Malignant salivary gland tumors: agreement between fine needle aspiration biopsy, incisional biopsy and final histopathological diagnostic / Tumores malignos de glándulas salivales: concordancia entre biopsia por aspiración con aguja fina, biopsia incisional y diagnóstico histopatológico final

Background: Incisional biopsy is indicated for intraoral tumors, but it is a contraindicated surgical procedure formajor salivary glands. To avoid complications and facilitate diagnosis, fine needle aspiration biopsy (FNAB) is atype of biopsy widely used for preoperative diagnosis in these glands.Material and Methods: The aim of this study was to analyze the agreement between the diagnosis by FNAB (ma-jor glands), incisional biopsy (minor glands) and histopathological analysis of the surgical specimen in salivarygland tumors from a database (medical records) of patients treated in a cancer treatment reference center in theNortheast region of Brazil.Results: The sample consisted of 110 cases, being 86 of them malignant tumors in major salivary glands (parotidgland=73; submandibular gland=13) and 24 cases in minor salivary glands (palate). The female gender was themost affected (57.3%), especially in patients over 60 years (42.7%). In the TNM classification, 41.8% of the caseswere in T2 at the time of diagnosis, with most of the regional lymph nodes in N0 (85.5%) and 87.3% of the casesin M0. FNAB was able to identify malignant neoplasms in 68.6% of the cases (n=59), while incisional biopsy inpalatal tumors obtained agreement of 75% of the cases (n=18). The analysis revealed that tumors classified as T3-T4 (p=0.012) showed greater agreement between pre- and post-surgical diagnosis.(AU)

Induction of perineural invasion in salivary adenoid cystic carcinoma by circular RNA RNF111

Objective Local recurrence, distant metastasis, and perineural invasion (PNI) viciously occur in salivary adenoid cystic carcinoma (SACC), resulting in a poor prognosis. This study aimed to explore the mechanism by which circular RNA RNF111 (circ-RNF111) regulates PNI in SACC by targeting the miR-361-5p/high mobility group box 2 (HMGB2) axis. Method Circ-RNF111 and HMGB2 were highly expressed in SACC specimens, while miR-361-5p was underexpressed. Functional experiments showed that ablating circ-RNF111 or promoting miR-361-5p hindered the biological functions and PNI of SACC-LM cells. Results HMGB2 overexpression induced the reversal of SACC-LM cell biological functions and PNI caused by circ-RNF111 knockout. Furthermore, reduction of circ-RNF111 suppressed PNI in a SACC xenograft model. Circ-RNF111 regulated HMGB2 expression through targeted modulation of miR-361-5p. Conclusion Taken together, circ-RNF111 stimulates PNI in SACC by miR-361-5p/HMGB2 axis and may serve as a potential therapeutic target for SACC (AU)

Adenocarcinoma mucinoso gigante de glándula submandibular: presentación de un caso extremadamente infrecuente / Giant mucinous adenocarcinoma of the submandibular gland. Report on an extremely rare case

El adenocarcinoma mucinoso es una entidad patológica poco frecuente dentro de las neoplasias que afectan a las glándulas salivales. Se presenta el caso de una paciente de 89 años con antecedentes de un aumento de volumen en el cuello. Al examen físico se constató una tumoración que abarcaba toda la región cervical derecha y se extendía desde la región submandibular hasta la región clavipectoral homolateral. El estudio histopatológico confirmó un adenocarcinoma mucinoso de bajo grado. Se efectuó una disección del cuello extendida a piel y luego se reconstruyó la zona con un colgajo miocutáneo de músculo pectoral mayor, debido al defecto generado por la eliminación del gran volumen tumoral.En conclusión, se presenta un caso extremadamente infrecuente de adenocarcinoma mucinoso de glándula submandibular de tamaño gigante. (AU)

Mucinous Adenocarcinoma is a rare pathological entity within neoplasms that affect the salivary glands. The case of an 89-year-old female patient with a history of increased volume in the neck is presented. Physical examination revealed a tumor that covered the entire right cervical region and extended from the submandibular region to the ipsilateral clavipectoral region. The histopathological study confirmed a low grade Mucinous Adenocarcinoma. A neck dissection extended to the skin was performed and then the area was reconstructed with a myocutaneous flap from the pectoralis major muscle due to the defect generated by the removal of the large tumor volume.In conclusion, an extremely rare giant-sized mucinous adenocarcinoma of the submandibular gland is presented.

