Carcinoma adenoide quístico / Adenoid cystic carcinoma

Introducción: El carcinoma adenoide quístico es una neoplasia maligna poco común y a menudo agresiva, que puede surgir en una variedad de órganos portadores de glándulas. Objetivo: Caracterizar la presentación de una neoplasia maligna agresiva infrecuente, el carcinoma adenoide quístico. Caso clínico: Paciente de 56 años que acudió a cuerpo de guardia con lesión en cuero cabelludo correspondiente con cilindroma maligno. Se estudia posteriormente y se constataron además lesiones a nivel de mamas, colon y recidiva en el cuero cabelludo, que dieron como resultado carcinoma adenoide quístico. Conclusiones: Es infrecuente; realizar su diagnóstico conlleva un alto índice de sospecha y su diagnóstico es histopatológico. Su pronóstico depende de la localización primaria y tiende a ser muy agresivo cuando se localiza fuera de la mama(AU)

Introduction: Adenoid cystic carcinoma is an uncommon and often aggressive malignant neoplasm that can arise in a variety of gland-bearing organs. Objective: To characterize the presentation of an uncommon aggressive malignancy, adenoid cystic carcinoma. Clinical case: 56-year-old patient who presented to the on-call department with a lesion on the scalp corresponding to malignant cylindroma. She was subsequently studied and lesions were also found in the breast, colon and recurrence in the scalp, which resulted in adenoid cystic carcinoma. Conclusions: It is infrequent; making its diagnosis involves a high index of suspicion and its diagnosis is histopathological. Its prognosis depends on the primary location and it tends to be very aggressive when located outside the breast(AU)

Carcinome adénoïde kystique de la fosse nasale : à propos d'un cas et revue de littérature

Parmi les tumeurs de la tête et du cou, le carcinome adénoïde kystique ne représenteque 1%. C'est la tumeur maligne la plus fréquente des glandes salivaires accessoires.Sa localisation dans la fosse nasale est exceptionnelle. Nous rapportons un cas de carcinome adénoïde kystique de la fosse nasale chez une patiente de 42 ans qui se présente pour une symptomatologie nasale. La tomodensitométrie objective un processus tissulaire de la fosse nasale droite, l'endoscopie montre une tumeur s'étendant au vestibule avec à la biopsie un carcinome adénoïde kystique. La prise en charge chirurgicale a consisté en une exérèse de la tumeur avec maxillectomie totale suivie d'une radiothérapie

Cytokeratin immunoprofile of primary and metastatic adenoid cystic carcinoma of salivary glands: a report of two cases

Distant metastases from salivary gland tumors are considered infrequent: the incidence of distant metastases ranges from 24% to 61% according to different histotypes and to the site of the primary mass. The most common site of distant metastases due to salivary gland malignancies is the lung. From the pathology point of view, cytokeratins (CK) are important differentiation markers in salivary gland tumors, which are often used for the diagnostic process. Their employment also may be useful to identify and confirm the diagnosis of their distant metastases. We report the expression of CK in two cases of primary and metastatic adenoid cystic carcinoma (ACC) and their CK profiles of the primary and metastatic masses. Both patients-one male and one female-were diagnosed with an ACC cribriform and tubular, respectively, with lung metastases. In case 1, the metastatic mass presented the same histotype and CK profile of the primary tumor. For case 2, the metastatic lung mass was distinct from the primary mass (a solid ACC) and presented a different CK profile. Although salivary gland metastatic disease presents a poor prognosis, both patients reported herein are alive despite the presence of the disease in long-term follow-up. Therefore, the modifications seen in the CK profiles do not appear to be predictive of tumor behavior and outcome. The use of a CK profile seems to be useful to identify the nature of a distant mass and its possible correlations with a primary salivary gland tumor.

