ABSTRACT
O Carcinoma de Células Acinares (CCA) é uma neoplasia epitelial maligna, composta por células com semelhança morfológica com células acinares e com evidências de produção de enzimas exócrinas pelas células neoplásicas. CCA é um tumor extremamente raro e agressivo: representa cerca de 1% de todas as neoplasias pancreáticas, e a taxa de sobrevida global de 5 anos encontra-se inferior a 10%. Esse relato justifica-se não só pela rara ocorrência da doença, mas também pela escassa literatura acerca dos conhecimentos da patologia estudada. Além disso, o CCA é um tumor com difícil diagnóstico e extremamente agressivo; o tratamento dessa doença continua sendo aprofundado, uma vez que novos estudos sugerem a presença de micrometástases indetectáveis em exames de imagem em pacientes submetidos à ressecção cirúrgica total com margens negativas. Dessa forma, há a necessidade de incrementar relatos de caso à bibliografia existente. O presente trabalho trata-se de um relato de caso de Carcinoma de Células Acinares no pâncreas de paciente do sexo masculino de 9 anos diagnosticado no Hospital da Criança Santo Antônio do Complexo Hospitalar Santa Casa de Misericórdia de Porto Alegre.
Acinar Cell Carcinoma (ACC) is an epithelial malignant neoplasm composed of cells with morphological similarity to acinar cells and with evidence of exocrine enzyme production by the neoplastic cells. ACC is extremely rare and an aggressive tumor: it represents about 1% of all pancreatic neoplasms, and the overall 5-year survival rate is less than 10%. This report justifies itself by the rare occurrence of the disease and by the scarce literature concerning the pathology studied. Moreover, ACC is a tumor that is difficult to diagnose and extremely aggressive; the treatment of this disease continues under investigation since new studies suggest the presence of micrometastases - undetectable in imaging studies - in patients undergoing total surgical resection with negative margins. Thus, there is a need to add case reports to the existing bibliography. The present study is a case report of Acinar Cell Carcinoma of the pancreas in a 9-year-old male patient diagnosed at Santo Antônio Children's Hospital, Hospital Complex Santa Casa in Porto Alegre (Hospital da Criança Santo Antônio do Complexo Hospitalar Santa Casa de Misericórdia).
Subject(s)
Carcinoma, Acinar CellABSTRACT
Resumen Los tumores malignos de glándulas salivales (TMGS) constituyen un grupo infrecuente de cánceres de una gran variedad histológica. Dentro de las neoplasias de estirpe epitelial encontramos al carcinoma de células acinares (CCA), que representa entre un 6%-10% de todos los TMGS. No posee patrones clínicos ni radiológicos específicos, pero comparte características comunes con otros TMGS que serán discutidas a lo largo del reporte. Cabe destacar que en términos generales es un cáncer de bajo grado y poco recidivante, por lo que un tratamiento oportuno y un seguimiento estricto mejoran el pronóstico para este tipo de pacientes. Su diagnóstico se establece con evidencia histopatológica que confirme la presencia de diferenciación epitelial de tipo acinar. El tratamiento consiste en la exéresis tumoral con márgenes libres por parotidectomía suprafacial o total, asociado a vaciamiento cervical si se detecta compromiso nodal. Adicionalmente, se debe sugerir terapia adyuvante ante la presencia de un factor de mal pronóstico. Se presenta un caso y se realiza revisión de literatura.
Abstract Malignant salivary gland tumors (TMGS) constitute an infrequent group of cancers of a wide histological variety. Within the epithelial lineage neoplasms, we find acinar cell carcinoma that represent between 6%-10% of all TMGS. It does not have specific clinical or radiological patterns, but it shares common characteristics with other TMGS that will be discussed throughout the report. It should be noted that in general terms it is a low-grade cancer with low recurrence rates, so timely treatment and strict follow-up improve the prognosis for this type of patient. Its diagnosis is established with histopathological evidence that confirms the presence of acinar-type epithelial differentiation. Treatment consists of tumor excision with free margins by suprafacial or total parotidectomy, associated with cervical lymph node dissection if nodal locoregional metastasis is detected. Additionally, adjuvant therapy should be suggested in the presence of a poor prognostic factor. A case is presented and a literature review is carried out.
Subject(s)
Humans , Male , Aged , Parotid Neoplasms/diagnosis , Carcinoma, Acinar Cell/diagnosis , Prognosis , Parotid Neoplasms/therapy , Tomography, X-Ray Computed/methods , Carcinoma, Acinar Cell/therapyABSTRACT
RESUMEN El cáncer pancreático es aquel que comienza en el páncreas y constituye la neoplasia más letal que puede padecer un ser humano. Aunque las células más comunes en el páncreas son las células acinares, la transformación maligna de estas es infrecuente. El adenocarcinoma de células acinares es un tumor maligno muy raro del páncreas exocrino, con menos de un caso por millón de habitantes en los Estados Unidos, y representa menos del 1 % de las neoplasias pancreáticas primarias. Se presentó una paciente femenina de 64 años de edad operada de un carcinoma de células acinares del páncreas. Se revisó la literatura sobre esta temática y se insistió en que dado la topografía pancreática, las neoplasias de este tipo, se diagnostican frecuentemente en la fase final de la enfermedad; por lo que resulta imprescindible, bajo contextos similares, pensar en este tipo de cáncer como posibilidad diagnóstica.
ABSTRACT Pancreatic cancer is a cancer that begins in the pancreas and is the most lethal neoplasm that a human being can suffer from. Although the most common cells in the pancreas are acinar cells, their malignant transformation is rare. Acinar cell adenocarcinoma is a very rare malignant tumor of the exocrine pancreas, with less than one case per million population in the United States, accounting for less than 1% of primary pancreatic neoplasms. We present a 64-year-old female patient operated on for acinar cell carcinoma of the pancreas. The literature on this topic was reviewed, emphasizing on this type of neoplasm, which is frequently diagnosed in the final stage of the disease given its pancreatic topography; that is why, under similar contexts, thinking about this type of cancer as a diagnostic possibility is essential.
Subject(s)
Carcinoma, Acinar Cell , Pancreatic NeoplasmsABSTRACT
Resumen El carcinoma de células acinares es una neoplasia poco frecuente que se presenta principalmente en las glándulas salivales. Presentamos el caso de un paciente femenino de 48 años con dolor, paresia palpebral derecha y aumento de volumen. Biopsia que confirma diagnóstico, manejada con exenteración orbitaria derecha más RT. Durante seguimiento seis años después se presenta dolor columna dorsal, RMN con lesión osteoblástica en T2 biopsia con metástasis de carcinoma de células acinares. Debido a su baja incidencia el comportamiento del carcinoma de células acinares de la glán dula lacrimal es incierto, no hay reportes en la literatura de lesiones metastásicas únicas en columna.
Abstract Acinar cell carcinoma is a rare neoplasm occurs primarily in the salivary glands. We report the case of a female patient of 48 years with pain, right palpebral paresis, and increased volume. Biopsy confirmed diagnosis, handled right exenteration more RT. During follow-up six years after dorsal spine pain, MRI with T2 lesion biopsy osteoblastic metastatic carcinoma of acinar cells. Because of its low incidence behavior acinar cell carcinoma of the lacrimal gland is uncertain, there are no reports in the literature of metastatic lesions unique column.