Tumor de glándula lacrimal: a propósito de un caso / Lacrimal gland tumor: a case proposal

INTRODUCCIÓN: El carcinoma adenoide quístico (CAQ) es conocido por su lento crecimiento, su tendencia a la recurrencia local y al desarrollo de metástasis a distancia incluso décadas después del diagnóstico inicial, y a pesar de terapias agresivas. OBJETIVO: Presentar el caso de una paciente con CAQ de la glándula lagrimal, tumor muy infrecuente que representa casi el 10% de las lesiones orbitarias ocupantes de espacio. De éstas el 20-30% son de origen epitelial, de las cuales el 55% son lesiones benignas y el 45% malignas. El CAQ es el más común de los tumores epiteliales malignos de la glándula lagrimal (65%). CASO CLÍNICO: Presentamos el caso clínico de una paciente con CAQ de glándula lacrimal diagnosticado de forma incidental tras la realización de TAC desde la consulta de psiquiatría. RESULTADOS Y CONCLUSIONES: El síntoma de presentación más frecuente suele ser la proptosis, acompañada o no de dolor, aunque también puede presentarse como ptosis mecánica, reducción de visión y diplopía. La cirugía radical no ha demostrado mejorar la supervivencia o reducir la recurrencia local en comparación con un abordaje quirúrgico, más conservador en pacientes con tumores de menor tamaño y sin afectación ósea. Las mayores tasas de supervivencia se han conseguido, en pacientes tratados con quimioterapia, con quimioterapia citorreductiva intra-arterial seguida de exenteración orbital y radioterapia.

INTRODUCTION:The adenoid cystic carcinoma (ACC) is known for its slow growth, a tendency to local recurrence and the development of distant metastases even decades after the initial diagnosis, and despite aggressive therapies. AIM:We present the case of a patient with a lacrimal gland tumor. They are very rare tumors that represent almost 10% of space-occupying orbital lesions, of which 20-30% are of epithelial origin, of these, 55% are benign and 45% are malignant. The ACC is the most common malignant epithelial tumor (65%). CASE REPORT :We present the clinical case of a patient with ACC of the lacrimal gland incidentally diagnosed after performing a CT scan from the psychiatric office. RESULTS AND CONCLUSIONS:The most frequent presentation symptom is usually proptosis, accompanied or not accompanied by pain, although it can also occur as mechanical ptosis, vision reduction, and diplopia. Radical surgery has not been shown to improve survival or reduce local recurrence compared to a more conservative surgical approach in patients with smaller tumors and without bone involvement. The highest survival rates have been achieved, in patients treated with chemotherapy, with intra-arterial cytoreductors (IACC) followed by orbital exenteration and radiotherapy.

Carcinoma adenoideo quístico pulmonar primario con metástasis precoces exclusivas hepáticas, un caso inusual / Primary adenoid cystic carcinoma of bronchus with early liver metastases, an unusual case

RESUMEN El carcinoma adenoideo quístico (CAQ) es una patología propia de las glándulas salivales, casos excepcionales se han descrito como primarios en otras partes de la economía. Este es un caso de CAQ primario pulmonar (CAQPP), con respuesta parcial a radioterapia, además, presenta metástasis hepáticas, sin evidenciarse lesiones de metástasis en otras partes del organismo. Tales características hacen que debamos prestar atención a las formas atípicas de CAQ sobre todo al CAQPP y a su comportamiento poco predecible.

ABSTRACT Adenoid cystic carcinoma (ACC) is a pathology of the salivary glands, exceptional cases have been described as primary of the bronchus. This is a case of primary adenoid cystic carcinoma of the tracheobronchial tree (PACCTBT), with partial response to radiation therapy, in addition, presents liver metastases, without evidence of metastasis injuries in other parts of the body. Such characteristics mean that we should pay attention to the atypical forms of ACC, especially PACCTBT and its unpredictable behavior.

