Primary pineal endodermal sinus tumor with elevated serum and CSF alphafetoprotein levels

1980 ◽  
Vol 53 (2) ◽  
pp. 244-248 ◽  
Author(s):  
Norio Arita ◽  
Shoji Bitoh ◽  
Yukitaka Ushio ◽  
Toru Hayakawa ◽  
Hiroshi Hasegawa ◽  
...  
Keyword(s):  
Therapeutic Response ◽  
Elevated Serum ◽  
Germ Cell Tumors ◽  
Pineal Region ◽  
Endodermal Sinus Tumor ◽  
Immunofluorescence Method ◽  
Content Type ◽  
Intracranial Germ Cell Tumors ◽  
Sinus Tumor ◽  
Sensitive Marker

✓ A 12-year-old girl with an alphafetoprotein-producing brain tumor in the pineal region is presented. The marker was detected in both serum and cerebrospinal fluid. The amount of the marker decreased after irradiation of the tumor and systemic chemotherapy and increased prior to tumor recurrence. At autopsy, the tumor was histopathologically diagnosed as an endodermal sinus tumor, and alphafetoprotein was demonstrated in the tumor cells by the immunofluorescence method. Alphafetoprotein may represent a specific and sensitive marker for predicting tumor histopathology and for monitoring the therapeutic response in patients with primary intracranial germ-cell tumors.

Pathogenesis of intracranial germ cell tumors reconsidered

1999 ◽  
Vol 90 (2) ◽  
pp. 258-264 ◽  
Author(s):  
Keiji Sano
Keyword(s):  
Survival Rate ◽  
Germ Cell ◽  
Survival Rates ◽  
Germ Cell Tumors ◽  
Yolk Sac ◽  
Endodermal Sinus Tumor ◽  
Content Type ◽  
Intracranial Germ Cell Tumors ◽  
Sinus Tumor ◽  
Fine Print

Object. To determine the pathogenesis of intracranial germ cell tumors (GCTs), the author studied 153 cases of these tumors encountered through 1994, 62.7% of which showed monotypic histological patterns and 37.3% of which were shown to be mixed tumors.Methods. Six patients died soon after admission and underwent autopsy; the other patients underwent surgery followed by radio- and/or chemotherapy. One hundred thirty-four cases were followed through the end of 1997. All patients with a choriocarcinoma died within 1 year. Patients with a yolk sac tumor (endodermal sinus tumor) or an embryonal carcinoma also had poor outcomes. Patients with a mature teratoma had 5- and 10-year survival rates of 93% each. Patients with an immature teratoma had 5- and 10-year survival rates of 86% each, whereas patients who had a teratoma with malignant transformation had a 3-year survival rate of 50%. Patients with a germinoma had a 5-year survival rate of 96% and a 10-year survival rate of 93%. These results may bring into question the validity of the germ cell theory because germinoma, which should be the most undifferentiated tumor according to the theory, was the most benign and choriocarcinoma and yolk sac tumor (endodermal sinus tumor), which should be the most differentiated tumors, were the most malignant according to results obtained during the follow-up study.Conclusions. Germ cell tumors other than germinomas may not originate from one single type of cell (primordial germ cells). The embryonic cells of various stages of embryogenesis may perhaps be misplaced in the bilaminar embryonic disc at the time of the primitive streak formation, becoming involved in the stream of lateral mesoderm and carried to the neural plate area to become incorrectly enfolded into the brain at the time of neural tube formation. The author propounds the following hypothesis: tumors composed of cells resembling the cells that appear in the earlier stages of embryogenesis (ontogenesis) are more malignant than those composed of cells resembling the cells that appear in the later stages of embryogenesis.

