The carcinoma of the nasopharynx is not uncommon. However its early detection is quite uncommon. The bizarre presentation of Nasopharyngeal carcinoma is due to the anatomical relations of the nasopharynx. The fossa of Rasenmuller, a lateral extension of the nasopharynx, is the commonest site of malignancy. The behaviour of the tumour in relation to the anatomy of this area is mainly responsible for the bizarre presentation and accounts for the difficulty in diagnosis. The nasopharynx is a difficult site for examination and some neoplasms may evade diagnosis even atnecropsy. This may be due to the tumour being small and inconspicuous as well as situated in a hidden corner of this concealed space. The symptoms in the early stage are vague and simulate many more Prof. A. Sinha, M. S. F. R. C. S.,D.L.O. Dr. B.M. Abroi, M. S. Dr. S. Gupta, M.B., B. S. From the Tumour Clinic, Department of Otolaryngology, All India Institute of Medical Sciences, New Delhi-16. Read at Twentieth annual Conference of the association o' Otolaryngologists of India 9-11 May 1968 held at Calcutta. Received for publication on 11.5.68.
common benign conditions. Symptoms occurring in later stages are dueto cervical lymph node metastasis or infiltration into the surrounding tissues, while the primary in the nasopharynx remains small, quiet and unobtrusive, and may tax the diagnostic acumen of the clinician. Palpation, the time honoured method of examination; is uncomfortable to the patient and misleads the examiner, small lesions are missed and large lesions are easily visualized. The instruments used to view thenasopharynx include the posterior Rhinoscopic mirror, Yankhauers'' speculum and the electric nasopharyngoscope. All these have blind spots, and the commonest site, the fossa of Rosenmuller, is especially elusive. Radiology is of litte help in early diagnosis, small lesions do not throw a soft tissue shadow, large lesions are visualized clinically and erosion of bones indicates an advanced lesion. Biopsy is difficult and blind, and may not be conclusive on first attempt. The concealed site and small size of tumour, the presence of submucous 17
lymphoid tissue may be contributory to the negative biopsy: It may be repeated and finally done by a transplatal approach. Anaplasia makes histological interpretation and classification difficult. Clinical Material ;
to different specialities. An unexplained cervical lymph node metastasis referred for irradiation from outside was sent to us from the radiotherapy department. Initial Symptom :
The main presenting symptoms The present papers is based on a were local i. e. nasal and aural followclinico-pathological study of 40 cases ed by cervical (lymph node) masses of malignant neoplasms involving the and neuro-ophthalmological symptoms. nasopharynx. These patients attended Comparitive figures of this classificathe out-patient department and the tion by Godtfredson (1944) are shown Tumour Clinic of the Department of in table No. 2. Otolaryngology of All-India Institute The nasal and aural symptoms are of Medical Sciences, New Delhi. The incidence of these neoplasms common to many benign conditions. was about 6.6% of all malignant tu- The symptomless cervical masses are often not alarming to the patient. mours of the head and neck. The male to female ratio was 4:1. A large In India, the patients often wait for number of patients were in the youn- the proptosis to subside spontaneously ger age group, 45% being below 40 before consulting a doctor. This is often responsible for a long delay years. between the onset of the presenting A minority of cases in our series, symptom and presentation to the 15 out of 40, visited the E.N.T. Dehospital. Surprisingly more than 50% partment on their first attendance, of of the cases reported to the E. N. T. these 5 were missed at the first examispecialist after 6 months (Table No.3). nation. (Table No. 1). Cervical The most important evidence suglymphadenopathy, headaches and neuralgia, cranial nerve palsies, pro- gestive of the bizarre presentation ptosis etc. made the patients present includes a galaxy of investigations Table No. 1 Department First Attended Department 1. E. N. T. 2. General Surgery 3. Neurology & Neurosurgery 4. Ophthalmology 5. Medicine 6. Dental 7. Radiotherapy Total
