Malignant melanoma of nasopharynx extending to the nose with metastasis in the neck.DS Grewal, SY Lele, SV Mallya, B Baser, NK Bahal, JD Rege
Dept. of Oto-Rhino-Laryngology, BYL Nair Charitable Hospital, Bombay, Maharashtra.
Malignant melanoma is a rare tumor in otorhinolaryngology. In this paper, we have reported the first case of melanoma of nasopharynx which we came across in 20 years of ENT practice. A 55 year old male patient with complaints of swelling of nose and left side of neck, nasal blockage and epistaxis was diagnosed as melanoma of nose with metastasis in the neck based on clinical and radiological examination. The tumour was excised by lateral rhinotomy with supraomohyoid block dissection on the left side. The tumour was found to originate from lateral wall of nasopharynx and middle turbinate. The surgery was followed by radiotherapy. Diagnosis of melanoma was confirmed on histopathology. During 30 months follow-up, no recurrence was noticed.
Keywords: Case Report, Combined Modality Therapy, Follow-Up Studies, Head and Neck Neoplasms, pathology,secondary,therapy,Human, Male, Melanoma, pathology,secondary,therapy,Middle Age, Nasopharyngeal Neoplasms, pathology,therapy,Prognosis,
Malignant melanomas of the nasopharynx and nasal cavity occur rarely in India; in 1965, Kully and Shreedharan reported the first case. Ravid and Esteeves reported that Lucke in 1869 operated on a 52 year old man suffering from melanotic sarcoma of the nasal mucosa. The first case in the American literature was reported by Lincoln in 1885. Melanomas are tumors arising from melanocytes which are neuroectodermally derived cells located in the basal layers of skin, skin adnexas and some of the mucosal membrane. Common sites for melanomas are head, neck and the lower extremities as they are exposed to sunlight, which is one of the predisposing factor. Less commonly they occur in the oral and genital mucosa, nail beds, conjunctiva, orbit, esophagus, nasal mucosa or nasopharynx, vagina and leptomeninges. In this paper we have described a case of malignant melanoma of the nasopharynx extending to the nose with metastasis in the neck.
A 55-year-old male patient came to us with the complaints of swelling of the nose, nasal blockage and epistaxis for two months. He noticed a small swelling one cm in diameter, on the left side of the nose 2 months back. The symptoms increased rapidly. Two weeks after this the patient noticed a swelling on the left side of neck which increased rapidly in size. On clinical examination, a huge swelling was noticed on the left side of the nose measuring 5 x 4 cm. There was a fleshy, bluish red, friable, non-tender mass in the left nostril, which was completely blocking the nasal passage and bled slightly on touch. The left ala was stretched and dilated blood vessels were seen over it. The septum was markedly deviated to the right by the mass [Figure - 1].
On posterior rhinoscopy the mass was found to be occupying the nasopharynx. Examination of the throat, ears and larynx were normal.
There were two soft, mobile, enlarged, non-tender, non-matted lymph nodes on the left side together measuring 4 x 4 x 3 cm in the jugulodigastric region [Figure - 2].
The routine hematological and biochemical investigations revealed that haemoglobin was 10.6 gm%, total W.B.C count was 7200/mm with polymorphs - 70%, lymphocytes - 28% and eosinophils - 2%. Fasting blood sugar was 105 mg%, and blood urea nitrogen was 14.2 mg%. Urine and stool examinations were normal. X -Ray of paranasal sinus (Water's view) showed a soft tissue shadow over the nasal region extending to the left maxillary sinus, frontal sinuses and right maxillary sinus [Figure - 3] X-ray chest was normal; CT scan was not done.
