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.

Youlk sac tumors

Yolk sac tumor [YST] is a unique category of germ cell tumors [GCTs] involving the gonadal and extragonadal regions. Although YSTs are extremely rare, they are important because they commonly affect children, display an unusual natural history, aggressive behavior and require specific treatment. This study included 3 cases with YSTs. One of them was ovarian pure YST that was discovered among 7 cases of ovarian GCTs [14.3%]. The age of the patients ranged from 4 to 41 years [mean age 21 years], all of them were presenting with palpable abdominal masses [one recurrent ovarian, one pelvic retroperitoneal, and one right hypochondrial mass]. Appropriate laboratory [including tumor markers] and radiological studies were performed. All patients underwent adequate surgical resection and postoperative chemotherapy. Local recurrence was reported in one case and distant metastases in 2 cases. They survive postoperatively for a period ranging from 8 to 36 months. The aim of this study was to throw light on this rare malignancy

Tumor del seno endodermal primario del ovario estadío IIIC tratada sólo con cirugía. Reporte de un caso / Primary endodermal sinus tumor of the ovary stage IIIC treated with surgery. Case report

Se comunica un caso de una paciente de 14 años de edad con un tumor del seno endodermal primario del ovario que fue tratado quirúrgicamente (estadiaje), siendo catalogado como estadío IIIc (micrometástasis en los ganglios retroperitoneales) requiriendo por lo tanto, tratamiento complementario quimioterápico, el cual no se cumplió por razones ajenas a la institución; sin embargo, se encuentra sin evidencia de la enfermedad a 8 años (último control: abril de 1994).

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