distinguish clinically. Lymphoepithe-
liom of the larynx share many characteristics with those of the 
nasopharynx. Both have significant potential for early regional 
and distant metastases. Sites of distant metastases included the 
mediastinum, lung, and abdomen.
Lymphoepithelioma is a highly radiosensitive tumor. Stanley et 
al8 used  primary  radiation  therapy  in  four  patients  with 
lymphoepitheliomas  of  the  larynx  with  good  local  control. 
Ferlito3 also reported a case of lymphoepithelioma of the larynx 
treated solely with radiation therapy in which his patient died in 
less than one year as a result of distant metastases. Our patient 
has  had  a  favorable  clinical  and  radiographic  response  to 
radiation  therapy  and  currently  shows  no  evidence  of 
locoregional  recurrence  or  distant  metastases.  Adjuvant 
chemotherapy  may  be  useful.  The  value  of  neoadjuvant  or 
concomitant  chemotherapy  for  lymphoepithelioma  is  still 
unknown.
Conclusion:
Lymphoepithelioma of the larynx, although is rarely found in 
the  larynx,  it  is  essential  to  distinguish  this  lesion  from 
squamous cell  carcinoma.  Radiation therapy is recommended 
as the sole treatment modality for local disease. Chemotherapy 
may  also  play  a  role  in  patients  with  advanced  disease. 
Carcinoma  nasopharyngeal  type  of  the  larynx  must  be 
considered as  a  distinct  'clinicopathological  entity'  and close 
cooperation  between  the  clinician  and  the  pathologist  is 
essential for the correct diagnosis of these tumour as regards to 
the correct classification and therapy.
References:
1. Shanmugaratnam K, Sobin LH. Histologic typing of 
upper  respiratory  tumor,  World  Health  Organiza-
tion, Geneva. 1978.
2. Micheau C, Luboinski B, Schwaab B et al Lympho-
epitheliomas of the larynx (undifferentiated carcino-
mas  of  nasopharyngeal  type).  Clin  Otolaryngol 
1979;4:43-48.
3. Ferlito  A.  Primary  lymphoepithelial  carcinoma  of 
the hypopharynx. J Laryngol Otol 1977;91:361-367.
4. Chapman  KE,  Patton  LL.  Nasopharyngeal  car-
cinoma: A 15-year retrospective study. Special Care  
in Dent 1993;13:163-170.
5. Toker C, Peterson DW. Lymphoepithelioma of the 
vocal cord. Arch Otolaryngol 1978;l04:161-162 .
6. Friedberg  SA,  Hass  GM.  Malignant  lymphoma 
presenting as primary pharyngeal lesion. Presbyt St  
Lukes Hosp Med Bull 1962;1:2-7.
7. Friedman M, Toriumi D. Chapter 40. In  Paparella 
M,  Shumrick  D,  Gluckman  J,  et  al  (eds): 
Otolaryngology,  Philadelphia, PA; Saunders. 1980. 
p 2512.
8. Stanley RJ, Weiland LH, DeSanto LW et al. Lvm-
phoepithelioma (undifferentiated carcinoma) of the 
laryngohypopharynx.  Laryngoscope 1985;95:1077-
1081.
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Undifferentiated Carcinoma of Larynx of Nasopharyngeal Type

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