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.

Unusual anatomic location of a primary intracranial yolk sac tumor

A nongerminomatous germ cell tumor occurring in the brain parenchyma is extremely rare. A 2-year-old boy presented with symptoms of abnormal movements in the hand and mouth. MRI scanning revealed a lesion occupying the left temporoparietal region. Craniotomy was performed and the tumor was removed by en bloc resection. Histological examination revealed that the tumor was arranged in a reticular pattern, and Schiller-Duval bodies were evident at the center of the tumor. Immunohistochemical study showed that the tumor cells were positive for alpha-fetoprotein and vimentin, but negative for glial fibril-lary acidic protein. The histological diagnosis was pure yolk sac tumor

Primary Yolk Sac Tumor Arising in the Pancreas with Hepatic Metastasis: a Case Report

Extragonadal yolk sac tumor (YST) is a relatively rare entity. We describe here the case of an extragonadal YST that occurred in the pancreas with hepatic metastasis in an adult woman. The contrast enhanced CT images of the abdomen revealed a heterogeneous, solitary mass occupying the pancreatic neck and body with slightly inhomogeneous contrast enhancement. Two low-density lesions in the liver were also displayed on the CT images. The patient underwent surgery and the diagnosis of YST was pathologically verified.

Tumores testiculares na infância: [revisão] / Testicular tumors in childhood: [review]

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.

Testicular mixed germ cell tumor metastasizing to heart.

A 21 years old male presented with low grade fever, hemoptysis and progressively increasing dyspnoea of four month duration followed by acutely developing dizziness, hypotension, convulsion and altered sensorium. He had been operated (left inguinal orchidectomy) for left testicular swelling two years back with high alpha-fetoprotein and normal beta-human chorionic gonadotropin (beta-hCG). In view of this a possibility of metastasis secondary to a malignant testicular tumor was considered. Echocardiography demonstrated a large intracardiac mass, chest computed tomography (CT) revealed intracardiac mass, mediastinal masses and left sided pleural effusion. The histopathology revealed testicular mixed germ cell tumor (MGCT). This case is presented to demonstrate uncommon cardiac manifestations of secondary spread of testicular malignancy.

Pineal yolk sac tumor: correlation between neuroimaging and pathological findings

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.

Prepubertal testicular tumours in Kashmir--a histopathological report of 15 cases.

A total of 15 cases of prepubertal testicular tumours were reported by the department of pathology, Government Medical College, Srinagar, Kashmir over a period of 15 years, from January 1984 to December 1998. The cases included yolk sac tumour (10 cases; 67%), teratoma [(mature) 2 cases (13%)], rhabdomyosarcoma [(paratesticular) 2 cases (13%)] and NHL-Burkitt's lymphoma [one case (7%)]. The youngest patient was 10 months old and the oldest was of 14 years age. Ten cases (67%) occurred at or below the age of 4 years. The youngest patient had yolk sac tumour and oldest had rhabdomyosarcoma. In 2 cases both the testes were involved with one of these two cases having bilateral undescended testes.

Cytodiagnosis of yolk sac tumor.

To analyze cytomorphologic features of yolk sac tumors of childhood. Four cases of pediatric yolk sac tumor (YST), diagnosed by fine needle aspiration cytology were reviewed (1998-2002). Age of patients ranged from 1(1/2) to 5 years. Three cases presented clinically with an intra-abdominal mass while one case presented with a testicular mass. Fine needle aspirates had been obtained directly as well as under radiologic (USG/CT) guidance. Smears were stained with H & E and Papanicolaou stain. In all cases values of serum alpha-fetoprotein and hCG were available preoperatively. Histopathologic diagnosis was correlated with cytologic findings in all the cases. Cytologic examination showed richly cellular smears with a combination of morphological patterns. Characteristically, tumor cells were arranged in papillary groups, tight cell clusters and formed acinar structures. Cells showed enlarged, moderately pleomorphic, hyperchromatic nuclei and moderate amount of cytoplasm, some of which displayed cytoplasmic vacuolation, displacing the nuclei eccentrically. Preoperatively, serum alpha-fetoprotein level was raised in all cases. Histopathology confirmed the cytologic diagnosis. Yolk sac tumor is common among the germ cell tumors of pediatric age group which presents a spectrum of cytomorphologic features having important differences with other germ cell neoplasm, e.g. embryonal carcinoma. Clinicoradiologic features and tumor markers are additionally helpful for an accurate cytologic diagnosis.