Carcinoma adenoideo quístico de mama: serie de casos / Adenoid cystic carcinoma of the breast: case series

El carcinoma adenoideo quístico (CAQ) de mama es un cáncer poco frecuente y representa entre el 0,1% y el 0,4% de todos los carcinomas de mama. A continuación se presentan dos casos con el objetivo de describir un tipo de carcinoma de mama de presentación poco frecuente con evolución clínica no común. El primero de una mujer de 34 años y el segundo de una mujer de 44 años, ambas con diagnóstico confirmado de CAQ de mama. El CAQ de mama es de predominio en el sexo femenino en la etapa postmenopáusica. Sin embargo, ambas pacientes presentaron el CAQ en edad fértil. Generalmente posee un pronóstico favorable con rara diseminación, a diferencia de estos dos casos presentados donde se observa varias metástasis a distancia y un curso muy agresivo de la enfermedad.

Adenoid cystic carcinoma (ACC) of the breast is a rare neoplasm and accounts for 0.1% to 0.4% of all breast carcinomas. This is a description of two clinical cases that describe a rare presentation with an uncommon clinical course. The patients are 34 and 44 year old women, both with a confirmed diagnosis of ACC of the breast. The ACC of the breast predominantly grows in postmenopausal women. However, both patients are still fertile. Commonly it has a favorable prognosis and a rare presentation of metastasis. Nevertheless, in both cases, distant dissemination and an aggressive course of the disease was observed.

Estudo imunoistoquímico de componentes da Via Sonic Hedgehog, Stat3 e Mcm3 em tumores de glândula salivar / Immunohistochemical study components of Via Sonic Hedgehog, Stat3 and MCM3 in salivary gland tumors

O adenoma pleomórfico (AP), o carcinoma mucoepidermóide (CME) e o carcinoma adenóide cístico (CAC) representam tumores frequentes em glândula salivar. A via de sinalização Sonic Hedgehog (Hh) e o Transdutor de sinal e ativador da transcrição 3 (STAT3) desempenham funções importantes na proliferação celular, favorecendo o desenvolvimento tumoral e a proteína MCM3 tem sido considerada uma nova classe de marcadores de proliferação celular. Portanto, o presente trabalho propõe-se a estudar componentes da via Hh, bem como o STAT3 e o MCM3 em neoplasias de glândula salivar, na tentativa de adicionar informações sobre as características biológicas dessas neoplasias. Foram utilizados 9 casos de AP, 17 casos de CAC e 20 casos de CME e, por meio da técnica imunoistoquímica, realizou-se a detecção das seguintes proteínas: SHH, GLI1, SUFU, HHIP, STAT3 e MCM3. No AP, observou-se alta expressão citoplasmática de SHH e SUFU, e baixa expressão de STAT3 e MCM3. No CAC, observou-se alta expressão de GLI1, HHIP e STAT3 e baixa expressão de SHH, SUFU e MCM3. No CME, observou-se alta expressão de SHH, GLI1, SUFU e HHIP e baixa expressão de STAT3 e MCM3. Quando comparado entre os tipos tumorais, observou-se diferença estatisticamente significante para expressão de SHH (p=0.0064), STAT3 (p=0.0003) e MCM3 (p=0.0257)...

The pleomorphic adenoma (PA), mucoepidermoid carcinoma (MEC) and the adenoid cystic carcinoma (ACC) are common tumors arising from salivary glands. The Sonic Hedgehog signaling pathway (Hh) and signal transducer and activator of transcription 3 (STAT3) play important roles in cell proliferation, favoring tumor growth. The MCM3 protein has been considered as a novel class of cell proliferation markers. The aim of this investigation was to study components of the Hh pathway, as well as STAT3 and MCM3 in salivary gland neoplasms in an attempt to add information about the biological characteristics of these neoplasms. We used 9 cases of PA, 17 cases of ACC and 20 cases of MEC. Using immunohistochemistry, were investigated: SHH, GLI1, Sufu, HHIP, STAT3 and MCM3. In PA, there was high expression of cytoplasmic SHH and Sufu, and low expression of STAT3 and MCM3. In the ACC, there was high expression of GLI1, HHIP and STAT3 and low expression of SHH, SUFU and MCM3. In the MEC, we observed high expression of SHH, GLI1, SUFU and HHIP and low expression of STAT3 and MCM3. There was a statistically significant difference between SHH (p=0.0064), STAT3 (p=0.0003) and MCM3 (p=0.0257) when all tumors were compared...

