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September 1980, Volume 30, Issue 9

Case Reports

NASAL MALIGNANT MELANOMA CASE REPORT AND REVIEW OF LITERATURE

M.H.A. Beg  ( Dept. of Otolaryngology, K.V. Social Security SITE Hospital, Karachi. )

Abstract

Primary Malignant Melanoma of Nose is a rare disease. A case is reported and literature is reviewed. Majority of cases are between 50-70 years of age though cases from Indian Subcontinent arc younger. It is more prevalent in Caucasians and in the north of Indo-Pakistan subcontinent where people are fairer. Nasal obstruction and epistaxis arc commonest symptoms. Surgery is the treatment of choice but immunity seems to play an active part in control of the dis­ease. Prognosis is bad at present.

Introduction

Nasal Malignant Melanoma is an unpleasant condition to treat. Variable figures have been quoted for the incidence of this disease from 0.5% to 1.7% of malignant melanometa of whole body (Pack et al., 1952; Allen and Spitz, 1953; Moore and Martin, 1955). Most of the reports on this disease are ill documented (Ravid and Esteves, 1960) and most of the larger number of cases have been review of pathological materials (Holdcraft and Gallagher, 1969). The largest single series is reported by Harrison (1976) in which he has reported 40 cases among 320 patients of malignant tumours arising in the nose and paranasal Sinuses. Thus malignant melanoma constitutes 12.5% of Nasal and Paranasal sinuses malignancies.
There have been three reports from India (Purandare, 1954; Kacker and Kohli, 1965; Kohli and Sachdeva, 1974), and none has been reported from Pakistan. Husain (1980) has mentioned a case of Paranasal Sinus Melanoma among 13 paranasal sinus malignant tumours in a table but no clinical details or histological evidence has been provided.
Here we report a case of Primary Malignant Melanoma of nasal cavity with clinical features and histological findings. It appears to be the first case reported in Pakistan.

Case Report

H.J. a 40 years old female born in a village near Rawalpindi presented on 8th April, 1978. from the right nostril and nasal blockage of one months duration. Anterior rihinoscopy showed a pale polypodal mass arising from right middle meatus. She was admitted and investigations revealed an opaque right antrum and a raised ESR to 30 mm. This Polyp was removed and she was discharged on the fifth postoperative day.
One month later she attended the hospital again with similar symptoms of nasal bleeding and blockage on the right side.
Examination under general anaesthesia revealed a pale coloured mass arising from right middle meatus which was sent for histology. Microscopy showed sheets of mostly round cells with large, vesicular nuclei. At places there were spindle cells. Some of the cells showed brownish pigment. Big collections of brown pigment laden cells were seen around large vascular channels in the fibrous septa separating tumour sheets.
For three months the patient did not attend the hospital. On 20th September, 1979 right lateral rhinotmy was performed the nasal cavity was full of melanomatous tissue. The right nasal wall was removed and there were satellite malanomatous foci all over the nasal septum which were removed by sleeving off nasal mucosa. Malanomatous foci were also seen in cribriform plate area which could not be removed.
Recurrences were visible with in three months and Immunotherapy was started by injecting BCG vaccine, 0.1 ml at monthly intervals. This was  followed by chemotherapy with Endoxan and Mitomycin. This did not prevent the natural history of the disease which now involved the right oribit (see Fig) and presented as a mass on right macilla which bled on touch.


Systemic Metastasis followed, and the liver became palpable 8 cm below the costal margin and pigmentation of skin was over fingers. The general condition of the patient deteriorated gradually and the natient died on 15th February", 1980.

