ABSTRACT
El tumor de Askin o tumor primitivo neuroectodérmico es una neoplasia de células pequeñas redondas que se origina de los tejidos blandos de la pared torácica, probablemente a partir de células embrionarias que migran de la cresta neural. Son tumores muy agresivos que metastatizan rápidamente y de forma diseminada. Clínicamente, los pacientes presentan una masa de tejidos blandos en la pared del tórax que puede cursar o no con dolor. Otras manifestaciones incluyen disnea, tos, pérdida de peso, síndrome de Horner y adenopatías regionales. La radiografía de tórax muestra una masa heterogénea extrapulmonar, por lo general de gran tamaño, que puede opacificar completamente el hemitórax. El pronóstico del tumor de Askin es pobre; sin embargo, el uso combinado de quimioterapia, cirugía y radiación ha mejorado el resultado de forma drástica.
Askin tumor or primitive neuroectodermal tumor is a small round cells' neoplasia, which originates in the chest's soft tissues probably from embryonic cells that migrate from the neural crest. They are very aggressive tumors that metastasize and disseminate quickly. Clinically, patients show a soft tissue mass in the chest that may or may not be accompanied by pain. Other manifestations include dyspnea, cough, weight loss, Horner syndrome and regional lymphadenopathy. Chest radiographies show a usually large extrapulmonary heterogeneous mass, which can completely opacify the hemithorax. The prognosis is poor; however, the combined use of chemotherapy, surgery and radiation has improved results dramatically.
O tumor de Askin ou tumor neuroectodérmico primitivo é uma neoplasia de pequenas células redondas que se origina dos tecidos moles da parede torácica, provavelmente de células embrionárias que tem migrado da crista neural. São tumores muito agressivos que metastatizam e se disseminam rapidamente. Clinicamente, os pacientes apresentam uma massa de partes moles na parede torácica que pode ou não causar dor. Outras manifestações incluem dispneia, tosse, perda de peso, síndrome de Horner e linfadenopatia regional. A radiografia de tórax mostra uma massa extrapulmonar heterogênea, geralmente grande, que pode opacar completamente o hemitórax. O prognóstico do tumor de Askin é ruim; no entanto, o uso combinado de quimioterapia, cirurgia e radiação tem melhorado drasticamente o resultado.
Subject(s)
Humans , Female , Adolescent , Neuroectodermal Tumors, Primitive, Peripheral/diagnostic imaging , Thoracic Wall/pathology , Radiography, ThoracicABSTRACT
Resumen El tumor de Askin es una rara neoplasia de la pared torácica que pertenece al grupo de tumores neuroectodérmicos primitivos que incluyen desde el sarcoma de Ewing hasta el neuroepitelioma. Su principal característica es el dolor. Los tumores neuroectodérmicos primitivos (PNET) derivan de células pluripotenciales neuroectodérmicas de la cresta neural y pertenecen al grupo de tumores de células pequeñas redondas y azules que se caracterizan por un comportamiento agresivo, con manifestaciones clínicas diversas que dependen de su localización anatómica. La localización más común es la toracopulmonar, seguida por el área pélvica. Las metástasis normalmente afectan a los pulmones, huesos, hígado y cerebro. Su diagnóstico se basa en estudios imagenológicos y se confirma con el estudio de histopatología en biopsia o en la resección quirúrgica.
Abstract The Askin's tumor is a rare neoplasm of the chest wall that belongs to the group of primitive neuroectodermal tumors (PNETs), which are derivatives from neuroectodermal pluripotent neural crest cells. The range of this type of tumors covers from Ewing's sarcoma to the neuroepithelioma and they belong to the group of small round blue cells tumors which are characterized for having an aggressive behavior with diverse clinical manifestations depending on their anatomical location. The most common location for the Askin's tumor is the thoracopulmonary area followed by the pelvic area and in the case of metastases the lungs, bones, liver and brain are the most affected parts. In order to identify this disease, the diagnosis of this type of tumor consists on image studies and the confirmation is made through a hispathology biopsy or surgical resection.
