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Axial MRI of the lesion showing a large invasive sinonasal mass centred in the left maxillary antrum.
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Melanoma of the sinonasal cavity has a high incidence of amelanotic presentation. Its diagnosis is difficult for clinicians and pathologists because of its hidden location and lack of pigmentation at the microscopic level. We reported a case of amelanotic melanoma of the maxillary sinus that showed aggressive extension to the oral cavity after extr...
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... melanoma (AM) is a malignant tumor of the skin and mucous membrane in which the tumor cells fail to produce melanin (1). This variant of melanoma with aggressive behaviour may involve the nasal cavity and paranasal sinuses (2). Microscopic diagnoses of these tumours are difficult as they may reveal variable histological patterns and the absence of melanin pigmentation. Immunohistochemistry (IHC) is an essential ancillary technique in the diagnosis of AM. S-100 and HMB-45 are sensitive immunohistochemical markers that confirm diagnosis (1,2). Our case of mucosal AM, centred in the maxillary sinus, extended to the nasal and oral cavities. A 72-year-old male patient presented with the chief complaint of swelling over the left cheek at duration of five months. He also had pain in the same region in the last six months, followed by a one-month history of decreased vision of the left eye. The patient was evaluated by a dentist one month previously for pain in the left upper tooth. Extraction of the tooth was conducted, following which the patient started noticing a progressively increasing mass associated with pain at the extraction site. The patient underwent an incisional biopsy procedure and was found to have poorly differentiated squamous cell carcinoma. Afterwards, the patient was referred to us for further evaluation. Clinical examination of the patient’s face revealed facial asymmetry, narrowing of the palpebral aperture of the left eye, and erythematous bulbar conjunctiva. Moreover, finger counting was not possible beyond a 3 feets distance. A hard, fixed irregular mass measuring approximately 8 x 6 cm was found in the left maxillary region that was not tender. Intraoral examination showed two separate masses palpated on the entire left hard palate and left vestibule. Both masses had smooth surfaces and firm consistency, and were pink to red in colour (Figure 1). They appeared to protrude from the left maxillary antrum. No clinically palpable lymphadenopathy was identified upon neck examination. The impression of carcinoma of the left maxilla was made upon clinical examination. Radiological examinations were conducted to rule out any metastatic disease in the lungs and to determine the extent of the lesion. Chest radiograph revealed no metastatic disease in the lungs. Using a Philips Magnetometer 1.5 Tesla system, contrast enhanced magnetic resonance imaging (MRI) of the head and neck was performed. A large invasive left sinonasal mass, measuring 7.2 x 4.2 x 4.7, centred in the left maxillary antrum was found to cause prominent osseous destruction. A massive inferior extension of the tumor was determined through the destruction of the floor of the maxillary antrum and left hard palate. Compression of the left posterior tongue from above, with tumor infiltration of the left buccal space, was observed. Medially, no tumoural extension across the midline to the right side was found. Superiorly, scalloping of the floor of the left orbital, without direct intraorbital tumor invasion, was observed (Figure 2). No metastatic lymph nodes were identified in the imaging. Radiological differential diagnoses were squamous cell carcinoma, adenoid cystic carcinoma, mucoepidermoid carcinoma, or undifferentiated malignant lesion of the left maxillary sinus. An incisional biopsy of the lesion was performed, and the specimen was referred for histopathological evaluation. The microscopic examination showed cells that were round and plasmacytoid with a fibrillary background along with sparsely arranged myxomatous stroma in several places. These cells showed a high frequency of mitotic activity (Figure 3). Blood vessels showed perivascular hyalinisation and endothelial proliferation. These microscopic features presented the impression of a malignant round cell tumor. To confirm these tumour cells, we performed IHC with Pan cytokeratin, vimentin, S-100, HMB-45, desmin, and myogenin. IHC examinations revealed that the tumor cells showed strong staining for S-100 and HMB-45 (Figure 4). These results indicated that the lesion was amelanotic malignant melanoma. Surgical innervations were not suggested as the lesion involved a large intracranial region and showed proximity to other vital structures. Radiotherapy was planned for the patient. Unfortunately, the patient did not respond to the radiotherapy and did not survive after one month of initiation of therapy. Mucosal melanoma of the head and neck (MMHN) region is uncommon and constitutes only 1% of all melanomas. MMHN commonly involves the nasal cavity, sinonasal complexes, and oral cavity. These tumours are associated with aging and commonly occur in the sixth to eighth decades of life (3,4). The incidence of amelanotic presentation (20%–25%) is higher than that of cutaneous melanoma (1.8–8.1%) in the mucous membrane (5). Amelanotic mucosal ...