Recurred Adenoid Cystic Carcinoma of Lacrimal Gland with Aggressive Local Invasion to the Maxillary Bone Marrow without Increased Uptake in PET-CT

No abstract available.

An Uncommon Primary Tracheal Tumor Masquerading as Chronic Obstructive Pulmonary Disease: Diagnosed at Autopsy.

Primary tracheal tumors are rare neoplasms and often are hard to diagnose early due to the non-specific clinical presentations. Prolonged symptomatic treatment for these can often lead to missing out the diagnosis and can prove fatal. A 35-year-old male presented with gradually increasing breathlessness and dysphagia to solids and was being treated with bronchodilators and antibiotics. Chest computed tomography (CT) revealed an intratracheal mass in the lower part of the trachea and the patient was referred to our institution in view of a primary tracheal tumor where he expired within 10 min of admission. On autopsy, the gross findings and histological examination revealed a diagnosis of adenoid cystic carcinoma in the trachea. To conclude, primary tracheal tumors, even though rare, should be suspected in cases of refractory chronic obstructive pulmonary disease and chest CT and bronchoscopy must be done to aid correct diagnosis.

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.

Carcinoma adenoideo quístico / Adenoid cystic carcinoma

El carcinoma adenoide quístico representa aproximadamente un 25 por ciento de los tumores de cabeza y cuello, ubicado principalmente a nivel de paladar, labio y mucosa bucal. Mantiene un curso lento pero con probabilidad de metástasis a largo plazo con diseminación de predominio vascular y raramente a nódulos linfáticos. Clínicamente se manifiesta como indolente manifestado principalmente como una masa. El método diagnóstico más importante es el examen físico con exámenes complementarios por medio de ultrasonido y dependiendo de características propias de la masa se solicitar resonancia magnética para delimitar patología como guía durante procedimiento quirúrgico. Como tratamiento de primera línea se utiliza la resección quirúrgica y se ha demostrado mejores resultados con la agregación de radioterapia.

Sinonasal adenoid cystic carcinoma: clinical case report and literature Review / Carcinoma adenoide quístico nasosinusal: caso clínico y revisión de la literatura

We present the case of a patient, a 59 year-old man, with Sinonasal Adenoid Cystic Carcinoma. Magnetic resonance exam revealed invasion of the right orbit and brain at the level of the anterior cranial fossa floor. Due to the large volume, we decided to perform radio-chemotherapy treatment to diminish the size of the lesion. On conclusion of the first stage of treatment, reduction in tumor size was confirmed by computerized tomography exam and we decided to perform surgical resection with right ocular globe preservation. At present the patient is under periodic control and without major complications...

Se presenta el caso de un paciente de sexo masculino de 59 años con carcinoma adenoide quístico nasosinusal. El examen de resonancia magnética reveló la invasión de la órbita derecha y el cerebro a nivel del suelo de la fosa craneal anterior. Debido al gran volumen, se decidió realizar el tratamiento de radio-quimioterapia para disminuir el tamaño de la lesión. Al término de la primera etapa del tratamiento, la reducción del tamaño del tumor fue confirmada por el examen de tomografía computarizada y se decidió realizar una resección quirúrgica con preservación del globo ocular derecho. En la actualidad el paciente se encuentra bajo el control periódico y sin mayores complicaciones...

