Primary intracranial yolk sac tumor: immunofluorescent demonstration of alpha-fetoprotein synthesis.

An autopsy case of 20-year-old male with primary intracranial yolk sac tumor (endodermal sinus tumor) is reported. Whereas the biopsy specimen obtained from the pineal region showed diffuse proliferation of atypical tumor cells, the metastatic subdural tumor removed from lumbar spinal region had the characteristic histologic appearance of yolk sac tumor. The histologic diagnosis was intracranial yolk sac tumor originating in the pineal gland. The elevated amount of alpha-fetoprotein in the cerebrospinal fluid and in the serum further supported the diagnosis. At autopsy, only metastatic tumor was present in the posterior fossa. The immunofluorescence study demonstrated the presence of intra- and extracellular alpha-fetoprotein globules in the tumor tissue. The intra- and extracellular distribution of alpha-fetoprotein, in general, appeared to coincide with that of the PAS-positive hyaline globules in the tumor.     

Chemotherapy of human yolk sac tumor heterotransplanted in nude mice

The chemotherapeutic effects of cis-diamminedichloroplatinum + vinblastine + bleomycin (PVB) on 3 human yolk sac tumors (YST-1, YST-2, and YST-3) of the ovary, which were heterotransplanted into BALB/c nude mice, were compared with the effects of vincristine + actinomycin D + cyclophosphamide (VAC), the combination currently favored for treatment of yolk sac tumors. PVB and VAC therapies were performed for 3 weeks. Both PVB and VAC significantly reduced the tumor volume of all the treated tumors. The mean weights of tumors in animals treated with PVB or VAC were, in percent of the mean tumor weight in untreated animals: 1.3 and 1.6 for YST-1, 2.5 and 3.3 for YST-2, and 5.5 and 2.7 for YST-3, respectively. A strong correlation was noted between tumor volume and alpha-fetoprotein level in the sera of mice bearing YST-1 or TST-2 tumors.     

A study on the lectin reactivity of alpha-fetoprotein produced by hepatoid adenocarcinomas and yolk sac tumors.

The carbohydrate structure of glycoproteins is considered to be tissue-specific or cell type-specific, but there have been no reports on the differences of the carbohydrate structure of alpha-fetoproteins (AFPs) produced by histologically identical tumors in different tissues. The lectin affinity electrophoresis of hepatoid adenocarcinomas and yolk sac tumors from different organs suggested that either the tumor heterogeneity or the tissue specificity is possibly involved, the lectin reactivity of the AFP sugar chain structure produced by the tumors in different tissues.     

Mediastinal yolk sac tumor in a woman

Primary endodermal sinus tumor (yolk sac tumor) of the mediastinum is uncommon and most patients are young and male. We report a yolk sac tumor with a mature teratoma of the anterior mediastinum in a 28-year-old woman with an intrathoracic mass. Four courses of combination chemotherapy were given and the tumor was resected. The patient's serum alpha-fetoprotein (AFP) level was elevated, to more 100 000 ng/ml. To the best of our knowledge, this is the first report of serum AFP level exceeding 100 000 ng/ml in a yolk sac tumor with a mature teratoma.     

Primary yolk sac tumor of the rectum

Extragonadal germ cell tumors are well recognized in men but have rarely been reported in women. Reports have Primarily, focused on the pediatric population and have suggested a poor prognosis for extragonadal yolk sac tumors. A 23-year-old woman with a yolk sac tumor arising in the rectum is described. A review of the English-language literature (MEDLINE 1966-1998) regarding extragonadal germ cell tumors in females is provided. Treatment with, four courses of cisplntin. etopo-side, and bleomycin was followed by surgical resection of the involved area. No residual tumor was identified. She remains disease free 3.5 years later. Previous reports are limited by the small number of patients, focus on the pediatric population, and treatment before the availability. of cisplatin. Extragonadal germ cell tumors in women are extremely rare but can be successfully treated with aggressive chemotherapy and surgery similar to testis cancer.     

Intrapericardial yolk sac tumor in an infant girl

This article is a report on the first known case of an intrapericardial yolk sac (endodermal sinus) tumor discovered in a 14-month-old girl. The patient presented with a systolic murmur, cardiomegaly, and manifestations of cardiac failure. At surgery a large, friable, intrapericardial mass with no evidence of extrapericardial spread was found. Histologic and immunohistochemical findings were diagnostic of yolk sac tumor. Following initial resection, chemotherapy, and a second resection 13 months after the first, the patient died 24 months after diagnosis. Tumor enlargement was associated with elevated serum alpha-fetoprotein levels.     

