Abstract
Non skin malignant melanomas are known to be more aggressive than the skin counterpart. A 50-year lady with recurrent epistaxis, had mass in right sphenoid, which was resected by endoscopic sinus surgery. After histopathological confirmation of malignant melanoma radiotherapy was given. She later developed metastasis in liver.
Keywords: Malignant, Melanoma, Sphenoid, Endoscopic
Introduction
Melanomas are tumours of melanocytes, which are normally present in the skin, mucous membranes, piamater, and uvea of humans. Malignant melanomas of sphenoid occur in less than 1% of all melanomas and less than 5% of all paranasal sinus neoplasms [1]. They usually present in the 5th–8th decade with symptoms like nasal obstruction, epistaxis or nasal discharge, polyp, and rarely pain [1].
Case Report
Here, we report a rare case of malignant melanoma arising from sphenoid sinus. A 50 years old lady presented to the surgical oncology outpatient department of Rajiv Gandhi Cancer Institute and Research Centre (RGCIRC) with recurrent headaches and nasal bleeding for 2 months. She was operated previously at Nepal; histopathological examination (HPE) of the resected mass showed malignant melanoma. On clinical examination, no growth or ulcer was found in nasal cavity, oropharynx. No palpable nodes were found in neck. Magnetic resonance imaging (MRI) of head and neck demonstrated a small nodular mass measuring 0.8 × 0.7 cm at the anterolateral aspect of right side of sphenoid sinus, at postoperative site. Whole body Position emission tomography Computed tomography (WBPETCT) was done to rule out any metastatic lesion. Trans-nasal endoscopic sinus surgery was performed; Narrow band imaging done per operatively did not reveal any other mucosal lesion. Postoperative (maxillary antrostomy, anterior and posterior Ethmoidectomy) status noted. A black pigmented lesion at sphenoid sinus (Fig. 1) was found which was removed completely. The surgical specimen was sent for HPE, which showed tissue lined by respiratory epithelium with anaplastic cells, showing intracytoplasmic brown pigment. Immunohistochemistry showed tumor cells expressing S100, HMB-45 and SOX-100, but negative for CK which confirmed malignant melanoma. She was given adjuvant 70 Gy/32# of Intensity Modulated Radiotherapy. WBPETCT done 3 months post treatment revealed no scan evidence of disease. Ten months post surgery, she was diagnosed to have space occupying lesions in liver on USG abdomen. WBPETCT suggested metastatic liver lesions. No other metabolically active disease locoregionally. The patient was negative for cKIT and BRAF mutation. She was given option for Immunotherapy but due to logistic issues she did not opt for it. She has been given 6 cycles of Nab-Paclitaxel and recent scans show stable disease.
Fig. 1.
Endoscopic photograph showing melanoma arising from right sphenoid sinus
Discussion
Malignant melanoma of the sphenoid sinus is an extremely rare tumour, comprising approximately 1.5% of all malignant tumors. It metastasizes primarily through haematogenic route. There are very few cases reported worldwide. The comprehensive review of the cases reported has been described in Table 1 [1–6].
Table 1.
| Sl no | Country, Year | Age, Sex | Symptoms | Treatment done | Outcome |
|---|---|---|---|---|---|
| 1 | New Delhi, India, 2019 | 50 year, F | Recurrent headaches, nasal bleeding | Endoscopic sinus surgery followed by CT, RT | Metastasis to liver, Patient alive with disease |
| 2 | Ohio, US, 2015 [1] | 70 year, M | Diffuse throbbing headache, lightheadedness and lassitude, bilateral blurry vision and diplopia | Endoscopic endonasal tumor resection foll by RT | Diffuse metastasis and death 7 later |
| 3 | China, 2014 [2] | 57 year, M | Nasal congestion and epistaxis | Subtotal tumorectomy of the left sphenoid sinus tumor using a nasal endoscope followed by RT, CT and Immunotherapy | Gastric metastasis and death 2 months later |
| 4 | Japan, 2013 [3] | 74 year, F | Loss of visual acuity in left eye | Endoscopic sinus surgery followed by CT | Metastasis to spine, ischium and death 3 months later |
| 5 | Philadelphia, US 2005 [4] | 75 year, M | occipital headache, double vision | Stereotactic RT and Palliative CT | Metastasis to liver, lower lobe lung, Patient alive with disease |
| 6 | Spain, 2004 [5] | 56 year, M | Ocular pain, Headache | FESS foll. by RT | Patient disease free and on follow up |
| 7 | Japan, 2000 [6] | 83 year, F | Headache, left nasal obstruction, left nasal bleeding, left eye visual disturbance | Conservative treatment, CT and RT | Death due to multiorgan failure |
The diagnosis of such cases is difficult owing to the wide spectrum of symptoms in patients, i.e., headache, blurriness of vision, nasal bleeding etc. Clinicians rely mainly upon radiological findings and histopathology of the resected tumour to confirm diagnosis. Computed Tomography (CT) findings of primary intracranial melanoma have varied results, hence not diagnostic. MRI of head and neck gave a clearer picture, with hyperintense signal on T1W1 and hypointense signal on T2W/STIR images in our case, which is true for many cases of primary intracranial malignant melanoma. The T1 and T2 relaxation times are both shortened by presence of free radicals which are paramagnetic, due to presence of melanin in malignant melanoma. This is important for diagnosis, except in amelanotic melanomas which do not produce melanin, and in cases of intratumor bleeding [6]. Histopathologically, malignant melanomas have to be differentiated from malignant lymphoma and metastatic tumors.
The 5 year survival rates in these cases are only 10–15%. Involvement of skull base and orbit are the most important prognostic factors, affecting the survival rates. Endoscopic resection of the tumor remains the treatment of choice, in feasible cases. However, in progressive cases associated with co morbidities, radiotherapy and chemotherapy are the only options available along with palliative support. Recently, newer modalities like biologic and immunomodulatory treatments like OK-432, interleukin 2, lymphokine-activated killer cells, and Bacille Calmette-Guerin (BCG) vaccine are being studied under trial [7].
Given the rarity of the case and the poor prognosis, the present case is an interesting report. Surgery followed by radiotherapy still continues to be standard of treatment. Surgical plan (endoscopic/open approach) is guided by the extent of disease. Follow up investigations in our case suggested no evidence of locoregional recurrence. Palliative care with chemotherapy helps patient to survive with disease.
Author Contributions
Dr. GM: Concept and design of study, acquisition of data/analysis and interpretation of data. Dr. VY: Manuscript review. Dr. VA: Manuscript review. Dr. AKD: Manuscript review and final approval of the version to be published.
Funding
None.
Compliance with Ethical Standards
Conflict of interest
All authors declare that they have no conflict of interest.
Footnotes
Publisher's Note
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References
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