No. of cases
37.5 25 15
3 1 1
7.5 2.5 2.5
18 Ind. J. Otol. Vol. XXI, No. 1, March, 1969
and a variety of treatment these tubuculous therapy for bilateral cervipatients have to undergo before a cal masses demonstrates a gross undefinitive diagnosis is made.. This is awareness of this condition resulting apparent from table No. 4 and 5. in delay in diagnosis. P. E. G. was done in two cases. AntiTable No. 2 Main Presenting Symptoms Presenting symptom AIIMS 1967
Amritsar 1963 Godtfredson 1944
Local 18 a) Rhinologic 12 b) Otologic 6 2. Cervical Lymph nodes 14 3. Ophthalmoneurologic 8 a) Neurologic 5 b) Ophthalmic 3
45 30 15
40.8 26.0 14.8
53.8 30.8 23.0
20 12.5 6.5
Table No. 3 Duration of Presenting Symptom Before Definitive Diagnosis Duration in months 0-1 1-3 3-6 6-12 13-24 25-36 37 and more
No. of cases — — — — — — —
3 9 8 8 8 3 1
Minimum 15 days
Maximum 4 years 40
Table No. 4 Causes of Delay In Diagnosis S. No. 1. 2. 3. 4. 5. 6. 7. 8.
Causes Delay in attending on the part of patients. Cervical lymphadenopathy investigations Neurological investigations Negative Biopsies Ophthalmic investigations Empirical anti-tubercular therapy for cervical lymphadenopathy. Undiagnosed Epistaxis Investigations and treatment of P. U. O.
Nasopharyngeal carcinoma/Sinha at el
No. of cases — — — — —
17 6 5 5 4
— — —
4 4 2 19
Clinical Findings on Presentation : The presenting symptoms were usually in combination and seldom solitary. It is apparent from Table No. 6 that most of the patients often presented late and with a large number of symptoms—the commonest being a mass in the neck. Despite there being only 5 cases with initial neurological symptoms and 3 cases with initial ophthalmological symptoms, 11 cases manifest neurological and 8 cases ophthalmological signs and symptoms in their clinical picture on presentation. (Table No.7 & 8). Difficulties in Clinical Diagnosis The Difficulties in diagnosing nasopharyngeal malignancies preclude early diagnosis. The malignant neoplasma of the nasopharynx are the ones most frequently missed in the early stages. (Table No. 9). Radiography : A mass in the nasopharynx did not add to the clinical findings in some cases and was absent when the primary was small. The destruction of bones in the base of the skull was a
significant finding. Sinusitis is nonspecific and was probably due to nasal obstruction. (Table No. 10). Cytodiagnosis : Exfoliative Cytology had been an important guide both in diagnosis and follow up, as can be seen from Table No. 11 as well as a case report (no. 1.) Biopsy and Histopathology : The initial biopsy was usually blind, occassionally this was repeated by various colleagues in the department to test their skill, which reveals the difficulty in confirming the clinical diagnosis (Table No. 12). On a few occasions the transpalatal approach for biopsy had to be resorted to for final confirmation. In some cases the examination of the nasopharynx revealed the primary after a metastatic lymph node had been excised fo i biopsy. The histopathology of malignant tumours of the nasopharynx showed a wide variety of epithelial types. Sarcomas were uncommon. Some rare and interesting tumours were encountered as shown in table No. 13. The varied histopatbological termi-
Table No. 5 Treatment Received Due To Erroneous Diagnosis S. No. 1. 2. 3. 4. 5. 6. 7. 8.
Treatment Nasal packing and cauterization for epistaxis. Symptomatic and indigenous treatment Anti-tuberculous therapy for cervical lymphadenopathy. A. W. O. and Antrostomy Irradiation for cervical masses Caldwell Luc operation Bronchoscopy Anti-allergic treatment
No. of cases
— — — — — —
4 4 3 1 1 1*
• The patient was found to have blood eosinophilia by a practitioner 20 Ind. J. Otol. Vol. XXI, No. 1, March, 1969
Table No. 6 Distribution of Symptoms and Phycial Signs in 40 Cases of Malignant Neoplasms of Nasopharynx S. No. 1. 2. 3. 4. 5. 6. 7. 8. 9. '10. 11. 12. 13. 14. 15. 16. 17. 18. 19.