MutSα expression predicts a lower disease-free survival in malignant salivary gland tumors: an immunohistochemical study

Ten cases of normal salivary gland (NSG) and 92 of SGT (54 benign and 38 malignant) were retrieved. Immunohistochemistry was performed for hMSH2, hMSH3, hMSH6. Scanned slides were digitally analyzed based on the percentage of positive cells with nuclear staining. Cases were further classified in MutSαhigh and MutSβhigh based on hMSH2-hMSH6 and hMSH3-hMSH6 expression, respectively. Results: hMSH3 expression was lower in malignant SGT compared to NSG and benign cases. Adenoid cystic carcinoma (ACC) cases with perineural invasion presented a lower percentage of hMSH3 positive cells. hMSH6 was downregulated in both benign and malignant SGT compared to NSG. Malignant SGT cases with MutSαhigh expression had lower disease-free survival compared to MutSαlow cases. A 10.26-fold increased risk of presenting local recurrence was observed. Conclusions: Our findings suggest that a lack of hMSH3 protein function is associated with a more aggressive phenotype (malignancy and perineural invasion) and that MutSα overexpression predicts a poor clinical outcome in malignant SGT.(AU)

Salivary gland cancer in Southern Brazil: a prognostic study of 107 cases / Cáncer de glándula salival en el sur de Brasil: un estudio pronóstico de 107 casos

Background: Salivary gland cancers (SGC) represent an uncommon group of heterogeneous tumors. We per-formed a retrospective survey of SGC diagnosed in a reference center for treatment of malignant tumors from thesouth of Brazil aiming to determine the prognostic value of demographic, clinic and pathologic features.Material and Methods: Cases diagnosed as SGC between 2006 and 2016 were retrospectively collected. Medicalrecords were examined to extract demographic, clinic, pathologic and follow-up information.Results: One-hundred and seven cases of SGC were identified. The most common SGC were mucoepidermoidcarcinoma (MEC) (n = 39) followed by adenoid cystic carcinoma (AdCC) (n = 29). Among AdCCs, 55.2% of caseswere classified as cribriform, 27.6% as tubular and 17.2% as solid. The tubular subtype had the highest percentageof cases with perineural invasion (p=0.01). Among MEC, 61.5% of cases were classified as low grade, 15.4% asintermediate grade and 19.9% as high grade. Low grade MEC had the lowest percentage of cases with perineuralinvasion (p=0.04). The 5-year survival for loco-regional control, disease-free survival (DFS) and disease-specificsurvival were 75%, 70% and 84%, respectively. The following features were associated with poor DFS: advanced age (p=0.03), rural residency (p=0.01), being a smoker or former smoker (p=0.01), pain (p=0.03), nodal metastasis(p<0.001), need for chemotherapy (p=0.02), neck dissection (p=0.04), perineural invasion (p=0.01), and being diag-nosed with AdCC compared to MEC (p=0.02).Conclusions: The clinco-demographic and pathologic features identified as prognostic factors reveal the profile ofpatients at increased risk of recurrence and who would benefit from closer follow-up.(AU)

Prognostic value of the immunohistochemical expression of vascular endothelial growth factors in malignant salivary gland neoplasms: a systematic review and meta-analysis / Valor pronóstico de la expresión inmunohistoquímica de vascular. Factores de crecimiento endotelial en neoplasias malignas de glándulas salivales: una revisión sistemática y un metanálisis