Intracranial germ-cell tumors

1984 ◽  
Vol 61 (5) ◽  
pp. 931-937 ◽  
Author(s):  
Hirofumi Naganuma ◽  
Hiroshi Inoue ◽  
Shuzo Misumi ◽  
Masaru Nakamura ◽  
Masaru Tamura
Keyword(s):  
Germ Cell ◽  
Chorionic Gonadotropin ◽  
Human Chorionic Gonadotropin ◽  
Embryonal Carcinoma ◽  
Germ Cell Tumors ◽  
Endodermal Sinus Tumor ◽  
Content Type ◽  
First Case ◽  
Intracranial Germ Cell Tumors ◽  
Sinus Tumor

✓ Histological and immunohistochemical findings in three cases with primary intracranial germ-cell tumors are reported. Based on histological examination, they were diagnosed, respectively, as an endodermal sinus tumor with immature teratoid differentiation, teratoma and embryonal carcinoma, and choriocarcinoma and germinoma. The first case was presumed to be a combination of endodermal sinus tumor and germinoma. An immunohistochemical examination showed positive reactions to alpha-fetoprotein (AFP) and carcinoembryonic antigen (CEA) in the endodermal sinus tumor, and to human chorionic gonadotropin (HCG) in the choriocarcinoma, but showed no reaction in the embryonal carcinoma. Human chorionic gonadotropin was demonstrated in syncytiotrophoblastic cells and CEA in a gland-like structure. The value of measuring CEA in cases of germ-cell tumors, in addition to AFP and HCG assays, is stressed, and the characteristics of CEA-positive tissue are discussed.

Intracranial germ-cell tumors in children

1991 ◽  
Vol 74 (4) ◽  
pp. 545-551 ◽  
Author(s):  
Harold J. Hoffman ◽  
Hiroshi Otsubo ◽  
E. Bruce Hendrick ◽  
Robin P. Humphreys ◽  
James M. Drake ◽  
...  
Keyword(s):  
Survival Rate ◽  
Adjuvant Therapy ◽  
Germ Cell ◽  
Germ Cell Tumors ◽  
Pineal Region ◽  
Pineal Tumors ◽  
Content Type ◽  
Suprasellar Region ◽  
Intracranial Germ Cell Tumors ◽  
Sick Children

✓ All patients with confirmed intracranial germ-cell tumors treated at the Hospital of Sick Children during the period January, 1952, to December, 1989, were reviewed. Of the 51 tumors reviewed, 16 were located in the suprasellar region, 32 in the pineal region, and three in both the pineal and the suprasellar regions. Forty-nine patients underwent surgical resection which was total in seven and partial in 20, and consisted of a biopsy in 22. Two patients were managed on the basis of serum and cerebrospinal fluid markers. Surgical tools such as the operating microscope, the ultrasonic surgical aspirator, and the laser beam allowed safe debulking and removal of the deep-seated tumors in the pineal region. There were no operative deaths in the 36 patients treated since 1972, who included 23 with pineal tumors. Twenty-five patients with germinomas received radiotherapy and had a 5-year survival rate of 85.1%. Thirteen patients with non-germinoma germ-cell tumors received radiotherapy and had a 5-year survival rate of 45.5%. On the basis of this review, the authors recommend resection of pineal and suprasellar germ-cell tumors in order to firmly establish an accurate histological diagnosis to guide the extent of adjuvant therapy. In the case of a pure germinoma without evidence of dissemination, adjuvant therapy consists only of local radiotherapy. On the other hand, for malignant non-germinoma germ-cell tumors, adjuvant therapy must include chemotherapy as well as craniospinal axis radiotherapy.

scholarly journals Pathogenesis of intracranial germ cell tumors reconsidered

1998 ◽  
Vol 5 (1) ◽  
pp. E3
Author(s):  
Keiji Sano
Keyword(s):  
Survival Rate ◽  
Germ Cell ◽  
Mature Teratoma ◽  
Germ Cell Tumors ◽  
Yolk Sac ◽  
Yolk Sac Tumor ◽  
Single Type ◽  
Endodermal Sinus Tumor ◽  
Intracranial Germ Cell Tumors ◽  
Sinus Tumor