Carcinoma adenoide quístico de cavidad nasal y senos paranasales: experiencia de 5 años en Clínica Las Condes de Santiago de Chile / Adenoid cystic carcinoma of nasal cavity and paranasal sinuses: 5 years experience in las Condes Clinic of Santiago of Chile

RESUMEN Introducción: El carcinoma adenoide quístico es una neoplasia originada en glándulas exocrinas de todo el cuerpo, principalmente en glándulas salivales mayores. En cavidad nasal y senos paranasales es poco frecuente y se caracteriza por presentar una alta frecuencia de recurrencia y de metástasis a distancia posterior a su remisión y a pesar de su tratamiento. Objetivo: Describir características de una serie de casos de pacientes con carcinoma adenoide quístico de cavidad nasal y senos paranasales operados. Material y método: Estudio descriptivo-retrospectivo. Período enero de 2012 y enero de 2017. La información se obtuvo a partir de las fichas electrónicas de la Clínica Las Condes. Se describen procedencia, edad y sexo, características clínicas, hallazgos anatomopatológicos, métodos diagnósticos, tratamiento, evolución. Resultados: Total de 5 pacientes operados, 3 mujeres y 2 hombres. La edad promedio fue de 63 años, con rango de 33 años a 90 años de edad. 100% de los casos sin factores asociados. La presentación clínica más frecuente fue la obstrucción nasal unilateral, seguida por el dolor facial. Dos pacientes se presentaron con diagnóstico inicial, dos por recurrencia y uno por persistencia. En tres casos el tumor se origina de seno maxilar y en dos en seno etmoidal. Todos presentaron enfermedad avanzada, etapa IV y III. Tres pacientes histológicamente fueron de bajo grado y dos de alto grado. En los cinco casos el tratamiento primario fue quirúrgico, en el 60% endoscópico, 20% abierto y 20% combinado. En cuatro casos se usó radioterapia posoperatoria y en tres de éstos, quimioterapia concomitante. Todos se encuentran sin signos de recidiva tumoral en último control. Conclusiones: El carcinoma adenoide quístico de cavidad nasal y senos paranasales es bastante infrecuente, su incidencia es menor a 1/100.000 casos por año. Es más frecuente en mujeres entre 40 y 50 años. Se identifica más con su origen en el seno maxilar (50%) y de patrón cribiforme. Clínicamente se presenta en estadíos avanzados ya que en etapa precoz es asintomático o presenta clínica inespecífica inflamatoria. El diagnóstico se realiza con biopsia complementada con imagenología. El tratamiento más utilizado es la cirugía endoscópica o abierta asociado a radioterapia posoperatoria, a pesar de la cual, se presentan con alta recurrencia a largo plazo.

ABSTRACT Introduction: Adenoid Cystic Carcinoma is a neoplasm originating in exocrine glands throughout the body, mainly in the major salivary glands. In the nasal cavity and paranasal sinuses is rare and characterized by a high frequency of recurrence and distant metastasis after remission and despite its treatment. Aim: To describe characteristics of a series of patients with operated adenoid cystic carcinoma of the nasal cavity and paranasal sinuses. Material and method: Descriptive-retrospective study. Period January 2012 and January 2017. The information was obtained from the electronic tabs of the Las Condes Clinic. It describes origin, age and sex, clinical characteristics, anatomopathological findings, diagnostic methods, treatment, evolution. Results: Total 5 patients operated, 3 women and 2 men. The average age was 63 years, ranging from 33 years to 90 years of age. 100% of the cases without associated factors. The most frequent clinical presentation was unilateral nasal obstruction, followed by facial pain. 2 patients presented with initial diagnosis, 2 due to recurrence and 1 due to persistence. In 3 cases the tumor originates from the maxillary sinus and in 2 in the ethmoidal sinus. All had advanced disease, stage IV and III. 3 patients were histologically low grade and 2 high grade. In all 5 cases, the primary treatment was surgical, 60% endoscopic, 20% open and 20% combined. In 4 cases, postoperative radiotherapy was used and in 3 of this concomitant chemotherapy. All are without signs of tumor recurrence in the last control. Conclusion: Adenoid Cystic Carcinoma of the nasal cavity and paranasal sinuses is quite infrequent; its incidence is less than 1/100,000 cases per year. It is more common in women between 40 and 50 years. It is identified more with its origin in the maxillary sinus (50%) and cribriform pattern. Clinically it presents in advanced stages since at an early stage, it is asymptomatic or it presents nonspecific inflammatory clinic. Diagnosis is performed with biopsy supplemented with imaging. The most commonly used treatment is endoscopic or open surgery associated with postoperative radiotherapy, despite which, they present with high recurrence in the long term.

