Cytokeratin-positive primitive neuroectodermal tumor of the prostate: Case report and review of literature.

Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) of the prostate is extremely rare. Here, we report a case of ES/PNET of prostate in a 24-year-old man presenting with dysuria and pelvic discomfort. Computed tomography scan revealed a heterogeneous mass involving the prostate without evidence of distant metastases. Histologically, the tumor was composed of small round blue cells strongly and diffusely positive for CD99 and epithelial markers. Fluorescence in situ hybridization confirmed rearrangement of the Ewing's sarcoma region on chromosome 22.

Sarcoma de Ewing: epidemiologia e prognóstico dos pacientes tratados no Instituto de Oncologia Pediátrica, IOP-GRAACC-UNIFESP / Ewing's sarcoma: epidemiology and prognosis for patients treated at the Pediatric Oncology Institute, IOP-GRAACC-UNIFESP

OBJETIVO: Traçar o perfil epidemiológico e o prognóstico do sarcoma de Ewing na população brasileira. Material e MÉTODO: Foram avaliados, retrospectivamente, os prontuários de 64 pacientes tratados, com sarcoma de Ewing intraósseo, no Instituto de Oncologia Pediátrica, IOP-GRAACC-Unifesp, no período de 1995 a 2010. RESULTADOS: A análise estatística dos dados obtidos não correlacionou fatores como sexo, trauma, fratura patológica e tempo ao diagnóstico com o desfecho do tratamento. Fatores como metástase inicial, metástase pulmonar, local do tumor, idade, recidiva e tipo de cirurgia indicam resultados que corroboram a literatura consagrada. CONCLUSÃO: O prognóstico no sarcoma de Ewing foi influenciado principalmente pela presença de metástase ao diagnóstico.

OBJECTIVE: To outline the epidemiological profile and prognosis for Ewing's sarcoma in the Brazilian population. Material and METHODS: The medical records of 64 patients with intraosseous Ewing's sarcoma who were treated at the Pediatric Oncology Institute, IOP-GRAACC-Unifesp, between 1995 and 2010, were retrospectively evaluated. RESULTS: The statistical analysis on the data obtained did not correlate factors such as sex, trauma, pathological fracture and time taken for case diagnosis with the treatment outcome. Factors such as initial metastasis, lung metastasis, tumor site, age, recurrence and type of surgery showed results corroborating what has been established in the literature. CONCLUSION: The prognosis in cases of Ewing's sarcoma was mainly influenced by the presence of metastases at the time of diagnosis.

How we performed chest wall reconstruction: analysis 31 cases.

OBJECTIVE: To see the results of patients who underwent chest wall resection and reconstruction (CWRR). SETTING AND DESIGN: Retrospective descriptional study. MATERIAL AND METHODS: We retrospectively reviewed all patients who underwent CWRR at Xingtai People's Hospital in China and B.P. Koirala Memorial Cancer Hospital in Nepal. A total of 31 patients were reviewed. Among them, 20 were male and 11 female. The median age was 63 years. The indications for resection were primary chest wall tumor in 21 patients (67.7%), lung cancer with invasion of chest wall 6 (19.4%), recurrence of breast cancer 2(6.3%), radiation necrosis 1(3.2%) and skin cancer 1(3.2%). RESULTS: The mean number of rib resected was 3.6 ribs, which induced a mean defect of 97.1 cm2. Concomitant resection was done in 13 patients, including lung resection 10, partial resection of diaphragm 2, and partial sternectomy 1. Seven patients underwent soft tissue reconstruction (STR) alone and 5 patients skeletal reconstruction (SR) alone. Simultaneous SR and STR were performed in 19 patients. Three patients (9.7%) developed postoperative complications. The median survival period was 22 months. CONCLUSION: Primary chest wall tumor and lung cancer invading chest wall are the most common diseases indicating CWRR. Simultaneous bony and soft tissue reconstruction was reliable for chest wall reconstruction in most cases and prevents postoperative complications.

Tumores ósseos malignos na criança e no adolescente / Malignant bone tumors in children and adolescent

Os tumores malignos primários dos ossos representam cerca de 10 porcento das neoplasias que acometem crianças e adolescentes. Os tumores de Ewing e o osteossarcoma säo os mais frequentes (95 porcento) nas duas primeiras décadas de vida. Enorme evoluçäo nas taxas de cura ocorreu nos últimos anos, graça a novos recursos da cirurgia e da moderna quimioterapia. A sobrevida livre de doença em cinco anos pode hoje ser conseguida, para ambas as doenças, em cerca de 60 pocento a 70 porcento dos casos. As cirurgias de conservaçäo do membro - endopróteses ou enxertos ósseos - podem oferecer condiçöes de manutençäo de funçäo adequada de membros, em lugar de amputaçöes, atualmente reservadas para tumores mais avançados. Para que estes objetivos possam ser alcançados, descrevemos neste artigo informaçöes para o diagnóstico precoce e a sequência terapêutica recomendada

Descriptive epidemiology of bone cancer in greater Bombay.

