Sinonasal mucosal melanoma: treatment strategies and survival rates for a rare disease entity

Abstract

Summary Background Sinonasal mucosal melanoma (SNMM) is a rare disease entity comprising 0.4–1.3% of all melanomas. Surgery with free margins has been the primary treatment over decades. Neither the addition of radiotherapy nor chemotherapy could significantly improve outcome rates of this devastating malignancy. This study presents our clinical experience with SNMM over a 19-year period and summarizes the current body of literature on SNMM. Methods This retrospective analysis included 12 patients with SNMM treated from 2001 to 2019 at an academic center. Additionally, a literature review of the last 29 years on treatment and survival data of SNMM was conducted. Results Main initial symptoms were epistaxis and nasal obstruction. Of the patients 9 underwent endoscopic surgery, 6 received adjuvant therapy. 3 patients who did not undergo surgery, received chemoradiotherapy, radiotherapy alone, and chemotherapy alone, respectively. At the time of diagnosis 2 patients had distant metastases and 4 patients developed distant metastases during the course of the disease. Mean overall survival (OS) was 30.6 months, 3‑year and 5‑year OS were 25%, and 18.2%, respectively. Conclusion Unspecific symptoms and hidden anatomic locations lead to delayed diagnosis and increased rates of metastatic dissemination. Distant metastasis is the main treatment failure in SNMM. Surgery with free margins remains the primary treatment for SNMM. Adjuvant radiotherapy might improve local control in individual cases but efficient systemic therapy is needed to improve outcome rates. To evaluate and define more effective targeted treatment options and improve outcome rates, homogeneous data and prospective multicentric analysis are needed.

Sinonasal malignant melanoma

“…In contrast, stereotactic radiotherapy (1,200 cGy in 2 fractions) is recommended for adrenal metastasis 15 . Systemic therapy does not bring the desired effects in survival 16 . Chemotherapeutic agents -actinomycin D and cisplatin -are used in advanced stages, usually for palliative purposes or when surgery is contraindicated.…”

Exceptional response to oral metronomic chemotherapy in a rare case of sinonasal mucosal melanoma

“…Features predictive of poor prognosis were vascular invasion, necrosis and a polymorphous population of tumour cells; while features like thickness of tumour, degree of invasion, ulceration, mitotic index and neural involvement had little effect on prognosis [1]. According to literature review done by Andrianakis et al [6], nasal cavity primary tumours have a better prognosis than primary tumours originating from paranasal sinus possibly because of delayed diagnosis of paranasal sinus tumour due to its 'hidden anatomic location'. Other significant prognostic factors are negative margin after resection which can be challenging due to complexity of the anatomy of sinonasal cavity and its proximity to vital structures, advanced stage, and interestingly, level of pigmentation.…”

“…Chemotherapy and immunochemotherapy can be used for adjuvant treatment for distant disease and also neoadjuvant chemotherapy. Most of the trials studied by Gore and Zanation [2] used a single agent chemotherapy regimen, dacarbazine (DTIC) (used in our patient), temozolomide or vindesine [1,[5][6][7]. Anti-angiogenic therapies also play a role in the management of mucosal melanoma, including bevacizumab.…”

“…According to literature review done by Andrianakis et al . [ 6 ], nasal cavity primary tumours have a better prognosis than primary tumours originating from paranasal sinus possibly because of delayed diagnosis of paranasal sinus tumour due to its ‘hidden anatomic location’. Other significant prognostic factors are negative margin after resection which can be challenging due to complexity of the anatomy of sinonasal cavity and its proximity to vital structures, advanced stage, and interestingly, level of pigmentation.…”

“…Meta-analysis done by Gore and Zanation [ 2 ] and literature review done by Andrianakis et al [ 6 ] recommend that aggressive surgical resection of the tumour with confirmed free margins is the primary modality of treatment. With technological advances, endoscopic resections are comparable to external approaches.…”