Background: The immunohistochemical expression of vascular endothelial growth factor is a prognostic markerin several cancer types. In salivary gland tumors, the association between vascular endothelial growth factor andprognosis remains unclear. The purpose of this study was to perform a systematic review and meta-analysis to as-sess whether the immunohistochemical expression of vascular endothelial growth factor in patients with salivarygland neoplasms presents prognostic value.Material and Methods: Immunohistochemical studies assessing the predictive value of vascular endothelialgrowth factor in salivary gland neoplasms were systematically reviewed using PubMed, Scopus, Embase, Co-chrane Library, and Web of Science databases. It was assessed any survival rates. The fixed-effect model withan adjusted hazard ratio (HR) and 95% confidence intervals (95% CI) as effect measures were performed in themeta-analysis. The Quality in Prognosis Studies (QUIPS) tool was used to assess the quality of the included stud-ies, and the evidence quality was assessed by the Grading of Recommendation, Assessment, Development, andEvaluation (GRADE) system.Results: The immunohistochemical overexpression of vascular endothelial growth factor in patients with salivarygland neoplasms was associated with shortened survival (HR=5.37, 95% CI: 2.67-10.83, P = 0.00001). In addition,the presence of vascular endothelial growth factor was tightly associated with tumor size, lymph node metastasis,clinical stage, perineural invasion, vascular invasion, poor local control of the disease, and recurrence.Conclusions: The immunohistochemical overexpression of vascular endothelial growth factor in patients withsalivary gland neoplasms has prognostic value and was associated with decreased survival time. However, moreprimary well-designed studies are necessary to increase the level of evidence.(AU)

Incidence and profile of benign epithelial tumors of salivary glands from a single center in Northeast of Brazil

BACKGROUND: Benign tumors of the salivary glands are a group of lesions with varied histopathological and clinical spectrum. The aim was to determine the incidence and clinicopathological characteristics of benign salivary gland neoplasms diagnosed between 2007 and 2016 in a single center located in northeastern Brazil. MATERIAL AND METHODS: Records regarding sex, age, anatomical location, histopathological subtype and treatment were retrieved, and data were analyzed using the Stata/IC software (version 12.0). RESULTS: There were above 7,100 cases of neoplasms in the head and neck region, of which 403 corresponded to salivary gland neoplasms. Of these, 238 (59%) were benign, being pleomorphic adenoma (PA) the most frequent neoplasm (n=178; 74.8%), followed by Warthin's tumor (WT) (n=23; 9.7%). Overall, most cases occurred in females (n=136; 57.1%) and age ranged from 11 to 83 years. The parotid gland (n=188; 79%) was the most common anatomical site, and all patients were treated by surgical excision. Of the cases diagnosed as PA, malignant transformation to carcinoma ex-pleomorphic adenoma (CAEXPA) occurred in 7 (3.9%) cases. CONCLUSIONS: The present study confirmed the clinical and demographic profile of benign salivary gland neoplasms, which contributes to the continuous knowledge of current data about these lesions

No disponible

Fine-needle aspiration cytology in the diagnosis of salivary gland lesions. The role of the Milan system for reporting cytopathology / Citología por aspiración con aguja fina en el diagnóstico de las lesiones de las glándulas salivales. El papel del sistema Milán en citopatología

BACKGROUND: Fine needle aspiration cytology (FNAC) is an established technique in the management of salivary gland lesions. The Milan System for reporting salivary gland cytopathology (MSRSGC) intents to standardize diagnostic categories. Current studies are trying to evaluate the diagnostic approach of this system. METHODS: FNAC of salivary gland lesions were retrieved over an 11-year period. 185 FNAC specimens from 182 patients were reviewed blindly and classified according to the criteria established by the MSRSGC. 136 (74.7%) patients had follow-up of their processes. RESULTS: The total number of diagnostic categories and risk of malignancy (ROM) in 185 specimens were the following: non-diagnostic 39 (21.1%; ROM 12%), non-neoplastic 35 (18.9%; ROM 0%), atypia of undetermined significance (AUS) 17 (9.2%; ROM 46.1%), benign neoplasm 75 (40.5%; ROM 4.9%), salivary gland neoplasm of uncertain malignant potential 4 (2.2%; ROM 100%), suspicious for malignancy 7 (3.8%; ROM 100%), malignant 8 (4.3%; ROM 100%). No false positives were observed in groups IVb, V, and VI in this series. The overall sensitivity, specificity, negative predictive value, positive predictive value and diagnostic accuracy were 88%, 91.8%, 96.3%, 76.7%, and 91% respectively. CONCLUSION: The ROM reported in our study was in keeping with ROM published by the MSRSGC. This system provides standardized information for risk stratification. The category AUS encompassed cases causing uncertainty representing a challenge in management. Defining criteria for AUS category need to be refined. The system facilitates communication between pathologists and clinicians favoring improvement in patient care

