Survival of patients with mucosal melanoma in Cali, Colombia: a retrospective cohort study.

BACKGROUND: Mucosa melanoma is a rare condition with aggressive behavior and a less favorable prognosis compared to cutaneous melanoma. The objective of this study was to estimate the overall survival and clinical outcomes of patients diagnosed with mucosal melanoma in a Colombian hospital. METHODS: A retrospective cohort study was conducted at Fundación Valle del Lili, a single center located in Cali, Colombia. Patients aged ≥ 18 years, both sexes, diagnosed with mucosal melanoma by histopathology study were included between 2010-2019. Patients who received extra-institutional treatment or whose vital status was unknown during follow-up were excluded. Demographic, clinical and laboratory data were obtained from medical records and laboratory and pathology databases. A descriptive analysis was performed. Survival analysis was conducted using the Kaplan-Meier method. RESULTS: A total of 23 patients were included. Median age was 63 years old (IQR: 57-68) and 52.2% were woman. Clinical stage was 34.8% early, 26.1% locally advanced and 39.1% metastatic. The main primary locations were nasopharynx (30.4%), genitals (26.1%), rectum (21.7%), oral cavity (13%) and paranasal sinuses (8.7%). The majority received surgery (30.4%) and immunotherapy (26.1%) as first line treatment. Overall survival at one year was 80.8%, at three years 44.3%, and at five years 36.9%. CONCLUSION: Mucosal melanoma is a rare, aggressive disease with adverse oncological outcomes due to late diagnosis and limited treatment options. This study provides real-world data in a single-center of Colombia.

Oral Mucosal Melanoma: A Case Report and Literature Review

ABSTRACT: Oral mucosal melanoma is an unusual and aggressive malignant tumor that mainly affects the palate of men aged between 50 and 60 years. We present a literature review focusing on the etiopathogenesis and the clinicopathologic features of this entity. In addition, we reported a rare case of an oral mucosal melanoma arising in the left cheek of a 60-yea r- old man. Computed tomography scan revealed infiltration of the tumor to other anatomic structures including the maxillary sinus, the zygomatic bone and the pterygoid processes. Based on its extension, the lesion was considered inoperable and treatment with three-dimensional conformal radiation therapy was proposed but the patient only attended to the first session and died from cancer progression 6 months after the diagnosis. This paper reinforces the importance of inclusion of this malignant tumor in the differential diagnosis of pigmented lesions of the oral mucosa.

RESUMEN: El melanoma de la mucosa oral es un tumor maligno inusual y agresivo que afecta principalmente al paladar de hombres de entre 50 y 60 años. Presentamos una revisión bibliográfica centrada en la etiopatogenia y las características clínico-patológicas de esta entidad. Además, reportamos un caso raro de melanoma de la mucosa oral que surgió en la mejilla izquierda de un hombre de 60 años. La tomografía computarizada reveló la infiltración del tumor a otras estructuras anatómicas, incluido el seno maxilar, el hueso cigomático y los procesos pterigoideos. En base a su extensión, la lesión se consideró inoperable y se propuso tratamiento con radioterapia conformada tridimensional pero el paciente solo asistió a la primera sesión y falleció por progresión del cáncer 6 meses después del diagnóstico. Este trabajo refuerza la importancia de la inclusión de este tumor maligno en el diagnóstico diferencial de las lesiones pigmentadas de la mucosa oral.

Melanoma nasofaríngeo: caso clínico / Nasopharyngeal melanoma: clinical case

Resumen El melanoma mucoso primario (MM) de la cavidad nasal y los senos paranasales sigue siendo un tumor maligno poco frecuente. El pico de incidencia se sitúa entre 50 y 60 años, siendo la epistaxis y la obstrucción nasal unilateral los síntomas más frecuentes. La resección quirúrgica es el tratamiento primario con radioterapia y quimioterapia adyuvante si es necesario, el pronóstico depende de la extensión. Presentamos el caso de una paciente mujer de 81 años con hemoptisis y halitosis de un año de evolución. La nasofibrolaringoscopia mostró una lesión parduzca, excrecente, irregular y friable, en el borde derecho del rodete tubárico derecho. El resultado histopatológico fue MM nasofaríngeo. Perfil inmunohistoquímico: S100 +, MelanA +, HMB45 +, SOX10 +, AE1AE3 negativo. El PET-CT mostró una hipercaptación a nivel del rodete tubárico derecho, sin evidencia de adenopatías o metástasis a distancia. Se realizó la resección tumoral completa mediante abordaje endoscópico. El Comité de Oncología del Melanoma decidió que no era necesario un tratamiento adyuvante debido a la resección completa de la lesión y considerando la edad y el deterioro cognitivo de la paciente.