Bone tumours are comparatively uncommon, constituting only 0.5% of the total world cancer incidence. As Bone tumors consist of several distinct clinico-pathological entities, descriptive epidemiology of tumors at this site can be based only on studies where they can be distinguished. Ewing's sarcoma Chondrosarcoma and Osteosarcoma are the principal tumors involving bones. The basic data utilized for this study was collected from the Bombay Cancer Registry which was established in 1963, and is the first population based registry to be established in India. For studying the descriptive epidemiological variables the most recent 5 year incidence rates have been used. As a group, bone cancers represent 0.9% of the total number of incident cancer are seen in Greater Bombay. Males in general are seen to have a higher incidence of bone cancers than females. Ewing's sarcoma was found to be the commonest bone cancer in Bombay. The age specific incidence curves present striking differences according to cell types of bone cancer. Time trends in the incidence of these cancers, over the past 30 years have been presented. Our data indicate that there is a decreasing trend in incidence of bone cancers in females, whilst the rates are stable in males. Ionising radiation is the only environmental agent to cause this cancer. The discovery of other risk factors is the key prevention and will depend upon the experimental work undertaken to develop sub-clinical measures of risk that can be applied in interdisciplinary studies to identify more completely the causes of bone cancers.

Primitive peripheral neuroectodermal tumors Mayagüez Medical Center experience

Primitive neuroectodermal or neuroepithelial tumors are names used to describe neoplasias composed of undifferentiated cells resembling germinal cells of the embryonic neural tube. These tumors are small round cell malignancies of the neural crest origin arising outside the central and sympathetic nervous system. They are described as peripheral and central neuroectodermal tumors related to the original malignant cell. A great number of tumors are described under this classification in spite of the fact that there is no an universal acceptance that these small-cell neoplasms, regardless of their primary site, are derived from immature neuroectoderm tissue. Because one tumor resembles others in terms of its phenotypic expression, multiple specific studies such as clinical profile, ultrastructural, immunocytochemical, and cytogenetic features should be studied, since no single clinical or laboratory marker is by itself diagnostic. However, there is a chromosomal reciprocal translocation, t(11;22)(q24;q12), which is unique to Primitive Neuroectodermal Tumor (PNET)

Tumores óseos malignos: estudio de 107 casos atendidos en el INOR durante el período 1964-1974 / Malignant bone tumors: study of 107 cases assisted at the Instituto Nacional de Oncologia y Radiobiologia during the period 1964-1974

Se estudiaron de forma retrospectiva 107 casos (100%) de tumores óseos malignos, inscriptos en el Instituto Nacional de Oncología y Radiobiología (INOR) desde enero l964 hasta diciembre de l974 que comprendían 44 osteosarcomas (41%), 28 condrosarcomas (26,2%), 26 sarcomas de Ewing (24,3%), 6 tumores de células gigantes (5,6%) y 3 fibrosarcomas (2,8%) en los cuales se tuvieron en cuenta aspectos epidemiológicos, diagnósticos y terapéuticos, con el fin de conocer el comportamiento de los mismos, concluyéndose que: el 73% de los tumores óseos malignos se presenta entre la primera y la tercera décadas de la vida, pero la edad de máxima incidencia se encuentra entre los 10 a 19 años, con un 44,9%. Predominan en el sexo masculino y la raza blanca. En el 43% de los casos, la aparición del tumor se relacionó con un trauma local previo de intensidad variable. El 47% de nuestros pacientes tuvo síntomas por más de 6 meses antes de acudir a un centro médico asistencial y sólo el 4,7% buscó atención antes del mes de aparición de las mismas, destacándose el dolor, la impotencia funcional, el tumor, los cambios locales de temperatura y más raramente las fracturas patológicas, como los síntomas de presentación. El 75% de las lesiones se localizaron en los huesos largos: fémur (36,4%), tibia (16,8%) y húmero (10,3%) como los sitios más importantes. La diáfisis y la metáfisis, respectivamente, fueron las localizaciones principales de origen tumoral. El tratamiento de elección fue la cirugía a la demanda asociada con la radioterapia y a la quimioterapia pre o posoperatoria, en dependencia con el tamaño de la lesión, el tipo histológico y el estado físico y psíquico de los enfermos. La supervivencia actuarial quinquenal fue de 69%