Cisplatin and Etoposide in Childhood Germ Cell Tumor: Brazilian Pediatric Oncology Society Protocol GCT-91

2009 ◽  
Vol 27 (8) ◽  
pp. 1297-1303 ◽  
Author(s):  
Luiz Fernando Lopes ◽  
Carla Renata Pacheco Macedo ◽  
Elitânia Marinho Pontes ◽  
Simone dos Santos Aguiar ◽  
Maria José Mastellaro ◽  
...  
Keyword(s):  
High Risk ◽  
Germ Cell ◽  
Children And Adolescents ◽  
Germ Cell Tumor ◽  
Multidisciplinary Treatment ◽  
Germ Cell Tumors ◽  
High Risk Group ◽  
Cell Tumor ◽  
Term Survival ◽  
Risk Patients

Purpose In 1988, we formed a consortium of Brazilian institutions to develop uniform standards for the diagnostic assessment and multidisciplinary treatment of children and adolescents with germ cell tumors. We also implemented the first childhood Brazilian germ cell tumor protocol, GCT-91, evaluating two-agent chemotherapy with cisplatin and etoposide (PE). We now report on the clinical characteristics and survival of children and adolescents with germ cell tumors treated on this protocol. Patients and Methods From May 1991 to April 2000, 115 patients (106 assessable patients) were enrolled onto the Brazilian protocol with a diagnosis of germ cell tumor. Results Patients were treated with surgery only (n = 35) and chemotherapy (n = 71). Important prognostic factors included stage (P = .025), surgical procedure at diagnosis according to resectability (P < .032), and abnormal lactate dehydrogenase value at diagnosis (P < .001). Conclusion The improvement in survival by the introduction of a standard protocol is an important achievement. This is of particular importance for smaller institutions with previous limited experience in the treatment of childhood germ cell tumors. In addition, the results of a two-agent regimen with PE were favorable (5-year overall survival rate is 83.3% for patients in the high-risk group [n = 36] who received PE v 58.8% for patients in the high-risk patients group who received PE plus ifosfamide, vinblastine, and bleomycin [n = 17; P = .017]). Thus for selected patients, complex three-agent regimens may not be necessary to achieve long-term survival, even for some patients with advanced disease.

Patients with advanced-stage germ cell tumors have a high risk of thromboembolic events.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e15621-e15621
Author(s):  
Anna Lina-Karin Gordy ◽  
Mary J. Brames ◽  
Lawrence H. Einhorn ◽  
Naveen Manchanda
Keyword(s):  
Logistic Regression ◽  
High Risk ◽  
Germ Cell ◽  
Germ Cell Tumor ◽  
Germ Cell Tumors ◽  
Cell Tumor ◽  
Advanced Stage ◽  
Tumor Patients ◽  
Poor Risk ◽  
Relapsed Disease

e15621 Background: Thromboembolism (TE) accounts for significant morbidity and mortality in cancer patients. Rates of TE have been reported from 0.9%-28.0%, depending on the population. Patients with germ cell tumors have a 4.0-8.4% risk of TE following platin chemotherapy. Our patients are high-risk, many with advanced disease receiving high-dose chemotherapy and peripheral blood stem cell rescue. We sought to more completely understand TE events in advanced germ cell tumor patients. Methods: Forty-four consecutive patients visiting our germ cell tumor clinic between 11/05/2012 and 1/22/2013 were selected. Data were collected by retrospective chart review from tumor diagnosis until TE (ranging from TE on diagnosis to 20 years later). A logistic regression model was fitted to determine variables that predispose patients to TE. Results: In our patient series, seven (15.9%) had venous TE and none had arterial events. Five patients (11.4%) had TE within 16 weeks of chemotherapy, and 2 at 10 and 19 years after diagnosis, respectively. Two had bilateral pulmonary emboli (PE) (4.5%), 3 had upper or lower extremity DVTs, or both, and 1 had bilateral PE and DVTs. Five patients with TE had nonseminomatous tumors, 2 had non-testis primaries, 4 had relapsed disease, 2 with late relapse (>7 years), 6 had metastatic disease, 3 had retroperitoneal lymph node dissection, and all 7 received platin chemotherapy. In logistic regression analysis, significant risk factors for TE included relapse (P= .016), bulky retroperitoneal lymphadenopathy (P= .006), alpha-fetoprotein >10,000 (P= .047) beta-HCG > 1,000 (P= .020), chemotherapy (P= .031), platin-refractory disease (P= .055), and poor risk disease compared to good risk disease (P=.020). Conclusions: Germ cell tumor patients have a high risk of venous TE. Those with relapsed disease, bulky retroperitoneal lymphadenopathy, platin chemotherapy, platin-refractory, or poor risk disease are at increased risk. Our estimates are higher than previously reported and in contrast to earlier studies, do not include arterial TE. To confirm our findings, we will extend this study to 100 patients. If confirmed, this pattern of TE events may be a consequence of advanced stage disease in our patients.