Symptoms and signs
Mass neck (cercical lymph nodes) Nasal obstruction and mass nose. Blood stained nasal discharge and bleeding nose. Headache Facial neuralgia Earache Toothache Change in voice, bulge & restricted movement of soft palate, (Nasality & Hoarseness) and nasal regurgitation. Tinnitus, impaired hearing, discharging ears, blue drum, retracted T.M. and perforation. Proptosis, Ptosis, Diplopia, Squint, epiphora and visual defects. Dysphagia and odynophagia Trismus Swelling temple and cheek Facial assymmetry Fever and cough with expectoration, haemoptysis. Loss of weight and weakness Mass nasopharynx and ulcer nasopharynx Multiple cranial nerve palsies Expansion bridge of nose.
Total No. of cases.
6 4 3 2 0
24 10 7 5 1
3 3 1 2 1
8 8 4 3 3
1 5 0
35 11 3
Table No. 7 Ophthalmic Manifestation in 8* Patients S. No. 1. 2. 3. 4. 5. 6. 7.
• Total exceeds 8 as more than one manifestations were seen in some patients. 1
Nasopharyngeal Carcinoma!Sinha at el
^ ^' ^ Y1 o
1^, 3`^ li
Ind. J. Otol. Vol. XXI, No. 1, March, 1969
Table No. 9. Major Stumbling Blocks in Early Diagnosis of Nasopharyngeal Cancer. 1. Anatomy:
2. Symptomatology :
Nasopharynx difficult site for examination. Growths evade early diagnosis and even at necropsy : i) inconspicuous and small. ii) situated in a hidden corner of this concealed space (Fossa of Rosenmuller). Vague especially in early stages. Mostly simulate other common benign conditions. Tumour may remain silent or present bizarre symptoms due to spread and may tax the diagnostic acumen of clinicians. Primary often small, quiet and unobtrusive. Rarely diagnosed when localized to nasopharynx only. Prominent signs due to metastasis.
-4. Diagnostic Aids:
i) Palpation : Time honoured method, unsatisfactory, uncomfortable to patients, misleads investigator. Small lesions often missed, large ones easily visualized. Bleeding from normal or abnormal mucosa obscures the view. ii) Instruments : includes posterior rhinoscopic mirror, Yankauer's speculum and electric nasopharyngoscope. All have blind spots. The common site posterolateral wall is specially elusive.
Little help in diagnosing early lesions. Erosion of bones indicates already advanced lesion.
S. Biopsy and Histopathology :
Difficult, may not be conclusive at first attempt. Must be repeated till clinicla suspicion is proved. Rapidly metastasizing tumour, biospy often from cervical lymph nodes and primary ignored. Anaplasia makes interpretation and histological classification difficult. Table No. 10
Significant Findings on Radiological Examination in 30 Cases. 3. No.
