|Year : 2015 | Volume
| Issue : 4 | Page : 543-546
Amelanotic melanoma of the nasal mucosa: A rare case report
Bharat Bhushan Dogra, Ashwani Kandari, Mahendra Kataria, Archana Buch
Department of Surgery, Padmashree Dr. D. Y. Patil Medical College Hospital and Research Centre, Pimpri, Pune, Maharashtra, India
|Date of Web Publication||14-Jul-2015|
Bharat Bhushan Dogra
Department of Surgery, Dr. D. Y. Patil Medical College Hospital, Pimpri, Pune - 411 018, Maharashtra
Source of Support: None, Conflict of Interest: None
Melanomas develop due to malignant transformation of melanocytes, which are derived from the neuroectoderm. Although the majority of melanomas are cutaneous, they occasionally arise from extra-cutaneous tissues as well which contain melanocytes, such as uvea, leptomeninges, or the mucosa. Melanomas can be melanotic or amelanotic. Approximately, 15-20% of all malignant melanomas arise in head and neck region and over 80% of these involve the skin. Mucosal malignant melanomas are very rare and may affect the upper aero-digestive tract (UADT). Their incidence is approximately as low as 0.5-3% of all malignant melanomas. Majority of noncutaneous head and neck malignant melanomas affect the ocular origin and only 6-8% originate in the mucous membranes of the UADT. Nasal mucosal melanomas account for <1% of all melanomas and <5% of all nasal tract neoplasms. The incidence of amelanotic melanomas (AMs) has been estimated to be between 1.8 and 8.1% of all melanomas. We present a case of 62-year-old lady presenting with epistaxis and a mass in left nasal cavity, diagnosed as AM and managed by wide local excision and primary reconstruction in our institution.
Keywords: Amelanotic mucosal melanoma, malignant melanoma face, nasal reconstruction
|How to cite this article:|
Dogra BB, Kandari A, Kataria M, Buch A. Amelanotic melanoma of the nasal mucosa: A rare case report. Med J DY Patil Univ 2015;8:543-6
| Introduction|| |
Malignant melanoma is a very rapidly growing tumor and its incidence is reported to be around 2:100,000.  Approximately, 15-20% of all malignant melanomas arise in head and neck region. Among the malignant melanomas affecting head and neck areas, over 80% involve the skin. Mucosal malignant melanoma is still rarer which affects the upper aero-digestive tract (UADT), and their incidence is approximately 0.5-3% of all malignant melanomas. Majority of noncutaneous head and neck malignant melanomas affect the ocular origin and approximately 6-8% originate in the mucous membranes of the UADT.  Nasal mucosal melanomas account for <1% of all melanomas and <5% of all nasal tract neoplasms.  The incidence of amelanotic melanomas (AMs) has been estimated to be between 1.8 and 8.1% of all melanomas. 
Mucosal malignant melanoma of the nasal mucosa is extremely rare. Amelanotic variety of malignant melanomas is generally difficult to diagnose because of lack of the melanin pigment. Histopathology report along with immuno-histochemistry may give an accurate diagnosis in such cases. Early diagnosis and radical surgery offer the only hope for controlling this disease. They carry very poor and unpredictable prognosis.  We report a case of mucosal malignant melanoma of the nasal cavity managed successfully in our hospital.
| Case Report|| |
A 62-year-old female patient was referred to us from the Department of ENT, for a swelling involving left naso-labial region of 6 months duration. She also complained of swelling in the left nasal cavity resulting in difficulty in breathing and three to four episodes of epistaxis.
Clinical examination revealed swelling of size 4 cm × 5 cm over left naso-labial area involving left nostril and naso-labial fold [Figure 1]. Anterior rhinoscopy revealed the presence of an irregular mass in lateral wall and floor of left nasal cavity which bled on manipulation and turbinates were not visualized. The mass was reddish pink in color without any brownish discoloration. Regional lymph nodes were, however, not enlarged. A wedge biopsy from the mass was suggestive of malignant melanoma, but immuno-histochemistry was positive for S-100 and HMB-45 and thus diagnosed as AM of the nasal mucosa [Figure 2]. Patient was also found to be diabetic, and the same was controlled by insulin before taking her up for surgery.
|Figure 1: A mass of size 4 cm × 5 cm over left naso-labial area involving left nasal cavity|
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|Figure 2: Photomicrograph showing nasal mucosa with underlying neoplasm, scanty eosinophilic cytoplasm and large vesicular nucleus with prominent nucleoli|
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Magnetic resonance imaging (MRI) of head and neck region showed a well-defined mass lesion in left subcutaneous fat planes lateral to nasal ala and extended into left nasal cavity sub-mucosally along the lateral wall and involving the vestibule [Figure 3]. However, there was no lymph node enlargement seen in the MRI.
