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PubMed2001

Adult testicular pure yolk sac tumor.

Here we describe the clinical, ultrasonographic and histological features of a rare pure adult yolk sac tumor detected in the right testis of a 44-year-old male. Due to the rarity of this neoplasm (less than 10 cases have been reported), there is no unanimous consensus for therapy following inguinal orchiectomy. We believe that nerve-sparing retroperitoneal lymph node dissection could be potentially curative and useful for future interpretations of this tumor's potential evolution.

Adult↗
PubMed1997

The familial occurrence of brain tumors.

This is a report of two parent-child families which developed intracranial neoplasm. There is no known history of brain tumor in other family members. Two instances of familial brain tumors are reported which are different from those seen in phakomatosis. Family one had posterior fossa tumors including astrocytoma and hemangioblastoma. The second family had endocrine-associated tumors consisting of pituitary adenoma and yolk sac tumor.

Adenoma↗
PubMed1999

Ovarian endometrioid-like yolk sac tumor treated by surgery alone, with recurrence at 12 years.

We describe the case of a stage Ia endometrioid-like yolk sac tumor (YST) of the ovary, which was originally misdiagnosed as a malignant struma ovarii and not treated with adjuvant chemotherapy. After 12 years, a contralateral dermoid cyst was excised along with a small omental nodule of partially necrotic and calcified endometrioid-like YST. No tumor was detected in several other biopsy specimens, and a peritoneal lavage was negative for tumor cells. Since there was no evidence of remaining tumor and the serum alpha-fetoprotein (AFP) level was normal after the second operation, the patient was followed. Serial serum AFP levels remained normal for 4 months. At a second-look laparotomy after 4 months, a small tumor nodule was removed from the cul-de-sac. Postoperatively, the patient received three cycles of BEP chemotherapy. The long disease-free interval after the first operation in spite of the presence of occult spread to the omentum and to the pouch of Douglas in this case indicates that some endometrioid-like YSTs may have an indolent course. The present case underscores the importance of careful surgical staging and of long-term follow-up in cases of primitive germ cell tumors of the ovary.

Adult↗
PubMed1994

Primary cerebellar yolk sac tumor: case report.

A rare case of cerebellar yolk sac tumor is described. A 4-year-old boy was admitted for the treatment of a cerebellar tumor and, following total removal of the tumor, he was treated with combination chemotherapy consisting of cisplatin, vinblastine, and bleomycin. He died 18 months after the primary diagnosis due to tumor recurrence. Serum alpha-fetoprotein level was well correlated with the clinical course and the amount of the tumor mass in neuroimaging.

Cerebellar Neoplasms↗
PubMed2005

Novel human alpha-fetoprotein mRNA isoform lacking exon 1 identified in ovarian yolk sac tumor.

OBJECTIVE: Alpha-fetoprotein (AFP) is a major fetal serum protein, the biologic role of which has not been not fully elucidated. Recently, existence of a novel AFP mRNA isoform (del.1 AFP mRNA isoform), which is transcribed from the intron A (the intron between exons 1 and 2), has been reported in murine yolk sac and fetal liver. In the present study, we intended to identify the human homologue of the murine AFP mRNA isoform in the yolk sac tumor. METHODS: To investigate the existence of the mRNA isoform (which we termed the "AFP-C mRNA isoform"), reverse transcription-polymerase chain reaction (RT-PCR) was used. Moreover, the expression analysis of the AFP-C cDNA isoform using the AFP-negative human cell line was carried out. RESULTS: RT-PCR revealed the existence of the AFP-C mRNA isoform in the yolk sac tumor and human hepatocellular carcinoma cells. The expression analysis clarified that the molecular size of the AFP-C was approximately 65 kd, and that the protein was not secreted, in contrast to the traditional AFP. CONCLUSION: From these results, the existence of the AFP-C mRNA isoform has been demonstrated for the first time in humans. The AFP-C located in cytoplasm possibly plays physiologic/pathogenic roles distinct from those of the traditional AFP in the yolk sac tumor and hepatocellular carcinoma.

Adenocarcinoma↗
PubMed1999

Intracardiac yolk sac tumor in an infant girl.