ANTECEDENTES: La citología por punción-aspiración con aguja fina (PAAF) está establecida en el tratamiento de las lesiones de las glándulas salivales. El sistema Milán (SM) intenta homogeneizar las categorías diagnósticas en los informes citológicos. Los estudios actuales están tratando de evaluar el enfoque diagnóstico de este sistema. MÉTODOS: Se revisaron las PAAF de las glándulas salivales obtenidas durante un período de 11 años. Se revisaron a ciegas 185 muestras de PAAF de 182 pacientes, y se clasificaron de acuerdo con los criterios establecidos por el SM. Se realizó seguimiento de los procesos de 136 (74,7%) pacientes. RESULTADOS: Los totales de categorías diagnósticas y el riesgo de malignidad (ROM) en 185 muestras fueron los siguientes: no diagnóstico 39 (21,1%; ROM 12%), no neoplásico 35 (18,9%; ROM 0%), atipia de significación indeterminada (ASI) 17 (9,2%; ROM 46,1%), neoplasia benigna 75 (40,5%; ROM 4.9%), neoplasia de potencial maligno incierto 4 (2,2%; ROM 100%), sospecha de malignidad 7 (3,8%; ROM 100%), malignidad 8 (4,3%; ROM 100%). No se observaron falsos positivos en los grupos IVb, V y VI. Los valores de sensibilidad, especificidad, el valor predictivo negativo, el valor predictivo positivo y la precisión diagnóstica fueron del 88%, 91,8%, 96,3%, 76,7% y 91%, respectivamente. CONCLUSIÓN: El ROM observado concordaba con el ROM publicado por el SM. Este proporciona información estandarizada para la estratificación del riesgo. La categoría ASI abarcó los casos que causaron incertidumbre, y representa un desafío en el manejo de los pacientes. Los criterios de definición de ASI deberían perfeccionarse. El sistema facilita la comunicación entre patólogos y clínicos, y mejora la atención al paciente

Nivolumab: «Tormenta inmune» en paciente con miastenia gravis previa / Nivolumab: An «Immune storm» in a patient with history of myasthenia gravis

No disponible

Adenoma pleomorfo de la mama. A propósito de un caso / Pleomorphic adenoma of the breast. Apropos of a case

OBJETIVO: El adenoma pleomorfo es un tumor con una gran heterogenicidad arquitectural y citológica, de ahí su denominación como tumor mixto. Compuesto característicamente por células epiteliales y mioepiteliales con estroma mesenquimal, presenta un comportamiento benigno, siendo su localización habitual las glándulas salivares. La mama es una localización muy infrecuente para este tipo de tumores. CASO CLÍNICO: Presentamos el caso clínico de una paciente de 76 años sin antecedentes de patología mamaria, valorada en consultas externas de cirugía por una secreción unilateral de aspecto serosanguinolento desde la mama derecha, asociada a un nódulo de unos 2cm de diámetro mayor. Tras completar su estudio, se decide exéresis de la tumoración, obteniendo como resultado en la anatomía patológica una tumoración heterogénea con predominio de células miopiteliales; descripción compatible con un adenoma pleomorfo. DISCUSIÓN: El adenoma pleomorfo de la mama, es una tumoración epitelial benigna que tiene un patrón histológico similar al del tumor de las glándulas salivares. Los rasgos histológicos más importantes es la metaplasia ósea y mixocondroide, junto con presencia de calcificaciones, tejido epitelial (con o sin diferenciación glandular), y tejido mioepitelial; siendo esta gran variedad estructural lo que le caracteriza. Hay que realizar un diagnóstico diferencial con patología maligna a la cual asemeja. Se presenta de forma poco frecuente a nivel mamario y suele ser una lesión bien delimitada y solitaria, elastica y no adherida. Pese a su comportamiento benigno (no ha presentado en ninguno de los casos documentados metástasis a distancia) el tratamiento es quirúrgico, asegurando buenos margenes de resección para evitar recidivas