Abstract Primary mucous melanoma (MM) of the nasal cavity and paranasal sinuses remains a rare malignancy. The peak of incidence is between 50 and 60 years old, being epistaxis and unilateral nasal obstruction the most frequent symptoms. Surgical resection is the primary treatment with radiotherapy and adjuvant chemotherapy if necessary, the prognosis depends on the spreading. We present the case of an 81-year-old female patient attending for hemoptysis and halitosis of a year of evolution. The nasofibrolaryngoscopy showed a brownish, excrescent, irregular and friable lesion, outgrowth of the right tube rim. Histopathology result was nasopharyngeal MM. Immunohistochemical profile: S100 +, MelanA +, HMB45 +, SOX10 +, AE1AE3 negative. PET-CT showed a hypercaptation at the level of the right tube rim, without evidence of adenopathies or distant metastases. Complete tumor resection was performed by endoscopic surgery. Melanoma Oncology Committee decided no need of adjuvant therapy due to the complete resection of the lesion and considering the age and cognitive deterioration of the patient.

Estudo da frequência da mutação BRAF p. V600E em nevos melanocíticos e melanomas mucosos orais / Study of BRAF p. V600E mutation frequency in oral melanocytic nevi and oral mucosal melanomas

Nevos melanocíticos são neoplasias benignas derivadas de melanócitos. O nevo melanocítico adquirido comum cutâneo é frequente na pele humana e apresenta maior incidência na terceira década de vida. E, embora uma taxa pequena de transformação maligna tenha sido estimada, considera-se que os nevos adquiridos sejam precursores de uma parcela dos melanomas cutâneos. A mutação somática BRAF p.V600E, que ativa a via MAPK/ERK e proliferação celular, está implicada na formação dos nevos adquiridos comuns de pele e de um grupo de melanomas cutâneos, de sítios não cronicamente expostos ao sol. A partir da caracterização molecular do melanoma seu tratamento foi aprimorado pelo uso de inibidores de Braf e Mek. O nevo melanocítico adquirido mucoso oral (NMO) e o melanoma mucoso oral (MMO) são lesões raras e de patogênese incerta. Há escassa literatura sobre aspectos moleculares do NMO e um número ligeiramente maior de estudos sobre os MMOs, em sua maioria em séries que englobam uma mistura de diferentes tipos de melanomas mucosos de diversos sítios. No presente estudo, investigou-se a mutação BRAF p.V600E em um grupo de 14 NMOs intramucosos e 7 MMOs primários, excluídas amostras de lábio, por meio de reação em cadeia da polimerase alelo-específico (PCR-AE). Realizou-se também uma revisão narrativa de literatura para calcular a frequência da mutação BRAF p.V600E em NMOs e MMOs. Foram incluídos artigos originais em língua inglesa que exibissem o sítio primário da lesão e status mutacional, seja por amostra ou sua frequência. Informações sobre a idade dos pacientes, país de origem e tipo de tumor, se primário, recorrente ou metastático, e técnica de análise do DNA utilizada também foram coletadas. Cinco das quatorze amostras de NMOs (35,7%) avaliadas no presente trabalho foram positivas para BRAF p.V600E, enquanto três das sete amostras de MMOs (42,8%) exibiram a mutação. Na revisão narrativa de literatura, em conjunto com nossos resultados, 19 NMOs foram avaliados e 8 NMOs apresentaram a mutação BRAF p.V600E, correspondendo a uma frequência de 42,1%. Dos 374 MMOs avaliados, 24 MMOs exibiram a mutação BRAF p.V600E, totalizando a frequência de 6,4%. Em conclusão, amostras de NMOs e MMOs foram analisadas quanto à presença da alteração genética oncogênica BRAF p.V600E. E junto à revisão da literatura pode- se calcular a frequência da mutação em NMOs e MMOs, contribuindo para uma melhor caracterização molecular dessas lesões.

