Amelanotic mucosal melanoma of the left sinonasal cavity: a case report

INTRODUCTION

Mucosal melanoma is rare type of melanoma accounted only 0.8-3.7 % of melanoma.¹ It defined as melanoma which occurs in mucous membrane, oral and nasal cavities, conjunctiva, genital sites, and rarely other mucosae.² Head and neck melanoma accounted 50% among other sites.³

Recent studies, amelanotic subtype account 13.2% of all sino-nasal melanoma. Nasal cavity melanoma usually occurs on lateral nasal area especially in middle turbinate and inferior turbinate.⁴

Among other location, sino-nasal originated primary lesion was more deadly compared to other.⁵

CASE REPORT

This was a case report of an unusual cases of amelanotic melanoma that presented to the outpatient clinic with advanced stage and non-specific clinical presentations. The rare and complexity of the medical and family history and clinical symptoms makes the diagnosis difficult, associating with delayed diagnosis and treatment. Here we study the clinical presentations, risk factor, and the diagnostic challenges in the diagnostic of amelanotic melanoma.

Case presentation

A 56-year-old Asian female presented to otolaryngology division clinic with nasal mass in left nose in July 2021. The mass firstly noticed around 5 months ago, then enlarge and occupied nasal cavity. The patient also reported recurring episodes of epistaxis, facial pain, epiphora, and blurred vision. She had no history of allergic rhinitis or chronic sinusitis, coagulopathy, illicit drug use, rheumatologic or any other systemic disease. There is also no history of recent infection. On physical examination, nasal dorsum was symmetric to no noticeable deformities. The nasal ala were symmetry and external valves were patent. There was no tenderness on maxillary and frontal sinus during palpable examination. No noticeable deformities were found in the septum wall. The right nasal cavity was patent with no noticeable abnormalities. In left nasal cavity was completely obstructed with mass with pink pale and moist mucous; the mucosa along lateral wall were inflamed with dried blood and crusting (Figure 1). For symptomatic patient given pain management only.

Before coming to our clinic patient already undergo biopsy with result of round blue cell tumor squamous cell carcinoma and non-Hodgkin lymphoma with immunohistochemistry suggest chronic infection with granulation. The patient then undergo another biopsy and immunohistochemistry for confirmation in August 2021. The biopsy result of non-Hodgkin lymphoma with large cell type (Figure 2). Then proceed with immunohistochemistry of LCA/CD45, CD3, CD20, Ki-67, Vimentin, S100, Synaptophysin, HMB45, EMA, and Melan-A with positive results in Vimentin, S100, HMB45, Melan-A. This finding result in the diagnosis of amelanotic melanoma.

Mucosal melanoma is very uncommon condition and malignant disease. Because of its low incidence and hard to diagnose, there is no sufficient guideline for its treatment and diagnose. Multiple modalities treatment such as radical removal, radiotherapy and chemotherapy does make a difference but still doesn’t effectively affect the patient survival. Ultimately, MM is an important differential in patient when nasal mass, and recurring episodes of epistaxis were presented.

Subsequently after physical examination patient also underwent CT scan which revealed the lesion already affected maxillary, ethmoidales, and sphenoid sinuses, with no metastasis on lymph nodes. The mass also expanded to the left eye orbit (Figure 3). Hence, we referred patient to eye department. After examination, they concluded proptosis of left eye with papilla edema and pre-papil atrophic.

In December patient undergo surgery for mass removal and orbitotomy and exenteration from eye department. Following the surgery patient to be scheduled for adjuvant radiotherapy and chemotherapy.

DISCUSSION

Classic presentation of MM lesions are dark pigmented, polypoid lesions with irregular borders. Nasal obstruction, mucosal bleeding, epistaxis, facial pain, rhinorrhea and even parathesias are usually presented in classic cases.⁶ In recent systematic review by Pontes et all on sino-nasal melanoma; epistaxis, nasal obstruction, and blurry vision were most common observed in patient with MM in sino-nasal. Same symptoms were observed in this patient.⁴ Blurry symptoms in our patient due to mass already expanded to left orbit, but we cannot conclude any neural involvement.

The diagnosis of mucosal melanoma can be challenging. In our patient alone, numerous immunohistochemistry was used to ensure our diagnosis. But vimentin, S-100, HMB-45, and Melan-A were the most common factor leading to diagnosis of amelanotic melanoma.⁴ In the latest systematic review, stage I and II were more likely to be observed.⁴ Contrary to the previous findings that patient usually already at stage III and stage IV when first diagnosed.⁷ These findings associated with the presence of recurring epistaxis and nasal obstruction which help specialist to further investigation.⁸

Melanoma already have higher death rate and poorer survival rate.⁹ MM have better outcome other than other in term of general type, but in sino-nasal is still inconclusive. Among other location, sino-nasal originated primary lesion was more deadly compared to other.⁵ Other prognostic factors such as clinical presentation such as age more than >67.6 years, middle turbinated primary tumor, advanced disease, radiotherapy alone, recurrence, presence of distant metastatic associated with less survival rate.⁴ Another finding, elevated lactate dehydrogenase (LDH) level and higher performance score were associated with poorer survival rate.⁵ Amelanotic melanoma have higher recurrence index than pigmented melanoma. But recurrence does differ depends on several risk factors, the higher stage tends to occur more, other factor are age more than >67.6 and gender. Although amelanotic melanoma occurrence higher in female, its likely to recurrence in male patient.⁸ Chemotherapy can be indicated if there were distant metastasis and in unresectable patients. But the therapeutic effect of chemotherapy on mucosal melanoma in head and neck remain inconclusive.¹⁰ The prognosis of mucosal melanoma remains uncertain leaning to poorer outcome due to its malignant nature. Even with multiple modalities treatment the prognosis and survival rate remain despicable. Hence further research in diagnosis and treatment were needed to achieve better outcomes

CONCLUSION

Amelanotic mucosal melanoma is very uncommon condition and malignant disease. Because of its low incidence and hard to diagnose, there is no sufficient guideline for its treatment and diagnose. Multiple modalities treatment such as radical removal, radiotherapy and chemotherapy does make a difference but still doesn’t effectively affect the patient survival. Ultimately, MM is an important differential in patient when nasal mass, and recurring episodes of epistaxis were presented. The diagnosis of mucosal melanoma can be challenging and vimentin, S-100, HMB-45, and melan-A were the most common factor leading to diagnosis of amelanotic melanoma.

Funding: No funding sources

Conflict of interest: None declared

Ethical approval: Not required

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