ABSTRACT
Objective To discuss the imaging manifestations and clinical characteristics of Askin tumor.Methods 12 cases of Askin tumor confirmed by surgically and histopathologically were included in this study.The MSCT signs and clinical data were summarized.Results In 12 cases of Askin tumor,7 cases located in the chest wall;7 case were hospitalised with rapid enlargementd painful lump(58.3%,7/12).9 cases were performed surgery and postoperative radio chemotherapy,6 patients experienced metastatic or recurrent tumors during the following year(66.7%,6/9).Askin tumor often occurred in the chest-lung junction region,had the diameters of 2.0-13.0 cm,11 cases showed inhomogeneous density (91.7%,11/12), 1 case showed calcification (8.3%,1/12), ill-defined margin(100%,12/12),12 cases showed intense but inhomogeneous enhancement following contrast administrationin (100%,12/12),11 cases had the feature of pleura invasion (91.7%,11/12), 9 cases with pleural effusion(75.0%,9/12), 1 case with swollen lymph nodes in mediastinum(8.3%,1/12), and 4 cases with lytic lesion of adjacent bone(33.3%,4/12).Conclusion Askin tumor often occurrs in the chest-lung junction region with rapid enlargementd painful lump as the primary symptom;the tumor show the infiltration diffuse growth;calcification and adjacent lymph node metastasis is not very common.
ABSTRACT
Objective To analyze CT manifestations of chest wall Askin tumor and to illustrate its CT diagnostic and differentially diagnostic key points.Methods Eleven cases of chest wall Askin tumors were pathologically confirmed in our hospital between May 2006 and November 2014.Of them,10 cases were children and adolescents,and 9 cases had chest pain as the first symptom.All patients received plain CT scan,while 7 cases received contrast enhanced scan.CT signs of this group were retrospectively analyzed,including the location,quantity,shape,size,density,adjacent tissue invasion and distant metastasis of the tumors.Results CT examination showed a single oval mass on the chest wall in all 11 cases.The tumor was located completely within the thoracic cavity in 10 cases,while a tumor's main part was within thoracic cavity.Nine tumors had long diameters of more than 9 cm.All the tumors were heterogeneous without calcification,wherein 10 cases showed necrosis and cystic degeneration.On contrast enhanced scan,7 cases showed heterogeneously mild to moderate enhancement.All tumors invaded adjacent tissues,including 7 cases of bone damage of a single rib,and 7 cases of pleural involvement,wherein 6 cases had pleural effusion.No remote metastases were found in this group.Conclusions CT manifestations of Askin tumors of chest wall have some characteristics,and familiarity with these manifestations is conducive to diagnosis and differential diagnosis of this disease.
ABSTRACT
Objective To analyze the clinicopathological features and enhance the knowledge of Askin tumor in children. Methods The clinicopathological features of 4 cases of children with Askin tumor were described. Results Clinically, the tumor was often seen in elder child patients with painful thoracopulmonary masses.Histopathologically, the nest-like or lobular structures composed of small round-to-oval cells were surrounded by sparse fibrovascular stroma. Among tumor cells there were no or few resticular fibers,however in the surrounding of tumor masses the fibers were found. PAS staining in the tumor in a part of cases was positive. The positive rates of CD99 and Syn detected by immunohistochemistry were high. Neurosecretory granules can be observed by electron microscopy. Two patients received both chemotherapy and radiotherapy three patients died of metastatic tumor of lung and brain and chemotherapy complication in 24,25 and 5 months, respectively. Conclusions Askin tumor is a highly malignant neurosecretory tumor in thoracopulmonary region, which often be misdiagnosed as neuroblastoma, lymphoma, embryonal rhabdomyo sarcoma, tuberculosis and lung abscess, and requires a synthetical treatment plan consisting of pre-and postoperative chemotherapy, radiotherapy and surgical resection. The treatment methods of Askin tumor needs to be further studied.
ABSTRACT
Malignant small cell tumor(MSCT) of the thoracopulmonary region(Askin tumor) is extremely rare and is seen predominantly in children and adolescents. This tumor represents a distinct clinicopathologic entity of neuroectodertnal origin, arising from the soft tissues of the chest wall or peripheral lung. This tumor tends to recur locally, but does not seem to disseminate widely. The overall survival is poor. Recently, we experienced a case of the MSCT of the thoracopulmonary region of a 12-year-old female. She was admitted because of a chest wall mass on radiographic examination, and a complaint of intermittent chest pain. Grossly, the mass was lobulated, round and had a solid appearance with focal necrosis and hemorrhage on the cut surface. Histologically, small round to oval cells were arranged in compact sheets, nests and lobular patterns with intervening fibrovascular stroma. Ultrastructurally, the presence of loose-fitting membrane-bound neurosecretory granules was noted.
Subject(s)
Child , Adolescent , Male , Female , HumansABSTRACT
The malignant small round cell tumor of the thoracopulmonary region was described by Askin in 1979 and called as Askin Tumor. The Askin tumor is a rare, arises from the soft tissues of the chest wall or peripheral lung which is predominantly in younger generation. Clinical and pathologic appearance were very similar to Ewing's sarcoma and rhabdomyosarcoma but when examined it by electron microscopy, there was some different in morphology. The tumor tended to recur locally and did not seem to disseminated widely but the median survival was only 8 months.