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| | CASE REPORT | | | | | Year : 2009 | Volume : 46 | Issue : 1 | Page : 67-70 | | | Mucosal malignant melanoma of the nasal cavity
KA Bothale, SA Maimoon, AD Patrikar, SD Mahore
Department of Pathology, NKP Salve Institute of Medical Sciences and Research Centre, Nagpur, India
Correspondence Address:
K A Bothale
Department of Pathology, NKP Salve Institute of Medical Sciences and Research Centre, Nagpur
India
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DOI: 10.4103/0019-509X.48600 PMID: 19282571 Mucosal malignant melanoma (MMM) of the nose is extremely rare. We report a case of MMM of the nasal cavity in a 60-year-old male patient presenting with a polypoidal mass in the right nasal cavity. It was increasing gradually and obstructing breathing. A biopsy of the lesion was done with a clinical suspicion of inverted papilloma/carcinoma. Microscopy revealed features suggestive of malignant melanoma with minimal melanin pigmentation. Subsequently wide local excision was done. Diagnosis of malignant melanoma was facilitated by histochemistry and immunohistochemistry.
Keywords: Mucosal malignant melanoma, nasal melanoma, sinonasal melanoma.
How to cite this article:
Bothale K A, Maimoon S A, Patrikar A D, Mahore S D. Mucosal malignant melanoma of the nasal cavity. Indian J Cancer 2009;46:67-70 |
| » Introduction | |  |
Mucosal malignant melanoma of the nose and paranasal sinuses is extremely rare. Prognosis is generally poor and unpredictable. Early diagnosis and radical surgical management appears to offer the best hope for curing the disease. Usually these neoplasms contain melanin pigment, but approximately one-third of cases have only focal, weak pigmentation or are non-pigmented amelanotic tumors.
Malignant melanomas may be misdiagnosed, especially when amelanotic. In such cases immunohistochemistry is useful in reaching the diagnosis. We report clinical and histopathological features of a case of MMM of nasal cavity in a 60-year-old male patient.
| » Case Report | | |
A 60-year-old male patient came to the outpatient department of otolaryngology with swelling in the right nasal cavity [Figure 1], causing obstruction to breathing and gradually increasing in size over three months. Frequent rhinorrhea and occasional episodes of epistaxis were present. He gave a history of tobacco chewing and long periods of exposure to sunlight, being a farmer by occupation. The swelling was reddish pink with black pigmentation at base, measuring 1.5 x 1.5 cm in size, firm in consistency with irregular surface. Turbinates were normal. Anterior rhinoscopy showed a right nasal mass measuring 1.5 x 1.5 cm, bleeding on manipulation. On clinical examination there was no regional lymphadenopathy, no pigmented lesions elsewhere and no history of regression of the previous pigmented lesion. X-ray of the paranasal sinuses showed a soft tissue mass in the right nasal cavity. There was no bony erosion or paranasal sinus involvement. White blood counts were within normal limits. There was no inflammatory syndrome. A biopsy of the lesion was done with a clinical suspicion of inverted papilloma/carcinoma. It was reported as malignant melanoma with minimal melanin pigmentation.
Operative findings : With a right lateral rhinotomy incision, the periosteum from the right wall of the pyriform aperture was elevated. The mass seemed to arise from the right lateral nasal wall showing focal black pigmentation at base. The mass was separate from turbinates. Wide local excision of the mass was done. The specimen was received for histopathology.
Gross-The tumor mass was received in multiple bits, soft to firm in consistency, grayish white with brownish black pigmentation on one aspect. One small bit of bony tissue was received which was hard in consistency. Decalcification was done.
Microscopy- Sections revealed a cellular tumor mass covered by partially ulcerated nasal mucosa. The tumor mass was composed of round or polygonal tumor cells and few spindle cells. They were arranged in nest-like pattern or lobules [Figure 2]. The tumor cells were moderately pleomorphic showing moderate amount of eosinophilic cytoplasm, high nuclear to cytoplasmic ratio and large hyperchromatic nuclei with prominent nucleoli. Nuclear molding was present. A few cells showed a small amount of melanin pigment. Bi or multinucleation was present in a few tumor cells. A few mitoses and areas of necrosis were seen. Intranuclear cytoplasmic inclusions were not seen.