scholarly journals The Role of Surgical Resection as A Salvage Therapy and Immunohistochemical Stain to Confirm Mediastinal Yolk Sac Tumor

2020 ◽  
Vol 2 (1) ◽  
pp. 49-55
Author(s):  
Dwi Agustina ◽  
◽  
Suryanti Pratiwi ◽  
Diah Retnani ◽  
Artono Isharanto ◽  
...  
Keyword(s):  
Case Report ◽  
Germ Cell ◽  
Germ Cell Tumor ◽  
Germ Cell Tumors ◽  
Vena Cava ◽  
Yolk Sac ◽  
Cell Tumor ◽  
Yolk Sac Tumor ◽  
Term Survival ◽  
Immunohistochemical Stain

Background: Mediastinal germ cell tumors are uncommon, representing less than 1% of all malignancies. They mostly occur in young men after puberty and have a poor prognosis with over all long term survival rate 42%. Case Report: We reported a case report in Saiful Anwar Malang Hospital, Indonesia, a young man, 26 years old with an anterior mediastinum tumor and VCSS The CT scan of the chest showed an anterosuperior mediastinal mass which compress the superior vena cava. The result from transthoracic FNAB with USG guidance showed an extragonadal germ cell tumor which resemble an Embryonal Carcinoma from mediastinum. The AFP serum level was elevated. Patient underwent a radiotherapy and a surgical debulking. The biopsy showed an Endodermal Sinus Tumor (Yolk Sac). Immunohistochemical stain for cytokeratin was positive in the tumor cells while stain for CD30 was negative. These were confirming the diagnosis of primary mediastinal yolk sac tumor. Patient received a combination chemotherapy for a total 6 cycles. Conclusion: Some procedures like biopsy, tumor marker, immunohistochemical stain, could differentiate the subtype of mediastinal germ cell tumor. The multimodality treatment by combining chemotherapy with surgical therapy or radiotherapy could increase the survival outcome of patients.

scholarly journals Leptomeningeal Metastases in a Patient with an Extragonadal Germ Cell Tumor

2008 ◽  
Vol 2 ◽  
pp. CMO.S687
Author(s):  
Yoshiaki Kinebuchi ◽  
Masakuni Ishikawa ◽  
Osamu Ishizuka ◽  
Osamu Nishizawa ◽  
Kazuhiro Hongo
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumor ◽  
Multidisciplinary Treatment ◽  
Germ Cell Tumors ◽  
Whole Brain Radiotherapy ◽  
Salvage Chemotherapy ◽  
Leptomeningeal Metastases ◽  
Cell Tumor ◽  
Extragonadal Germ Cell Tumor ◽  
Brain Radiotherapy

We present a case of leptomeningeal metastases in a 30-year-old man with an extragonadal germ cell tumor. The patient was referred to our hospital for treatment of an occipital brain metastasis. This lesion was resected, followed by whole brain radiotherapy and further chemotherapy, and a temporary complete remission was achieved. However, leptomeningeal recurrence developed, and despite salvage chemotherapy, the patient died of disease. Although multidisciplinary treatment is given to treat brain metastases of germ cell tumors, the patients’ prognosis has been unsatisfactory. The identification of a standard/effective treatment is required.

scholarly journals Germ Cell Tumors in the Intersex Gonad: Old Paths, New Directions, Moving Frontiers

