High-Grade Transformation in Adenoid Cystic Carcinoma of the Bartholin Gland: Case Report / Transformação de alto grau em carcinoma adenoide cístico de glândula de Bartholin: relato de caso

Abstract Introduction In the present study, we report a case of primary adenoid cystic carcinoma (ACC) of the Bartholin gland with high-grade transformation (HGT). Adenoid cystic carcinoma of the Bartholin gland is a rare tumor and HGT has only been reported in head and neck tumors. Case Report A 77-year-old woman with a non-ulcerated vulvar lesion on the topography of the right Bartholin gland. The patient was submitted to tumor resection followed by V-Y island flap and adjuvant radiotherapy. The histopathological examination revealed primary ACC of the Bartholin gland, with areas of HGT and extensive perineural invasion. The immunohistochemical study with p53 showed a diffuse and strong positive reaction in areas with HGT. After 24 months of follow-up, the patient presented distant metastases and died, despite having undergone to chemotherapy. Conclusion As far as we know, this case is the first description in the literature of HGT in ACC of the Bartholin gland, and HGT appears to be associated with tumor aggressiveness.

Resumo Introdução Este estudo relata o caso de um carcinoma adenoide cístico (CAC) de glândula de Bartholin com transformação de alto grau. O CAC de glândula de Bartholin é um tumor raro, e sua transformação de alto grau é relatada somente em tumores de cabeça e pescoço. Relato de caso Paciente de 77 anos de idade, do sexo feminino, com lesão vulvar não ulcerada na topografia da glândula de Bartholin direita. A paciente foi submetida a ressecção do tumor e realização de retalho em V-Y, seguidas de radioterapia adjuvante. O exame histopatológico revelou CAC primário de glândula de Bartholin, com áreas de transformação de alto grau e invasão perineural. O estudo imunohistoquímico com p53 mostrou reação positiva difusa e intensa em áreas com transformação de alto grau. Após 24 meses de seguimento, a paciente apresentou metástases à distância e faleceu, apesar de ter sido submetida a quimioterapia. Conclusão Pelo que sabemos, este caso é a primeira descrição na literatura de transformação de alto grau em CAC de glândula de Bartholin, e a transformação de alto grau parece estar associada à agressividade do tumor.

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.

Adenoid cystic carcinoma of the lacrimal sac: case report / Carcinoma adenóide cístico do saco lacrimal: relato de caso

ABSTRACT Lacrimal sac tumors are rare with a clinical presentation that typically includes obstruction of the lacrimal drainage system and epiphora as the most frequent symptom. Cribriform adenoid cystic carcinoma (ACC) is the most common malignant epithelial tumor of the lacrimal gland and minor salivary glands; however, its occurrence in the lacrimal drainage apparatus is extremely rare. Given the rarity of ACC, definitive diagnosis is almost invariably late conferring a poor prognosis. Herein we report the case of a 41-year-old woman with primary ACC of the lacrimal sac and describe the ophthalmological examination, diagnosis, and multidisciplinary treatment of this rare type of tumor.

RESUMO Tumores do saco lacrimal são raros. A apresentação clínica muitas vezes mostra uma obstrução no sistema de drenagem lacrimal sendo a epífora o sinal mais frequente. Carcinoma adenóide cístico cribriforme (ACC) é o tumor epitelial maligno mais comum da glândula lacrimal e glândulas salivares menores, mas a sua ocorrência no aparelho de drenagem lacrimal é extremamente rara. Infelizmente, devido a raridade destes tumores, o diagnóstico preciso é quase sempre atrasado, o que por sua vez leva a um pior prognóstico. Nós relatamos o caso de uma mulher de 41 anos de idade, com ACC primário do saco lacrimal e analisamos o exame oftalmológico, diagnóstico e tratamento multidisciplinar deste tipo de tumor.

A retrospective study of 18 cases of adenoid cystic cancer at a tertiary care centre in Delhi.

