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.

Primary adenoid cystic carcinoma arising from an ectopic lacrimal gland involving both nasal orbits: a rare clinical entity.

Adenoid cystic carcinoma (ACC) is a slow-growing, locally invasive tumor of epithelial origin. The common sites of origin are the minor and major salivary glands and also the lacrimal glands. ACC accounts for 4.8% of primary orbital neoplasms and commonly arises superolaterally from the main lacrimal gland. Primary ACC of the orbit from an extra lacrimal region is quite rare. We present a case of ACC that occurred in ectopic lacrimal gland tissue involving the medial region of both orbits. A 74-year-old woman was admitted with a 4-week history of progressive painful proptosis in her left eye. Examination revealed a medium-size bilateral nasal canthal mass. Computed tomography (CT) scan revealed a nasal mass in both orbits with bone erosion. The patient underwent bilateral anterior orbitotomy and incisional biopsy was performed. Histopathology exam revealed an ACC with perineural and bone invasion. She refused orbital exenteration and radiation therapy was initiated.

Adenoid Cystic Carcinoma of the Trachea: A Case Report.

BACKGROUND Adenoid cystic carcinoma of trachea (ACCT) is an unusual low-grade tumor from the tracheal and bronchial wall glands. The histological structure and biological behavior of ACCT are similar to that of tumors found in the salivary glands. ACCT occurs most commonly in the upper trachea, being found in the articular cartilage in the posterior aspect of the trachea. CASE REPORT We describe the case of a 62-year-old male ex-smoker 25 years/pack (abstinence 20 years ago), who began with mild dyspnea 2 years ago, with intense evolution and cough. He was evaluated as an outpatient and was treated for sinusitis and later bronchitis after pulmonary function tests. With the worsening of the aforementioned symptoms, he sought prompt care, with hospitalization and computed tomography (CT) of the chest being indicated, showing an expansive lesion in the anterior wall of the trachea with an extension of approximately 3.1 cm, ending at the level of the carina, measuring 3.4×2.8 cm, with moderate stenosis of the tracheal lumen, with an exophytic component to the mediastinum. Bronchoscopy of the lesion was done, and later immunohistochemistry showed a probable pleomorphic adenoma. However, in a new analysis, after the removal of the surgical specimen, it was observed that it was a ACCT. CONCLUSIONS ACCT is a rare tumor that should be diagnosed as soon as possible in order to ensure its best prognosis. Moreover, it is evident that the analysis of the surgical specimen is sovereign to immunohistochemistry with regard to histological typing.

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.

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.

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.

Definitive radiotherapy for unresected adenoid cystic carcinoma of the trachea.

Adenoid cystic carcinoma is a rare malignancy that usually originates in the salivary glands of the head and neck but has rarely been known to originate in the trachea. This histology has a predilection for perineural invasion and a tendency for both local and distant recurrences. While surgical resection is the mainstay of treatment of tracheal adenoid cystic carcinoma, tumor size, location, and patient comorbidities may preclude surgery, and the optimal nonsurgical management remains undefined. In the absence of locoregional lymph node metastases, we recommend highly conformal radiotherapy alone to a dose of 80 Gy. We report on two patients with unresectable disease who were treated with definitive radiotherapy: one using conventional photons and one treated with a combination of photon and proton beams. Both patients were treated to a dose of 80 Gy with acceptable toxicities and objective clinical and radiographic response. The patient treated with conventional photons has no evidence of recurrent disease at 5 years; the patient treated with protons has continued evidence of response without evidence of disease recurrence 11 months after treatment.

An unusual case of adenoid cystic carcinoma with hard palate perforation.

Adenoid cystic carcinoma is a malignant tumor which occurs frequently in hard palate associated with minor salivary glands. The lesion generally presents as a painful slow growing mass and it is characterized by recurrences and distant metastasis resulting in a poor prognosis for the patient. This paper reports an atypical adenoid cystic carcinoma with palatal perforation which occurred in a young woman. Initial diagnostic hypothesis were necrotizing sialometaplasia and lues. Although adenoid cystic carcinoma is common in hard palate, cases with palatal perforation are uncommon and may lead to delay in diagnosis and therapy.

[Tumor of ceruminous gland with intracranial invasion: case report]. / Tumor de glândula ceruminosa com invasão intracraniana: relato de caso.

Ceruminous glands are modified apocrine glands, confined to the skin lining of the cartilaginous part of the external auditory meatus. Tumors arising from these glands are rare. Controversy exists regarding the term "ceruminoma". Actually this neoplasia should be classified as adenoma, adenocarcinoma, adenoid cystic carcinoma and pleomorphic ceruminous adenoma. We report a 39-year-old woman first seen at Santa Casa of Belo Horizonte, in 1998, presenting with headache, nausea, vertigo, hearing loss and tinnitus on the right for the past two years. CT scan showed a tumor eroding cartilaginous and bony limits with intracranial invasion. She was submitted to multidisciplinary treatment with surgery followed by radiotherapy (6000 cG). Histology showed a ceruminous adenoid cystic carcinoma. The patient manifested a right peripheral facial palsy and had no recovery of the previous deficits. After one year from surgery she is clinically stable.