• Title/Summary/Keyword: germ cell tumor

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Intracranial Germ Cell Tumor in the Molecular Era

  • Phi, Ji Hoon;Wang, Kyu-Chang;Kim, Seung-Ki
    • Journal of Korean Neurosurgical Society
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    • v.61 no.3
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    • pp.333-342
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    • 2018
  • Intracranial germ cell tumors (iGCTs) are a heterogeneous group of tumors with peculiar characteristics clearly distinguished from other brain tumors of neuroepithelial origin. Diverse histology, similarity to gonadal GCT, predilection to one sex, and geographic difference in incidence all present enigmas and fascinating challenges. The treatment of iGCT has advanced for germinoma to date; thus, clinical attention has shifted from survival to long-term quality of life. However, for non-germinomatous GCT, current protocols provide only modest improvement and more innovative therapies are needed. Recently, next-generation sequencing studies have revealed the genomic landscape of iGCT. Novel mutations in the KIT-RAS-MAPK and AKT-MTOR pathways were identified. More importantly, methylation profiling revealed a new method to assess the pathogenesis of iGCT. Molecular research will unleash new knowledge on the origin of iGCT and solve the many mysteries that have lingered on this peculiar neoplasm for a long time.

Prognostic factors in children with extracranial germ cell tumors treated with cisplatin-based chemotherapy

  • Kim, Jinsup;Lee, Na Hee;Lee, Soo Hyun;Yoo, Keon Hee;Sung, Ki Woong;Koo, Hong Hoe;Seo, Jeong-Meen;Lee, Suk-Koo
    • Clinical and Experimental Pediatrics
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    • v.58 no.10
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    • pp.386-391
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    • 2015
  • Purpose: To evaluate the outcomes and prognostic factors in children with extracranial germ cell tumors (GCTs) treated at a single institution. Methods: Sixty-six children diagnosed with extracranial GCTs between 1996 and 2012 were included in the study. Primary treatment was surgical excision, followed by six cycles of cisplatin-based chemotherapy. The survival rates were compared according to the International Germ Cell Cancer Cooperative Group classification used for GCTs in adults to validate the classification guidelines for GCTs in children. Results: The median patient age was 4.4 years. In 34 patients (51.5%), the primary tumor site was the gonad. Extragonadal GCTs were detected in 32 patients. The 5-year overall survival and event-free survival (EFS) were $92.0%{\pm}3.5%$ and $90.4%{\pm}3.7%$, respectively. In univariate analysis, tumor histology, metastasis, and elevated alpha-fetoprotein were not prognostic factors in children with extracranial GCTs. However, EFS was poorer in patients with mediastinal disease (n=12, $66.7%{\pm}13.6%$) than in those with nonmediastinal disease (n=54, $96.0%{\pm}2.8%$) (P=0.001). The 5-year EFS was lower in patients older than 10 years, (n=21, $80.0%{\pm}8.9%$) compared with those younger than 10 years (n=45, $95.2%{\pm}3.3%$) (P=0.04). Multivariate analysis identified the mediastinal tumor site as the only independent prognostic factor. Conclusion: The prognosis of children with extracranial GCTs was favorable. However, nongerminomatous mediastinal tumors were associated with poor survival in children. Further research is needed to improve the prognosis of children with malignant mediastinal GCTs.

Outcomes of Malignant Ovarian Germ-Cell Tumors Treated in Chiang Mai University Hospital over a Nine Year Period

  • Neeyalavira, Vithida;Suprasert, Prapaporn
    • Asian Pacific Journal of Cancer Prevention
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    • v.15 no.12
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    • pp.4909-4913
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    • 2014
  • Malignant ovarian germ cell tumors (MOGCT) are rare neoplasms that most frequently occur in women at a young reproductive age. There have been limited data regarding this disease from Southeast Asian countries. We therefore conducted a retrospective study to analyze the clinical characteristics and the treatment outcomes of MOGCT treated at our institute between January, 2003 and December, 2012. Seventy-six patients were recruited from this period with the mean age of 21.6 years and 11.8% were pre-puberty. The two most common symptoms were pelvic mass and pelvic pain. Two-thirds of the studied patients presented at an early stage. The most common histology was immature teratoma (34.2%) followed by endodermal sinus tumor (28.9%), dysgerminoma (25%), mixed type (10.5%) and choriocarcinoma (1.3%). Over 80% of these patients received fertility sparing surgery and about 70% received adjuvant chemotherapy with the complete response rate at 73.3% and partial response at 11.1%. The most frequent chemotherapy was BEP regimen (bleomycin, etoposide, cisplatin). With the mean follow up time at 56.0 months, 12 patients (15.8%) developed recurrence and only an advanced stage was the independent prognostic factor. The ten year progression free survival (PFS) and overall survival rate of our study were 81.9% and 86.2%, respectively. In conclusion, MOGCT often occurs at a young age. Treatment with fertility sparing operations and adjuvant chemotherapy with a BEP regimen showed a good outcome. An advanced stage is a significant prognostic factor for recurrence.