2006 ◽  
Vol 27 (5) ◽  
pp. 468-484 ◽  
Author(s):  
Martine Cools ◽  
Stenvert L. S. Drop ◽  
Katja P. Wolffenbuttel ◽  
J. Wolter Oosterhuis ◽  
Leendert H. J. Looijenga
Keyword(s):  
High Risk ◽  
Germ Cell ◽  
Germ Cell Tumor ◽  
Germ Cells ◽  
Germ Cell Tumors ◽  
Tumor Development ◽  
Disorders Of Sex Development ◽  
Cell Tumor ◽  
Maturation Delay ◽  
Made In

The risk for the development of germ cell tumors is an important factor to deal with in the management of patients with disorders of sex development (DSD). However, this risk is often hard to predict. Recently, major progress has been made in identifying gene-products related to germ cell tumor development (testis-specific protein-Y encoded and octamer binding transcription factor 3/4) and in recognizing early changes of germ cells (maturation delay, preneoplastic lesions, and in situ neoplasia). The newly recognized “undifferentiated gonadal tissue” has been identified as a gonadal differentiation pattern bearing a high risk for the development of gonadoblastoma. It is expected that the combination of these findings will allow for estimation of the risk for tumor development in the individual patient (high risk/intermediate risk/low risk). This article reviews the recent literature regarding the prevalence of germ cell tumors in patients with DSD. Some major limitations regarding this topic, including a confusing terminology referring to the different forms of intersex disorders and unclear criteria for the diagnosis of malignant germ cells at an early age (maturation delay vs. early steps in malignant transformation) are discussed. Thereafter, an overview of the recent advances that have been made in our knowledge of germ cell tumor development and the correct diagnosis of early neoplastic lesions in this patient population is provided. A new classification system for patients with DSD is proposed as a tool to refine our insight in the prevalence of germ cell tumors in specific diagnostic groups.

Multiple Tumor-Associated Intracranial Aneurysms Adjacent to a Suprasellar Germ Cell Tumor: Case Report and Review of Literature

2021 ◽  
pp. 1-10
Author(s):  
John K. Yue ◽  
Diana Chang ◽  
Taemin Oh ◽  
Ethan A. Winkler ◽  
Alex Y. Lu ◽  
...  
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumor ◽  
Intracranial Aneurysms ◽  
Multidisciplinary Treatment ◽  
Clinical History ◽  
Cell Tumor ◽  
Paracrine Signaling ◽  
Current Case ◽  
Case Presentation ◽  
Multiple Tumor

<b><i>Introduction:</i></b> Tumor-associated intracranial aneurysms are rare and not well understood. <b><i>Case Presentation:</i></b> We describe a 4-year-old female with multiple intracranial aneurysms intimately associated with a suprasellar germ cell tumor (GCT). We provide the clinical history, medical, and surgical treatment course, as well as a comprehensive and concise synthesis of the literature on tumor-associated aneurysms. <b><i>Discussion:</i></b> We discuss mechanisms for aneurysm formation with relevance to the current case, including cellular and paracrine signaling pertinent to suprasellar GCTs and possible molecular pathways involved. We review the complex multidisciplinary treatment required for complex tumor and cerebrovascular interactions.

scholarly journals GCT-06. DIAGNOSIS OF A RARE CASE OF RECURRENT GERM CELL TUMOR BY CSF PLACENTAL ALKALINE PHOSPHATASE PRESENTING WITH DIFFUSE INTRAAXIAL ABNORMALITY IN THE LOWER BRAINSTEM

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii329-iii329
Author(s):  
Hiroki Yamada ◽  
Tomohiro Abiko ◽  
Hirokazu Fujiwara ◽  
Kazunari Yoshida ◽  
Hikaru Sasaki
Keyword(s):  
Alkaline Phosphatase ◽  
Germ Cell ◽  
Germ Cell Tumor ◽  
Rare Case ◽  
Cervical Spinal Cord ◽  
Germ Cell Tumors ◽  
Cell Tumor ◽  
Placental Alkaline Phosphatase ◽  
Steroid Pulse ◽  
Platinum Based Chemotherapy