1. Mass in nasopharynx 2. Destruction of Bone (base of skull etc.) 3. Sinusitis 4. Involvement of Optic foramen 5. Metastasis chest b. No significant finding Nasopharyngeal Carcinoma!Sinha at el
No. of cases. —
— — —
17 9 15 3
Table No. 11 Evaluation of Exfoliative Cytology in Diagnosis of Nasopharyngeal Cancer. No. of cases. Total number of cases done 16 True positive 13 2 — True negative 1 False negative 0 False positive Not done 24 Total 40 —
Table No. 12 Role of Surgery in the Management of Nasopharyngeal Cancer. No. of cases. Procedure S. No. — 35 Multiple Biopsie s=8 1. Biapsy primary growth i) Nasal Route (Blind and mirror control) — 23 ii) Post nasal (after retraction of palate) — 10 — iii) Transpalatal 13 Biopsy metastatic lymph node. 2. Table No. 13. Histopathological Diagnosis S. No. Histopathology Report 1. Primary Tumour i) Poorly differentiated carcinoma ii) Anaplastic carcinoma iii) Transitional cell carcinoma iv) Squamous cell carcinoma -v) Lymphoepithelioma vi) Papillary adeno-carcinoma vii) Malignant melanoma viii) Malignant Lymphoma ix) Reticulum cell sarcoma — x) Inconclusive xi) Report not available xii) Negative :
nology used was confusing, but they bore no correlation to the clinical behaviour of the neoplasm. Case Reports : Case No. 1—R.C.K., a 54 year old male presented in the Neurology Department with pain and cranial nerve palsies. He was referred to the E.N.T. Department where history revealed that the onset of illness was with dysphagia 2 years ago accompanied by sanguinous nasal discharge. He had neuralgic pain (R) half of the face and pain (R) ear for three months. On examination there were multiple cranial nerve paralysis, the motor division of (R) 5th, 10th, 11th and 12th cranial nerves were involved. There was a small, firm, mobile, nontender lymph node in the (L) jugulodigastric group, and a tumefaction on the (R) side of the nasopharynx. Cytology of a smear from the nasopharynx revealed malignant cells, a biopsy however taken through the nose was reported chronic inflammatory tissue. Another blind biopsy was negative, and the excised (L) jugulo-digastrie lymph node showed sinus histiocytosis. Biopsy through the transpalatal approach revealed undifferentiated carcinoma of the (R) fossa of Rasenmuller. Comments : The clinical suspicion of malignancy of nasopharynx was high. The positive exfoliative cytology was a pointer. Repeat biopsies from the primary as well as biopsy of the lymph node had to be done to prove the clinical impression supported by exfoliative cytology. The importance of transpalatal approach needs emphasis when blind biopsies have been negative. Nasopharyngeal Carcinoma/Sinha at el
Case No. 2. : G. D. S., a 20 year old male was referred from the Neurology Clinic. for investigation of his deafness. His illness started with hoarseness. 9 months ago accompanied by pain,. tinnitus and deafness (L) ear and followed by occassional epistaxis for 2. months and squint (L) eye for 20 days. On examination there was a bluedrum on the (L) side. The Audiogram showed sensorineural deafness. A mass was seen in the nasopharynx, a biopsy from which was reported as. undifferentiated carcinoma. Comments : The patient presented to thedepartment of Neurology and was referred to the E.N.T. department for otoneurological tests. When a detailed. E.N.T. examination was done a nasopharyngeal mass was discovered. This case baffled the neurologist. The aural and nasal symptoms as well as multiple cranial nerve palsies could haveindicated nasopharyngeal carcinoma. Case No. 3 B.S., a 56 year old male was under the treatment of a general practitioner for one year, who had on routineexamination of blood found eosinophilia. The patient felt a mass in thenasopharynx himself by finger palpation and presented to the Otolaryngology Department. - The history started with a cervical mass behind the angle of jaw on the (R) side 4 years ago, followed by intermittent tinnitus and deafness for 1 year. On examination a solitary firm mobile, non-tender (R) jugulo digastric node was palpated_ A fungating mass from the (R) lateral wall of the nasopharynx extending into the soft palate was seen and 25