Citations
... [2,4] Amelanotic mucosal melanoma has been reported as case reports and forms a small part of large reported series. [2,3,[7][8][9] The present case was unusual with a much younger age of presentation of 35 years. The lesion was polypoidal and nonpigmented, which suggested a clinical diagnosis of inverted papilloma. ...
Mucosal melanomas are rare head‑and‑neck tumors and have a distinct etiology and genetic profile different from cutaneous counterparts. They are most commonly seen in sixth–seventh decade of life and have aggressive behavior. The patients usually present with nodal metastasis, which is the most important poor prognostic indicator for mucosal melanomas. We present a case of mucosal melanomas in a young male (30 years), who presented with nonpigmented, polypoidal mass in the
right nasal cavity simulating an inverted papilloma. Histological examination showed small to large pleomorphic cells and no melanin pigment. Based on the immunopositivity of the tumour cells were positive for HMB45 and S100 confirming a diagnosis of amelanotic mucosal melanoma. The partied had a localized disease to the right nasal cavity with no nodal involvement and no recurrences in the available follow‑up of 24 months.
... The most frequent symptoms are nasal obstruction, epistaxis or nasal discharge, polyp, and rarely pain, so they are often diagnosed in the late stages when the lesions are already large and lymph node metastases have developed 1,6,8 . Up to 10-30% of subjects with sinonasal melanomas will present positive lymphadenopathy at diagnosis, resulting in a poor prognosis [9][10][11] . Microscopically, the presence of melanin on the epithelial surface derived from neoplastic pigmented melanocytes is the main characteristic of this lesion 12,13 ; however, there is an amelanotic variant that does not present melanin, which represents a diagnostic challenge for pathologists 6 . ...
... Regarding the location of the lesion, the nasal cavity was the most affected site, followed by the maxillary sinus, ethmoid sinus, and paranasal sinus not specified; this is in agreement with the literature 1,11,21 . The main symptoms observed were epistaxis, nasal obstruction, and blurry vision, which is in accordance with previous reports, and these occur due to the expansive process of the tumour and neurovascular invasion 6,22 . ...
... In malignant melanoma, most tumour cells appear pigmented and hence the diagnosis of melanin-rich melanoma is not difficult 8,19 . In contrast, a diagnostic challenge occurs in amelanotic melanoma 11,12,24 . In melanoma, tumour cells are arranged in sheets with oval to round nuclei and prominent nucleoli. ...
The objective was to evaluate the available published data on sinonasal melanoma and analyse its clinical features, treatment modalities, and prognostic factors. An electronic search was undertaken in March 2018 in multiple databases. Eligibility criteria included publications with sufficient clinical, histological, and immunohistochemical information to confirm the diagnosis. Seventy-three publications (439 cases) were included. The lesion was more prevalent in females than in males. There was a higher prevalence in the seventh and eighth decades of life. The lesions mainly presented as epistaxis and commonly involved the nasal cavity. Age (>67.6 years; P=0.0012), primary location (middle turbinate; P=0.0112), disease stage (advanced disease stage; P=0.0026), treatment (radiotherapy; P=0.0111), recurrence (recurrence presented; P=0.0137), and distant metastasis (distant metastasis presented; P=0.0011) were independently associated with a lower survival rate. Recurrence was significantly correlated with age (>67.6 years; P=0.0021), sex (males tended to present a higher recurrence rate than females; P=0.0051), disease stage (stages III and IV presented a higher recurrence rate than stages I and II; P=0.0331), and histological type (amelanotic lesions presented a higher index of recurrence than melanotic lesions; P=0.0095). In conclusion, sinonasal melanoma is a neoplasm with a poor prognosis, presenting a 30.69% possibility of survival after 5 years.
