VERRUCOUS CARCINOMA OF LARYNX

Saurabh Varshney1, Jasprit Singh2, R. K. Saxena3, Anoop Kaushal4, V. P. Pathak5

INTRODUCTION Verrucous carcinoma is an uncommon variant form of epithelial cancer. Since it was first delineated as a clinico-pathologic entity by Ackerman in 1948, this uncommon variant (1 or 2 percent) of laryngeal squamous cell carcinoma has presented a dilemma to pathologists and head-neck surgeons alike, to the former because of the very high differentiation of this tumor, which can make a firm diagnosis of malignancy quite difficult, and to the latter because of controversy about its proper treatment.

CASE REPORT A 55 years old male patient was admitted in January 2000 with complaints of change in voice (4 months), cough with expectoration (1 month) and difficulty in breathing (15 days). There was no history suggestive of antitubercular treatment in past, chest pain, heamoptysis and difficulty in swallowing. Patient was a chronic smoker (30 years).

Clinical examination revealed an exophytic, bulky growth on the right vocal cord, with restricted mobility, the left vocal cord was normal and mobile. Rest of the ENT examination was normal. There was no palpable neck node. Chest was normal. An elective tracheostomy was done the same day to relieve stridor. A flexible laryngoscopy with retrograde endoscopy (through tracheostomy) was done which showed a exophytic, mass involving right vocal cord, right arytenoid, (inter arytenoid area and anterior commissure were spared), and hanging in the subglottic area (approximately 3mm anteriorly and 4mm posteriorly) without involving the mucosa, right vocal cord had restricted mobility, rest of the areas were normal. Biopsy of the mass was suggestive of verrucous carcinoma. A pre-operative Contrast Enhanced C. T. Scan (CECT) of neck was done and 5 mm contiguous axial

sections were obtained from just above the hyoid bone to the root of neck in quite respiration, i.v. contrast was given and scans repeated from the level of the soft palate to the root of neck with 3 mm sections of the region of interest. Findings were suggestive of mass involving the right true vocal cord and extending into both the supraglottic and infraglottic compartment with extensions in to anterior commissure, right paraglottic space and thickening of right aryepiglottic fold (carcinoma). Contralateral vocal cord was normal, with no significant regional adenopathy. Rest of the haematological, biochemical, radiological (Xray chest PA) investigations were normal.

Patient was treated surgically under general anaesthesia by laryngofissure approach. Curved horizontal incision was given, (after marking the surgical landmarks with gentian violet), at the level of middle of thyroid cartilage, superior flap raised up to hyoid bone and inferior flap raised up to cricoid cartilage along the subplatysmal plane. Deep cervical fascia was slit open vertically and strap muscles retracted laterally there were enlarged prelaryngeal lymph nodes which were excised and sent for

1,2Assistant Professor, 3Professor & Head, 4Senior Resident, Department of ENT, 5Professor & Head, Department Pathology, Hima-layan Institute of Medical Sciences, Jollygrant, Dehradun, U.P. 248140

Fig. I & II : CECT of the patient

Verrucous Carcinoma of Larynx 55

Fig. IV : Excised tumor mass.

histopathological evaluation. Thyroid and cricoids cartilages exposed. Thyroid cartilage perichondrium cut vertically 2 mm on left lateral side and laryngofissure done and the growth exposed in the laryngeal cavity. Cricothyroid membrane was cut horizontally to define lower limit of the growth. Left vocal cords, anterior commissure, bilateral arytenoids were found free from any pathology. Subglottic mucosa was normal. The broad based growth on the right vocal cord separated from its attachments above with 4 mm extension in subglottis, the inner thyroid perichondrium laterally, the vocal process of arytenoid posteriorly and the growth excised with a healthy margin all around. Right false cord was preserved as it was not involved. Post operative period was uneventful. Patient was discharged on 10th post operative day, after closing the tracheostomy on 6th post operative day. Histopathological examination of excised mass showed tumour tissue made up of well differentiated squamous epithelium exhibiting marked papillomatosis, hyperkeratosis, parakeratosis as well as downwards

extension of nests of squamous cells infiltrating the deeper tissue which contains heavy lymphoplasmacytic and eosinophilic infiltrate. Consistent with verrucous carcinoma. Prelaryngeal nodes showed non-specific reactive hyperplasia. Patient has a reasonable functional voice, with no recurrence after 2 months of follow up.

DISCUSSION Verrucous squamous cell carcinoma (verrucous carcinoma, Ackerman's tumor) is a malignant neoplasm usually defined as verrucoid, highly differentiated, squamous cell carcinoma of mucosal or skin surface. It tends to produce prominent surface keratin, and even though capable of local tissue destruction and invasion, does not usually metastasize. Clinical and histiological differentiation from conventional squamous cell carcinoma is of prime importance in diagnosis.