The author studied 153 cases of intracranial germ cell tumors (GCTs) through 1994, 62.7% of which showed monotypic histological patterns and 37.3% of which were shown to be mixed tumors. All of these cases, except for six patients who died soon after admission and underwent autopsy, underwent surgery followed by radio- and/or chemotherapy. All patients with choriocarcinoma died within 2 years. Patients with yolk sac tumor (endodermal sinus tumor) and embryonal carcinoma also had poor outcomes. Patients with mature teratoma had 5- and 10-year survival rates of 92.9% each. Patients with immature teratoma and malignant teratoma had a 5- and 10-year survival rate of 70.7% each. Patients with germinoma had a 5-year survival rate of 95.4% and a 10-year survival rate of 92.7%. These results may bring into question the validity of the germ cell theory, because germinoma, which should be the most undifferentiated according to the theory, was the most benign and choriocarcinoma and yolk sac tumor (endodermal sinus tumor), which should be the most differentiated, were the most malignant according to results obtained during follow-up study. Therefore, GCTs other than germinoma may not originate from one single type of cell (primordial germ cells). The embryonic cells of various stages of embryogenesis may perhaps be misplaced in the bilaminar embryonic disc at the time of the primitive streak formation, becoming involved in the stream of lateral mesoderm and carried to the future cranial area to become incorrectly enfolded into the brain at the time of the neural tube formation. The authors propound the following law: tumors composed of cells resembling the cells that appear in the earlier stages of embryogenesis (ontogenesis) are more malignant than those composed of cells resembling the cells that appear in the later stages of embryogenesis.

Primary intracranial endodermal sinus tumor

1979 ◽  
Vol 50 (2) ◽  
pp. 246-252 ◽  
Author(s):  
Thomas J. Eberts ◽  
Robert C. Ransburg
Keyword(s):  
Pineal Region ◽  
Yolk Sac ◽  
Endodermal Sinus Tumor ◽  
Cranial Cavity ◽  
Content Type ◽  
Suprasellar Region ◽  
Sinus Tumor ◽  
Fine Print

✓ Endodermal sinus tumor (yolk sac carcinoma), a germinal neoplasm, is rarely primary in the cranial cavity. The authors add a third case with the tumor located in the anterior third ventricular (suprasellar) region to the literature. Seventeen previously reported intracranial cases, mostly in the pineal region, are briefly reviewed.

Precocious puberty in a girl with an hCG-secreting suprasellar immature teratoma

1994 ◽  
Vol 81 (4) ◽  
pp. 601-604 ◽  
Author(s):  
Chifumi Kitanaka ◽  
Masao Matsutani ◽  
Shigeo Sora ◽  
Sachiko Kitanaka ◽  
Ayako Tanae ◽  
...  
Keyword(s):  
Brain Tumors ◽  
Precocious Puberty ◽  
Germ Cell Tumors ◽  
Immature Teratoma ◽  
Content Type ◽  
Intracranial Germ Cell Tumors ◽  
Hcg Secretion ◽  
High Level ◽  
Very High ◽  
Fine Print

✓ Although precocious puberty is common in boys with human chorionic gonadotropin (hCG)-secreting brain tumors, it is extremely rare in girls. The authors describe a 6-year-old girl with an hCG-secreting suprasellar immature teratoma who presented with diabetes insipidus, increased intracranial pressure, and precocious puberty. On admission, breast budding was observed. The serum hCG level was 1230 mIU/ml. Both luteinizing hormone (LH) and follicle-stimulating hormone (FSH) remained below detectable levels, even after gonadotropin-releasing hormone stimulation. Serum estrogen and androgen were moderately elevated. After chemotherapy, breast budding disappeared with normalization of serum hCG. It has been believed that hCG does not produce precocious puberty in girls in the absence of FSH, and this has been used as an explanation for the rarity of precocious puberty in girls with hCG-secreting brain tumors. However, it has also been reported that hCG has not only LH activity but also intrinsic, although weak, FSH-like activity. In the present case, this FSH-like activity was considered to have played a role in the development of precocious puberty. It is speculated that a very high level of serum hCG can produce precocious puberty in girls. The rarity of intracranial germ-cell tumors with a high potential of hCG secretion may be one of the reasons why hCG-induced precocious puberty is uncommon in girls.