Estudo da expressão imuno-histoquímica das MMps -2, -7, -9 e TIMPs -1 e -2 em adenomas pleomórficos e carcinomas adenóides císticos de glândulas salivares menores / Study of immunohistochemical expression of MMPs -2, -7, -9, and TIMPs -1 and -2 in pleomorphic adenomas and adenoid cystic carcinoma of minor salivary glands

0 balanço entre a expressão das metaloproteinases da matriz (MMPs) e seus inibidores teciduais (TIMPs) tem sido relacionado a vários processos fisiológicos e patológicos, incluindo a morfogênese de glândulas salivares e os processos de invasão e metástase tumoral. O adenoma pleomórfico (AP) e o carcinoma adenóide cístico (CAC) representam,respectivamente, neoplasias benignas e malignas de glândulas salivares que, embora compartilhem a mesma origem celular, apresentam comportamentos biológicos distintos. O propósito deste estudo foi comparar a expressão imuno-histoquímica das MMPs -2, -7, -9 e -26 e dos TIMPs -1 e -2 em casos de AP e CAC de glândulas salivares menores. Vinte casosde AP e vinte casos de CAC foram avaliados quanto à presença, intensidade e localização das MMPs e TIMPs no parênquima tumoral. A maioria dos APs e CACs apresentaram alta expressão das MMPs e dos TIMPs, predominantemente localizada nas células tumorais. Nãohouve diferença estatisticamente significativa na expressão das MMPs -2 (p=0,359), -7 (p=0,081) e -26 (p=0,553), bem como dos TIMPs -1 (p=0,657) e -2 (p=0,248), entre o parênquima dos APs e CACs. A MMP-9 demonstrou uma diferença significativa de expressão entre os dois tumores, apresentando o CAC uma marcação mais intensa para esta gelatinase(p=0,041). A forte expressão da MMP-9 observada no parênquima dos CACs sugere que esta gelatinase possa desempenhar um papel importante no comportamento biológico destes tumores. Por outro lado, apesar de não ocorrer uma diferença significativa entre as médias das MMPs -2, 7 e 26 nos tumores estudados, os dados quando analisados em conjunto sugeremque estas proteases podem estar participando de processos de remodelação tecidual em ambos os tumores, mas não apresentam uma relação direta com o padrão de agressividade do CAC...

Metastatic pleural effusion: A rare presentation of salivary gland adenoid cystic carcinoma.

Adenoid cystic carcinoma, an uncommon malignant salivary gland neoplasm, is known for its long clinical course, indolent growth, local recurrence and late distant metastasis. We report an unusual case of adenoid cystic carcinoma of the palate in a 64-year-old woman, undiagnosed for more than 15 years, who presented as malignant pleural effusion.

Carcinoma adenoideo quístico de la mama: a propósito de un caso / Adenoid cystic carcinoma of the breast: a purpose of a case

El carcinoma adenoideo quístico de la mama es inusual se presenta con una frecuencia menor al 1 por ciento. La presentación de este caso está relacionada con la rareza del mismo, incidencia de 0,1 por ciento. Paciente femenina 56 años de edad, menarquía: 15 años. IIIG, IP, IC, IA. Menopausia: 2002. No recibe terapia hormonal. No antecedentes personales y/o familiares de patología mamaria. Consulta a nuestra unidad por control, examen físico: normal. Mamografía: mamas con moderada cantidad de tejido fibroglandular, evidencia opacidad nodular en unión de cuadrantes inferiores mama derecha. Eco mamario: imagen aspecto sólido bordes regulares, ubicada en cuadrantes inferiores de mama derecha de 6,1 mm x 7,1 mm x 7,0 mm, se corresponde con opacidad nodular mamográfica. Evaluación mastológica clase IV. Se recomienda evaluación histológica de la lesión a través de técnica de biopsia ecoguiada. Anatomía patológica: carcinoma adenoideo quístico. Grado nuclear II, actividad mitótica atípica presente. Desmoplasia estromal severa. Estudio de inmunohistoquímica: CK7+ (células secretoras), CK7/CK14+(células adenoescamosas), colágeno tipo IV. Se realiza mastectomía parcial oncológica derecha, previa localización y confirmación por imagen márgenes por corte congelado más ganglio centinela. Anatomía patológica: carcinoma adenoideo quístico de 0,7 cm. Grado nuclear I. Actividad mitótica atípica presente. Bordes de resección quirúrgica, margen adicional libres de lesión. Patología mamaria adicional: ectasia ductal y condición fibroquística. Ganglio linfático centinela uno sin evidencia de neoplasia. En marzo de 2009, paciente acude a control, donde se realiza evaluación mastológica concluyéndose clase IIRF.

