NEURILEMMOMA OF LARYNX

(A Case Report)

Karan Sharma, Senior Lecturer,K. K. Duggal, Professor E.N.T.M. S. Bal, Asstt. Professor Pathology,E.N.T. Department, Medical College, Amritsar

A Rare case of neurilemmoma arising from laryngeal surface of epiglottis and adjoiningaryepiglottic fold is being reproted. Its clinical features, histopathological findings andmanagement is being discussed.

INTRODUCTION

Neurilemmomas are neurogenic tumours aris-ing from the Schwan sheath of peripheral, cranialor sympathetic nerves. Approximately 25-30%of neurilemmomas occur in the region of headand neck (Putney et al, 1964) and have occurredat almost every site. However, neurilemmomasof larynx are quite uncommon. Souchanek (1925)reported the first case. Cali (1965) reviewed allthe published cases and found the total numberof such cases to be sixty one. Very few individualcase reports have been documented thereafter.

Most of the neurilemmomas are situated inthe supraglottic structures arising from the branchesof superior laryngeal nerve, the aryepiglottic foldbeing the commonest site. The nerve functionsremain intact except in very large tumours becauseusually, with the expansion of the tumour, nervefibre gets splayed over the capsule rather thanbecoming incorporated within the tumours.

CASE REPORT

An eighteen year old, moderately built younggirl presented with the history of foreign bodysensation and dysphagia for the last six monthsand occasional difficulty in breathing for the lasttwo months. On being inquired, she also re-vealed slight hoarseness of voice. On indirectlaryngoscopic examination, a smooth pinkishpolypoidal mass was seen in the supraglotticlarynx. The mass was attached to the laryngealsurface of the epiglottis and its lower extent wasnot apparent. The glottic and sub-glottic regionscould not be visualized as were obscured bythe mass. There was slight supralaryngeal ex-

pansion. However, there was no mass palpableeither in the neck or in any other region of thebody. Also, there were no signs of any res-piratory distress. X-ray soft tissue neck, lateralview revealed an oval soft tissue shadow about4.00 cm. x 3.0 cm in the supraglottic region ofthe larynx . . . X-ray chest and all other routineblood and urine investigations were within normallimits but for Hb to be 9.0 mg%. One unit offresh blood transfusion was given preoperatively.A provisional diagnosis laryngeal cyst/mass wasmade.

An elective tracheostomy under localanaesthesia was performed. After 48 hours, generalanaesthesia was given via the tracheostome.The encapsulated pinkish pedunculated polypoidalmass was firm in consistency with its pediclealong the laryngeal surface of epiglottis (Righthalf) and right aryepiglottic fold. Trans-orally(Endoscopically), whole tumour mass was sur-gically removed after excising the pedicle withthe scissor. The glottic and subglottic regionswere normal. The surgical specimen consistedof a reddish brown, globular, firm mass mea-suring about 4.0 cm x 3.0 cm x 2.5 cm, thinlyencapsulated with a homogenous yellowish cutsurface. The excised mass weighed twelve grams.On histological examination, it turned out to bea neurilemmoma. Decanulation of tracheostomywas done after 4 days and the post operativeperiod was uneventful. Patient is asymptomaticand growth free after two and a half years offollow-up. Microscopically, there were bands ofSchwann cells accompanied by reticulin fibreswith their nuclei aligned in rows i.e. palisadingof nuclei. This arrangement resembled a tactile

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Neurilemmoma of Larynx (A Case Report)—K. Sharma et al.

corpuscle and has been termed a verocay bodyand their presence clinches the. diagnosis of aneurilemmoma.

(DISCUSSION

Neurilemmomas develop from the neural sheathof peripheral motor, sensory, sympathetic andcranial nerves, the exceptions being the opticand olfactory cranial nerves that lack Schwanncell sheaths. Verocay (1910) establishedneurilemmoma as a pathological entity and calledthem neurinomas. Stout (1935) coined the termneurilemmoma believing tumour to arise fromthe sheath of Schwann or neurilemmoma cells.Many still called them as Schwannoma. Othernames are encapsulated neurofibroma, perineuralfibroblastoma, Penglioma, neurilemmoblastoma,etc. Although these tumours have been foundto involve any region or structure in Head andNeck, yet laryngeal involvement is quite un-usual.

The characteristic lesion is a solitary encap-sulated mass which is benign and seldom re-curs. When the nerve of origin is small its associationwith a given tumour may be difficult to demon-strate. It may appear pink, yellow or pearly greyin colour. The small tumours are usually solid,though the larger tumours may become soft,cystic and spongy, because of spontaneousdegeneration and haemorrhage. The cut surfacevaries from homogenous solid grey-white to amottled red to yellow colour with or without cysticspaces. It varies in size and usually ovoid offusiform in shape. Histologically the tumour iscomposed of distinct portions. The more solidportion consists of cords of Schwann cells,accompanied by reticulin fibres with their nucleiarranged in parallel rows. This arrangement i.e.

REFERENCES1. Cali, G (1965) : Clinical2. Cumming, CW; Montogom

Laryngology, 78 : 76-95.3. Kragh, LV; Soule EH and Masson, JK (14. Loon, E.L.; Van & Diamonds, S. (1940)5. Putney, FJ; Moran, JJ and Thomas,6. Souchnek (1925) : Quoted by Whittam

84 : 747-749.7. Stout, AP (1935) : American Journal of8. Verocay, J (1910) : Quoted by Gore et al

: 193-201.

palisading of nuclei often resembles a tactilecorpuscle, termed as a verocay body. This wholesolid complex is called Antoni A tissue orfasciculatedtype. In another arrangement, Schwann cells arearranged loosely in a meshwork of reticulin fibresand microcysts. Coalescence of these microcystsproduce gross cystic spaces. This second ar-rangement is called Antoni B tissue or reticulartype.

In differential diagnosis, there are some otherconditions. A more frequent benign condition islaryngeal cyst which contains fluid and diagnosiscan be made by puncture and aspiration duringdirect laryngoscopy. Laryngocoele is a rare conditionwhich can be easily diagnosed by laryngealtomography. Another rare tumour is intrinsic lipomawhich can be differentiated from neurilemmomaon gross examination. However, histopathologicalexamination will make the final diagnosis.

As for as the management is concerned, surgicalexcision is the only effective way to cure thecases of neurilemmoma of larynx. Various externalsurgical approaches have been described bydifferent authors. These include laryngofissure(Whittam & Morris, 1970), and median thyrotomy'route (Loon and Diamonds, 1942). Cumming etal (1969) preferred another way of uncoveringthe laryngeal framework externally, including upperpart of the thyroid cartilage and enucleation ofencapsulated neurilemmoma without entering intothe larynx. Although most authors remove thetumour by external approach, yet small lcc?^n"can be removed in toto, endoscopically (Putneyet al, 1962). Recurrence is not expected evenif a portion of capsule remains (Kragh et al,1960).

Otorhinolaryngoiatrica, 17 : 200-212.erey, WW and Balogh, K (1969) : An nals of Otology, Rhinology an

960) : Surgery, Gynaecology and Obstetrics, 111: 211-218.Annals of Otology, Rhinology & Laryngology, 51 : 122-126.GK (1964) : Laryngoscope, 74 : 1037-1059., DE et al (1970) : Journal of Laryngology and Otology,

Cancer, 24 : 751-796.(1956) : Surgery, Gynaecology and Obstetrics,

9. Whittam, DE and Morris, TMO (1970) Journal of Laryngology and Otology, 84 : 747-749

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