Paraganglioma of Larynx DEVI PRASAD, R. C. NAYAR, V. NIRMALA, K. M. NALINESHA, E~ K. G. S. RAJU

A case of Paragangl ioma of region is no tewor thy , as this is

The histologic features and

The paragangliomas are collec- tions of neural crest cells which arise in association with the autonomic ganglia. They are widely dispersed in the body, in the adrenal medulla, the carotid and aortic bodies, vagal body and groups of cells associa- ted with the thoracic, intra-abdo- minal and retroperitoneal ganglia.

Tumours of paraganglia may be functional or nonfunctional. The nomenclature of the tumours is confusing. Paragangliomas are best classified according to their anatomical sites, as carotid body tumour, glomus jugulare tumour, vagal body tumour, mediastinal paragangliomas and retroperitoneal paragangliomas.

Von Hailer in 1743 first noted the presence of the carotid body. Its chemoreceptor function was suggested by De Castro in 1928 (Zachariah and Shah 1972). Aggregates of tissue with similar structure has been described in a variety of areas in the Head and Neck, including the middle ear, jugular foramen, ciliary ganglion and the larynx (Andrews 1955).

Marchand in 1891 first described a carotid body tumour arising from chemoreceptor tissue. Such tu-

Devi Prasad, Associate Professor and Head R. C. Nayar, Assistant Professor. V. Nirmala, Lecturer. K. M. Nalinesha, Lecturer. K. G. S. Raju, Resident, Department of E.N.T. V. Nirmala, Associate Professor, Depart- ment of Pathology. St. John's Medical College Hospital, Bangalore.

Address for Correspondence : Dr. Ravi C. Nayar Assistant Professor Department of E.N.T. St. John's Medical College Hospital BANGALORE-560 034.

Larynx in an elderly female is reported. Its locat ion in the interarytenoid not a site of normal aggregation of paragangl ionic t issue in the Larynx.

origin of this rare turnout are discussed.

mours have now been described in various sites in the Head and Neck. They have been reported in sites where paraganglia have yet to be identified in normat tissues, such as tongue, soft palate, facial skin choncha, nasal cavity, parana- sal sinuses, temporalis muscle, mandible, temporomandibular joint and thyroid (All, Aird, and Bihari 1983).

On 21.02.87 she underwent de- finitive surgery. A conservative surgery, partial laryngectomy was planned. However, on entry into the larynx the tumour was seen to be a pedunculated mass arising from the interarytenoid region, extending into subglottic, hence, perforce, a total laryngectomy had to be performed .(Fig.I).

Paragangliomas in the larynx were first described in 1955 inde- pendently by Andrews, Blanchard and Saunders and Zeitlhofer (Ste- arns 1982). We report a case of paraganglioma of larynx, with an unusual location in the larynx, to recapitulate certain noteworthy features of this rare tumour.

Case Report

R.K. a 67 year old female, presented on the 12th of February 1987, with complaints of dyspha- gia and a foreign body sensation in the throat for the last one year: She had undergone direct laryngoscopy and biopsy elsewhere for these complaints about 8 months ago, and a laryn- geal lesion was noted, which on biopsy was reported as chronic inflammatory tissue. No further details were available.

On 13.02.87 she underwent a repeat direct laryngoscopy and a large globular swelling was noted, which bled profusely on biopsy necessitating a tracheostomy. The site of origin of the tumour was diffi- cult to pinpoint. Histopathological examination revealed round cell neoplasm and a possibility of a paraganglioma was considered.

Fig. 1. Showing site of origin of tumour from the interarytenoid region viewed through a laryngofissure incision.

On cut section, the tumour showed a "beefy" red appearance.

On microscopic examination, the tumour was composed of cells having abundant pale granular cy- toplasm, and finely clumped nu- clear chromatin. The cells ware arranged in nests of varying sizes which were separated by a con- spicuously vascular stroma. The cell morphology and arrangement were uniformly similar throughout the section except at the ulcerated

'30 Indian Journal of Otolaryngology, Volume 40, No. 1, March, 1988

PARAGANGt=IOMA OF LARYNX--PRASAD et a l

surface where some hyperchroma- sia and pleomorphism of nuclei were evident. A peculiar feature seen in this tumour was the presence of cells where the cy- toplasm of one engulfed the other, a feature typically described in carotid body tumour. There were no glandular structures or squamo- id elements in the tumour. (Fig.2)

Fig. 2. Photomicrograph (H 8 E x 400) showing cell nests separated by vascular stroma. Cells with cytoplasmic processes engulfing other cells are also seen.

Histochemical studies revealed that the chromaffin reaction was negative. Arygrophil granules were however demonstrable by Modi- fied Gremelius stain. Electron microscopy and flurorescent studies were not done in this case.