revealed poorly differentiated squamous cell carcinoma on biopsy. Comments : Palpation of the nasopharynx is an uncomfortable procedure and it is very unusual for a patient to do so. The eosinophilia was discovered accidentally by a general practitioner and ,diverted attention, from the important symptoms and signs including tinnitus and deafness. The awareness of this entity may have led to an early diagnosis of this serious condition. Case No. 4 V. T., a 18 year old girl had been having troublesome epistaxis. She was treated with nasal decongestants, packing as well as cauterization. She had been having nasal obstruction as long as she could remember. Headache with bleeding from both nostrils started 2 year ago. On .examination she had nasal obstruction more on the (L) side with a pink polypoidal mass in the posterior part of (L) nose. This mass was extending on to the (L) lateral wall of the nasopharynx. It was suspected to be a benign condition and excised under general anesthesia by transpalatal approach, the biopsy report was lympho—epithelioma. Comments : The age, sex and symptomatology were misleading. The condition was diagnosed on biopsy following an attempt to excise the mass. This case also baffled the E.N.T. surgeons. Case No. 5
M. S., an 11 year old boy was admitted in the neuro-surgery ward as
a case of brain abscess. An E. N. T. consultation was requested and the history revealed fever with headache and vomiting for I5 days starting 2j months ago, followed by pain (L) in temporal region and ptosis of the (L) eye and bleeding from the nose 4 days prior to admission. On examination there was cranial nerve paralysis of the (L) 3rd, 4th, 6th and 5th cranial nerves, bulging of the palate with restricted mobility, and firm discreetc mobile jugulo-disastric nodes on both sides. On examination there was (L) nasal obstruction with mass attached to the (L) lateral wall of the nasopharynx. X-ray showed thinning of the dorsum sellae and floor of the pituitary fossa. Biopsy from the nasopharynx was reported to be poorly differentiated Transitional cell carcinoma. Comments : The history of fever was misleading, which baffled the neurologist and neuro-surgeon. However bleeding from the nose, nasal obstruction a bulge in the palate with restricted mobility and bilateral cervical lymph nodes were good pointers to the clinical diagnosis. Discussion : The causes for delay and difficulty in diagnosis of malignancy of the nasopharynx needs emphasis. The following are the points to possible early diagnosis of this condition. Suspicion should be aroused when there is unexplained cervical Iymphadenopathy, especially bilateral and pain and neuralgias of the head and neck. The bizarre combinations of cranial nerve palsies should suggest this
26 Ind. J. Otol. Vol. XXI, No. 1, March, 1969
condition to the neurologist. Ophthal^noplegia and proptosis cases must have an E.N.T. examination. The E.N.T. surgeon must examine the nasopharynx in unexplained tinnitus, ear block, blue drum and nasal obstruction where anterior rhinoscopy is not helpful. Bleeding on digital palpation of the nasopharynx or eustachian catheterisation should suggest this possibility. Exfoliative cytology done routinely in all cases above 30 years may be an useful screening procedure to an early diagnosis and follow-up.
diagnosis. In this context the important role of exfoliative cytology has. been stressed. REFERENCES
The importance of understanding this clinical entity and the difficulties encountered in its diagnosis have been
The clinical features of 40 cases of nasopharyngeal carcinoma are presented, enumerating the delays, erroneus treatment and difficulties encountered in the diagnosis of these cases. Reports of the cases which illustrate the bizarre nature of this disease are given. The clinical features which should make us suspect this disease are discussed, for only a high degree of suspicion will lead to an early
Nasopharyngeal Carcinoma/Sinha et al
Godtfredson, E.: Ophthalmic and neurologic symptoms of malignant nasopharyngeal tumours; clinical study comprising of 454 cases, with special reference to histopathology and possibility of earlier recognition. Acta. Path. Microbiol. Scandinev (Suppl 55) Lederman, M. : Cancer of the nasopharynx, Charles Thomas, Springfield, U.S.A. 1961. Pang, L. Q. : Carcinoma of the nasopharynx : experiences with 66 cases. Arch Otolaryng. 82:622,, Dec. 1965.
Taneja, G. M., Kohli, G. S.,. Gupta, S. N. : Cancer of the. nasopharynx; incidence in Punjab, presentation of 27 cases. with interesting case reports.. Ind. J. Otolaryng. 15 : 1-16,1963. 5. Wang, C. C. and Schulz, M. D.:. Cancer of the nasopharynx; its clinical course and management_ Geriatrics, 20:864, Oct., 1965.