Yolk sac primary tumor of mediastino: a rare case in a young adult / Tumor de saco vitelínico primário do mediastino: raro caso em um adulto jovem

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.

Yolk sac primary tumor of mediastino: a rare case in a young adult.

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.

Morphometrical and immunohistochemical study of intrahepatic bile ducts in biliary atresia.

BACKGROUND: Extrahepatic biliary atresia results from a progressive destruction of the bile ducts by an inflammatory fibrosing process which leads ultimately to cirrhosis of biliary type. The etiology of the disorder remains unknown. The histological features include cholestasis, ductular proliferation, eventual loss of intrahepatic bile ducts, and ducts with primitive embryonic shape (ductal plate malformation). PURPOSE: To examine the morphological changes of the biliary intrahepatic ducts, we aimed at investigating the cell proliferation and the diameter of the interlobular bile ducts in extrahepatic biliary atresia, and in normal liver children. METHODS: Liver samples from 35 patients with biliary atresia and 10 from control normal children were used. Immunoexpression of cytokeratin 19 was evaluated and a double-staining procedure was performed with cytokeratin 8/proliferating cell nuclear antigen. The stereological measurements of the intrahepatic bile ducts diameter were evaluated by a computerized system of image analysis. RESULTS: The patterns of intrahepatic cholangiopathy in biliary atresia were obstructive features (42.86%), paucity of intrahepatic bile ducts (20%), ductal plate malformation (28.57%), and ductal plate malformation associated with paucity of intrahepatic bile ducts (8.57%). The average external diameter of interlobular bile ducts in biliary atresia was smaller than that of the control infant livers. Among the four patterns of biliary atresia cholangiopathies, those associated with ductopenia showed the smallest bile duct diameter. There was a negative correlation between the bile duct to portal space ratio and the age of the child at the time of Kasai portoenterostomy. Only in biliary atresia are the bile duct cells stained with proliferating cell nuclear antigen. CONCLUSION: (i) In biliary atresia, both ductular metaplasia and ductular proliferation were observed; (ii) biliary atresia associated with ductopenia showed narrowing of interlobular ducts, probably as a consequence of degeneration with atrophy and fibrosis.