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Testicular germ cell tumors are a diverse group of neoplasms, consisting of the prepubertal type 1 tumors, pure teratoma, and pure yolk sac tumor, the type 2 tumors, which are biologically malignant, preceded by germ cell neoplasia in situ, and harbor chromosome 12p abnormalities, and the type 3 tumor, spermatocytic tumor, which features chromosome 9p amplification. These arise in distinct clinical settings, and harbor distinct genetic abnormalities, immunohistochemical properties, and morphologic features. Here we have attempted to unify embryology, morphology, immunohistochemistry, and genetics in order to distill this fascinating group of neoplasms into what we hope is a useful framework for understanding their classification.

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ABSTRACT Germ cell tumors are rare neoplasms that mostly occur in the gonads, although they can also affect other body sites, especially the anterior mediastinum (50 to 70% of all extragonadal germ cell tumors). We report a case of a primary mediastinal yolk sac tumor, a rare and aggressive germ cell tumors subtype. This was a 38-year-old man who was admitted to Hospital do Servidor Público Estadual "Francisco Morato de Oliveira", complaining about dyspnea and dry cough for 1 year. The computed tomography scan of his chest revealed a large mass in the anterior mediastinum with heterogeneous enhancement to the contrast associated with pleural effusion. There were also high serum levels of alpha-fetoprotein. After neoadjuvant chemotherapy, the patient underwent surgical resection of the mass, followed by pathological examination, which confirmed a primary mediastinal yolk sac tumor, a nonseminomatous subtype of germ cell tumors. Primary mediastinal yolk sac tumors have poor prognosis, despite advances in therapy with surgical resection and cisplatin-based chemotherapy. This poor prognosis is due to the degree of invasion and unresectability in most patients by the time of the diagnosis.

RESUMO Os tumores de células germinativas são neoplasias raras que acometem mais frequentemente as gônadas, embora possam também ocorrer em outras localizações do corpo, destacando-se o mediastino anterior (50 a 70% de todos os tumores de células germinativas extragonadais). No presente artigo, relatamos um caso de tumor de saco vitelínico mediastinal primário, de subtipo raro e agressivo de tumor de células germinativas. Tratava-se de um homem, 38 anos, admitido no Hospital do Servidor Público Estadual "Francisco Morato de Oliveira", com quadro de dispneia e tosse seca há 1 ano. Na investigação clínica, foi solicitada tomografia computadorizada de tórax, que mostrou volumosa massa no mediastino anterior com realce heterogêneo ao meio de contraste associada a derrame pleural. Havia ainda aumento dos níveis séricos da alfafetoproteína. Após quimioterapia neoadjuvante pré-operatória, o paciente foi submetido à ressecção cirúrgica, seguida de estudo anatomopatológico da peça, no qual demonstrou tratar-se de um tumor de saco vitelínico primário do mediastino. Os tumores de saco vitelínicos primários do mediastino têm prognóstico reservado, apesar do avanço na terapêutica com a ressecção cirúrgica e a quimioterapia à base de cisplatina. Isto se deve ao grau de invasão e de irressecabilidade na maioria dos pacientes no momento do diagnóstico.

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Germ cell tumors are rare neoplasms that mostly occur in the gonads, although they can also affect other body sites, especially the anterior mediastinum (50 to 70% of all extragonadal germ cell tumors). We report a case of a primary mediastinal yolk sac tumor, a rare and aggressive germ cell tumors subtype. This was a 38-year-old man who was admitted to Hospital do Servidor Público Estadual "Francisco Morato de Oliveira", complaining about dyspnea and dry cough for 1 year. The computed tomography scan of his chest revealed a large mass in the anterior mediastinum with heterogeneous enhancement to the contrast associated with pleural effusion. There were also high serum levels of alpha-fetoprotein. After neoadjuvant chemotherapy, the patient underwent surgical resection of the mass, followed by pathological examination, which confirmed a primary mediastinal yolk sac tumor, a nonseminomatous subtype of germ cell tumors. Primary mediastinal yolk sac tumors have poor prognosis, despite advances in therapy with surgical resection and cisplatin-based chemotherapy. This poor prognosis is due to the degree of invasion and unresectability in most patients by the time of the diagnosis.

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INTRODUCTION: Neoplasms originating from the urachus are rare. The most common urachal malignancy is adenocarcinoma, whereas extragonadal germ cell tumors, primarily of the urachus, are an extremely rare finding. OBJECTIVE: To describe a primary yolk sac tumor (YST) of the urachus in an adult. CASE REPORT: A 44-year-old woman presented with 6 months of pelvic pain associated with a sensation of progressive mass growth. At the time of tumor resection, the tumor was found to be attached by a pedicle to the dome of the bladder, with no injury to the adjacent organs. Pathological study showed a neoplasm with epithelioid cells, pseudocysts, a myxomatous background, and Schiller-Duval body formations. Immunohistochemistry stains showed positivity to AE1/AE3, α-1-fetoprotein, and α-1-antitrypsin and negativity to other markers. CONCLUSION: An unusual case of a YST in the urachus is presented. This is the first reported adult case based on the authors' bibliographic search.

