Alpha‐1‐fetoprotein (AFP) and Germ Cell Tumors: Biological and Histological Correlation
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Abstract:
The level of α1FP has been evaluated in the sera of 155 children aged 1 month to 16 years with germ cell tumors. Abnormal α1FP levels were found in 63 cases. Reexamination of histological slides from more than 120 cases, in a double blind study has revealed a close connection between the presence of abnormal α1FP levels in the serum and the presence of vitelline components on the tissue sections: 54 yolk sac tumor out of 60 are α1FP positive (that is 90% and 100% in the acute phase), whatever the site: testis, ovary, vagina, sacrococcygeal, intra‐ or retro‐peritoneal region, mediastinum or intracranium. Immunohistological localization (immuno‐fluorescence and immuno‐peroxydase) was performed in this study.Keywords:
Alpha-fetoprotein
Polyembryoma in pure form is a rare entity, more often associated with other germ cell tumor components, commonly observed as a part of malignant mixed germ cell tumor with predominant yolk-sac component. We report a case of a 23 year old male having malignant mixed germ cell tumor of testis in which major component was polyembryoma (>65%) and rest were teratomatous and yolk sac component with an elevated level of serum ?-fetoprotein (AFP) and human chorionic gonadotrophin (HCG). He was treated with surgery followed by combination chemotherapy and doing well till now.
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Germ-cell tumors occurring primarily in the mediastinum have been well recognized in the literature for many years. Unfortunately, most of what has been written on these tumors has been in the form of case reports and small series of cases, thus precluding a better understanding of their clinical and demographic features, as well as of their biologic behavior in this particular location. In addition, mediastinal germ-cell tumors not only have never had a standardized nomenclature, but they also have lacked the benefit of a clinical staging scheme for assessment of prognosis. Another important aspect of these tumors that has required attention is the frequency with which teratomatous lesions are associated with malignant components in the mediastinum. Comprehensive studies of mediastinal germ-cell tumors to determine whether these lesions recapitulate the distribution, histopathologic features, and biologic behavior of their counterparts in other areas, such as the gonads, have been lacking for many years. In this article, we review our experience with a large series of cases of primary mediastinal germ cell tumors and examine recent concepts and advances in our understanding of these unusual and diagnostically challenging neoplasms.
Anterior mediastinum
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Plasma α-fetoprotein (AFP) and human chorionic gonadotropin (HCG) concentrations were assayed in patients with intracranial teratomas or germinomas. Plasma AFP was significantly elevated in one patient with a yolk sac tumor, and in four with embryonal carcinomas, while in 14 patients with germinomas, the plasma AFP level was in the normal range. Plasma HCG concentrations were significantly elevated in two patients with embryonal carcinomas and in seven with germinomas. In one patient with a yolk sac tumor, the HCG concentration in CSF was elevated. Pertinent literature was reviewed and it was concluded that AFP is specific to yolk sac tumors and embryonal carcinomas, while HCG is common in all germinal tumors. Blood or CSF AFP and HCG are quite useful for detecting germinal tumors. They are also useful in evaluating the efficacy of the treatment.
Human chorionic gonadotropin
Embryonal carcinoma
Gonadotropin
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Malignant germ cell tumors constitute about 3%-4% of all neoplasms occurring before the age of 15. They arise in the ovaries, the testes, and in several other locations, including the lower back, the chest, the brain, and the abdomen. In infants and young children, the sacrococcygeal region is the most common site for extragonadal germ cell tumors, and teratomas account for the vast majority of sacrococcygeal germ cell tumors. Neonatal sacrococcygeal teratomas are usually benign and rarely they may contain a malignant component that is predominantly a yolk sac tumor. In this article, we describe a rare case of a male newborn with a giant sacrococcygeal mixed germ cell tumor composed of grade 3 immature teratoma and malignant yolk sac elements.
Sacrococcygeal Teratoma
Immature teratoma
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Abstract Background Germ cell tumors prefer gonadal regions, but they can be expected in extragonadal sites such as the anterior mediastinum, which is the commonly involved region after gonads. Amongst germ cell tumors, teratomas are the rarer subtypes which develop in the anterior mediastinum. Case presentation The authors present two adult patients, a male and a female, both complaining of chronic chest pain and cough. Chest CT scans were performed revealing characteristic CT features of mature teratomas in the anterior mediastinum which were extending to the superior mediastinum in both patients. Conclusion Germ cell tumors are involving the mediastinum; however, amongst all germ cell tumors, teratoma is the least common type arising in the mediastinum, and a chest CT scan considered the imaging investigation of choice. Surgery is the best option and patients may have a very good prognosis.
Anterior mediastinum
Presentation (obstetrics)
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Mediastinal teratoma is a rare germ cell tumor. The mediastinum is the most common extragonadal site. Malignant tumors of the mediastinum account for 4 % of all extragonadal tumors of the mediastinum. We present a case of successful surgical treatment of a 24‑year-old man with a gigantic immature mediastinal teratoma complicated by severe cardiorespiratory disorders.
Anterior mediastinum
Mediastinal tumor
Mature teratoma
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Germinoma
Human chorionic gonadotropin
Histology
Mediastinal mass
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Endodermal sinus tumor
Alpha-fetoprotein
Teratocarcinoma
Sinus (botany)
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During the last 6 1/2 years, serum AFP has been determined by radioimmunoassay in 387 patients with germ cell tumors of the gonads and extragonadal sites. The histological appearances of all these neoplasms were carefully reviewed. Highly elevated levels of serum AFP were noted in patients with tumors containing endodermal sinus (yolk sac) tumor elements irrespective of the location of the neoplasm or presence or absence of metastatic disease. There was good correlation between the presence and quantity of endodermal sinus (yolk sac) tumor elements within the primary tumor or its metastases and elevated levels of serum AFP. All patients with tumors composed of pure seminoma or dysgerminoma, and teratoma, had normal serum AFP levels. Slightly elevated levels of serum AFP up to 60 ng/mg (upper limit of normal 20 ng/ml) were noted in a few patients with testicular tumors composed of pure embryonal carcinoma, whereas patients with tumors composed of or containing endodermal sinus (yolk sac) tumor elements had serum AFP levels that could be measured in 100's or 1000's of ng/ml. Serum AFP was elevated only in patients with active disease. Serum AFP was determined in 81 patients with gonadal tumors of non germ cell origin and was normal in all these patients. Serum AFP is a very good tumor marker in patients with germ cell tumors composed of or containing endodermal sinus (yolk sac) tumor, irrespective of their location. Serial serum SFP determinations can be used for diagnostic purposes, for monitoring the results of treatment, and for early detection of metastases and recurrences. Serial serum AFP determination is a useful procedure in all patients with germ cell neoplasms and is highly recommended.
Endodermal sinus tumor
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Yolk sac tumors (also known as primitive endodermal tumors or endodermal sinus tumors) are malignant primitive germ cell tumors. They are histologically similar to the mesenchyme of the primitive yolk sac.Yolk sac tumor is a type of germ cell tumor. Germ cell tumors commonly arise in the gonads but sometimes can occur outside the gonads and are called extragonadal germ cell tumors. The most common testicular tumor in young children under the age of 3 is yolk sac tumor, and it is also known as infantile embryonal carcinoma. Yolk sac tumors have a good prognosis in this age group.Compared to pure type in infants and young children, yolk sac tumors in adults are often found in combination with other types of germ cell tumors (e.g., teratoma and embryonal carcinoma).
Endodermal sinus tumor
Embryonal carcinoma
Mesenchyme
Immature teratoma
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