... Among nasal MM cases rare were describe as AMs. 1 Thus, some authors presented a case of AM in female patient with a mass in left nasal, and other a case of sinonasal cavity AM with intraoral extension. 4,5 Local recurrences in MM occur in about 50% of cases. 2,6 This could be the main reason for poor prognosis and survival. ...
The aim of this report is to present a case of a patient with a recurrent nasal cavity amelanotic melanoma (AM), with emphasis on diagnosis and therapy options of this clinical entity. A 65-year-old female patient presented with pain in the right cheek region and nasal obstruction. In 2013, she was diagnosed with mucosal melanoma (MM) of the left nasal cavity. After endoscopic surgery and radiotherapy, the patient was followed by the oncology team. Five years after the initial diagnosis, rhinoscopy showed a tumorous formation in the right nasal cavity. The tumor mass was without black discoloration and was the same color as the surrounding nasal mucosa. Microscopic examination after biopsy of the tumor confirmed amelanotic MM. The patient underwent an additional endoscopic surgery. A complete standard diagnostic workup for MM found metastases in head and neck lymph nodes, on both sides. MMs of head and neck are uncommon malignancies. Unique biology of MM cells causes a high rate of recurrences. This report presents an example of recurrent AM of the nasal cavity, in treatment with checkpoint inhibitor (pembrolizumab), which could provide a good therapy option for patients with MM.
Oral amelanotic melanoma (OAM) is a rare, non-pigmented mucosal neoplasm representing less than 2% of all melanoma. The present study analyses the available data on OAM and describes its clinicopathological features, identifying potential prognostic factors. Online electronic databases such as PubMed-Medline, Embase, and Scopus were searched using appropriate keywords from the earliest available date till 31st March 2021 without restriction on language. Additional sources like Google Scholar, major journals, unpublished studies, conference proceedings, and cross-references were explored. 37 publications were included for quantitative synthesis, comprising 55 cases. The mean age of the patients was 59.56 years, and the lesions were more prevalent in males than in females. OAM’s were most prevalent in the maxilla (67.2%) with ulceration, pinkish-red color, nodular mass, and pain. 2 patients (3.36%) were alive at their last follow-up, and 25 were dead (45.4%). Univariate survival analysis of clinical variables revealed that age older than 68 years (p = 0.003), mandibular gingiva (p = 0.007), round cells (p = 0.004), and surgical excision along with chemotherapy & radiation therapy (p = 0.001) were significantly associated with a lower survival rate. Oral Amelanotic Melanoma is a neoplasm with a poor prognosis, presenting a 6.25% possibility of survival after 5 years. Patients older than 68 years, lesions in the mandibular gingiva, round cells, and surgical excision along with chemotherapy and radiotherapy, presented the worst prognosis. However, they did not represent independent prognostic determinants for these patients.
Malignant melanoma is a malignant tumor derived from melanocytes, and typically occurs in the skin. Malignant mucosal melanoma rarely arises in the oral cavity, accounting for 0.5％ of all malignant melanomas. Amelanotic malignant melanoma lacking melanin pigment accounts for 2.0％ of malignant mucosal melanomas in the oral region. Here, we describe a patient who exhibited amelanotic malignant melanoma in the maxillary gingiva and palate.
A 64-year-old woman was referred to our department for examination and treatment of a nodular lesion in the right maxillary gingiva in February 2018. At the initial visit, a nodular lesion measuring 60×45mm was observed in the right maxillary gingiva. A biopsy was performed on the lesion, and histopathological evaluation yielded a diagnosis of amelanotic malignant melanoma. On preoperative analysis, the lesion was classified as cT4aN0M0, so we performed right maxillectomy. Postoperative histopathological analyses identified the tissue as an amelanotic malignant melanoma with venous and lymphatic invasion. Therefore, postoperative adjuvant therapy was planned. However, adjuvant therapy could not be performed in this case for economic reasons and because the patient did not consent to further treatment. Metastasis in the small intestine occurred 1 year postoperatively, and the patient died 6 months later.