Melanocytic nevi are benign neoplasms derived from melanocytes. Common cutaneous acquired melanocytic nevus is frequently in human skin and it has a higher incidence in the third decade of life. Although a low rate of malignant transformation is estimated, a portion of cutaneous melanoma is preceded by a melanocytic acquired nevus. BRAF p.V600E somatic mutation activates the MAPK/ERK pathway and cell proliferation. It is implicated in the cutaneous melanocytic acquired common nevus pathogenesis and cutaneous melanoma that arise in sites not chronically sun-exposed. After melanoma molecular description, its therapeutic was improved by Braf and Mek inhibitors. Oral mucosal acquired melanocytic nevus (NMO) and oral mucosal melanoma (MMO) are rare lesions with uncertain pathogenesis. There is scanty literature about NMOs molecular features and few studies on MMOs. Most articles are series that evaluate mucosal melanomas from several sites collectively. In the present study, BRAF p.V600E mutation was assessed in 14 intramucosal NMOs and 7 primary MMOs, excluding lip samples, by allele specific quantitative polymerase chain reaction (AS-qPCR). A narrative literature review had been performed to calculate BRAF p.V600E frequency in NMOs and MMOs. Original articles in English language were included, since it was possible to identify the primary sample site and mutational status, by sample or its frequency. Data about patient age, country, type of tumor (primary, recurrent or metastatic) and sequence technique used also were collected. Five in fourteen NMOs samples (35.7%) analyzed in the present study were BRAF p.V600E positive and three in seven MMOs samples (42.8%) showed the mutation. In the narrative literature review, added to our results, 19 NMOs were evaluated and 8 NMOs presented BRAF p.V600E mutation, corresponding to a frequency of 42.1%. Between 374 MMOs evaluated, 24 MMOs showed the mutation totalizing the frequency of 6.4%. In conclusion, BRAF p.V600E oncogenic mutation was assessed in NMOs and MMOs samples. Additionally, in combination with the literature review, it calculated the mutation frequency in NMOs and MMOs, improving the molecular characterization of those lesions.

Uncommon Subtypes of Malignant Melanomas: A Review Based on Clinical and Molecular Perspectives.

Malignant melanoma represents the most aggressive type of skin cancer. Modern therapies, including targeted agents and immune checkpoint inhibitors, have changed the dismal prognosis that characterized this disease. However, most evidence was obtained by studying patients with frequent subtypes of cutaneous melanoma (CM). Consequently, there is an emerging need to understand the molecular basis and treatment approaches for unusual melanoma subtypes. Even a standardized definition of infrequent or rare melanoma is not clearly established. For that reason, we reviewed this challenging topic considering clinical and molecular perspectives, including uncommon CMs-not associated with classical V600E/K BRAF mutations-malignant mucosal and uveal melanomas, and some unusual independent entities, such as amelanotic, desmoplastic, or spitzoid melanomas. Finally, we collected information regarding melanomas from non-traditional primary sites, which emerge from locations as unique as meninges, dermis, lymph nodes, the esophagus, and breasts. The aim of this review is to summarize and highlight the main scientific evidence regarding rare melanomas, with a particular focus on treatment perspectives.

Melanoma anal, patología infrecuente, reporte de un caso / Anal melanoma uncommon pathology, case report / Melanoma anal, patologia infreqüente, relato de um caso

El melanoma anal (MA) es un tumor infrecuente, caracterizado por presentar un comportamiento agresivo y mal pronóstico(1). La sintomatología inespecífica con la cual se presenta y su similitud con etiologías benignas anorrectales, dificulta el diagnóstico y puede conducir a un retraso en el mismo(2)(3). Por lo tanto, es necesaria una exploración física minuciosa y un elevado índice de sospecha. Se discute el caso de una paciente que consultó por tumoración anal dolorosa, no pigmentada y rectorragia; a la que se le realiza diagnóstico de melanoma anal.

Anal melanoma (MA) is an infrequent tumor, characterized by aggressive behavior and poor prognosis(1). The nonspecific symptomatology and its similarity with benign anorectal etiologies, makes the diagnosis difficult and leads to a delay in it(2)(3).Therefore, a thorough physical examination and a high index of suspicion are necessary. The case of a patient who consulted for a non-pigmented and painful anal mass with rectal bleeding is discussed; which is diagnosed with anal melanoma.

O melanoma anal (MA) é um tumor infrequente, caracterizado por apresentar um comportamento agressivo e ter um mauprognóstico(1). A sintomatologia inespecífica da sua apresentação e a sua similaridade com etiologias benignas anorretais dificultam o diagnóstico e pode levar a um atraso do mesmo(2)(3). Por tanto é necessário uma exploração física minuciosa e um indice de suspeita elevado. Discute-se o um caso de uma paciente que consultou por uma tumoração anal, dolorosa, não pigmentada e retorragia, a qual se realizou o diagnostico de melanoma anal.