We report about a two and a half year old girl with a primary yolk sac tumor (YST) of the heart, located in the interventricular septum. The girl was admitted to the hospital with a systolic cardiac murmur. Echocardiography revealed a smoothly surfaced tumor which based on the interventricular septum below the aortic valve and filled out more than half of the left ventricular cavity. Diagnosis of a yolk sac tumor was established on a frozen section of a biopsy taken at surgery and confirmed by the examination of the resected material. Excessively elevated serum alpha-fetoprotein (AFP) levels contributed to this diagnosis. Follow up examinations of the patient including computed tomography (CT) of head, thorax and abdomen, did not indicate an extracardiac tumor manifestation. We found no previous report of a primary intracardial yolk sac tumor in the literature.

Child, Preschool↗
PubMed1998

[Treatment of malignant germ cell tumors of the ovary: a clinical analysis of 233 cases].

OBJECTIVE: To evaluate the treatment methods of malignant germ cell tumors of the ovary. METHODS: Two hundred thirty three patients with malignant germ cell tumors of the ovary treated during 1958 through 1993 were retrospectively analyzed. The number of patients in different stage of the disease was as follows: stage I, 94; stage II-IV, 43; recurrence or metastasis, 96. Unilateral salpingo-oophorectomy was performed in 78 patients, or total abdominal hysterectomy and bilateral salpingo-oophorectomy with or without omentectomy and appendectomy was performed in 151 cases, and biopsy in 4 cases. Operation alone was done in 17 cases, operation plus radiotherapy with or without chemotherapy in 65, operation plus chemotherapy in 151 cases. RESULTS: Of the 233 patients 127 were disease-free. The number of survivors in stage I patients was 78, in stage II-IV was 17. There were 32 cases with recurrence and metastasis. The overall 5-year survival rate was 55.6%. The 5-year survival rate differed between dysgerminoma and nondysgerminomatous tumurs being 84.2% for the former and 44.6% for the latter. In patients with nondysgerminomatous tumors, chemotherapy with PVB and BEP regimens gave a 5-year survival rate of 66.0% and 73.3%, respectively. CONCLUSION: Malignant germ cell tumors of the ovary is very sensitive to chemotherapy. The PVB and BEP regimens result in excellent response in these patients. Complete remission and preservation of fertility can be obtained in patients with early tumors. Preoperative chemotherapy and postoperative adjuvant chemotherapy can improve survival in patients with advanced tumors.

Adolescent↗
PubMed2001

Morphologic features of human chorionic gonadotropin- or alpha-fetoprotein-producing germ cell tumors of the central nervous system: histological heterogeneity and surgical meaning.

Our study of germ cell tumors (GCT) of the central nervous system (CNS) investigated the relationship between tumor histology and patient serum titers of human chorionic gonadotropin (HGC) and alpha-fetoprotein (AFP). Thirty-five patients were enrolled. Their serum titers of HCG (mlU/ml) and/or AFP (ng/ml) before initial treatment were available, as were tumor specimens obtained before the administration of adjuvant therapy. They were divided into three groups, depending on whether HCG alone (group H), AFP alone (group A), or both HCG and AFP (group HA) were detected. Each group was subdivided into three groups: patients in group I had H, A, and/or HA titers below 9.9; patients in group II/III had titers from 10.0 to 999; and those in group IV had titers of 1000 or more. Serial sections of tissue specimens were repeatedly stained, mainly with hematoxylin and eosin (H-E) stain, HCG immunostain, and AFP immunostain. There were seven patients in the H-I group and five in H-II/III. Of these 12 patients, 11 had germinomas (G) and one had an embryonal carcinoma (EC). Five patients were included in group A: one was classified as A-II/III and had a germinoma, and the remaining four patients were in A-IV and had yolk sac tumors (YST) or mixed GCT consisting mainly of YST or EC (MXGCT-YST, EC). The HA group consisted of 18 patients. Three were classified as HA-I and had germinomas; nine HA-II/III patients had T or MXGCT-T; and six HA-IV patients had choriocarcinoma (CC), YST, MXGCT-CC, or MXGCT-YST. Throughout the study, the situations for the elevated serum titers could be elucidated in only four cases (three in group A-IV and one in group HA-IV). These results led to the conclusion that serologic evaluation is superior to morphologic evaluation in diagnosing marker-producing GCTs. From a diagnostic perspective, the role of surgery is to verify the HCG- and AFP-immunonegative tissue in patients with G, T, and EC.

Adolescent↗
PubMedDate not supplied

Intracranial germ cell tumors in children with and without Down syndrome.