Clinical Experience of the Mediastinal Tumors - Report of 40 Cases - (종격동 종양의 임상적 경험 - 40례 보고 -)

  • 송진천
    • Journal of Chest Surgery
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    • v.23 no.1
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    • pp.137-140
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    • 1990
  • This report is an review of 40 cases of mediastinal tumors which were treated surgically in the Department of Thoracic and Cardiovascular Surgery, Chonnam University Hospital from January, 1985 to September, 1989. In this series, male to female sex distribution was 1.35:1 with the male predominant. The main clinical manifestations were chest pain and dyspnea, and there was no definitive symptom in 5 cases [12.5 %]. The most common mediastinal tumor was thymoma, 11 cases [27.5 %], and next common mass was germ cell tumor, 9 cases [22.5 %]. The half of these were malignant. In operating, all of the benign masses were removed.

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Clinicopathological Features and Survival of Young Turkish Patients with Testicular Germ Cell Tumors

  • Ozgun, Alpaslan;Karagoz, Bulent;Tuncel, Tolga;Emirzeoglu, Levent;Celik, Serkan;Bilgi, Oguz
    • Asian Pacific Journal of Cancer Prevention
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    • v.14 no.11
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    • pp.6889-6892
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    • 2013
  • Background: Testicular germ cell tumors (TGCTs) are a relatively common malignancy in young men. The aim of this study was to investigate the clinicopathological features and survival of young Turkish patients with TGCT. Materials and Methods: In this retrospective study, the clinical and pathological characteristics of young Turkish patients with TGCT who were monitored by the Department of Medical Oncology of a military hospital between 2008 and 2013 were investigated. Overall survival data were analyzed. Results: Ninety-six patients were included in the study. The mean age was 26.4 years. Among the patients, 17.7% had seminoma and 43.8% had mixed non-seminomatous germ cell tumors. Some 46.9% were Stage I, 30.2% were Stage II, and 22.9 were Stage III. Of the patients, 83.3% received chemotherapy, 25% underwent retroperitoneal lymph node dissection (RPLND), 3.1% received radiotherapy, and 12.5% were followed-up without treatment. In addition, 18.8% of the patients were administered salvage chemotherapy due to relapse or progression. The 5-year overall survival rate was 90.2% for all patients. The 2-year overall survival rate was 100% for Stage I patients, 94% for Stage II patients, and 70.2% for Stage III patients. The difference between the survival curves of stages was statistically significant (p=0.029). Conclusions: In young Turkish patients with TGCT, good results were obtained with appropriate treatment, most receiving chemotherapy. The prognosis of the disease was good even in the advanced stage.

Diagnosis of immunohistochemical marker expressed by a canine Sertoli cell tumor case (개 세르토리세포종 케이스에서 면역조직화학적 마커를 통한 진단)

  • Kim, Sung-Jae;Han, Jeong-Hee
    • Korean Journal of Veterinary Service
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    • v.34 no.3
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    • pp.273-278
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    • 2011
  • Sertoli cell tumor (SCT) of the testicle arises from the supporting cells within the seminiferous tubules. SCT is common in dogs, especially in cryptorchid testicles, but also has been reported in the stallion, ram, cat, and bull. Sertoli cell tumor sample was collected from 7-years male german shepherd. In this study, SCT arose from one testicle. Sample size is approximately 1.7 cm in diameter and it has a round form. In the microscopic, cells within the tumor variably resemble Sertoli cells (SCs) that normally populate the seminiferous tubules and interstitial area. There is abundant stroma of dense, mature fibrous connective tissue in SCT. In the immunohistochemical staining, cytokeratin AE1/AE3 was not expressed in the control and SCT. S-100 protein was expressed by SCs, germ cells and fibrous connective tissue of SCT. Melan A was expressed by leydig cells (LCs) of SCT. A study by using S-100 and melan A in canine SCT was almost never carried out. S-100 and melans A is considered to suggest for diagnosis and pathogenesis of canine SCTs. Inhibin-alpha and Vimentin were well known as the marekers of SCTs. Also, they were expressed by Sertoli cells and LSs of SCT in this study.

Primary endodermal sinus tumor of the mediastium -A case report- (종격동에 발생한 원발성 내배엽동종 -수술치험 1예 보고-)

  • 최영호
    • Journal of Chest Surgery
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    • v.19 no.3
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    • pp.494-499
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    • 1986
  • Malignant germ cell tumors which have the same morphological appearance as germinal tumor of the gonad are sometimes found in the anterior mediastinum. Pure endodermal sinus tumor among them is very rare. It is highly malignant and usually extensively invasive at the time of surgical consideration. The prognosis is poor, but improvements in systemic chemotherapy regimens may improved the outlook. A case of primary endodermal sinus tumor of the mediastinum was presented. He was a 23-year-old male with anterior chest pain, tenderness and exertional dyspnea for 1 month. The tumor was well encapsulated and adhered to pericardium. The microscopic appearance was indistinguishable from that of the analogous gonadal neoplasm. The patient has treated with combination of surgery and radiotherapy as daily 180 rad for 6 weeks schedule, and then has follow up about 3 months with early recurrence.