OBJECTIVE: Pleomorphic adenoma shows wide architectural and cytological heterogeneity and is consequently classified as a mixed tumour. These benign adenomas are characteristically composed of epithelial and myoepithelial cells with mesenchymal stroma and are usually located in the salivary glands. Localisation in the breast is extremely infrequent for this type of tumour. CASE REPORT: We report the case of a 76-year-old female patient with no prior history of breast disease who was assessed in the surgery outpatient clinic for unilateral serosanguinous secretion from the right breast associated with a nodule of approximately 2cm in diameter. After workup, it was decided to extirpate the tumour. Histological findings indicated a heterogeneous tumour with a predominance of myoepithelial cells, compatible with pleomorphic adenoma. DISCUSSION: Pleomorphic adenoma of the breast is a benign epithelial tumour whose histological pattern is similar to that of pleomorphic adenoma of the salivary glands. The most important histological features are osseous and myxochondroid metaplasia, together with the presence of calcifications, epithelial tissue (with or without glandular differentiation) and myoepithelial tissue. This wide structural variety is what characterises these tumours. The differential diagnosis should be performed with malignant disease, which these adenomas frequently mimic. Pleomorphic adenomas are very infrequent in the breast and are usually well-demarcated, solitary, elastic and non-adherent tumours. Despite their benign behaviour (there have been no reported cases of distant metastases), treatment is surgical, with good resection margins to avoid recurrences

Epidemiologic analysis of salivary gland tumors over a 10-years period diagnosed in a northeast Brazilian population

BACKGROUND: Salivary gland tumors (SGT) correspond to a heterogeneous group of lesions with variable biological behavior. The present study aimed to determine the distribution and demographic findings of salivary gland neoplasms in a northeast Brazilian population. MATERIAL AND METHODS: A retrospective descriptive cross-sectional study was performed. A total of 588 cases of SGT were diagnosed between 2006 and 2016 of 4 pathology services in the state of Sergipe, Brazil. All cases were reviewed, and data such as sex, age, anatomical location, and histopathological diagnosis were collected. RESULTS: A total of 470 (79.9%) tumors were benign and 118 (20.1%) were malignant. The majority of the patients were females (n = 328, 55.8%) with an overall female: male ratio of 1.2:1. The major salivary glands were affected more than the minor glands (69.5% vs. 30.5%). Pleomorphic adenoma (n = 419, 71.3%) and mucoepidermoid carcinoma (n = 29, 4.9%) were the most frequent benign and malignant tumors, respectively. In addition, both benign and malignant tumors occurred more frequently in the parotid gland (n = 300, 51%, p < 0.05). CONCLUSIONS: The epidemiologic profile and clinical characteristics of SGT were similar to those described in other countries and other regions of Brazil. Epidemiological studies of SGT help to understand their clinical and pathological features and are essential to establish the proper management and prognosis

No disponible

A rare case of a recently described entity: mammary analogue secretory carcinoma (MASC) of the parotid gland / Un caso raro de una entidad descrita recientemente: carcinoma secretorio análogo mamario (CSAM) de la glándula parótida

MASC is a salivary gland tumour which shares histological, immunologic and genetic characteristics with mammary secretory carcinoma including an ETV6 translocation and immunocytochemical positivity for S-100 protein, CK7, and mammaglobin as well as negativity for DOG1. This is a rare tumour with uncommon characteristics when compared to other salivary gland tumours. The case reported here is of a 28-year-old female patient who presented in the ER due to a palpable mass in the left parotid region. She underwent a superficial parotidectomy with using a mini-lifting approach, with tumour resection, followed by radiotherapy. The identified tumour shared most of the clinical characteristics with other cases of MASC described in the literature