PURPOSE: Two Chinese children with Down syndrome affected by intracranial germ cell tumors are described. Because they represent two of eight affected patients in the current series from 1990 to 1996, it is postulated that such occurrence may be more than a coincidental event. PATIENTS AND METHODS: Two children with Down syndrome developed germ cell tumors in atypical intracranial sites that affected basal ganglion and cerebellum. The pathology showed germinoma and yolk sac tumor, respectively. These were treated by radical surgical resection and chemotherapy with cisplatin, etoposide, and bleomycin, but without radiotherapy. RESULTS: One patient survived 3 years without radiologic evidence of tumor. The other died from infective complications caused by severe myelosuppression after chemotherapy. CONCLUSIONS: Subtle neurologic manifestations in developmentally handicapped patients with intracranial space-occupying lesions could result in delayed diagnosis. Children with Down syndrome suffering from brain tumors may have a higher chance for germ cell tumors. Assay for alpha-fetoprotein and beta-human chorionic gonadotrophin could hasten diagnosis in some cases. This observation and review of literature suggest an increased risk of developing intracranial germ cell tumors in subjects with Down syndrome.

Antineoplastic Combined Chemotherapy Protocols↗
PubMedDate not supplied

Pathology and molecular biology of teratomas in childhood and adolescence.

The biologic behaviour of teratomas depends on various interdependent clinical and epidemiologic variables such as the age at diagnosis, sex, tumor site, histology which all correlate to different cytogenetic and molecular biologic aberrations. Thus, testicular teratomas of infancy are generally benign. Accordingly, prepubertal teratomas show no cytogenetic or molecular genetic aberrations. In contrast, postpubertal testicular teratomas can present as clinically malignant tumors and may show complex cytogenetic aberrations such as the isochromosome 12p, which is pathognomonic of malignant germ cell tumors. Notably, teratomas of both age groups show an at least partial erasure of the genomic imprinting, correlating with their origin from primordial germ cells. The Kiel Pediatric Tumor Registry includes 541 teratoma specimens, and among these, the most frequent tumor sites (in descending order) are: the sacrococcygeal region (33.8 %), the ovaries (31.2 %) and the testes (10.5 %). Rare localizations include the mediastinum, the retroperitoneum, the head and neck region as well as the central nervous system. The WHO classification of germ cell tumors distinguishes mature and immature teratomas as well as teratomas with malignant transformation. In immature teratomas, primitive neuroectodermal structures predominate. According to the grading system (Gonzalez-Crussi, 1982), mature teratomas (G0) are more frequent (54.5 %) than immature teratomas (G1-G3, 45.5 %). Only 7.8 % of all teratomas show the highest grade of immaturity (G3). The frequency of additional microscopic foci of malignant yolk sac tumor correlates with the grade of immaturity. In sacrococcygeal teratomas, the yolk sac tumor microfoci may give rise to a malignant relapse after incomplete resection. The rare teratomas with malignant transformation contain components with "conventional" somatic type malignancy such as leukaemia, carcinoma or sarcoma. Here, molecular genetic analysis has demonstrated the origin of the somatic malignancy from a malignant transformation within the germ cell tumor with retention of the cytogenetic changes characteristic of malignant germ cell tumors.

Adolescent↗
PubMed1993

Establishment and characterization of a rat yolk sac tumor cell line, NMT-1, producing alpha-fetoprotein, with potential for lymphatic metastasis.

A new cell line producing alpha-fetoprotein, designated NMT-1, was established from rat yolk sac tumor with the potential for lymphatic metastasis in inbred Wistar rats. In this paper, we investigated the characteristics of NMT-1 cells. The cell line grew in a monolayer and had a polygonal epithelioid appearance in phase-contrast microscopy. The doubling time of NMT-1 cells during exponential growth was approximately 16 h in RPMI-1640 with 10% fetal calf serum. The D0 value for radiation sensitivity was 97 +/- 3 cGy. The extrapolation number, n, for NMT-1 was 1.08 +/- 0.15. In the log phase, the G0/G1, S and G2/M fractions were 23.2%, 62.1% and 14.3%, respectively. Tumor take was observed in all of the rats inoculated with NMT-1 cells. In the case of flank tumor, the mean survival time was 104 days after inoculation with 10(6) tumor cells. Inguinal, axillary, paraaortic, mesenteric and mediastinal lymphnode metastases were observed in all rats inoculated with NMT-1 cells. Rats which survived for a long time developed metastases in a lymphatic vessels of the liver, lung and/or kidney microscopically. The biological behavior and the histopathological features of the tumor induced by inoculation with NMT-1 cells were the same as those of the original tumor induced by fetectomy.