卵黄囊瘤的CT及MRI表现

目的:探讨卵黄囊瘤CT及MRI的表现及在诊断中的应用价值。方法:回顾性分析2005年1月至2015年5月经病理证实的40例卵黄囊瘤的CT或MRI表现及临床特征。结果:发病年龄9个月~50岁,平均年龄12.7岁;其中男17例,女23例。肿瘤位于卵巢18例,睾丸12例,骶尾部6例,阴道2例,膈肌1例,腹股沟1例。影像学表现为类圆形或不规则形肿块,呈实性或囊实混合性,肿瘤最大径1.5~28cm。增强扫描实性部分明显不均匀强化,实性部分及囊壁内可见迂曲血管影。结论:卵黄囊瘤在CT及MRI存在特征性的影像学表现,结合发病年龄、AFP升高等临床特征,可以提高对该病的鉴别诊断。     

Structure of the sugar chain of alphafetoprotein purified from a human yolk sac tumor and its reactivity with concanavalin A

We have attempted to determine the carbohydrate moiety of human alphafetoprotein (AFP) produced by a yolk sac tumor. AFP was obtained from the cystic fluid of human yolk sac tumors grown in nude mice and was purified using an immunoadsorbent column coupled with monoclonal anti-AFP antibody. Then, the carbohydrate chain of the purified AFP was quantitatively released from the polypeptide chain. The resulting oligosaccharide was labeled and, by sequential exoglycosidase digestion in combination with methylation analysis and periodate oxidation, the structure was determined to be: Sia alpha 2----6Gal beta 1----4GlcNAc beta 1----2Man alpha 1----6 (GlcNAc beta 1----4) (Sia alpha 2----6Gal beta 1----4GlcNAc beta 1----2Man alpha 1----3) Man beta 1----4GlcNAc beta 1----4 (Fuc alpha 1----6) GlcNAcOT Compared with the known structure of the sugar, chain of human hepatic AFP, it was found that the sugar chain of yolk sac AFP contained an additional sugar, N-acetylglucosamine (bisect GlcNAc) linked to the beta-mannose. In the light of recent knowledge, this result indicates that the Concanavalin A (Con-A) binding site of the sugar chain is blocked by this GlcNAc in human yolk sac AFP. This fact forms the basis for the clinical use of the Con-A binding test to determine the origin of AFP in patients.     

Primary chemotherapy in strongly suspected yolk sac tumor of the ovary

We report a case of a right ovarian tumor measuring 10.8 × 10 × 8.5 cm (diagnosed as a yolk sac tumor) in a 31-year-old nulligravid woman with serum α-fetoprotein (AFP) level of 25 000 ng/ml and pleural effusion. Seven courses of primary chemotherapy (PVB; cisplatin, vinblastine, bleomycin) were given from May 16 to November 11, 1994. No surgical procedures were conducted. After the second course of PVB, the pleural effusion resolved; after the third course, the tumor disappeared, and after the fourth course, serum AFP decreased to the normal range. At the true of writing (May 11, 1998), the patient is in good health without recurrence, with an AFP level below 20 ng/ml. This paper reports, for the first time, a patient with yolk sac tumor in whom remission was achieved by primary chemotherapy alone. Received: September 3, 1997 / Accepted: June 3, 1998     

Combined hepatoblastoma and yolk sac tumor of the liver.

The authors report a liver tumor that occurred in a 6-month-old boy in which areas of yolk sac and hepatoblastoma were identified. To the best knowledge of the authors, this morphologic pattern has not been reported previously. Theories of histogenesis are discussed.     

Production of 13 plasma proteins by human testicular yolk sac tumor transplanted into nude mice

Thirteen human proteins were found in plasma and cystic fluid of mice bearing human testicular yolk sac tumor. Six of them, alpha-fetoprotein, prealbumin, albumin, alpha 1-antitrypsin, hemopexin and transferrin, had been previously demonstrated to be produced by yolk sac tumors. The syntheses of the remaining seven, namely apolipoprotein A1, retinol-binding protein, alpha 2HS-glycoprotein, haptoglobin, apolipoprotein B, C5 component and anti-hemophilic factor, have been demonstrated for the first time.     

Serum lactate dehydrogenase isoenzyme-1 in children with yolk sac tumor

Although in recent years the evidence of an increase in serum lactate dehydrogenase isoenzyme-1 (LDH-1) in patients with germ cell tumor (GCT) has attracted attention, there have only been a few reports concerning yolk sac tumor (YST), which is a frequent type of GCT in childhood. Serum LDH isoenzymes were determined in eight children with YST, and an increase in LDH-1 was found in seven of them, excluding one in an early stage. On the basis of the findings of serial serum LDH-1 levels during treatment and of LDH isoenzyme pattern in tumor tissues, it is presumed that the increased serum LDH-1 is derived from tumor tissues. LDH-1 seems to be useful as a tumor marker, not specific for YST but associated with the whole spectrum of GCT, for monitoring of YST.     