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Prognosis of Eight Chinese Cases of Primary Vaginal Yolk Sac Tumor with a Review of the Literature

  • Tang, Qiong-Lan;Jiang, Xue-Feng;Yuan, Xiao-Ping;Liu, Yong;Zhang, Lin;Tang, Xiao-Feng;Zhou, Jia-Jia;Li, Hai-Gang;Fang, Jian-Pei;Xue, Lin
    • Asian Pacific Journal of Cancer Prevention
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    • v.15 no.21
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    • pp.9395-9404
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    • 2014
  • Background: Primary vaginal yolk sac tumor is a rare malignancy in the pediatric population, and a diagnostic challenge and appropriate initial treatment remains unsolved. The aim of this study was to investigate the clinicopathologic features, treatment and prognosis of this tumor. Materials and Methods: Eight cases of primary vaginal yolk sac tumor were reported with a literature review. Results: There were 4 pure yolk sac tumor cases and four mixed germ cell tumors containing yolk sac tumor element, including two cases with embryonal carcinoma and two cases with embryonal carcinoma and dysgerminoma. Partial vaginectomy was performed in four cases and all patients received chemotherapy. 85 cases in literatures were reviewed and 9 cases were misdiagnosed. Follow-up data was available in 77 cases and 5-year overall survival rate was 87.6%. 5-year survival rate of biopsy with chemotherapy, conservative surgery with chemotherapy and radical surgery with chemotherapy was 91.1%, 100% and 28.6%, respectively (p<0.001). Compared to cases without relapse or metastasis after initial treatment, patients with relapse or metastasis had a shorter overall survival (35.6% vs 96.6%, p<0.001). Conclusions: Mixed germ cell tumor containing yolk sac tumor element was not uncommon and partial vaginectomy may be a good choice for primary vaginal mixed yolk sac tumor type to eradicate local tumor cells and provide complete information for pathological diagnosis and postoperative adjuvant therapy.

Anterior Mediastinal Tumor

  • Lee, Jae-Kyo
    • Journal of Yeungnam Medical Science
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    • v.27 no.2
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    • pp.98-104
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    • 2010
  • Primary anterior mediastinal neoplasms comprise a diverse group of tumors and account for 50% of all mediastinal masses. Thymic epithelial neoplasm are most common and classified into thymoma, invasive thymoma, and thymic carcinoma. Neuroendocrine differentiation of thymic epithelial neoplasm are rare malignancies. Germ cell tumor (GCT) is second most common anterior mediastinal tumor and most of them are mature cystic teratoma. Malignant mediastinal GCT are rare than benign. Primary thoracic lymphoma is rare than thoracic involvement of systemic lymphoma and most common location of primary thoracic lymphoma is anterior mediastinum. The clinical and radiologic appearance of the most common masses are reviewed.

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Percutaneous Fine Needle Aspiration Cytology of Thymoma (흉선종의 경피 세침흡인 세포학적 검색)

  • Park, Weon-Seo;Park, In-Ae;Ham, Eui-Keun;Lee, Sang-Kook
    • The Korean Journal of Cytopathology
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    • v.4 no.1
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    • pp.16-24
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    • 1993
  • The fine needle aspiration (FNA) cytologic findings in 16 cases of histologically confirmed thymoma are reported. The aspirates were obtained under fluoroscopic guidance. The cytologic diagnoses were inadequate sample in one case, thymoma in 12(75%), small cell carcinoma or thymoma in 1, benign mesenchymal tumor in 1, and germ cell tumor in one. The cytologic features were detailed according to the constituent epithelial cell type, and into 4 small of epithelial cells and lymphocytes. Fifteen cases were classified into 4 small epithelial cell type, 6 intermediate epithelial cell type, 1 large epithelial cell type, 1 large pleomorphic epithelial cell type, and 3 spindle-shaped epithelial ceil type. Cytologic differential diagnosis was discussed, and the important criteria for the cytologic diagnosis of thymoma were reviewed. This review leads us to think that nonoperative cytologic approaches in the diagnosis of the thymoma are possible, and that correct cytologic diagnosis of thymoma with FNAs can easily be made, if adequate samples are obtained However the invasiveness and histologic type could not be predicted by cytological features only. Knowing various cytologic and histologic features of thymoma will be helpful for the diagnosis of thymoma and the differential diagnosis of modiastinal tumors.

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