Tumor lobules were separated by thin fibrous septa. There was no evidence of angioinvasion. Bone showed a single small microscopic focus of invasion by tumor cells. Adequacy of margins was difficult to evaluate due to poorly oriented bits of specimen and frequent ulceration of surface mucosa. Histochemistry and immunohistochemistry were done. Fontana Masson stain was positive for melanin pigment, Prussian blue stain for hemosiderin was negative and tumor cells were immunoreactive to S-100 protein and HMB-45.
Histopathological diagnosis of primary malignant melanoma of right nasal cavity with a microscopic focus of infiltration in bone was given.
This tumor was considered as primary melanoma of nasal cavity due to the absence of previous or concurrent pigmented lesions elsewhere.
Postoperatively, in view of microscopic focus of invasion of the bone by tumor cells, the patient underwent a full course of radiotherapy. He is under regular monitoring for nearly two years. Quarterly anterior rhinoscopy and yearly radiography of paranasal sinuses are being done as per follow-up protocol. To date there is no local recurrence or systemic metastasis.
| » Discussion | | |
Malignant melanomas are neural crest-derived neoplasms originating from melanocytes and demonstrating melanocytic differentiation. Approximately 15-20% of all malignant melanomas arise in head and neck sites. Of the head and neck malignant melanomas, over 80% are of cutaneous origin. Mucosal malignant melanoma (MMM) of the upper aerodigestive tract (UADT) represents 0.5-3% of malignant melanomas of all sites. Of the non-cutaneous head and neck malignant melanomas, the majority are of ocular origin, and approximately 6-8% originate in the mucous membranes of the UADT. [1] Sinonasal mucosal melanomas are rare, accounting for less than 1% of all melanomas and less than 5% of all sinonasal tract neoplasms. [2] UADT MMMs are more common in men than in women.
In the nasal cavity, the most frequent site of occurrence is the nasal septum (anterior portion) and the lateral nasal wall. [1] Clinically most patients have symptoms of nasal obstruction or epistaxis or both. On physical examination nasal melanomas tend to be large, bulky, friable masses which bleed with manipulation. [3] Clinical appearance of the tumors may be indistinguishable from benign polyposis. [4] The nasal melanomas project into the involved cavity and may have a somewhat polypoidal configuration.
The consistency has been described as firm, friable or gelatinous. Most of these tumors grow in sheets or nests of polygonal cells of variable size. They may have vesicular nuclei and prominent nucleoli. In a few cases, spindle cells predominate. The amount of melanin pigment varies considerably. [3]
Small round cell neoplasms of the sinonasal tract often generate considerable diagnostic difficulty. Differential diagnoses of such undifferentiated small blue cell tumors of sinonasal tract include olfactory neuroblastoma, sinonasal undifferentiated neuroendocrine carcinoma and Ewing's sarcoma/peripheral neuroectodermal tumor (PNET) and rhabdomyosarcoma. [5]
In our case, clinically the patient presented with non-pigmented, reddish pink polypoidal lesion and histologically there was minimal melanin pigment in tumor cells. Clinically and morphologically we also considered the above-mentioned differential diagnosis. Then histochemistry (Masson Fontana and Prussian blue) for identification of melanin and immunohistochemistry for differentiation from other similar tumors was done. Tumor cells were immunoreactive to HMB-45 and S-100 proteins. For both, intensity of staining was strong and diffuse. Cytokeratin, EMA and muscle actin were negative. HMB-45 is a superb diagnostic marker for poorly differentiated melanomas. Its immunoreactivity in our case eliminated all other differential diagnoses.
Peripheral neuroectodermal tumor (PNET) shows variable positivity for S-100 proteins but is negative for HMB-45. [1] So finally the diagnosis of malignant mucosal melanoma was facilitated by histochemistry and immunohistochemistry.
Immunohistochemistry remains the diagnostic gold standard. In our case the tumor mass was confined to the nasal cavity, but histologically there was a single small microscopic focus of bone invasion, which could not be assessed on X-ray.