Ovarian Sertoli-Leydig cell tumor,endometrioid-like yolk sac tumor,and Y-chromosomal material

Sertoli-Leydig cell tumors (SLCTs) are rare neoplasms, accounting for less than 0.2% of ovarian tumors. The endometrioid-like variant of yolk sac tumor (YST) is very rare, and the most extensive series reported only 8 cases. We present a case of ovarian SLCT with endometrioid-like YST in a patient with a 46,XX karyotype with Y-chromosomal material. A 26-year-old woman had undergone a right salpingo-oophorectomy for SLCT with endometrioid-like YST. Chromosomal analysis revealed a 46,XX karyotype with Y-chromosomal material insertion into chromosome 1. The patient's father and sister, and 7 other paternal relatives (4 male and 3 female) presented the same chromosome variant without evidence of cancer. The YST component relapsed to the right side of the uterine wall and then metastasized to the peritoneum and liver, while SLCT was eradicated with primary surgery. Several chemotherapeutic regimens were totally ineffective to control tumor progression. She died of disease progression 54 months after the diagnosis. We adopted the policy of a close surveillance for ovarian neoplasms for the 22-year-old sister of the patient, who presented the same Y-chromosomal material in her karyotype. In very rare tumors, new methods, based on molecular and cytogenetic models, are requested to define recommended management.     

Transformation of ovarian yolk sac tumor to immature teratoma following chemotherapy

The patient, a 28-year-old woman, in her ninth week of pregnancy, was operated on for stage Ia, mixed germ cell tumor (grade 3 immature teratoma + yolk sac tumor) of AFP decreased to the normal level. Eight months later, an intrapelvic mass and raised AFP were found. The extirpated recurrent tumor in the pouch of Douglas was a grade 2 immature teratoma with no yolk sac element. FAM chemotherapy was given again, and the patient is alive and well after taking oral UFT. As in testicular germ cell tumors, ovarian germ cell tumors can be converted to a more differentiated tumor following chemotherapy.     

Human yolk sac tumor of the thymus heterotransplanted into nude mice

A human yolk sac tumor of the thymus (YSK-1) was transplanted into athymic nude mice. Histological and ultrastructural investigations revealed that the YSK-1 tumor, even after serial transplantation in nude mice, characteristically displayed Schiller-Duval bodies, endodermal sinus structures and ultrastructural profiles, numerous microvilli, desmosome-like cell attachments, annulate lamellar structures, many lipid droplets and interspersed glycogen particles. Serological study by radioimmunoassay demonstrated AFP, CEA, HCG, alpha 1-AT and transferrin in the sera and cyst fluid of the tumor-bearing nude mice. Immunohistochemical investigation using the PAP method showed that the YSK-1 cells produced CEA, HCG, alpha 1-AT, transferrin as well as AFP. The increase in the level of AFP paralleled the increase in tumor size.     

Pure gastric yolk sac tumor that was diagnosed after curative resection

We recently experienced an 87-yr-old man with gastric yolk sac tumor. Preoperative diagnosis was poorly differentiated adenocarcinoma in the cardia of stomach without apparent metastasis. A total gastrectomy was performed. The precise histological examination after surgery revealed the tumor was composed of pure gastric yolk sac tumor without adenocarcinomatous components. The surgical margin and the resected lymph nodes were histologically negative for the tumor and a curative resection was performed. Five months after the operation, enlargement of the intraabdominal lymph nodes occurred with elevation of serum alpha fetoprotein (AFP), and the patient died 2 mo later. Gastric yolk sac tumors are very rare, and only six cases of gastric yolk sac tumors have been previously reported in the literature. Five out of six cases are accompanied by components of adenocarcinoma, and our present case is the second report of pure gastric yolk sac tumor to the best of our knowledge.     

Chemotherapy for primary retroperitoneal yolk sac tumor: report of a case

A case of primary extragonadal yolk sac tumor occurring in the retroperitoneum of a young adult male was studied. The chemotherapy of this tumor has not previously been described for cases of extragonadal origin. A combination of cyclophosphamide, vinblastine, bleomycin, cis-diamminedichloroplatinum, actinomycin-D, and chlorambucil was used. A partial response and dramatic prolongation of survival was achieved, compared with previously reported cases.