Context: Adenoid cystic carcinoma (ACC) is a rare neoplasm that usually arises from the salivary, lacrimal, or other exocrine glands. It is characteristically locally infiltrative in nature and has a tendency toward local recurrence, high propensity for perineural invasion, and prolonged clinical course. Aim: To analyze the presentation and natural history of cases of adenoid cystic tumors of salivary glands in our institution; and to compare with the existing literature. Design and Setting: Retrospective study at the Department of Radiotherapy. Materials and Methods: Data on 18 patients of ACC of the salivary glands treated between 2004 and 2008 were reviewed with respect to clinical presentation, stage, and histology. Results: There were 8 cases of major salivary gland tumors (47%), of which 2 were in the submandibular and 6 were involving the parotid. Ten patients (53%) had minor salivary gland involvement. Two patients had metastasis at the time of presentation. All patients underwent surgery. Radiotherapy was delivered to 16 patients and chemotherapy to 6 patients (concurrent, n = 3 and adjuvant, n = 3) and no adjuvant therapy was given to 2 patients. All patients were alive at a median follow-up of 3 years. No patient developed local or distant failure during the study duration. Conclusion: ACC has locally aggressive behavior. Radiotherapy adjuvant to surgery improves local control in locally advanced disease. Longer follow-up is mandatory in view of incidence of late metastasis.

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.

Parotidectomía: experiencia en 3 años / Parotidectomy: experience in 3 years

Este trabajo descriptivo observacional expone la casuística de parotidectomías realizadas en el Hospital Universitario de Los Andes entre los años 2004 y 2007. De 23 pacientes con tumor de parótida 18 fueron mujeres. A todos se les practicó parotidectomías. El 64,3 por ciento eran mayores de 45 años, el aumento de volumen postoperatorio prevaleció en 22 pacientes. La PAAF fue benigna en 60,86 por ciento. La variedad histológica mas frecuente fue el Adenoma Pleoformórfico. La complicación mas frecuente fue lesión del nervio facial. El 80 por ciento de tumores de glándulas salivales afecta a la parótida. Aparecen en promedio a los 50 años de edad. La PAAF ofrece un diagnóstico preoperatorio cercano al definitivo, lo que evita una cirugía innecesaria. En este estudio existió concordancia del 100 por ciento entre PAAF e histopatología. La cirugía es el tratamiento indicado, preservando el nervio facial.

Laryngeal adenoid cystic carcinoma: case report

CONTEXT: Adenoid cystic carcinomas are malignant tumors that occur in both the major and the minor salivary glands. A laryngeal location is rare because of the paucity of accessory salivary glands in this area. Adenoid cystic carcinomas account for less than 1 percent of all malignant tumors in the larynx, and only about 120 cases have been reported in the literature. These tumors have a slight female predisposition, and their peak incidence is in the fifth and sixth decades of life. In this article, we describe a case of laryngeal adenoid cystic carcinoma and discuss its clinical characteristics and treatment. CASE REPORT: We report on a case of laryngeal adenoid cystic carcinoma in a 55 year-old female patient who presented with dyspnea and hoarseness. Features of the diagnostic and therapeutic evaluation are described and the clinical management of such cases is outlined. The clinical course, definitive treatment strategy and surgical procedure, and also adjuvant treatment with irradiation are discussed. Although the tumor is radiosensitive, it is not radiocurable.

CONTEXTO: Carcinoma adenóide cístico (CAC) é um tumor maligno que ocorre tanto nas glândulas salivares maiores quanto nas menores. Localização laríngea é rara devido à paucidade de glândulas salivares acessórias nesta região. O carcinoma adenóide cístico representa menos de 1 por cento das lesões malignas da laringe e apenas 120 casos foram relatados na literatura. O CAC tem freqüência discretamente superior no sexo feminino e seu pico de incidência ocorre entre a quinta e sexta décadas de vida. Neste artigo, descrevemos um caso de CAC laríngeo e discutimos suas características clínicas e seu tratamento. RELATO DE CASO: Relatamos o caso de CAC numa paciente de 55 anos que apresentava disfonia e dispnéia. Características diagnósticas e avaliação terapêutica são descritas e a conduta clínica definida. Evolução clínica, estratégia terapêutica e procedimento cirúrgico são discutidos, bem como o tratamento adjuvante com radioterapia. O tumor, apesar de radiossensível, não é radiocurável.

Carcinoma adenoide quístico de la mama / Adenoid cystic breast carcinoma

Se presenta el cuadro clínico, manejo diagnóstico y tratamiento de una paciente de 45 años portadora de una tumoración de mama derecha EC II A, catalogada por los patólogos como carcinoma adenoide quístico. El presente artículo documenta los hallazgos clínicos, radiológicos, patológicos entre otros que permitieron un adecuado manejo diagnóstico y terapéutico de la paciente.

This is the clinical feature, diagnosis management and treatment of a 45 years old woman with a right breast tumor EC II A which was named by the patologist ad adenoid cystic breast carcinoma. This article shows all clinic, radiological and pathological features of the disease wich let an adequate diagnostic and terapeutic patient management.