CSAM es un tumor de glándula salival que comparte características histológicas, inmunológicas y genéticas con el carcinoma secretor mamario, que incluye una translocación ETV6 y positividad inmunocitoquímica para la proteína S-100, CK7 y mamaglobina, así como negatividad para DOG1. Este es un tumor raro con características poco comunes en comparación con otros tumores de glándulas salivales. El caso referido aquí es el de una paciente de 28 años de edad que se presentó en la sala de emergencias debido a una masa palpable en la región parotídea izquierda. Se sometió a una parotidectomía superficial con un abordaje de mini-lifting, con resección tumoral, seguida de radioterapia. El tumor identificado compartía la mayoría de las características clínicas con otros casos de CSAM descritos en la literatura

Epithelioid hemangioendothelioma of the parotid gland: a case report in an unusual location with a review of the literature / Hemangioendotelioma epitelioide de la glándula parótida: reporte de un caso de una localización inusual y revisión de la literatura

Epithelioid hemangioendothelioma (EHE) is a malignant vascular tumor that usually affects the liver, lung, bone and deep soft tissues of the extremities or trunk. To our knowledge, only 3 cases in the parotid gland have been reported to date. We report a case of a 62-year-old woman who presented with a 1-year history of a slow-growing, painless mass over the left mandibular angle. Imaging studies showed a 2cm mass over the left parotid gland with peripheral calcifications. The patient underwent a superficial parotidectomy. Sections displayed neoplastic epithelioid cells with cytoplasmic vacuoles containing erythrocytes, surrounded by a myxohyaline stroma. Immunohistochemistry was positive for CD31, CD34, ERG, and factor VIII, but negative for cytokeratin AE1/AE3, CK7, EMA, SMA, and S100. The findings were those of an EHE involving the parotid gland. This case demonstrates an EHE in a rare location and emphasizes the need to consider this tumor when diagnosing uncommon soft tissue tumors of salivary glands

El hemangioendotelioma epitelioide (HEE) es un tumor vascular maligno, que generalmente afecta el hígado, los pulmones, los huesos y los tejidos blandos profundos de las extremidades o el tronco. Por lo que sabemos, solo 3 casos reportados en la literatura han descrito este tumor en la glándula parótida. Presentamos el caso de una mujer de 62 años que presentó una historia de un año, de una masa indolora, de crecimiento lento sobre el ángulo mandibular izquierdo. Los estudios de imagen mostraron una masa de 2cm sobre la glándula parótida izquierda con calcificaciones periféricas. La paciente se sometió a una parotidectomía superficial. Los cortes mostraron células epitelioides neoplásicas con vacuolas citoplasmáticas que contenían eritrocitos, rodeadas por un estroma mixohialino. Se demostró mediante técnicas de inmunohistoquímica positividad en la celularidad neoplásica para CD31, CD34, ERG y factor VIII, pero negatividad para la citoqueratina AE1/AE3, CK7, EMA, SMA y S100. Estos hallazgos fueron los de un HEE comprometiendo la glándula parótida. Este caso demuestra un HEE en una localización rara y enfatiza la necesidad de considerar este tumor al diagnosticar tumores raros de tejidos blandos de glándulas salivales

Carcinoma análogo del secretor mamario de glándulas salivales: a propósito de un caso clínico / Mammary analogue secretory carcinoma of salivary glands: Report of clinical case

No disponible

Metástasis coroideas de un carcinoma adenoide quístico de glándula salival menor: caso clínico / Choroidal metastasis of a minor salivary gland adenoid cystic carcinoma: A case report