Abstract INTRODUCTION Germ cell tumors in the central nervous system (CNS) typically arise either at suprasellar and/or pineal region, and occasionally at basal ganglia. We report a case of diagnostically challenging, recurrent germ cell tumor presented with diffuse intraaxial abnormality in and across the lower brainstem, which was diagnosed by the elevated placental alkaline phosphatase (PLAP) level in cerebrospinal fluid (CSF). CASE DESCRIPTION: A 28-year-old man had been treated by chemoradiotherapy at the previous hospital for bifocal suprasellar and pineal lesions with the provisional diagnosis of germinoma without histological confirmation. Three years later, he presented with progressive weakness of bilateral extremities for weeks. Magnetic resonance imaging showed a diffuse, bilaterally symmetric high intensity lesion on T2-weighted image with slight contrast enhancement across the ventral side of the medulla oblongata to the upper cervical spinal cord. Serum and CSF hCG, hCG-β, and AFP were all negative. Since the image findings were atypical for recurrent germ cell tumor, some kind of myelitis was initially suspected. Therefore, steroid pulse therapy was administered. However, the patient’s symptom was still gradually progressing. Then, the CSF PLAP turned out to be positive, indicating the recurrence of germinoma. Accordingly, platinum-based chemotherapy was administered, and the imaging findings, patient’s symptoms, and CSF PLAP began to improve. The patient is to be treated with radiotherapy following chemotherapy. CONCLUSION We report a rare case of CNS germ cell tumor that presented with diffuse intraaxial lesion in the lower brainstem in which examination of CSF PLAP was extremely useful.

scholarly journals A rare case of mixed germ cell tumor in a teenage girl: a case report

2017 ◽  
Vol 6 (6) ◽  
pp. 2663
Author(s):  
Faraz S. Vali ◽  
Amit Kyal ◽  
Parul I. Chaudhary ◽  
Sujatha Das ◽  
Aprateem Mukherjee ◽  
...  
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumor ◽  
Embryonal Carcinoma ◽  
Germ Cell Tumors ◽  
Cell Tumor ◽  
Ca 125 ◽  
Gestational Choriocarcinoma ◽  
Mixed Germ Cell Tumor ◽  
Initial Surgery ◽  
Alpha Feto Protein

Germ cell tumors represent only 20% to 25% of all benign and malignant ovarian neoplasms. Mixed germ cell tumors are a rare variety of non–dysgerminomatous germ cell tumors. They contain two or more elements; the most frequent combination being a dysgerminoma and an EST (Endodermal Sinus Tumor). We present a case of malignant mixed germ cell tumor comprising of yolk sac tumor, embryonal carcinoma and choriocarcinoma. A 13-year-old girl presented with a huge 25 x 18 cm mass in abdomen with raised values of CA-125, hCG, AFP (alpha-feto protein) and LDH (lactate dehydrogenase). She underwent laparotomy followed by unilateral salpingoopherectomy and infracolic omentectomy. Histopathology report revealed malignant mixed germ cell tumor comprising predominantly of EST with elements of embryonal carcinoma and non-gestational choriocarcinoma. Following surgery, she was started on adjuvant chemotherapy (Bleomycin, Etoposide and Cisplatin regimen). Mixed germ cell tumor (YST/EST, non-gestational choriocarcinoma and embryonal carcinoma) is a very rare tumor. Careful initial surgery with adequate staging biopsies followed by combination chemotherapy can greatly improve the prognosis of these patients

scholarly journals Pediatric Germ Cell Tumors: An Experience of 7 Years in a Tertiary Hospital of Bangladesh

2020 ◽  
Vol 35 (2) ◽  
pp. 119-122
Author(s):  
SM Rashed Zahangir Kabir ◽  
Md Waheed Akhtar ◽  
Farida Yasmin
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumor ◽  
Tertiary Care ◽  
Germ Cell Tumors ◽  
Yolk Sac ◽  
Cell Tumor ◽  
Yolk Sac Tumor ◽  
Treatment Modalities ◽  
Care Hospital ◽  
Childhood Malignancies