A provisional diagnosis of melanoma of nose with metastasis in the left neck was made. The patient was operated and tumour was excised by lateral rhinotomy with supraomohyoid block dissection on the left side. On operation it was found that the middle and inferior turbinates on the left side were lacerated and there was a septal perforation measuring 2 x 1 cm. The septal perforation was probably caused by pressure necrosis. The melanoma was polypoidal and capsulated which ruputured during removal and hence it was removed in pieces. The final attachment was broad based and was found on the lateral wall of nasopharynx, anterior to the Eustachian tube More Details orifice, extending to the posterior end of the middle turbinate. The left maxillary antrum was inspected by Caldwell-Luc approach and was normal. Anterior and posterior nasal packing was done. Supraomohyoid block dissection was done on the left side and it was found that the lymph nodes were transformed into a mass, which was blue in colour and filled with thick bluish fluid. Radiotherapy, was given following complete wound healing. The patient was followed for a period of 2 ½ years and there has been no recurrence.
After removal of tumour, the gross examination revealed multiple bits of size 2.5 x 2 x 1 cms, totally aggregating to form 7 x 5 cm mass. It was firm and dark blue to black in colour. The lymph nodes were two in number, together measuring 4 x 3.5 x 1.5 cm and had thick capsule with dark areas of extensive necrosis.
On microscopic examination, tumour showed extensive areas of necrosis and nests of tumour cells. Individual tumour cells had moderate to abundant eosinophilic cytoplasm, enlarged pleomorphic nuclei with prominent nucleoli, cytoplasm and nucleus was covered with large amount of melanin pigment [Figure - 4] and [Figure - 5].
Lymph nodes showed destruction of normal architecture and replacement by sheets and nests of tumour cells with scattered areas of necrosis. Individual tumour cells showed moderate to abundant eosinophilic cytoplasm, small amounts of melanin pigment and pleomorphic, hyperchromatic nuclei showing increased mitotic activity. Few cell nests showed central areas of necrosis [Figure - 6].
Presence of melanin was confirmed by histochemical method (Masson Fontana and melanin bleach) and immunohistochemical study to demonstrate Vimentine, S100 protein was done.
The incidence of melanomas of the head and neck and nasal cavities varies from 0.4 to 4%. The tumour occurs between 50-70 years of age and is slightly more common in males than females, although age and sex do not affect the prognosis,.
This is the first case of melanoma we have seen in 20 years of ENT practice. In the upper respiratory tract the commonest sites are the nose and paranasal sinuses with decreasing incidence as the respiratory tract is descended. The site most frequently involved is the nasal septum in our case the tumour originated from the lateral wall of the nasopharynx and the middle turbinate, which is one of the less common sites. Malignant melanomas of the nasal cavities and sinuses are characterized by early and repeated recurrences. Regional metastasis to lymph nodes is uncommon in Negroes whereas it is very frequent in the white races. In this case as the tumororiginated from the nasopharynx, a very early metastasis was seen and it was rapidly progressive.
According to Batsakis, et al, of the mucosal melanomas in head and neck, 56% occur in upper respiratory tract and only 0.6% cases occur in the nasopharynx. In a survey of 158 cases of melanomas of upper respiratory tract, Batsakis found only one such case, which was reported by Conley and Pack. Besides this, they also recorded two cases of melanoma of eustachian tube orifice with metastasis to the middle ear. The interesting fact about our case was that the meianoma was polypoidal with a broad based origin in the lateral wall of nasopharynx anterior to the eustachian tube opening, extending to the middle turbinate and because of the involvement of the nasopharynx, a very early metastasis was observed (within two weeks).
Various methods of therapy, including surgery, irradiation alone, irradiation with surgery and chemotherapy have been used in treating malignant melanoma of the nose. Surgical exclusion is the best treatment, as malignant melanoma are considered to be radioresistant. Different chemotherapeutic regimens have been tried which include - vinca alkaloids, alkylating agents, antimetabolites, levamisole, DTIC (dimethyl - trizeno - imidazolecarboxamide) and dactinomycin but all with unsatisfactory results.
The prognosis for these tumours is very poor. Ravid and Esteeves described 5 patients with melanoma of the nose, who survived for 5 years but died of the same in the sixth year. The 5 year survival rate varies between 6 and 17%.
We are grateful to our Dean Dr (Mrs) KD Nihalani for allowing us to publish this paper.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]