Melanoma ano-rectal amelanótico primario. reporte de un caso / Primary amelanotic ano-rectal melanoma. a case report

RESUMEN: Se presenta el caso de una mujer de 37 años, que acude a la consulta por sensación de bulto anal y hematoquecia de 3 meses de evolución, con antecedente de estreñimiento. No presenta prurito, dolor anal ni pérdida de peso. A la inspección, presenta lesión tumoral de 3 cm, en cuadrante superior izquierdo de la región anal, de aspecto denudado, color rosado y mucosa anal hiperpigmentada. La histopatología reporta un tumor de células redondas, sugestivo de melanoma. La inmunohistoquímica confirma finalmente el diagnóstico, pues reporta positividad a los marcadores S100, HMB45 y Vimentina. Al realizar el estudio a los cuatro meses de evolución de extensión de la enfermedad, se encuentran implantes de la neoplasia a nivel pulmonar e inguinal, lo que demuestra la agresividad y el pobre pronóstico de esta patología.

SUMMARY: We present the case of a 37-year-old woman, who came to the clinic for a sensation of anal bulge and hematochezia of 3 months of evolution, with a history of chronic constipation. No pruritus, anal pain or weight loss. On inspection, she presented a 3 cm tumor in the upper left quadrant of the anal region, pink and denuded in appearance. The adjoining mucosa was hyperpigmented. Histopathology reports a round cell tumor, suggestive of melanoma. Immunohistochemistry finally confirms the diagnosis, since it reports positivity to S100, HMB45 and vimentin markers. When performing the disease extension study, implants of the neoplasm are found at the pulmonary and inguinal level, which demonstrates the aggressiveness and poor prognosis of this pathology.

Analysis of KIT gene mutations in patients with melanoma of the head and neck mucosa: a retrospective clinical report.

Unlike their cutaneous counterparts, head and neck mucosal malignant melanomas (HNMM) are more aggressive, and their prognostic markers have not been fully elucidated. This study, comprising 28 patients with HNMM, aimed to establish the relationship between different mutations and outcome, define the incidence of KIT mutations in HNMM, and identify the correlation among therapeutic options, histopathological findings, demographic data, and clinical response. Clinical analysis included patient characteristics, staging, primary and palliative treatments, and disease-free survival and overall survival (OS). Progression-free survival and OS were analyzed. Paraffin blocks were selected following histologic analyses, enabling DNA extraction. PCR amplification of exons 9, 11, 13, and 17, with different DNA concentrations, was performed. Patients were predominantly females (57%) and aged 27-85 years. All patients underwent surgery; 17 received adjuvant radiotherapy, and recurrences occurred in 82% patients. Oncologic mutations in KIT were found in 7 of 7 tumors, 3 in exon 9, 3 in exon 11, and 1 in exon 13. Predictive factors for recurrence were mitotic rate, vascular invasion, and perineural spread. There were no significant differences in DFS and OS according to KIT mutation. Our study results suggest that some patients might benefit from appropriate targeted therapy with kinase inhibitors.

Utilidad de imagen de banda estrecha en un caso de melanoma mucoso de septum nasal / Utility of narrow band Imaging in a nasal septum mucosal melanoma case

En los últimos años, se está utilizando la fibrolaringoscopía con imagen de banda estrecha (NBI) como técnica novedosa para observar el patrón específico de microvas-cularización de una lesión concreta a evaluar. Es conocida por su utilidad en el diagnóstico de otras lesiones de vías aerodigestivas superiores, fundamentalmente laríngea y digestiva. Los melanomas mucosos son tumores infrecuentes, que suelen localizarse a nivel del área rinosinusaly que comportan un manejo y pronóstico distinto con respecto a los melanomas cutáneos. Se presenta el caso clínico de una paciente mujer con anamnesis, exploración y fibrolaringoscopía con imagen de banda estrecha, compatible con melanoma mucoso de fosa nasal izquierda. El tratamiento realizado fue quirúrgico, sin necesidad de tratamiento coadyuvante, y no presenta evidencia de enfermedad al año postseguimiento.

