Nasal Septum Deviation Due to Nasal Melanoma: Case Report

Document Type

Article

Abstract

INTRODUCTION: Mucosal melanomas are very rare tumors and majority of them occur in the nasal cavity. Due to their unique location and delayed presentation treatment of such tumours is very challenging. Here we present one such unique case of nasal cavity melanoma.

CASE PRESENTATION: A 60 year old male presented with difficulty breathing through the right nostril. He was hemodynamically stable. Physical examination revealed mass in the right nostril. Maxillofacial computed tomography (CT) scan revealed 3.9x2.5 cm soft tissue mass in the right nasal cavity extending to right nasal sinuses and nasopharynx causing deviation of nasal septum to the left and destruction of right maxillary sinus. Flexible endoscopy revealed near complete obstruction of the right nasal cavity. Endoscopic resection of nasal mass with total ethmoid and maxillary antrostomy was done. Biopsy revealed cT4a N0 M0, Stage IVA malignant melanoma with NRAS gene mutation Q61L. He was started on Nivolumab 240 mg IV q2w and received adjuvant radiotherapy. Five month follow up Positron Emission Tomography (PET) /CT revealed resolution of previous metabolic lesions. One year follow up PET/CT revealed 1.2 x 0.9 cm soft tissue density in the right uvula and bone metastasis in the sacrum and L femur. Immunotherapy was switched to Pembrolizumab 200 mg IV and Ipilimumab 1 mg/kg IV q3v. After 1 year of this regimen, repeat PET/CT revealed metastasis to left anterior fourth rib, L anterior acetabulum, L parapharyngeal and post belly of digastric muscle lymph nodes. R femur extending into the neck and intrathoracic region. Regimen was switched to oral Binimetinib 45 mg daily for advanced NRAS mutant melanoma. Follow up PET/CT revealed further metastases with prominent hypermetabolic activity. Patient currently follows up with an oncologist outpatient.

DISCUSSION: Nasal melanoma is a very rare and aggressive variant, representing 0.02-0.2 cases per 100,000 per year. It has a poor prognosis due its delayed diagnosis with a 5 year mean survival rate of 30%. Most commonly it presents with epistaxis, nasal obstruction , rhinorrhea, headache and nasal deformity. CT of the head and neck is the first line modality to diagnose the tumor and definitive diagnosis is made with biopsy and immunohistochemistry. Conventional treatment includes wide resection with adjuvant radiotherapy and systemic therapy. Systemic therapy decreases recurrence and prevents metastasis however it does not affect the overall survival rate. Specific mutations like NRAS, B-RAF or c-KIT could help in selecting the immunotherapy drug. Immune checkpoint inhibitors Pembrolizumab, Ipilimumab and Nivolumab are the most common immunotherapeutic agents used for mucosal melanomas. More recently Talimogene Laherparepvec (T-VEC) intra-lesional therapy is used to invoke local immune response which has promising results. Cases like these underline the pertinence of history taking and physical examination in a world increasingly dependent on technology.

CONCLUSIONS: Rapid progression of mucosal melanomas require early detection and intervention to prevent metastasis and recurrence however overall survival rate is not affected.

First Page

A2961

Last Page

A2962

DOI

10.1016/j.chest.2023.07.1939

Publication Date

10-1-2023

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