Turner syndrome is a congenital condition affecting 1 in every 2500 female live births. This condition is characterized by complete or partial loss of the X chromosome. They commonly present with normal female external and internal genitalia and may develop hypogonadism and streak ovaries later in life. We describe an unusual presentation of a case of Turner syndrome – a 31-year-old Filipino with male phenotype mosaic Turner syndrome, with 46,X,+mar[46]/45,X[4] chromosome, presenting with ambiguous genitalia and a pelvoabdominal mass. The patient underwent exploratory laparotomy, peritoneal fluid cytology, adhesiolysis, tumor debulking (gonadectomy) appendectomy, omentectomy, identification and inspection of bilateral ureters and bladder, gonioscopy and biopsy of the urogenital cavity (bladder vs. vagina). Histopathology revealed a mixed gonadal tumor, consisting of 70% yolk sac tumor, and 30% dysgerminoma. The patient eventually succumbed to postoperative complications. Postmortem fluorescence-in situ hybridization revealed a 46,X,+mar[46]/45,X,[4].ish der (Y) (DYZ3+), a marker of chromosome Y origin, consistent with a mosaic type Turner syndrome, associated with increased risk for gonadal malignancy.
Dysgerminoma ; mixed germ cell tumor ; mosaicism ; yolk sac tumor

No abstract available.
Endodermal Sinus Tumor* ; Lung* ; Neoplasm Metastasis* ; Yolk Sac*

The Beckwith-Wiedemann syndrome, which included congenital anomalies such as macroglossia, exomphalos, postnatal somatic gigantism, have a substantially increased risk for the development of tumor. We report a case of testicular yolk sac tumor associated with Beckwith-Wiedemann syndrome, a previously unreported association. Pathologic examination showed Schiller-Duval body with evidence of testicular yolk sac tumor. This finding appears to represent a previously unreported association between Beckwith-Wiedemann syndrome and testicular yolk sac tumor.
Beckwith-Wiedemann Syndrome* ; Endodermal Sinus Tumor* ; Gigantism ; Hernia, Umbilical ; Macroglossia ; Testis ; Yolk Sac*

Primary intracranial yolk sac tumor usually arises in the pineal or suprasellar area and shows poor prognosis. Several children with primary yolk sac tumor in the cerebellum have been reported in the literature, and none of these survived beyond 30 months. We report a case of primary yolk sac tumor in the cerebellum showing longer survival with intensive multimodal combined therapy using surgery, preirradiation chemotherapy, and irradiation.
Cerebellum ; Child ; Drug Therapy ; Endodermal Sinus Tumor* ; Humans ; Prognosis ; Radiotherapy ; Yolk Sac*

Extragondal germ cell tumors are rare, which develop outside of the testes, but histologically identical to primary testicular germ cell tumors. There have been much interest and controversies about their etiology, definition, treatment and care of testes. We recently experienced a case of primary mediastinal yolk sac tumor in a 16-years-old male, who was cured by intensive chemotherapy and cytoreductive surgery. Herein we report the case with review of the literature.
Drug Therapy ; Endodermal Sinus Tumor* ; Humans ; Male ; Neoplasms, Germ Cell and Embryonal ; Testis ; Yolk Sac*

Endodermal sinus tumors(EST) have also been referred to as "yolk sac tumors" because they are derived from the primitive yolk sac. These lesions are the third most frequent malignant germ-cell tumors of the ovary. Recent advances in treating the endodermal sinus tumor of the ovary with the combination chemotherapy result in improvement of the prognosis. We had experienced a case of the endodermal sinus tumor of 15 year old woman. We report this case with a brief review of literatures.
Adolescent ; Drug Therapy, Combination* ; Endoderm* ; Endodermal Sinus Tumor* ; Female ; Humans ; Ovary* ; Prognosis ; Yolk Sac