In recent years, it is being used fibrolaryngoscopy with narrowband image (NBI) as a novel technique to observe the specific pattern of microvasculature of a particular lesion. NBI is known for its usefulness in the diagnosis of other lesions of the upper aerodigestive tract, (primarily laryngeal and digestive lesions). Mucosal melanomas are rare tumors, which are usually located at the level of rhino-sinusal area and involving a different prognosis and management regarding cutaneous melanomas. We report a female patient case with anamnesis, clinical examination and NBI compatible with mucosal melanoma of left nostril. Surgical treatmentwas performed without adjuvant therapy, and there is no evidence of disease at one year post-monitoring.

In Situ Melanoma of the Gingiva Associated with Dense Inflammation and Pigment Deposition: A Potential Diagnostic Pitfall in Evaluating Stromal Invasion.

Melanoma is a highly aggressive neoplasm in which the neoplastic cells display melanocytic differentiation. Less than 1 % of all melanomas arise in the mucosal surfaces of the oral cavity, with purely in situ lesions being exceedingly rare tumors, but with a favorable prognosis compared with invasive lesions. We describe the clinical, histopathological and immunohistochemical findings in an uncommon case of in situ mucosal melanoma of the oral cavity with intense tumor-associated pigment deposition and inflammation complicating the morphological assessment of infiltrative areas.

Head and neck mucosal melanoma: clinicopathological analysis of 51 cases treated in a single cancer centre and review of the literature.

Head and neck mucosal melanoma (HNMM) is a rare and aggressive malignancy. The objective of this study was to describe the outcomes of patients with HNMM. Clinical and pathological data from 51 patients with primary HNMM were reviewed. All patients were treated at a single cancer centre between 1954 and 2012. Most tumours involved the nasal cavity (35.3%) and upper gingiva (29.4%). The majority of lesions were ulcerated (54.9%) and pigmented (84.3%). Forty-three patients underwent surgical treatment and 21 (41.2%) underwent adjuvant chemotherapy and/or radiotherapy. Eight patients (15.7%) received palliative treatment. The median follow-up period was 21 months. During this period, 30 (58.8%) patients had tumour recurrences. At the last clinical evaluation, only seven (13.7%) patients were alive with no evidence of disease and three (5.9%) were alive with HNMM. There were significant differences in overall survival probability according to the presence of ulceration (P=0.004), metastatic lymph nodes (P=0.003), and treatment including a radical surgical procedure (P<0.001). On multivariate analysis, ulceration was the only variable associated with an increased risk of death. Despite the poor prognosis, there was significant improvement in overall survival in the most recent years in this sample, mainly due to advances in diagnosis and reconstruction techniques.

Melanomas no cutáneos de cabeza y cuello enfermedad de pronóstico sombrío / Non-cutaneous melanoma of the head and neck disease of grim prognosis

Antecedentes: Los melanomas no cutáneos representan el 1-7% del total. Su pronóstico es malo, a pesar de lo cual, los avancestecnológicos pueden haber cambiado su evolución.Objetivo: Descubrir factores que permitan determinar los progresos de los últimos 20 años.Diseño: Retrospectivo, observacional.Lugar de aplicación: Hospital Público de atención terciaria.Población: 30/56 pacientes pertenecían al género masculino, con una relación 1.15/1. La edad media fue 61.07 años. El sitiodominante de los mucosos fue paladar con 22 (39.2%) y senos paranasales con 9 (16%). Los oculares con 24/56 representaron el(42.8%) de la muestra. El 94.6% se hallaban en E I.Métodos: La extensión de la cirugía varió desde la simple resección local en 13/56 -23.2%- pacientes, a la enucleación ocular en14/56 (25%), exenteración orbitaria en 5/56(9%), maxilectomía superior en 20/56 (35.7%), resección craneofacial en 3 y amputaciónnasal en 1.Resultados: La morbilidad fue 51.7% y la mortalidad 1.7%. El 67.8% desarrolló recurrencias. El análisis univariado para recurrenciademostró que los orígenes rinosinusal y conjuntival, influyeron desfavorablemente en el evento recurrencia. Al cierre de laobservación 42/56 (75%) habían fallecido, (85.8%) de ellos por causa específica. Solo 2/42 (4.7%) muertes se produjeron despuésde los 60 meses. La supervivencia libre de enfermedad a 5 años fue 31.9%. El paladar y el E I, fueron las variables que a su favorconservaron significación estadística.Conclusiones: Urgen nuevos paradigmas de tratamiento para esta enfermedad cuya evolución no se ha modificado sustancialmenteen los últimos 20 años.