Bronchial adenoid cystic carcinoma masquerading as bronchial asthma: a case report / 北京大学学报(医学版)

Here we reported a case of bronchial adenoid cystic carcinoma from Peking University Third Hospital. A 40-year-old female presented with dry cough for 1 year and nocturnal paroxysmal attacks of wheezing for 4 months. She was a non-smoker, and did not have past histories of asthma or allergy. On physical examination, no stridor, wheezing and cyanosis were present and the general appearance was good. The results of the laboratory analysis, including blood eosinophils count, immunoglobulin E level and chest X-ray were normal. Spirometry revealed reversible airflow obstruction, and post-bronchodilator forced expiratory volume in one second (FEV1) showed an increase of 12% and 230 mL from baseline. Bronchial asthma was diagnosed, however, she responded poorly despite the adequate anti-asthma therapy including high dose inhaled corticosteroid plus long-acting beta2-agonist, theophylline and montelukast. Then chest computed tomography (CT) was performed which showed a polypoid mass occupying the lumen of left main bronchus. Then the bronchoscopy revealed a polypoid endo-bronchial mass arising from the left main bronchus, causing subtotal obstruction of the lumen. Biopsy was carried out through the bronchoscopy, the pathological findings showed characteristic cribriform and tubular pattern which was formed by two-layered cells with ductal and myoepithelial phenotypes, which were consistent with adenoid cystic carcinoma. Re-examining the patient, the lung was clear without any wheeze when she was seated. However, inspiratory wheeze was heard in her left upper lung when she was supine, and disappeared after sitting up again. Subsequently the patient underwent a resection surgery. At the operational site, the tumor was seen on the anterolateral wall of the left main bronchus, without submucosally expanding histologically. Therefore, a sleeve resection surgery of the left main bronchus was performed. Following surgery, chest CT scan revealed complete resolution of the tumor. Her symptoms improved significantly, as did her pulmonary function tests, although all the medicines for asthma were stopped. Now, two years after the operation, the patient remained asymptomatic, and spirometry was performed again which showed normal completely. The presenting case report emphasizes the fact that not all wheezes and reversible airflow obstruction are asthma. It is critical to bear in mind that if a "difficult asthma" patient does not respond to appropriate anti-asthma therapy; localized obstructions should be differentiated.

Metastasis pulmonares tardías de carcinoma adenoideo quístico de la glandula de bartolino / Late lung metastases of cystic adenoid carcinoma of the bartolino gland

El carcinoma adenoideo quístico de la glándula de Bartolino (GB) es una entidad infrecuente, de crecimiento lento y comportamiento agresivo con alta recurrencia local y metástasis. Presentamos el caso de una paciente de 48 años, con antecedente de tratamiento quirúrgico de un absceso Bartolino derecho. La biopsia demostró un carcinoma adenoideo quístico (CAQ) de la GB, por lo que se realizó hemivulvectomía radical derecha y linfadenectomía inguinofemoral. El resultado de la biopsia mostró 4 ganglios positivos de 12 y margen lateral positivo a menos de 2 mm. Por lo que se realizó adyuvancia con radioterapia. La paciente no se presenta a su control y acude luego de tres años por dolor urente desde cadera izquierda hacia rodilla y cara lateral de la pierna y pie. Se realiza cintigrama óseo que muestra lesión osteoblástica sacroilíaca izquierda de 2 cm, por lo que se completó estudio de diseminación con tomografía computada de tórax, abdomen y pelvis con contraste que evidenció múltiples lesiones nodulares pulmonares bilaterales de distribución generalizada en parénquima central, periférico y subpleurales. Se deriva al Instituto Nacional del Tórax, completando estudio preoperatorio y se realizó una biopsia quirúrgica por video toracoscopía resecando un nódulo subpleural, el informe histológico señaló una metástasis de CAQ concordante con primario en GB. La paciente fue presentada en el comité oncológico y se indicó radioterapia paliativa a lesión sacroilíaca y control clínico ambulatorio. Ha evolucionado estable desde el punto de vista funcional pulmonar.