Tumor del seno endodermico primario de hígado: presentación de un caso y revisión de la literatura / Endodermal sinus tumor primary of liver: presentation of a case and review of the literature

El tumor del seno endodérmico es una neoplasia maligna poco frecuente, con localización extragonadal extremadamente rara. Presentamos un caso de un paciente femenino de 58 años de edad, con diagnóstico clínico de absceso hepático. Los estudios imagenológicos mostraron una lesión neoplásica en los segmentos VI-VII del lóbulo hepático derecho. Además presentaba niveles elevados de alfa-fetoproteína (6616 ng/mL). Se le realizó hepatectomía derecha y drenaje toráxico. El estudio histopatológico e inmunohistoquímico concluyó que se trataba de un tumor del seno endodérmico. No se demostró la presencia de tumor primario extrahepático

Primary mixed germ cell tumour of the liver--a case report.

Germ cell tumours in liver are uncommon. Fewer than 20 cases have been reported in the literature. Primary mixed malignant germ cell tumours of liver are even rare. Here we describe a case of primary mixed malignant germ cell tumour of left lobe of liver in a two and half year old male boy. This is the first reported case of primary mixed malignant germ cell tumour with components of yolk sac tumour and mature teratoma in an infant. The patient complained of gradual increase in abdominal distension, upper abdominal pain and loss of weight. Ultrasonography and computed tomography of the abdomen revealed a large hepatic mass. Serum alpha-feto protein levels were raised. Left lobectomy of liver was done and chemotherapy was initiated. Histopathology of specimen disclosed yolk sac tumour and mature teratoma. Levels of serum alpha-feto protein declined rapidly. Widespread intrahepatic metastasis developed and patient died due to liver dysfunction after six months of left lobectomy.

Primary pleuro-pulmonary malignant germ cell tumours.

Lungs and pleura are rare sites for malignant germ-cell tumours. Two cases, pure yolk-sac tumour and yolk sac-sac tumour/embryonal carcinoma are described in young males who presented with rapid progression of respiratory symptoms. The malignant mixed germ cell tumour occurred in the right lung, while the yolk-sac tumour had a pseudomesotheliomatous growth pattern suggesting a pleural origin. Alpha-foetoprotein was immunohistochemically demonstrated in both.

Endodermal sinus tumour of cervix--case report.

Endodermal sinus tumor of Cervix is an extremely rare cause of vaginal bleeding. Because of its rarity and interesting features, it is reported with a brief review of literature.

Tumor de seno endodérmico del ovario. Presentación de un caso / An endodermal sinus tumor of the ovary. A case report

The authors present the clinical history of a 15-year-old young woman, student, from David, Chiriquí, who complained of menstrual irregularities associated with the sensation of a mass in the right iliac fossa. Exploratory laparotomy revealed a right ovarian tumor, which was totally resected. The histopathological examination showed an endodermal sinus tumor, stage one, in the right ovary. The patient received four cycles of cytotoxic i.v. therapy every 3 weeks, after surgery, with vinblastine 6 mg/m2 body surface (B.S.) on days 1 and 2, bleomycin 15 u daily for 5 days, cisplatin 20 mg/m2 B.S. daily for 5 days, plus hydration with normal saline (5 to 6 l/day). Follow up studies with abdominal and pelvic sonograms, chest x-ray, feto protein and physical examination have not shown evidence of tumor activity 36 months after diagnosis