Background: Non cutaneous are 1-7% of all melanoma. The prognosis is poor in spite of which, technological progress maychange the outcome.Objective: To discover factors which allow to define progress of last 20 years.Design: Retrospective, observational.Setting: Public Hospital of tertiary care.Population: 30/56 male patients with 1.15/1 rate. Mean age 61.07 years old. Priority sites in mucosal melanoma were palate with22-39.2% and paranasal sinus with 9 (16%). Ocular melanomas were 42.8% of the sample. 94.6% were EI.Methods: Extention of surgery was simple resection in 13/56 (23.2%), ocular enucleation in 14/56 (25%), orbit exenteration in 5/56(9%) maxilectomy in 20/56 (35.7%), craniofacial resection in 3 and nasal amputation in 1.Results: Morbility was 51.7% and mortality 1.7%. 67.8% of the sample recurred. Multivariate analysis for recurrence showed thatrinosinusal and conjuntival sites unfavorably influence on that event. At close of observation 42/56 (75%) was dead, 85.8% of themfor specific cause. Only 2 death happened after 60 months. Five years specific survival was 31.9%. Palate and stage I were favorablesites with statistic significance.Conclusions: Other paradigms of treatment are urgently necessary for this disease which outcome was not substantially modifiedin the last 20 years.

Melanomas no cutáneos de cabeza y cuello: Enfermedad de pronóstico sombrío

Antecedentes: Los melanomas no cutáneos representan el 1-7% del total. Su pronóstico es malo, a pesar de lo cual, los avances tecnológicos pueden haber cambiado su evolución. Objetivo: Descubrir factores que permitan determinar los progresos de los últimos 20 años. Diseño: Retrospectivo, observacional. Lugar de aplicación: Hospital Público de atención terciaria. Población: 30/56 pacientes pertenecían al género masculino, con una relación 1.15/1. La edad media fue 61.07 años. El sitio dominante de los mucosos fue paladar con 22 (39.2%) y senos paranasales con 9 (16%). Los oculares con 24/56 representaron el (42.8%) de la muestra. El 94.6% se hallaban en E I. Métodos: La extensión de la cirugía varió desde la simple resección local en 13/56 -23.2%- pacientes, a la enucleación ocular en 14/56 (25%), exenteración orbitaria en 5/56(9%), maxilectomía superior en 20/56 (35.7%), resección craneofacial en 3 y amputación nasal en 1. Resultados: La morbilidad fue 51.7% y la mortalidad 1.7%. El 67.8% desarrolló recurrencias. El análisis univariado para recu-rrencia demostró que los orígenes rinosinusal y conjuntival, influyeron desfavorablemente en el evento recurrencia. Al cierre de la observación 42/56 (75%) habían fallecido, (85.8%) de ellos por causa específica. Solo 2/42 (4.7%) muertes se produjeron después de los 60 meses. La supervivencia libre de enfermedad a 5 años fue 31.9%. El paladar y el E I, fueron las variables que a su favor conservaron significación estadística. Conclusiones: Urgen nuevos paradigmas de tratamiento para esta enfermedad cuya evolución no se ha modificado sustancial-mente en los últimos 20 años.(AU)

Background: Non cutaneous are 1-7% of all melanoma. The prognosis is poor in spite of which, technological progress may change the outcome. Objective: To discover factors which allow to define progress of last 20 years. Design: Retrospective, observational. Setting: Public Hospital of tertiary care. Population: 30/56 male patients with 1.15/1 rate. Mean age 61.07 years old. Priority sites in mucosal melanoma were palate with 22-39.2% and paranasal sinus with 9 (16%). Ocular melanomas were 42.8% of the sample. 94.6% were EI. Methods: Extention of surgery was simple resection in 13/56 (23.2%), ocular enucleation in 14/56 (25%), orbit exenteration in 5/56 (9%) maxilectomy in 20/56 (35.7%), craniofacial resection in 3 and nasal amputation in 1. Results: Morbility was 51.7% and mortality 1.7%. 67.8% of the sample recurred. Multivariate analysis for recurrence showed that rinosinusal and conjuntival sites unfavorably influence on that event. At close of observation 42/56 (75%) was dead, 85.8% of them for specific cause. Only 2 death happened after 60 months. Five years specific survival was 31.9%. Palate and stage I were favorable sites with statistic significance. Conclusions: Other paradigms of treatment are urgently necessary for this disease which outcome was not substantially modified in the last 20 years.(AU)