Dados do Trabalho

Título

MANAGEMENT OF A RARE CASE OF SINONASAL MUCOSAL MELANOMA: THE EXPERIENCE OF A BRAZILIAN CANCER CENTER

Apresentação do caso

Mucosal melanoma (MM) is a rare and aggressive malignant neoplasm, which represents 1% of all melanoma cases that 55% are located in the head and neck. The pathogenesis molecular comprehension by clinicians can lead to the use of targeted therapies. This case report aims to describe an atypical case of nasal mucosal melanoma (NMM) in an advanced clinical stage. A 64 years old female patient, who had started for 03 months with sporadic episodes of nasal congestion and epistaxis, progressed with edema and ocular proptosis two months later. The Magnetic Resonance Imaging exam showed the presence of an expansive nasal/orbital lesion on the right side that infiltrated the encephalic's parenchyma. After histopathological assessment, the diagnosis of primary MM was confirmed. On the other hand, the molecular analysis showed mutation in codon 13 of the NRAS. The treatment was established after the medical multidisciplinary reunion based on 04 cycles of immunotherapy with Nivolumab 3mg/kg and Ipilimumab 1mg/kg concomitantly with Radiotherapy (RT) by the Intensity Modulated Technique (IMRT) with a total dose of 40Gy into the lesion and 30Gy to the holocephalic metastasizing lesion programmed in 10 sessions. However, only 7 sessions and 1 cycle of immunotherapy were performed. The patient developed intratumoral and intraparenchymal cerebral hemorrhage that caused brain death.

Discussão

NMM is extremely rare with poor prognosis that arises into the nasal cavity that involves the turbinates and nasal walls without symptoms at the initial stages. Surgical resection is the primary treatment chosen; however, due to its location closer to a complex anatomy and microscopic infiltration observed, it has become complicated to obtain free surgical margins by oncologic surgeons. RT has been used at total doses ranging from 35 to 60Gy for local control and to avoid clinical failures. Target therapies have been addressed to inhibit mutations of genes such as BRAF and KIT, besides immunotherapy protocols with anti-CL-4 and anti-PD-1 used for MM management that recent studies have shown increased rates of overall survival (OS).

Comentários finais

Advanced-stage of NMM showed a poor prognosis and its early diagnosis and multimodal management are mandatory for locoregional control. For metastasized lesions, targeted therapies and immunotherapy protocols are recommended in order to increase rates of OS, mainly when associated with surgical resection. In cases of unresectable tumors, RT could be considered together.

Palavras-chave

head and neck neoplasms, melanoma, combined modality therapy