Introduction: Germ cell tumors are a group of tumors with different clinical presentation and histological and biological characteristics. Malignant germ cell tumors occur at all ages with a trend of bimodal distribution in infancy and adolescence. Objective: To evaluate the demographic characteristics, distribution of different types of germ cell tumor, treatment modalities and outcome of germ cell tumor in children in a tertiary care hospital of Bangladesh. Methods: In this retrospective study, data regarding age and sex distribution, location, types of tumors, management of germ cell tumor in children were retrieved from the medical records of pediatric oncology department in NICRH, Dhaka from 2008 to 2014. Results: Out of total 87 patients female were 50 and male 37. Most of the patients were up to 5 years of age. The gonadal germ cell tumors (80%) were more than extragonadal tumor (20%) in both male and female patients. The most common germ cell tumor was dysgerminoma (32%) followed by yolk sac tumor (29.8%) and teratoma (19.5%). Yolk Sac Tumor (51.4%) was the most common in male and dysgerminoma (56%) the commonest in female. Out of 87, seventy two (82.7%) received chemotherapy following surgery. Among those 72 patients who received chemotherapy 49 (68 %) patients completed their treatment. Until the last follow up 71.4% patients remained alive and tumor free. Conclusion: Germ cell tumors are the most variable tumor of all childhood malignancies that has difference in age, sex, location and histological subtypes. Gonadal tumors have better prognosis than extragonadal tumors in both the sex. DS (Child) H J 2019; 35(2) : 119-122

scholarly journals MPC-08 CLINICOPATHOLOGICAL ANALYSIS OF 12P GAIN IN INTRACRANIAL GERM CELL TUMORS

2019 ◽  
Vol 1 (Supplement_2) ◽  
pp. ii23-ii23
Author(s):  
Kaishi Satomi ◽  
Hirokazu Takami ◽  
Shintaro Fukushima ◽  
Yoichi Nakazato ◽  
Shota Tanaka ◽  
...  
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumor ◽  
Copy Number ◽  
Mature Teratoma ◽  
Methylation Status ◽  
Germ Cell Tumors ◽  
Copy Number Variations ◽  
Cell Tumor ◽  
Testicular Germ Cell ◽  
Intracranial Germ Cell Tumor

Abstract BACKGROUND Gain of short arm of chromosome 12 (12p) is commonly observed in testicular germ cell tumors (tGCTs). 12p gain is also frequently seen in intracranial GCTs (iGCTs). However, little is known about the clinical significance of 12p gain in iGCTs. MATERIALS AND METHODS We have collected over 200 fresh frozen tissue samples of iGCTs through the Intracranial Germ Cell Tumor Genome Analysis Consortium in Japan. Firstly, we analyzed DNA methylation status in 83 iGCTs, 3 seminomas and 6 normal control samples using Infinium Human Methylation 450K BeadChip array (Illumina, CA). Idat files were processed using R (Version 3.5.3) and minfi package (1.30.0) to generate copy number variations. Compared with average genome-wide copy number level, 12p gain was determined. Then, 58 iGCTs with clinicopathological information were analyzed for progression-free survival (PFS) and overall survival (OS). Those tumors that consist of only either germinoma and/or mature teratoma components were classified as Favorable Histology (FH) and all the others that contains malignant histological components were classified as Unfavorable Histology (UFH). RESULT 12p gain was observed in 100% (3/3) of seminoma, 13.6% (3/22) of germinoma, 16.7% (1/6) of mature teratoma, 25% (1/4) of immature teratoma, 55% (11/20) of mixed germ cell tumor, 100% (4/4) of yolk sac tumor, 100% (1/1) of embryonal carcinoma, and 100% (1/1) of choriocarcinoma. In total, 44.6% (37/83) of iGCT showed 12p gain. Regarding histological classification, the 12p gain rate in UFH (72%, 18/25) was significantly higher than that in FH (12.1%, 4/33, P&lt;0.01). Both PFS and OS were significantly worse in iGCTs with 12p gain (PFS: P=0.027, OS: P=0.0012). DISCUSSION 12p gain can be a molecular marker to predict prognosis and histological malignancy in iGCTs.

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