CASO CLÍNICO: Varón de 61 años con un carcinoma adenoide quístico de glándulas salivales menores de labio inferior presentó deterioro unilateral y progresivo de la agudeza visual debido a una metástasis coroidea. DISCUSIÓN: El carcinoma adenoide quístico es un tumor infrecuente con un importante potencial mestastásico. Nuestro paciente presentó una metástasis coroidea unilateral. Actualmente hay descritos 8 casos de metástasis coroidea de un carcinoma adenoide quístico de glándulas salivales. Este es el segundo caso descrito de metástasis coroidea con origen en un tumor de glándulas salivales menores y el primero con origen en las glándulas salivales menores del labio inferior

CASE REPORT: A 61-year-old man with a lower lip minor salivary gland adenoid cystic carcinoma, suffered from a unilateral progressive visual acuity loss due to choroidal metastasis. DISCUSSION: Adenoid cystic carcinoma is a rare primary tumour with significant metastatic potential. Our patient presented with a unilateral choroidal metastasis. According to the current literature, 8 cases of choroidal metastasis of salivary gland adenoid cystic carcinoma have been reported. This is the second case reported of choroidal metastasis with origin in a minor salivary gland, and the first one with origin in the minor salivary glands of the lower lip

Carcinoma análogo secretor de mama: revisión de diagnóstico y tratamiento a propósito de 2 casos / Mammary analogue secretory carcinoma: presentation of 2 cases and a review of its diagnosis and treatment

Introducción. El carcinoma análogo secretor de mama (MASC: mammary analogue secretory carcinoma) es una entidad neoplásica de las glándulas salivares caracterizada por presentar importantes similitudes histológicas e inmunohistoquímicas con el carcinoma secretor de mama. Hasta su descripción en 2010, el MASC era frecuentemente clasificado como carcinoma de células acinares por su solapamiento morfológico. Ante la falta de evidencia científica en la literatura respecto al tratamiento óptimo de estos tumores, el objetivo de este artículo es presentar 2casos tratados recientemente en nuestro servicio y revisar la literatura descrita acerca de su diagnóstico y tratamiento. Material y métodos. Se trata de un paciente varón de 41 años con diagnóstico de MASC de glándula salivar menor en paladar duro y de una paciente de 56 años con el mismo diagnóstico en la glándula submaxilar. Resultados. En ambos casos se realizó resección quirúrgica con márgenes de la lesión. En uno de ellos, al presentar bordes libres, se decidió control clínico, mientras que en el otro se decidió tratamiento adyuvante con radioterapia al presentar un borde cercano a la lesión. Ambos pacientes tras 2años están libres de enfermedad y mantienen seguimiento clínico. Discusión. En la literatura se había descrito la existencia de un tumor de glándulas salivares con unas características morfológicas comunes entre el carcinoma de células acinares y el carcinoma secretor de mama, caracterizado inmunohistoquímicamente por ser positivo a vimentina y a la proteína S-100, pero no fue conocido como MASC hasta su descripción en 2010. La alteración genética asociada al MASC es la presencia de la translocación t(12;15)(p13;q25) en el oncogén ETV6-NTRK3, lo que lo convierte en un marcador prácticamente patognomónico de este tumor. Respecto al tratamiento, existe consenso en el tratamiento quirúrgico de la lesión primario, y no existe consenso respecto a la disección cervical. El valor de la RT postoperatoria es difícilmente valorable por los escasos casos en la literatura. Conclusiones. El MASC es una enfermedad neoplásica de glándula salivar que precisa un diagnóstico correcto previo a la elección de su tratamiento. Debido a su comportamiento como una neoplasia maligna de bajo grado, consideramos que su tratamiento debe ser el quirúrgico, con unos márgenes de seguridad a la lesión, pero en algunos casos su comportamiento puede ser agresivo (AU)