Adenoid cystic carcinoma of Bartholin glands (BG) is a rare, slow-growing but a highly aggressive tumor with remarkable capacity for local recurrence and distant metastasis. We present the case of a 48 year-old female patient with a history of surgical treatment for right Bartholin abscess. The biopsy showed a cystic adenoid carcinoma of the BG, which led to radical hemivulvectomy and inguinofemoral lymphadenectomy. The biopsy show inguinal lymph node metastasis on 4 of 12 lymph nodes and positive surgical resection margin. Adjuvant radiotherapy was applied. The patient doesnt present to her clinical follow up, and consult three years later with radiological evidence of sacroiliac metástasis on bone scintigraphy. Study was completed with thorax, abdomen and pelvis tomography scan, and showed multiple bilateral lung nodules. The patient was transfered to National Thoracic Institute, and preoperative study was performed. We decide to obtain histologycal confirmation by a video thoracoscopy, it was performed without incidents. Histology show metastasis of adenoid cystic carcinoma pattern, concordant with primary tumor. The patient was submitted to the oncology committee and palliative radiotherapy was indicated for sacroiliac lesion. The patient evolve stable and asintomatic at ambulatory clinical control.

A rare case of primary adenoid cystic carcinoma of lung.

A 33-year-old male presented with repeated episodes of blood-streaked sputum for last one-and half-year. Chest radiograph showed consolidation in the right lower zone. Fibreoptic bronchoscopy revealed an endoluminal growth in the right lower lobe bronchus. Histopathological examination of bronchoscopic biopsy specimen confirmed adenoid cystic carcinoma.

Tumor híbrido parotídeo / Parotid hybrid tumor

Los tumores de glándulas salivales representan el 3%-10% de las neoplasias de cabeza y cuello. La localización más común es en la glándula parótida, representando el 50%-85% de los casos, siendo 20%-30% de ellos malignos. Los siguientes son indicadores de malignidad: Crecimiento acelerado, masa dolorosa, parálisis facial asociada y linfoadenopatía. La mayoría de las neoplasias de parótida derivan de un único tipo histológico, pero eventualmente puede ocurrir el desarrollo de más de un tipo en la misma glándula. Este trabajo presenta un caso de una neoplasia en parótida con dos tipos histológicos diferentes, con una presentación clínica atípica. El paciente se presentó inicialmente con otalgia y otorrea, al examen destacaba un tumor en el canal auditorio externo. El estudio complementario evidenció una neoplasia de parótida y se realizó resección total de la glándula. La biopsia informó un carcinoma adenoideo-quístico con áreas basaloideas diferenciadas. Se administró radioquimioterapia adyuvante, y el control imagenológico con PET-TC mostró la ausencia de recurrencias o diseminación del tumor.

Tumors of the salivary glands represent 33%-10% of head and neck neoplasms. The most common location is the parotid gland, accounting for 50%-85% of the cases, with 20%-30% of them being malignant. The following are known to be indicative of a malignant tumor: fast growing, painless mass, associated facial paralysis and lymphadenopathy. Most parotid neoplasm derive from a single histological type but eventually the development of more than one type on the same gland can occur. This paper presents a case of a parotid neoplasm with two different histological tumors, with uncharacteristic clinical presentation. The patient presented initially with ear pain and otorrhoea, in the clinical examination highlighted an external auditory canal tumor. The complementary study revealed a parotid neoplasm and a total resection of the gland was performed. The biopsy revealed an adenoid-cystic carcinoma with differentiated basaloid areas. Adjuvant radio-chemotherapy was administered, and the imaging control with PET-CT showed no evidence of recurrence or dissemination of the tumor.