scholarly journals Blood to cerebrospinal fluid human chorionic gonadotropin-beta ratios in intracranial germ cell tumors

1998 ◽  
Vol 5 (1) ◽  
pp. E6 ◽  
Author(s):  
Paul B. Rogers ◽  
Eliot C. Sims ◽  
Nicholas Plowman
Keyword(s):  
Cerebrospinal Fluid ◽  
Germ Cell ◽  
Chorionic Gonadotropin ◽  
Human Chorionic Gonadotropin ◽  
Germ Cell Tumors ◽  
Intrathecal Administration ◽  
Pineal Region ◽  
Elevated Blood ◽  
Intracranial Germ Cell Tumors ◽  
Human Chorionic Gonadotropin Beta

Levels of human chorionic gonadotropin-beta (HCG-beta) are elevated in up to 43% of patients with intracranial germ cell tumors (GCTs) and are useful in the diagnosis of these tumors and the follow up of such patients. The ratio of blood HCG-beta to lumbar cerebrospinal fluid (CSF) HCG-beta in these patients at presentation has not been defined. Twenty-two patients with intracranial GCTs have been treated at St. Bartholomew's Hospital over the past 15 years. Two (17%) of 12 germinomas and seven (70%) of 10 nongerminomatous GCTs had elevated blood HCG-beta at presentation. Four cases of pineal region GCTs (one of 12 germinomas and three of 10 nongerminomatous GCTs) had paired, elevated, blood and lumbar CSF HCG-beta levels. The mean blood to CSF ratio was 1:10 (range 1.7-18.4), which is substantially lower than the ratio of 286:1 reported in systemic GCTs. The authors confirm the finding of a previous single report showing that ventricular CSF HCG-beta sampling via an accessible ventriculoperitoneal shunt reservoir may give a spuriously negative result, and they discuss the pathophysiology of the blood-brain barrier in the pineal region and the implications of the intrathecal administration of chemotherapy.

Diagnosis and management of pineal tumors

1983 ◽  
Vol 58 (5) ◽  
pp. 654-665 ◽  
Author(s):  
Rashid Jooma ◽  
Brian E. Kendall
Keyword(s):  
Tumor Markers ◽  
Germ Cell Tumors ◽  
Ct Scanning ◽  
Individual Case ◽  
Pineal Region ◽  
Pineal Tumors ◽  
Content Type ◽  
Pineal Region Tumors ◽  
Primary Surgery ◽  
Fine Print

✓ The management of pineal region tumors remains controversial. Advocates of a conservative approach emphasize the excellent results of radiotherapy, particularly with germinomas, while a number of recent reports have demonstrated the safety of direct surgery. In order to improve treatment planning, attempts have been made to distinguish the various tumor types by computerized tomography (CT) and by the use of markers for germ-cell tumors. This paper reports a study of 35 patients with pineal tumors including two ectopic germinomas. Pretreatment CT was performed in all 35 patients, and human chorionic gonadotropin and alpha-fetoprotein levels were assayed in 11. Histological verification was available for 33 tumors; the other two were characterized by clinical and CT features plus tumor markers. Of the 27 tumor operations, 21 were for a pineal mass and six for ectopic germinoma or metastasis. There was no operative mortality. Morbidity was minimal, and surgery did not increase the incidence of subarachnoid seeding. Each histological type of tumor has a typical appearance on CT scanning, although, in the individual case, a firm diagnosis is not always possible. However, consideration of CT scans together with the clinical features, cerebrospinal fluid cytology, tumor markers, and (if there is still doubt) response to a small dose of irradiation will generally allow a specific diagnosis with a high degree of probability. In this way, germinomas may be selected for radiotherapy and the tumors less likely to respond may be subjected to primary surgery.

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