Introduction. Mammary analogue secretory carcinoma (MASC) is a neoplastic tumour of the salivary glands, characterised by having important histological and immuno-histochemical similarities with secretory breast carcinoma. Until its description in 2010, MASC was often diagnosed as carcinoma of acinar cells due to their morphological overlapping. As there is a lack of evidence in the scientific literature concerning the optimal treatment of these tumours, the aim of this article is to present 2recently treated cases, and review the described literature about their diagnosis and treatment. Material and methods. The first case concerns a 41 year-old male with MASC of the minor salivary gland of the hard palate, and the other a 56 year-old patient with the same diagnosis at the level of the submandibular gland. Results. Surgical resection with lesion-free margins was performed in both cases. Clinical surveillance was decided for one case with free margins, while in the other one, it was decided to give adjuvant therapy with radiotherapy due to the narrow lesion free margin. Both patients are disease free and continue on clinical follow-up. Discussion. The literature describes the existence of a salivary glands tumour with common morphological characteristics between acinar cell carcinoma and breast carcinoma, and immunohistochemically characterised by being positive to vimentin and S-100 protein, but it was not known as MASC until its description in 2010. The genetic alteration associated with MASC is the presence of a translocation t(12;15) (q25; Q13) in ETV6-NTRK3, making it a pathognomonic tumour marker. There is consensus in the surgical treatment of primary lesions, but there is no agreement as regards neck dissection. The value of post-surgical radiotherapy is difficult to assess by the few cases in the literature. Conclusions. MASC is a recently described salivary gland tumour characterised by ETV6 translocation. Due to its behaviour as a low grade malignant neoplasm, it is recommended that its treatment should be excision with surgical margins, but in some cases its behaviour can be aggressive (AU)

Diagnóstico, tratamiento y seguimiento de un tumor de reciente descripción: el carcinoma análogo secretor de mama (MASC) de glándula salival. A propósito de 2 nuevos casos / Diagnosis, treatment and follow-up of a recently described tumour: Mammary analogue secretory carcinoma (MASC) of the salivary gland. A report of 2 new cases

Introducción. El carcinoma análogo secretor de mama (MASC) es un tumor de glándula salival de reciente aparición con pocos casos descritos. Tiene un diagnóstico preoperatorio difícil y su tratamiento y seguimiento todavía siguen en estudio. Material y métodos. Revisión sistemática de 367 tumores parotídeos intervenidos entre enero de 2011 y diciembre de 2015 en el Hospital Universitario Miguel Servet. De los 367 casos operados, 45 eran malignos, y de estos, encontramos 2 únicos casos de MASC. Resultados. Cuarenta y cinco de los 367 tumores parotídeos eran malignos, siendo 2 de ellos MASC. La parotidectomía subtotal y superficial asociada a la radioterapia ha sido el tratamiento de elección. Tras 12 y 18 meses, no se han evidenciado recidivas. Discusión. Revisión de los estudios publicados en la literatura sobre su diagnóstico diferencial con otros tumores de glándula salival y su tratamiento, teniendo en cuenta los resultados citológicos e inmunohistoquímicos de cada uno de ellos, y destacando cuáles son los marcadores más específicos para el diagnóstico de MASC. Conclusión. El diagnóstico preoperatorio de este tumor casi nunca es acertado, y todavía no se conoce su tratamiento y supervivencia a largo plazo, por lo que más estudios prospectivos y nuevos casos deben ser documentados y seguidos (AU)

Introduction. Mammary analogue secretory carcinoma (MASC) is a new salivary gland tumour with few cases reported. Pre-operative diagnosis is difficult, and treatment and follow-up are still under study. Material and method. A systematic review was conducted on 367 parotid tumours treated between January 2011 and December 2015 at the University Hospital Miguel Servet, Spain. Of the 367 cases operated on, 45 were malignant, and of these, only two cases were MASC. Results. Of the 367 parotid tumours reviewed, 45 were malignant, and two of them MASC. Sub-total and superficial parotidectomy combined with radiation therapy has been the treatment of choice. They showed no recurrence after 12 and 18 months. Discussion. A review is presented on the studies published in the literature on the differential diagnosis with other salivary gland tumours, taking into account their cytological and immunohistochemical results, focussing on the most specific markers for the diagnosis of MASC. Conclusion. Pre-operative diagnosis of MASC is rarely successful. Its treatment and long-term survival is largely unknown, thus there is a need for more prospective studies, as well as new cases that should be documented and followed-up (AU)