Animals↗
PubMed2003

[Retroperitoneal germ cell tumor with testicular calcification indicating tiny testicular origin: consideration of the origin of retroperitoneal germ cell tumors: report of two cases].

Two cases of germ cell neoplasm retrospectively considered to have been of testicular origin are reported. Case 1. A 19-year-old male with brain, liver and retroperitoneal tumors was diagnosed with yolk sac tumor by retroperitoneal tumor biopsy. After multidisciplinary treatment, a region of calcification was detected in the left testis on scrotal sonography and left high inguinal orchiectomy was performed. Case 2. A 57-year-old male with neck, lung and retroperitoneal tumors was diagnosed with yolk sac tumor by supraclavicular biopsy. From initial examination, scrotal sonography revealed a small calcified lesion in the right testis. After chemotherapy, high inguinal orchiectomy and retroperitoneal lymphadenectomy were simultaneously performed. Pathologic evaluation of these testicular specimens revealed calcification and a fibrous scar in correspondence with the clinical diagnosis. These changes were considered as scars of the primary testicular tumor due to burned-out tumor or the result of reaction to chemotherapy. Since a primary tumor of testicular origin may exist in the extragonadal germ cell tumor, it is important to examine the intrascrotal contents in detail in the case of so-called extragonadal germ cell tumors with palpably normal testes. In such cases, there are two possible conditions, an occult testicular tumor and a burned-out testicular tumor. We briefly reviewed 42 such cases in the Japanese literature. It appears that there are very few true extragonadal germ cell tumors, and that the possibility of primary testicular origin metastasizing from viable occult testicular tumor or burned-out testicular tumor with spontaneous regression is high in retroperitoneal germ cell tumors.

Adult↗
PubMed2002

Fine-needle aspiration cytology of unusual germ cell tumors of the mediastinum: atypical seminoma and parietal yolk sac tumor.

A 30-yr-old man presented with a mediastinal germ cell tumor that combined the histologic pattern of seminoma with the immunohistochemical profile of embryonal carcinoma (beta-HCG+/PLAP-). In FNA smears, this atypical seminoma presented as fragile large cells with scanty cytoplasm, vesicular nuclei, irregular and indistinct nucleoli, scattered singly and in loosely cohesive fragments without the characteristic lymphocytic or tigroid background. The cytologic features were more suggestive of a poorly differentiated carcinoma than seminoma, potentially leading to misdiagnosis and mismanagement. The second case was a 51-yr-old female smoker who presented with mediastinal parietal yolk sac tumor with extension to the lung, a rare occurrence, and contrary to the clinical impression of lung cancer with hilar lymph node metastasis. This case illustrates the value of using the unique cytologic features of parietal yolk sac tumor, i.e., the abundant, viscous, stringy metachromatic extracellular hyaline material associated with the tumor cells in reaching the accurate diagnosis.

Adult↗
PubMed2006

Metastasizing testicular germ-cell tumor with infiltration of the right heart: indication for primary metastasectomy.

Cardiac intracavitary metastases are very uncommon. The case of a 42-year-old male patient with a testicular germ cell tumor extending into the superior caval vein, the left brachiocephalic vein, and the right heart, which manifested as a mild form of pulmonary embolization, is presented. Due to the perceived high risk of continuous embolization and the urgent need to begin systemic chemotherapy, a complete cardiac tumor resection was performed, utilizing a cardiopulmonary bypass, followed by a simultaneous orchiectomy. Histology revealed a 61-cm long vascular tumor as a metastasis of a yolk sac tumor originating from the left testis. There were no postoperative complications, and the patient is alive and without tumor recurrence 12 months after four cycles of systemic chemotherapy according to the PEB (cisplatin, etoposide, bleomycin) scheme. We conclude that in this special case aggressive surgical management following chemotherapy was very effective in controlling the disseminated testicular tumor.

Adult↗
PubMed2001

Primary retroperitoneal pure yolk-sac tumor in an adult male.

Pure extragonadal tumors are rare in adult males. A 24-year-old male with an extremely high level of serum alpha-fetoprotein (32.795 ng/ml) was diagnosed by abdominal computed tomography as having a retroperitoneal tumor. A case of primary retroperitoneal pure yolk-sac tumor in an adult male is described.

Adult↗