Comments

Review of Literature: The first case was reported by Lucke in 1869 operated on a 52 years old man with Melanotic Sarcoma arising from nasal mucous membrane. Ravid and Esteves (1960) reviewed world literature and found 117 cases and added one case of their own. In 1969 Holdcraft and Gallagher found another 77 cases and added 39 of their own cases all of them being reviews of histological slides and clinical features at Armed Forces Medical Institute in U.S.A. There have been a number of individual reports of one or few cases (Kohli and Sachdeva, 1974; Ghamrawi and Glenie, 1974; Wright and Heenan, 1974). The first large series of Harrison was reported in 1976. In all there are not more than 300 cases reported all round the world (Pearman, 1979).
Most of the cases reported have been between the ages of 50-70 years, the youngest patient reported is 15 (Harrison, 1976) and the oldest 90 (Pearman, 1979). The cases reported from India are of a much younger age most of them between 30-40 (Kohli and Sachdeva, 1974). This is in keeping with the finding that atleast Head and Neck Cancer effects in Indo Pakistan Subcontinent at an earlier age. The case reported in this paper is 40 as well.
Caucasians more commonly suffer from this disease. Ravid and Esteves (1960) found six Negroes in 118 patients and Holdcraft and Gallagher (1969) found six Negroes out of 30 patients. We have no figures about Pakistan but it is known that skin cancer is more common in Northern Races (Jafrey, 1977). The patient in this report was also from the north born near Rawalpindi.
Slight predominance has been shown of both sexes in various reports but it appears there is no definite preponderance of sex.
Clinical Features: Nasal obstruction and epistaxis are commonest symptoms. It is a vascular tumour and tend to bleed easily and ulcerate. A substantial number of patients had various previous nasal polypectomies (Ravid and Esteves, 1960; Holdcraft and Gallagher, 1969) and malignancy was suspected when recurrence occurred soon after polypectomy. This is what happened to our patient as well.
The duration of symptoms varies from 3-24 months, our patient had symptoms for one month before she presented. Common sight of origin is nasal septum inferior or middle meatus or turbinates. Cervical Lymphadeno-pathy at the time of presentation has not been found commonly. Harrison (1976) found it in two patients out of 40.
Histogenesis of pigmented tumours has remained obscure. It is supposed to be derived from neural crest (Stell and Maran, 1972). Though Ravid and Esteves (1960) believe in Epithelial origin of this malignancy and they propose that there is metaplasia of pseudostratified columnar epithelium to stratified squamous variety which contain melanocytes.
Management: Surgery to be the treatment of choice (Harrison, 1976) but surgical excision with preservation of nose is not always possible particularly when cribriform plate is involved as happened in the case reported. Earlier papers particularly of Holdcraft and Gallagher (1969) reported few cases where surgery and radiotherapy both have been combined and two of five patients survived for 5 years. Harrison (1976) feels that radiotherapy is not worth while as the tumour is radio resistant although, there have been isolated reports of dramatic cures after radiotherapy (Ghamrawi and Glennie, 1974).
Chemotherapy offers little for treatment of this lesion. BCG immunotherapy has been shown to improve results in cutaneous melanomas (Mathe, 1977). We cannot say that immunotherapy made any improvement in the lesion in our patient.
Prognosis: Death is common out come. Harrison reported 27.5% of his 40 cases surviving for 5 years. This is in complete contrast to skin melanomata where 77% survive for 5 years. Personal immunity though seems to play a strong part in prognosis of these patients as there are reports (Wright and Heenan, 1974; Mason and Friedman, 1955) of patients with slow growing tumours which made them survive upto 10 years following diagnosis but this is rare.

Acknowledgement

I am thankful to Professor N.A. Jafrey Head Department of Pathology, Basic Medical Science Institute, Jinnah Postgraduate Medical Centre, Karachi for reporting on histology of this case.

References

1. Allen, A.C. and Spitz, S. (1953) Malignant Melanoma: A clinicopathological analysis of the criteria for diagnosis and prognosis. Cancer, 6:1.
2. Ghararawi, K.E.A. and Glennie, J.M. (1974) The Value of Radiotherapy in the management of Malignant Melanoma of the Nasal Cavity. J. Laryngol. Otol., 88:71.
3. Harrison, D.F. (1976) Malignant melanometa arising in the nasal mucous membrane. J. Laryngol. Otol., 90:993.
4. Holdcraft, J. and Gallagher, J.C. (1969) Malignant melanomas of the nasal and paranasal sinus mucosa. Ann. Otol. Rhinol. Laryngol., 78:5.
5. Husain, S.S. (1980) Malignant disease of paranasal sinuses. Doctor, (Special Issue on ENT Diseases) 1:4.
6. Jafrey, N.A. (1977) Frequency of Malignant Tumours in Seven Centres in Pakistan. JPMA, 27:335.
7. Kacker, S.K. and Kohli, LP\\. (1965) Primary malanomas of the nose and paranasal air sinuses. J. Indian Med. Ass., 45:203.
8. Kohli, G.S. and Sachdeva, O.P. (1974) Malignant Melanoma of the Nose and Paranasal Sinuses. J. Laryngol. Otol., 88:693.
9. Lucke, A. (1869) Die Lehre Von den Geschwulsten in anato-mischer and Klimischer Beziehung, in Handbuch D. allg. u. Spec. Chir Erlangen P. 244.
10. Mason, M. and Friedmann, I. (1955) Melanoma of the nose and ear. J. Laryngol. Otol., 69:98.
11. Mathe, G. (1977) Cancer Chemotherapy and Immuno-therapy. Traingle, 16:151.
12. Moore, E.S. and Martin, II. (1955) Melanoma of the upper respiratory tract and oral cavity. Cancer, 8:1167.
13. Pack, G.T., Gerber, D.M. and Scharanagel, I.M. (1952) End results in the treatment of malignant melanoma. A report of 1190 Cases. Ann. Surg., 136:905.
14. Pearman, K. (1979) Malignant melanoma of nasal mucous membrane. J. Laryngol. Otol., 93:1003.
15. Purandare, N.M. (1954) Malignant melanoma in nose; case report. Indian Med. Gaz., 89:159.
16. Ravid, J.M. and Esteves, J.A. (1960) Malignant melanoma of nose and paranasal sinuses and juvenile melanoma of the nose. Arch. Otolaryngol., 72:431.
17. Stell, P.M. and Maran, A.G.D. (1972) Head and Neck Surgery. William Heinemann Chichester, P. 225.
18. Wright, J.L.W. and Heenan, P.J. (1974) Prolonged survival in malignant Melanoma of the nose. J. Laryngol. Otol.,

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