Verrucous squamous cell carcinoma occurs most often in the oral cavity, but the next most common area of involvement is the larynx (0.7% - 1.0% of laryngeal carcinomas), but similar lesions have also been described on the genitalia, in the nasal passages, and in the oesophagus. Age may range from the fourth to eight decades, with a mean age of 60 years. Four out of five patients are male. Demographics are similar to other types of squamous cel l carcinoma. Et iology and symptomotology is same as laryngeal squamous cell carcinoma. Typical lesion is a pale, warty, fungating, locally aggressive, ulcerated tumor attached by a broad base, is well circumscribed and it is clearly demarcated from the adjacent mucosa. Metastasis is rare, but growth is inexorable if untreated and the tumor can result in the patient's death. Ninety percent of laryngeal involvement is in the glottis. Pathological diagnosis can be very difficult, especially if the biopsy material provided to the pathologist does not show an area of junction between tumor and normal tissue. Because of the bulkiness of these lesions, frozen section monitoring of repeated biopsies is often necessary in order to obtain tissue that will permit a firm diagnosis. Microscopically, verrucous carcinoma tends to be broadly based with an irregular surface sometimes thrown up into papillary fronds. The surface is usually heavily keratinised. The presence of keratin on an irregular moist mucosal surface gives the lesion its white, warty clinical appearance. In the typical lesion the deeper portions are locally invasive and destructive, with the infiltrative margin composed of blunt, well demarcated, well differentiated squamous cells. Typically, but not

Indian Journal of Otolaryngology and Head and Neck Surgery Vol. 56 No. 1, January - March 2004

Fig. Ill : Per operative photograph (arrow shows the tumor)

56 Verrucous Carcinoma of Larynx

invariably, there is an associated mononuclear in-flammatory reaction in the stroma immediately adjacent to the advancing margin. The inflammatory cells are usually plasma cells and lymphocytes; but giant cell reaction to extruted keratin is also commonly encountered.

Probably in no other neoplasm of the larynx is there more need for co-operation between surgeon and pathologist than in the diagnosis of verrucous squamous cell carcinoma. The lesion is difficult to differentiate either clinically or histiologically from well differentiated squamous cell carcinoma, except for identification of dysplastic epithelium and infiltration of irregular cords of dysplastic squamous cells into adjacent stroma rather than the broad, pushing bands of uniform squamous cells of verucous squamous cell carcinoma, should help clear the difficulty, when biopsy in done the pathologist usually uses the words such as "hyperplasia" and "heperkeratosis", but is reluctant to use the word carcinoma. These lesions should be quantified by CT examination if any significant infiltration is suspected.

Treatment of choice for laryngeal squamous cell carcinoma appears to be surgical removal. One must remember that in this non-metastasizing neoplasm, incomplete excision is not necessarily disastrous. Although this may lead to later recurrence, it is not life threatening, and perhaps more radical approaches may then be selected. There are those who advocate radiation therapy alone, but others have reported anaplastic transformation after radiotherapy. Certainly the well differentiated keratin surfaced verrucous squamous cell carcinomas would not seem very susceptible to cure by radiation. Only 50 percent of verrucous carcinoma respond primarily to radiation even when small. Properly performed surgery (partial/subtotal/ total Laryngectomy) has a very high cure rate.

Batsaka & Associates cite Felito and Retcher, who surveyed the literature and in collected series they found 90 patients treated with radiation, of those treated, 71 percent had lesions that persisted or returned. In contrast, of 103 treated surgically, seven (6.8 percent) returned.

Fortunately most verrucous cancers can be diagnosed

early enough for a conservation operation. Bryce believes that patients who require laryngectomy because of the stage of the growth should be offered radiation instead and that laryrngectomy should be reserved for those who fail such treatment. He believes the concern over anaplastic changes is sufficient to justify the loss of larynx.

The follow up of verrucous carcinoma is particulary deceptive, and critical radiologic imaging should be employed as indicated. The prognosis of verrucous squamous cell carcinoma is excellent.

REFERENCES 1. Ferlito A, Recker G (1980) : Ackerman's tumor (verrucous

carcinoma of the larynx. A clinico. Pathological study of 77 cases. Cancer, 46 : 1617-1630.

2. Abramson A. L., Brandsma J., Steinberg B. (1985): Winkler B: verrucous carcinoma of the larynx. Arch. Otolaryngol, 111: 709-715.

3. Ackerman L. V. (1948): Verrucous carcinoma of the oral cavity. Surgery, 23 : 670.

4. Van Nostrand AWP, Olofsson J. (1972) : Verrucous carcinoma of the larynx. A clinical and pathological study of 10 cases. Cancer, 30 : 691.

5. Tucker H. M. (1987) : The Larynx, Published by Thieme medical Publishers, 63-65.

6. Pradhan S. (1996) : Voice conservation surgery in laryngel cancer. Lioyd Publishing house, Mumbai.

7. Burns H. P., Van Nostrand A. W. P. ,Brycenp( 1976): Verrucous carcinoma of the larynx-Management by radiotherapy and surgery : Ann Otol Rhin. Laryngol 85 : 1.

8. Stanley E. Thawley, Panje W. R. (1987) : Comprehensive Management of Head Neck Tumors. W. B. Saunders Co ( vol. 1).

9. Batsaki's J.C.; Hybels R.; Crissman J. P. and Rice D. H.(1982): The pathology of head and neck tumors verrucous carcinoma part 15 Head Neck Srugery 5 :29.

10. Ryan R. E. Jr., De Santo L. W., Devine K. D., Weiland L. H. (1989,1977): Verrucous carcinoma of the larynx. Laryngoscope 87.

Address for Correspondence : Dr. Saurabh Varshney Assistant Professor ( ENT) Himalayan Institute of Medical Sciences Jollygrant ; Dehradun, U. P. 248140

Indian Journal of Otolaryngology and Head and Neck Surgery Vol. 56 No. I, January - March 2004