She had an uneventful post operative period, and since dis- charge was on a close follow up. On the 14th July 1987 she presen- ted with multiple painful nodules on the skin suggestive of metasta- sis. She has since been lost to fol low up.

D i s c u s s i o n

Laryngeal paragangliomas, ori- ginate from the paraganglionic tissue present within the larynx. These are situated at two sites.

1. The submucosal superior laryngeal glomus bodies which are situated at the

R e f e r e n c e s 1. All, S., Aird, D.W. and Bihari, J.

(1983) : Pain inducing laryngeal paragangliomas CNon chromaffin). The Journal of Laryngology & Otology, 97 : 181-188.

2. Andrews, A.H. (1955) : Glomus Tumours (Non Chromaffin Para- ganglioma) of the Larynx. Case Report. Annals of Otolaryngology, 64 : 1034-1036.

anterior end of the vocal cords. (Described by Watza in 1963).

2. The paired inferior laryngeal glomus bodies which lie between the anterior and pos- terior branches of the re- current laryngeal nerves in the space between the thyroid and cricoid. (Described by Kleinsasser in 1964).

Review articles, comment on the relative predominance of tumour presentations in the supraglottic region, mostly visualised at the right aryepiglottic fold. (Stearns 1982 and Justrabo et al 1980). A careful review of indexed literature has revealed no report of the tumour arising from the interary- tenoid region.

All, Aird and Bihari (1983) have lecommended the classification of the case in to two types (Type I II). The latter characterised by a smaller lesion but presenting with severe spasmodic pain while swal- lowing. These patients often mis- takenly labelled as having neura- lgia, have a poorer prognosis be- cause, of a high incidence of metastasis.

The present case would be classi- fied clinically as a Type 1. However conservative surgery, usually recommended for Type I was not feasible due to the tumour's peculiar location. Inspite of redical surgery a recurrence was noted within 5 months of primary surgery. The incidence of metastasis in Type I is over 12%. The exquisitely painful, subcutaneous metastasis is a described feature which was observed in this case. (Stearns 1982). The tumour is believed to be radioresistant, hence post opera- tive radiotherapy was withheld (Zachariah and Shah 1972).

Pre operative diagnosis is often difficult to establish. Zachariah and Shah (1972) cautioned that

3. Justrabo, E. Michiels, R. Calmettes, C., Cubanne F., Bastein H, Noriot J.C. and Gurrein, J. (1980) : An Uncommon APUDOMA: A func- tional Chemodectoma of the Larynx Acta Otolaryngologica 89 : 135-143.

4. Stearns, M.P. (1982) : Chemodec- toma of Larynx. The Journal of Laryngology and Otology, 96 : 1181-1185.

because of the submucosal location of tumour, repeated and deep bio- psies are necessary to enable the pathologist to clinch the diagnosis. The tumour bleeds heavily on biopsy as was noted in the present case also.

Functional activity, as noted by fluctuations of Blood Pressure, VMA assays and the Falck Test for catecholamines, and presence of secretory granules on electron mi- croscopy has been reported by Justrabo et al (1982), enabling identification of these tumours as belonging to Pearse's APUD cell lines. The term non chromaffin paraganglioma, was suggested on the basis of a yellow staining of the cells by chromic salts. However as it now been established as a non- specific reaction, the term is best discarded (Stearns 1982). Chro- maffin reaction does not corres- pond to the functional activity of these tumours and is usually in- validated by formalin fixation. Formaldehyde induced fluoures- cence in fresh or frozen tissue and detection of argyrophil granules using Modified Gremelius stain are seen to be more reliable and sensitive. Electron microscopic demonstration of dense intra cytoplasmic granules is diagnostic. Though the more sensitive diagnostic techniques such as electron microscopy and formalin induced fluorescence were not carried out, the light microscopic pictures and demo- nstration of arygrophil granules justify the diagnosis of Paragang- lioma of Larynx in the present case.

The characteristics of cell mor- phology and pattern of arrange- ment of cells in the biopsy speci- men of a highly vascular laryngeal growth should alert the histopatho- Iogist to the posibility of a laryngeal paraganglioma.

5. Tobin H.A. and Harris, H.H. (1971) : Non chromaffin paraganglioma of the larynx, Case Report and Review of the literature. Archieves of Oto- laryngology, 96 : 1 54-157.

6. Veters, J.M. and Toner, P.G. (1970) : Chemodectoma of Larynx. Journal of Pathology, 101 : 259-265.

7. Zachariah, K.J. and Shah, J.H. (1972) : Chemodectoma of Larynx. Journal of Laryngology and Otology, 86 : 1213-1218.

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