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Testicular and paratesticular prepuberal tumors are rare. They represent around 1 percent of the total of tumors of infancy. They subdivide in 2 groups: germ cells tumors and non germ cells tumors, being able to occur in all the ages, and about 75 percent are malignant, and about 19 percent of these they present metastasis. The tumors of germ cells tumors represent 60 75 percent of the tumors testiculars in infancy, having as main example the yolk sac tumor (65 percent of the neoplasms), followed for teratomas (14 percent); although some works to exist where teratoma, if presents as most common .The non germ cells tumors include the Leydig cell tumor and Sertoli cell tumor. The Leydig cell tumor, are most frequent between the non germ cells tumors testicular. This review article on epidemiology, diagnosis and treatment of to testicular and to paratesticular tumors in child.

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Testicular and paratesticular prepuberal tumors are rare. They represent around 1% of the total of tumors of infancy. They subdivide in 2 groups: germ cells tumors and non germ cells tumors, being able to occur in all the ages, and about 75% are malignant, and about 19% of these they present metastasis. The tumors of germ cells tumors represent 60 75% of the tumors testiculars in infancy, having as main example the yolk sac tumor (65% of the neoplasms), followed for teratomas (14%); although some works to exist where teratoma, if presents as most common .The non germ cells tumors include the Leydig cell tumor and Sertoli cell tumor. The Leydig cell tumor, are most frequent between the non germ cells tumors testicular. This review article on epidemiology, diagnosis and treatment of to testicular and to paratesticular tumors in child.

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Salivary gland tumors are uncommon and most of them are of epithelial origin. Mesenchymal tumors affecting the parotid are extremely rare, and we present a series of 19 cases. All parotid tumors (600 cases) treated at the Department of Head and Neck Surgery from A.C. Camargo Hospital, Brazil from 1953 to 2003 were reviewed and 19 cases of nonlymphoid mesenchymal origin were selected. The histological characteristics were reviewed and clinical features were obtained from the medical charts. 15 out of 19 were benign tumors, including 5 lymphangiomas, 5 neurofibromas, and one case each of schwannoma, lipoma, solitary fibrous tumor, meningioma and giant cell tumor. Four malignant tumors were classified as rhabdomyosarcoma, fibrosarcoma, Langerhans cell histiocytosis and endodermal sinus tumor. From the malignant cases, only the patient with fibrosarcoma died due the tumor, the other three are alive with no signs of recurrence. In our series of 600 cases of parotid gland tumors, nonlymphoid mesenchymal tumors corresponded to 3.16% (19 cases; 15 benign and 4 malignant). All cases were treated by surgery with no recurrences, except one case of fibrosarcoma whose patient died of distant metastasis.

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A 17-year-old boy presented with somnolence and mental confusion. Physical examination demonstrated motor disturbances. Laboratory investigation showed elevated levels of alpha-fetoprotein in serum and cerebrospinal fluid. The CT scan revealed a heterogeneous mass at the pineal region. At the MRI, this lesion was hypointense on T1 and hyperintense on T2-weighted images, enhancing after contrast administration. The patient underwent a surgical biopsy, which defined the diagnosis of yolk sac tumor. We emphasize the correlation of neuroimaging and pathological findings of this rare pineal region tumor.

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A 17-year-old boy presented with somnolence and mental confusion. Physical examination demonstrated motor disturbances. Laboratorial investigation showed elevated levels of alpha-fetoprotein in serum and cerebrospinal fluid. The CT scan revealed a heterogeneous mass at the pineal region. At the MRI, this lesion was hypointense on T1 and hyperintense on T2-weighted images, enhancing after contrast administration. The patient underwent a surgical biopsy, which defined the diagnosis of yolk sac tumor. We emphasize the correlation of neuroimaging and pathological findings of this rare pineal region tumor.

Um menino de 17 anos de idade apresentou-se com sonolência e confusão mental. O exame físico demonstrou distúrbios motores. A investigação laboratorial revelou aumento dos níveis de alfafetoproteína no soro e no líquor. A TC de crânio revelou massa heterogênea na região pineal. A RM, a lesão era hipointensa em T1 e hiperintensa em T2, com realce após a administração de contraste. O paciente foi submetido a biópsia cirúrgica, a qual definiu o diagnóstico de tumor do seio endodérmico. Enfatizamos a correlação entre os achados patológicos e de neuroimagem deste raro tumor da região pineal.

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