|Figure 3: Magnetic resonance imaging showing mass lesion lateral to nasal ala and extending into left nasal cavity sub-mucosally along the lateral wall and involving the vestibule|
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She was taken up for ablative surgery and primary reconstruction. Mass was removed en bloc by lateral rhinotomy approach with a minimum of I cm margin all around beyond the area of induration. Another mass found at posterior end of left inferior turbinate was also removed, and its bed was cauterized. Surgery left a large full thickness soft tissue defect over the left half of the nose, infraorbital and naso-labial regions, and upper half of left upper lip [Figure 4].
|Figure 4: Post excision soft tissue defect over the left half of the nose, infraorbital, naso-labial regions and upper half of left upper lip|
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Reconstruction was planned by utilizing local tissues for better cosmetic match. Midline forehead flap lined with split skin graft was used for reconstruction of the nasal defect. A transposition flap from malar region was used for upper lip defect and cheek rotation flap was used to provide cover for cheek defect [Figure 5].
Postoperative recovery was uneventful. Histopathological examination of excised specimen revealed small to large nests of malignant cells with oval and spindle cells presenting with eosinophilic mitotic nucleoli. She was advised to undergo radiotherapy under care of radiotherapist, but she expressed her unwillingness due to poor socioeconomic status of the family.
She reported for regular monthly review on outpatient basis and developed local recurrence 6 months later.
| Discussion|| |
Melanomas arise due to malignant transformation of melanocytes, which are in-turn derived from the neuro-ectoderm. Therefore, it is not surprising that mucosal melanoma is more common in ectodermally derived mucosal tissues such the nasopharynx, larynx, trachea-bronchial tree, and esophagus, than in nonectodermally derived tissues. 
80% of all malignant melanomas are cutaneous in origin. Primary mucosal melanomas of UADT are uncommon and represent only in 1.7-3% of all melanomas. 
Such patients generally complain of asymptomatic swelling with or without bleeding. Lesions are by and large painless and may or may not be pigmented.  On examination, nasal melanomas tend to be large, bulky, friable masses which bleed with manipulation. At initial presentation, these tumors may be fairly advanced, due to the ample space available to accommodate their growth and the obscure anatomy of the sinuses.  Histological examination and immune-histochemical staining analysis for S-100, HMB-45, Melan-A, microphthalmic transcription factor, tyrosinase, and Mart-1 are critical for making an accurate diagnosis of mucosal melanoma.  Other histopathologic features of mucosal melanomas include frequent angioinvasion, multicentricity, and the relative lack of lympho-plasmacytic reaction to the tumor.
The natural course of malignant melanoma is marked by early local recurrence, local extension, and frequent metastasis to skin, lymph nodes, and skeletal muscles.  Microscopic features of the melanoma occasionally resemble lymphomas, sarcomas, and undifferentiated carcinomas, hence, immunohistochemical studies are required to reach the diagnosis. Fludeoxyglucose (FDG) positron emission tomography (PET) scan provides a more comprehensive whole-body assessment as compared to the conventional cross-sectional imaging since PET scan has better capability of detecting regional, nodal, and distant metastasis. FDG PET/computed tomography is also reported to be a better modality for diagnosing occult metastases. 
Surgery has been the mainstay of the treatment of these tumors, provided they are resectable as in the case being reported here. We carried out wide local excision of the mass by open rhinotomy approach with 1 cm margin all around beyond the area of induration. Reconstruction was carried out by utilizing local tissues for better cosmetic match. Midline forehead flap lined with split skin graft was used for reconstruction of the nasal defect. A transposition flap from malar region was used for upper lip defect and cheek rotation flap was used to provide cover for large cheek defect.
Surgery, along with adjuvant radiotherapy, should be used for patients with either regional metastases or large bulky primary disease (with or without positive neck nodes). Malignant melanoma is considered to be radio-resistant tumor, but recent observations suggest that radiotherapy may have a role in their treatment. Literature shows overall initial response rate of 50-75% if radiotherapy alone is used for treating localized mucosal melanomas.  However, long-term survival remains a big issue. Prophylactic neck irradiation should be used in such cases. 
A variety of chemotherapeutic agents have been evaluated, however, the most widely used chemotherapeutic agent in the treatment of metastatic melanoma is the dacarbazine.
The most common combined chemotherapy regimens used as a standard for the treatment of metastatic melanoma are Dartmouth regimen, CVD (cisplatin + vinblastine + dacarbazine) and BOLD (bleomycin + vincristine + lomustine + dacarbazine). 
| Conclusion|| |
A very rare case of AM of the nasal mucosa in a 62-year-old female patient is being reported here. She had a locally advanced tumor involving left nasal cavity and naso-labial region with no regional lymph node involvement. She was managed by wide local excision and primary reconstruction by local flaps. Post op recovery was uneventful. She was advised adjuvant radiotherapy but expressed her unwillingness for the same due to her poor socioeconomic status. She has been followed up on outpatient basis and developed local recurrence after 6 months.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]