Primary mucosal melanoma of the nasal cavity: An uncommon cause of epistaxis in the elderly

Abstract

An 87-year-old woman presented with a 3-month history of recurrent epistaxis. She required operative intervention (nasal packing, blood transfusion) because of chronic epistaxis that had resulted in anemia and weakness. Physical examination, nasal endoscopy, and computed tomography (CT) showed a 3.5 × 3 cm, ulcerated, black-pigmented mass in the left nasal cavity arising from the anterior part of the nasal septum without signs of bone erosion (Fig 1). A metastatic workup revealed no evidence of locoregional and distant metastasis. A diagnostic biopsy was performed.

On microscopy, the section of the specimen showed epitheloid and spindle cells arranged in lobules and small bundles. The nuclei were pleomorphic, hyperchromatic, and contained prominent nucleoli. Cytoplasm showed abundant melanin pigment.

Immunohistochemical stainings for S100, Vimentin, Melan-A, and HMB45 antigens were strongly positive. A diagnosis of nasal mucosal melanoma (NMM) was made.

The patient underwent a paralateronasal surgical “box resection” of the mass with the septum, the lateral nasal wall, and a partial maxillectomy. A reconstruction of the nasal pyramid was achieved through a nasal flap rotation. Postoperative course was uneventful. She has been followed for 12 months and has presented no evidence of recurrence or epistaxis.

Discussion

NMM represents less than 1 percent of all melanomas and from 2 percent to 9 percent of head and neck melanomas. More than 75 percent of the lesions originate in the nasal cavity. Both genders are affected equally, with the peak age incidence between fifth and eighth decade.¹

NMM arises from the melanocytes that have migrated during embryologic development from neural crest to the nasosinusal mucosa. One third of the NMMs are amelanotic lesions. Epistaxis, nasal obstruction, and headache are the most common presenting symptoms. NMM is liable to be mistaken for other nasosinusal malignancies, particularly when melanin is scanty or absent. The differential diagnosis of NMM includes several neoplasms as the nasopharyngeal carcinoma, the large B-cell lymphoma, the extramedullary plasmocytoma, the sarcomatoid squamous carcinoma, the rhabdomyosarcoma, the neuroendocrine carcinoma, and the olfactory neuroblastoma. Immunohistochemistry is essential for the correct diagnosis. NMM is positive for S100 protein, vimentin, and specific melanocytic markers such as melan-A and HMB45 antigens.²

Figure 1

Preoperative image of nasal mucosal melanoma of the left cavity.

Prognosis of NMM is uniformly poor. Tumor size, site of origin, bone invasion, histologic aggressiveness, and metastasis at the time of diagnosis are the main prognostic factors.^2,3 Wide surgical excision is the treatment of choice. The local recurrences are common because of the difficulty in achieving complete tumor removal with wider negative margins at this anatomic site.² Chemotherapy should be reserved for patients with systemic disease. Therapeutic regimens with dacarbazine, cisplatin, ranimustine, and tamoxifen have been used with varying results.^4,5

The elderly patients present treatment dilemmas that include prognosis quoad vitam, quality of life, life expectancy, surgical and anesthesiological risks, and prolonged postoperative care. In our case, the leading problem of the patient was epistaxis, which was potentially life-threatening. Decisions with respect to radical surgery in an elderly population must encompass quality-of-life issues in addition to that of disease-free survival. This is a classic demonstration of how the surgery may improve the quality of life rather than extend it.

Author Information

Author Contributions

Emanuele Ferri, writer, clinical and therapeutic analysis; Ida Pavon, histopathologic analysis; Enrico Armato, writer, clinical and therapeutic analysis; and Ferdinando Ianniello, writer, clinical and therapeutic analysis.

Financial Disclosure

None.

References

Lund

. Malignant melanoma of the nasal cavity and paranasal sinuses. Ear Nose Throat J 1993;72:285–90

Hofbauer

Boni

Simmen

. Histological, immunological and molecular features of a nasal mucosa primary melanoma associated with nasal melanosis. Melanoma Res 2002;12:77–82

Chang

Karnell

Menck

. The National Cancer Data Base report on cutaneous and noncutaneous melanoma: a summary of 84,836 cases from the past decade: the American College of Surgeons Commission on Cancer and the American Cancer Society. Cancer 1998;83:1664–78

Lund

Howard

Harding

. Management options and survival in malignant melanoma of the sinonasal mucosa. Laryngoscope 1999;109:208–11

Seo

Ogasawara

Sakagami

. Chemohormonal therapy for malignant melanomas of the nasal and paranasal mucosa. Rhinology 1997;35:19–21