We report herein a case of gastric undifferentiated carcinoma with focal yolk sac tumor and hepatoid carcinoma differentiation. A 57-year-old man was referred after a gastroscopy for anemia evaluation. Gastroscopy revealed an approximately 3 cm ulcerofungating mass occupying the greater curvature of stomach body. Biopsy results revealed a poorly differentiated adenocarcinoma. Left gastric lymph node was enlarged, but there were no evidence of distant metastasis on the abdominal CT. He underwent a radical subtotal gastrectomy and gastroduodenostomy with dissection of the enlarged lymph nodes. Postsurgical histological examination revealed an undifferentiated carcinoma with focal yolk sac tumor and hepatoid carcinoma differentiation. Immunohistochemical staining revealed that the tumor cells were focal positive for AFP, anti-hepatocyte antibody.
Adenocarcinoma ; Anemia ; Biopsy ; Carcinoma ; Endodermal Sinus Tumor ; Gastrectomy ; Gastroscopy ; Lymph Nodes ; Neoplasm Metastasis ; Stomach ; Yolk Sac

Ovarian cancer is the second most common gynecologic cancer worldwide and the six most common cancer among females. Germ cell tumorbs are the most common ovarian neoplasm in the first two decades of life constituting approximately two-thirds of all ovarian tumors. Malignant germ cell tumors constitute one-third of germ cell origin tutors and two-thirds of all ovarian malignancy in this age-group. This paper presents a case of a 19 year-old nulligravid who presented at the emergency room with abdominal pain, and was intraoperatively diagnosed with yolk sac tutor of the right ovary, stage 1A mature cystic teratoma of the left ovary. She subsequently underwent unilateral salpingooophorectomy and contralateral oophorocystectomy, left. Patient is advised chemotherapy postoperatively, with Bleomycin, Etoposide and Paclitaxel. This paper discusses the incidence, risk factors, prognosis and management of yolk of sac tutor in a young nulligravid.

Polyvesicular vitelline tumor of the ovary is an extremely rare variant of yolk sac tumor. We present a case of pure polyvesicular vitelline tumor in a 43-year-old woman. Light microscopy revealed a predominantly polyvesicular pattern embedded in mesoblastic stroma with the cysts showing two type of lining; tall columnar and cuboidal, or mesothelioid cells. The lining atypical cells showed occasional mitoses and intracytoplasmic PAS positive hyaline globules. In some areas, the cystic space contained a large amount of intraluminal hyaline material. Immunohistochemically, alpha- fetoprotein and alpha-1-antitrypsin were detected as a fine granular deposit in the cytoplasm of epithelial cells and hyaline globules. Electron microscopically, marked specialization of the vesicular lining cells suggested a differentiation toward gut structures and mature yolk sac.
Adult ; Cytoplasm ; Endodermal Sinus Tumor ; Epithelial Cells ; Female ; Fetal Proteins ; Humans ; Hyalin ; Microscopy ; Mitosis ; Ovary* ; Vitellins* ; Yolk Sac

Polyvesicular vitelline tumor of the ovary is an extremely rare variant of yolk sac tumor. We present a case of pure polyvesicular vitelline tumor in a 43-year-old woman. Light microscopy revealed a predominantly polyvesicular pattern embedded in mesoblastic stroma with the cysts showing two type of lining; tall columnar and cuboidal, or mesothelioid cells. The lining atypical cells showed occasional mitoses and intracytoplasmic PAS positive hyaline globules. In some areas, the cystic space contained a large amount of intraluminal hyaline material. Immunohistochemically, alpha- fetoprotein and alpha-1-antitrypsin were detected as a fine granular deposit in the cytoplasm of epithelial cells and hyaline globules. Electron microscopically, marked specialization of the vesicular lining cells suggested a differentiation toward gut structures and mature yolk sac.
Adult ; Cytoplasm ; Endodermal Sinus Tumor ; Epithelial Cells ; Female ; Fetal Proteins ; Humans ; Hyalin ; Microscopy ; Mitosis ; Ovary* ; Vitellins* ; Yolk Sac