Adenoid cystic breast carcinoma is rare variant occurring less than 1 percent. The presentation of this case is related to the rarity of it, because the incidence of this tumor in the breast is 0.1 percent. Female patient, 56 years old, menarche: 15 years. III pregnancy: IB, IP, IA. Menopause: 2002. Not receiving substitutive hormone therapy. No personal and family history of breast pathology. She came in August, 2007 for breast evaluation, physical examination: Normal. Mammography: Breast with moderate amount of fibroglandular tissue, nodular opacity in union of lower quadrants right breast. Breast ultrasound: Solid image, with regular edges, located at junction of lower quadrants right breast, measuring 6.1 mm x 7.1 mm x 7.0 mm, which corresponds with nodular mammographic opacity. Mastological evaluation class IV. Histological evaluation of lesion, using the technique of microbiopsia ecoguide. Pathology report: Adenoid cystic carcinoma, nuclear grade II. This atypical mitotic activity, stromal desmoplasia severe. Immunohistochemistry: CK7 + (secretory cells), CK7/CK14 + (Adenosquamous cell), type IV collagen. Partial mastectomy was performed in oncology right after echolocate confirmation by imaging, margins cut more frozen sentinel node. With pathology report: Adenoid cystic carcinoma of 0.7 cm. nuclear grade I, atypical mitotic activity, edge of surgical resection and additional margin free of injury. Additional mammary pathology: Fibrocystic status, ductal ectasia. A sentinel lymph node without evidence of malignancy; March 2009, the patient comes to control, where assessment is completed mastological class IIRF.

Tumor pseudopapilar del páncreas: reporte de caso / Pancreatic pseudopapilar tumor: case report

El tumor pseudopapilar del páncreas es una entidad muy poco frecuente que afecta preferentemente a mujeres jóvenes. Se trata de un carcinoma quístico con bajo potencial maligno que se diagnostica generalmente de forma incidental pues no hay signos ni síntomas patognomónicos de dicha enfermedad. La resección quirúrgica es curativa en la mayoría de los casos y el pronóstico es excelente aun si hay metástasis al momento del diagnóstico. Se presenta el caso de una paciente de sexo femenino de 21 años de edad, sin antecedentes personales de importancia, quien consultó por dolor abdominal agudo. Se realizó tomografía computarizada, la cual reportó una masa de 75 x 80 x 76 mm en la cola del páncreas, por lo que se llevó a cirugía realizándose pancreatrectomía distal con resección completa del tumor y preservación esplénica. El estudio de anatomía patológica reportó tumor pseudopapilar del páncreas. La paciente tuvo una evolución satisfactoria y hasta el seguimiento a los 16 meses persiste asintomática y no ha presentado recurrencia.

Pseudo papillary tumor of the pancreas is a rare entity most commonly seen in young females. This neoplasm it is a cystic carcinoma of low malignant potential usually diagnosed incidentally because an abscense of typical signs or symptoms. Surgical resection it is usually curative with excellent prognosis even when metastasis are present at the time of diagnosis. We report the case of a 21-year-old female who presented with abdominal pain with no previous history of any abdominal discomfort or pain. Computed tomography showed a 75 x 80 x 76 mm solid mass located at the tail of the pancreas. A distal pancreatectomy with preservation of the Spleen was performed. Pathology reported: pseudo papillary tumor of the pancreas. Evolution was satisfactory with no complication and the follow up at 16 months showed no recurrence. Histological examination confirmed the diagnosis and the complete resection of this tumor has an excellent prognosis with rare recurrences.