HMB-45 expression seems to be 100% specific for the diagnosis of melanoma. Melan-A is slightly less specific. Microphthalmia transcription factor (MITF) has limited specificity in the diagnosis of melanocytic tumors. [6] Morris et al. analyzed the results of immunohistochemistry in primary mucosal melanoma of head and neck and came to the conclusion that PNL-2 is a highly sensitive marker for mucosal melanoma, likely superior to Melan-A and MITF and comparable to HMB-45, with specificity superior to S-100. Therefore the authors advocated inclusion of PNL-2 as an important adjunctive marker in the immunohistochemical evaluation of primary mucosal melanomas. [7] Yu et al . studied the expression of three melanocytic markers HMB-45, S-100 and Melan-A in primary oral and nasal mucosal melanomas. Their results indicated that both HMB-45 and S-100 show a high positive rate and labeling index in mucosal melanomas and therefore may be good markers for immunohistochemical diagnosis of primary oral and nasal mucosal melanomas. HMB-45 shows a significantly higher staining intensity than S-100. [8]
Metastatic melanoma of the sinonasal tract, although highly uncommon, must always be excluded, as the prognosis is even poorer. [2] Irrespective of the site of origin, MMM are highly aggressive and lethal tumors. Radical surgical excision is the treatment of choice. Radiotherapy or chemotherapy in the treatment of MMM is felt to have little effect on local or distant disease, and presently is used as adjuvant therapy. [1] Surgery accompanied by radiation and/or chemotherapy is the mode of treatment to control the disease. [9] Overall for MMM of sinonasal tract, the 5-year survival ranges from 17 to 46%. There is no time period after which a patient with MMM should be considered as cured. Malignant melanoma is notorious for remaining quiescent for long periods following the initial diagnosis only to resurface years to decades later. Recurrence, metastasis and death may occur decades after curative therapy. [1] At presentation, 70-80% of cases are localized, 10-20% have regional lymph nodes and less than 10% have distant metastasis. However during the course of disease, an additional 20% may develop nodal metastasis and 40-50% may develop distant metastasis to lungs, brain, bone and/or liver. Local recurrence is frequent (67-92%), may be repeated, and is a harbinger of adverse prognosis. Other poor prognostic factors include advanced age, obstructive symptoms, tumor size >3 cm, location in paranasal sinuses and nasopharynx, vascular invasion into skeletal muscle and bone, high mitotic count, marked cellular pleomorphism and regional and distant metastasis. [2]
| » References | | |
| 1. | Wenig BM. Tumors of the upper respiratory tract, Part A- Nasal cavity, paranasal sinuses and nasopharynx. In: Fletcher CD, editor. Diagnostic histopathology of tumors. Vol 1. 3rd ed. Elsevier: Churchill Livingstone; 2007. p. 83-149.  | | 2. | Wenig BM, Prasad ML, Dulguerov P, Fanburg JC, Kapadia SB, Thomson LD. Neuroectodermal tumors. In: Barnes L, Eveson JW, Reichart P, Sidransky D, editors. WHO classification of tumors. Pathology and Genetics, Head and Neck Tumors. Lyon: IARC Press; 2005. p. 65-75. | | 3. | Fu YS, Perzin KH. The nasal cavity, paranasal sinuses and nasopharynx. In: Silverberg SG, Delellis RA, Frable WJ. editors. Principles and practice of Surgical Pathology and cytopathology. Vol 2. 3rd edition. New York: Churchill Livingstone; 1997. p. 1039-71. | | 4. | Sil A, Chatrath P, Warwick-Brown. Malignant melanoma of the nose. Indian J Otolaryngol Head Neck Surg 2004;56:59-62. | | 5. | Iezzoni JC, Mills SE. "Undifferentiated" small round cell tumors of the sinonasal tract: Differential diagnosis update. Am J Clin Pathol 2005;124:S110-21. [PUBMED] | | 6. | Gleason BC, Nascimento AF. HMB-45 and Melan-A are useful in the differential diagnosis between granular cell tumour and malignant melanoma. Am J Dermatopathol 2007;29:22-7. [PUBMED] [FULLTEXT] | | 7. | Morris LG, Wen YH, Nonaka D, DeLacure MD, Kutler DI, Huan Y, et al . PNL-2 Melanocytic marker in immunohistochemical evaluation of primary mucosal melanoma of the head and neck. Head Neck 2008;30:771-5. [PUBMED] [FULLTEXT] | | 8. | Yu CH, Chen HH, Liu CM, Jeng YM, Wang JT, Wang YP, et al . HMB-45 may be a more sensitive marker than S-100 or Melan-A for immunohistochemical diagnosis of primary oral and nasal mucosal melanomas. J Oral Pathol Med 2005;34:540-5. [PUBMED] [FULLTEXT] | | 9. | Thomson LD, Wieneke JA, Miettinen M. Sinonasal tract and nasopharyngeal melanoma: A clinicopathological study of 115 cases with a proposed staging system. Am J Surg Pathol 2003;27:594-611. |
[Figure 1], [Figure 2]
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