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OJHAS Vol. 10, Issue 1:
(Jan-Mar 2011) |
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Undifferentiated
Carcinoma of Larynx of Nasopharyngeal Type |
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Sandeep Bansal, Amit Shankar, Ashok K Gupta,
Department of
Otolaryngology, Head and Neck Surgery, Post Graduate
Institute of Medical Education and Research, Chandigarh,
INDIA. |
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Address for Correspondence |
Assistant Professor,
Department of Otolaryngology,
PGIMER, Sector 12,
Chandigarh – 160012, India.
E-mail:
drsandeepb@rediffmail.com |
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Bansal S, Shankar A, Gupta AK. Undifferentiated
Carcinoma of Larynx of Nasopharyngeal Type. Online J Health Allied Scs.
2011;10(1):24 |
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Submitted: Jan 31,
2011; Accepted: March 29, 2011; Published:
April 15, 2011 |
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| Abstract: |
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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.
Key Words:
Nasophryngeal type
of carcinoma larynx; Chemotherapy; Rare tumour
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In 1978 the World Health Organization
(WHO) classified carcinomas 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 lymphoepithelial 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 undifferentiated
carcinoma of nasopharyngeal type in the larynx.
A 62 years
old man was referred with a six months history of progressive hoarseness
and occasional dysphagia and odynophagia. He complained of a progressive
cough but denied dyspnea, hemoptysis and weight loss. He had a 60 pack/year
history of tobacco use and a significant history of alcohol abuse. Indirect
laryngoscopy showed mucosa covered growth involving 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 metastatic. The results
from metastatic work up, including a CT scan of the chest and abdomen
were negative. A computed tomography of neck confirmed the location
of the mass and also showed involvement of the preepiglottic space and
thyroid cartilage invasion (Fig 1). Endoscopy under general anaesthesia
was then performed and findings were confirmed. Biopsy was taken from
the representative sites which revealed undifferentiated 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 completion
of therapy, fiberoptic evaluation of the larynx showed near complete
regression of the submucosal mass in the medial aspect 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.
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Fig. 1: Computed tomography
showing left hemilarynx submucosal tumor mass |
Fig. 2: H& E staining (original
magnification x 25) showing pleomorphic tumor cells surrounded
by mononuclear inflammatory cells.
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Lymphoepithelioma is diagnosed
most often in the nasopharynx. It compromises 40% of all nasopharyngeal
tumors and is associated with Epstein - Barr virus (EBV).3
EPV genomes are found in over 90% of nasopharyngeal carcinomas.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 reported 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 lymphoepitheliomas
is unknown; although it may arise from lymphatic 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 lymphoepithelial 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 lymphoid 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 component
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 patient who underwent two negative
biopsies before a supraglottic laryngectomy was performed. Pathology
showed a lymphoepithelioma in an intramural diverticulum without ulceration
of the mucosa.’ Laryngeal lymphomas may also present as submucosal
masses. In a summary of 18 cases of malignant lymphoma confined to the
upper aerodigestive tract, Friedberg and Has6 reported four
cases in which the lesions occurred submucosally in the hypopharynx.
Most lymphomas of the larynx present as smooth supraglottic masses,
usually involving the epiglottic or aryepiglottic fold.7
Lymphomas and lymphoepitheliomas may be difficult to distinguish clinically.
Lymphoepitheliom 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.
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.
- Shanmugaratnam K, Sobin LH. Histologic typing of upper respiratory tumor, World Health
Organization, Geneva. 1978.
Micheau C, Luboinski B,
Schwaab B et al Lymphoepitheliomas of the larynx (undifferentiated
carcinomas of nasopharyngeal type). Clin Otolaryngol 1979;4:43-48.
Ferlito A. Primary lymphoepithelial
carcinoma of the hypopharynx. J Laryngol Otol 1977;91:361-367.
Chapman KE, Patton LL. Nasopharyngeal
carcinoma: A 15-year retrospective study. Special Care in Dent
1993;13:163-170.
Toker C, Peterson DW. Lymphoepithelioma
of the vocal cord. Arch Otolaryngol 1978;l04:161-162 .
Friedberg SA, Hass GM. Malignant
lymphoma presenting as primary pharyngeal lesion. Presbyt St Lukes Hosp
Med Bull 1962;1:2-7.
Friedman M, Toriumi D. Chapter
40. In Paparella M, Shumrick D, Gluckman J, et al (eds): Otolaryngology,
Philadelphia, PA; Saunders. 1980. p 2512.
Stanley RJ, Weiland LH,
DeSanto LW et al. Lvmphoepithelioma (undifferentiated carcinoma) of
the laryngohypopharynx. Laryngoscope 1985;95:1077-1081.
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