Neoplasia de tráquea: presentación de cuatro casos / Tracheal neoplasms: presentation of four cases

Se presenta la casuística de pacientes con neoplasia de la traquea que fueron admitidos al Departamento de Tórax del Instituto de Enfermedades Neoplásicas, tratados con diferentes modalidades terapéuticas, incluyendo, argón plasma y radioterapia externa, braquiterapia, previa citoreducción endoscópica del tumor a través del broncoscopio flexible. La histología de la neoplasia traqueal fue documentada como adenocarcinoma en un caso y tumor adenoide quístico en tres pacientes. Luego del tratamiento se apreció adecuado control de la enfermedad, sin evidencia de recurrencia objetiva endobronquial y completamente asintomáticos a largo plazo.

The case of patients with primary tracheal neoplasia is presented, who were admitted to the Department of Thorax of the Instituto Nacional de Enfermedades Neoplásicas. They received different therapeutic modalities, according to the case, including braquiterapy, argon and external radiation therapy, previous endoscopic citoreducción of the tumor through flexible broncoscopy. The histology of the tracheal neoplasia was documented as adenocarcinoma in a case and adenoid cystic tumor in three patients. After the respective treatmen we documented appropriate control of the disease without evidence of objective endobronchial recurrency and completaly asymptomatic to long term.

[Report 9 cases of adenoid cystic carcinoma of trachea]

Primary malignant neoplasm of trachea are very rare and there is limited information about them. Adenoid cystic carcinoma is a slow-growing malignant tracheal tumor and the best method of treatment is surgical resection. This study was conducted for evaluation of patients with adenoid cystic carcinoma of trachea after treatment. In this descriptive study, all patients treated for adenoid cystic carcinoma between 1995 to 2006 in Mashad Ghaem hospital and Tehran Imam Khomeini hospital, were analyzed statistically. Of nine patients, female to male ratio was 2/1,the mean age of patients was [56.3] years, dyspena and stridor were the most common presenting symptoms [88.8%] and CT scan was the best diagnostic imaging method. The most common site of involvement was in lower third of trachea [44.4%]. [77.7%] of patients underwent surgical resection, hospital mortality after tracheal resection occurred in one patient due to complication [14.2%] aspiration pneumonia. During three years follow up just one patient [11.1%] had tumor recurrence and resection with post operative radiotherapy was performed in this cases overall three-year survival was 88.8%. Because of the nature of adenoid cystic carcinoma in trachea, surgical resection is the best method of treatment, but if surgical margin is positive, post operative radiotherapy will be necessary. In patients who are not candidates for resection, radiotherapy can be an effective alternative treatment

Carcinoma adenóide cístico: relato de caso / Adenoid cystic carcinoma: case report

O carcinoma adenóide cístico é uma neoplasia maligna rara de crescimento lento, caracterizando prognóstico reservado, devido a sua agressividade e grande potencial recidivante. A lesão é mais prevalente em pacientes na faixa etária entre 50 e 70 anos, sendo incomum em jovens. O artigo relata um caso de carcinoma adenóide cístico de glândulas salivares menores localizado no palato duro em paciente com 26 anos, do sexo masculino que foi encaminhado para tratamento no Serviço de Cirurgia de Cabeça e Pescoço

Carcinoma adenóide cístico de traquéia: tratamento pela laringotráqueo-esofagectomia e traqueostomia mediastinal / Adenoid cystic carcinoma of the trachea: treatment by removing the larynx, trachea, and esophagus, and mediastinal tracheostomy

Os autores descrevem o caso de uma paciente de 54 anos de idade com carcinoma adenóide cístico de traquéia ao nível da cricóide, na qual foi realizada ressecçäo extensa, incluindo a laringe, parte da traqéia e esófago. A reconstruçäo do trato gigestivo foi através do tubo gástrico e a via aérea pela construçäo de uma traqueostomia mediastinal. Cinco anos mais tarde removida uma recorrência tumoral cutânea no pescoço. Sete anos depois do procedimento original, foi notada nova recorrência no mediastino, que respondeu à irradiaçäo. A paciente encontra-se muito bem atualmente, oito anos e cinco meses após o procedimento

Carcinoma adenóide-cístico da laringe: diagnóstico, prognóstico e tratamento / Laryngeal adenoid cistic carcinoma: diagnosis, prognosis and treatment

Carcinoma adenóide-cístico da laringe é uma patologia rara, com origem em glândulas salivares menores, cujo diagnóstico geralmente é tardio e as metástases distantes säo frequentes. O tratamento é cirúrgico, com radioterapia complementar. O prognóstico é reservado, ocorrendo as recorrências locais ou metástases distantes, precoces ou tardiamente.