Tumours of the submandibular salivary gland: a clinicopathologic review of cases over a 17-year period / Tumores de la glándula salival submandibular: una revisión clínico-patológica de casos por un período de 17 Años

OBJECTIVE: This study presents a clinicopathologic review of cases of submandibular salivary gland tumours at the Lagos University Teaching Hospital, Nigeria, over a period of 17 years. METHODS: The records of all patients with histological diagnosis of submandibular salivary gland tumours at the Lagos University Teaching Hospital over a period of 17 years (January 1990 to December 2006) were retrospectively reviewed. Parameters studied were; age and gender of patients, symptoms and duration ofsymptoms, and histological diagnosis. RESULTS: A total of 36patients with submandibular gland tumours were seen during the period. Maleto-female ratio was 1.8:1 (male = 23, female = 13). Mean age (± SD) at presentation was 43 (± 19) years (age range, 17-84 years). There were 19 malignant tumours and 17 benign ones. Pleomorphic adenoma (36.1%) was the most frequent tumours, followed by adenoid cystic carcinoma (11.1%), anaplastic carcinoma (11.1%) and malignant lymphoma (11.1%). Patients with histological diagnosis of malignant tumours were significantly older than those with benign tumours (p = 0.01). Most patients (80.6%) presented with painless swelling. CONCLUSION: Malignant submandibular salivary gland tumours were slightly more than the benign ones in the studied population. Painful swelling or ulceration is indicative ofa malignant submandibular gland tumour.

OBJETIVO: Este estudio presenta una revisión clinicopatológica de casos de tumores de la glándula salival submandibular en el Hospital Docente de la Universidad de Lagos, Nigeria, por un periodo de 17 años. MÉTODOS: Se revisaron retrospectivamente las historias clínicas de todos los pacientes con el diagnóstico histológico de los tumores de la glándula salival submandibular en el Hospital Docente de la Universidad de Lagos, correspondientes a un periodo de 17 años (de enero de 1990 a diciembre de 2006). Los parámetros estudiados incluyeron: la edad y el género de los pacientes, síntomas y duración de síntomas, y diagnóstico histológico. RESULTADOS: Un total de 36 pacientes con tumores de la glándula de submandibular fueron atendidos durante ese periodo. La proporción varón:hembra fue 1.8:1 (varones = 23, hembras = 13). La edad promedio (± SD) al momento de su presentación fue 43 (± 19) años (rango de edad, 17-84 años). Hubo 19 tumores malignos y 17 benignos. El adenoma pleomórfico (36.1%) fue el tipo de tumor más frecuente, seguido por el carcinoma cístico adenoideo (11.1%), el carcinoma anaplástico (11.1%) y el linfoma maligno (11.1%). Los pacientes con diagnóstico histológico de tumores malignos eran significativamente de más edad que los que tenían tumores benignos (p = 0.01). La mayoría de los pacientes (80.6%) presentaban hinchazón sin dolor. CONCLUSIÓN: Los tumores malignos de la glándula salival submandibular fueron ligeramente mayor en número que los benignos en la población estudiada. Hinchazón dolorosa o la ulceración dolorosas son indicios de un tumor maligno de la glándula submandibular.

Adenoid cystic carcinoma of the external auditory canal

This is a rare case of a young male with biopsy proven adenoid cystic carcinoma of the external auditory canal who underwent excision of the lesion with superficial parotidectomy sparing the facial nerve. Histopathology showed perineural invasion, which is a diagnostic hallmark of adenoid cystic carcinoma. Clinical examination, chest X-ray and CT scan showed no signs of recurrence or metastasis 2 years postoperatively